Viva ENT

Download as pdf or txt
Download as pdf or txt
You are on page 1of 343

Viva Guide in ENT

The traffic light approach

RICARDO AP PERSAUD
FRCS ORL-HNS (Eng) CCT Otol. (UK)
MBBS MSB CBiol MPhil MRCS (Gen) DO-HNS
Consultant ENT Head Neck & Rhinoplastic Surgeon
Al Zahra Medical Group, Sharjah and Dubai, UAE.

OWEN JUDD
FRCS ORL-HNS (Eng) CCT Otol. (UK)
MRCP FRCS (Edin) PGDipClinEd DCH
Consultant Neurotologist and Laryngologist
Royal Derby Hospital, Derby, UK.

YOGESH M BHATT
FRCS ORL-HNS (Edin)
MB ChB (Hons) BSc (Hons) 1st Neuroscience (Lond)
Interface Fellow in Head and Neck Surgical Oncology
University Hospitals Birmingham NHS Foundation Trust, UK
Council Member for the British Association for Paediatric
Otolaryngology.

JULIAN A GASKIN
FRCS ORL-HNS (Edin) CCT Otol. (UK)
MBChB DO-HNS
Fellow in Paediatric Otorhinolaryngology
University of Manchester Medical School Examiner
Royal Manchester Children's Hospital, Manchester, UK.
- 12 -
Viva Guide in ENT Surgery:
The traffic light approach

CONTENTS

• Preface x
• Foreword x
• Acknowledgements x
• Dedication x
CHAPTER 1: PAEDIATRIC ENT SURGERY x
1.1 Periorbital cellulitis x
1.2 Hypovolaemic shock X
1.3 Epiglotitis X
1.4 Foreign body wheezing x
1.5 Acute otitis media (AOM) x
1.6 Acute mastoiditis x
1.7 Neonatal nasal obstruction x
1.8 Retropharyngeal abscess x
1.9 Juvenile respiratory papillomatosis x
1.10 Pre-auricular sinus x
1.11 Microtia x
1.12 Otitis media with effusion (Glue ear) x
1.13 Branchial cleft sinus x
1.14 Drooling x
1.15 Laryngomalacia x
1.16 Down syndrome x
1.17 Haemangiomas x
1.18 Cystic hygroma x
1.19 Thyroglossal duct cyst x
1.20 Lingual thyroid x
1.21 Congenital midline nasal mass x
1.22 Cleft lip and palate x
1.23 Congenital cholesteatoma x
1.24 Waardenburg syndrome x
1.25 Ranula x
1.26 Cervical lymphadenopathy X

-5-
1.27 Traumatic perforation of tympanic membrane
and non-accidental injury (NAI) x
1.28 Caustic ingestion and NAI x
1.29 Juvenile nasopharyngeal angiofibroma (JNA) x
1.30 Airway foreign body x
1.31 Sleep-disordered breathing in a child x

CHAPTER 2: RHINOLOGY AND FACIAL PLASTICS x


2.1 Anosmia x
2.2 CSF rhinorhoea x
2.3 Fungal rhinosinusitis x
2.4 Hereditary Haemorrhagic Telangiectasia (HHT) x
2.5 Antrochoanal polyp x
2.6 Sarcoidosis x
2.7 Sinonasal tumour x
2.8 Olfaction x
2.9 Septal perforation and septal button x
2.10 Nasal trauma x
2.11 Limited Wegener disease x
2.12 Systemic Wegener disease x
2.13 Empty nose syndrome x
2.14 Frontal mucoele x
2.15 Inverted papilloma x
2.16 Atrophic rhinitis x
2.17 Cavernous sinus thrombosis x
2.18 Allergic rhinitis x
2.20 Rhinitis medicomentosa x
2.21 Scarring disorder x
2.22 Septorhinoplasty x
2.23 Saddle nose deformity x
2.24 Nasal reconstruction after trauma x
2.25 Alar basal cell carcinoma x
2.26 Pinnaplasty x
2.27 Rhomboid flap x
2.28 Rhinophyma x
2.29 Lower lip SCC and Karapandzic flap x
2.30 Z-Plasty

-6-
CHAPTER 3: HEAD AND NECK x
3.1 Salivary gland neoplasm x
3.2 Neck lumps x
3.3 Hoarse voice x
3.4 Carcinoma of unknown primary (CUP) x
3.5 Tonsil carcinoma x
3.6 Nasopharyngeal carcinoma x
3.7 Laryngeal carcinoma and neck dissection x
3.8 Supraclavicular metastatic squamous cell carcinoma x
3.9 Black hairy tongue x
3.10 Benign parotid lesion x
3.11 Malignant parotid lesion x
3.12 Vocal cord dysplasia/early glottic SCC x
3.13 HPV and oropharyngeal lesion x
3.14 Facial pain x
3.15 Pharyngeal pouch x
3.16 Parapharyngeal mass x
3.17 Hemi-tongue atrophy x
3.18 Cervical mycobacterial lymphadenitis (scrofula) x
3.19 Pseudomenbraneous colitis (C. Diff infection) x
3.20 Reinke’s oedema x
3.21 Radiotherapy and side effects x
3.22 SCC Tongue x
3.23 Vocal process granuloma x
3.24 Parapharyngeal space abscess x
3.25 Goitre X
3.26 Nasolabial cyst x
3.27 Frey’s syndrome and Botox x
3.28 Giant plexiform neurofibroma of scalp x

CHAPTER 4: OTOLOGY AND NEURO-OTOLOGY x


4.1 Acute otitis externa x
4.2 Exostoses and osteomas x
4.3 Perforation of tympanic membrane x
4.4 Tympanic membrane retraction x
4.5 Cholesteatoma of the middle ear x
4.6 Otosclerosis x

-7-
4.7 Meniere’s disease x
4.8 Benign paroxysmal positional vertigo x
4.9 Temporal bone fracture x
4.10 Noise induced hearing loss x
4.11 Non-organic hearing loss x
4.12 Drug induced hearing loss x
4.13 Bone anchored hearing aid (BAHA) x
4.14 Cochlear implant x
4.15 Chronic otitis externa x
4.16 Vestibular schwannoma x
4.17 Ramsay Hunt Syndrome x
4.18 Mastoidectomy fistula x
4.19 High jugular bulb x
4.20 Glomus tympanicum x
4.21 Meningioma x
4.22 Bell’s palsy x
4.23 Chronis otitis media active mucosal disease x
4.24a Otoacoustic emissions (old graph) x
4.24b Otoacoustic emissions (new graph) x
4.25 Universal Neonatal Hearing Screening x
4.26 Auditory Brainstem Response x
4.27 Presbyacussis x
4.28 Sudden sensory neural hearing loss x
4.29 Tinnitus x
4.30 Clinical anatomy of the temporal bone x

-8-
CHAPTER 1

Paediatric ENT Surgery

1.1 PERIORBITAL CELLULITIS

CLINICAL
PHOTOGRAPH

Introductory Answer
question

What is the • Orbital cellulitis with associated sinusitis


differential
• Trauma/insect bite induced infection
diagnosis of this
child in the • Dysthyroid eye disease
photograph?
• Orbital pseudo-tumour
• Mucocele
• Non-accidental injury

- 17 -
Competency Answer
question(s)

How would you • This is a sight threatening emergency


manage this and is best managed together with an
patient? ophthalmologist and depends upon the
grade of the infection.
• History – determine likelihood of orbital
cellulitis and associated sinusitis. Pain,
diplopia, impaired vision, duration of
symptoms. DH/allergy.
• Examination – determine likelihood of
post-septal disease by identifying
proptosis, diplopia, chemosis or pain on
eye movement. Assess visual acuity with
Snellen chart and red desaturation with
Ishihara plates. Gross test of CN2
function using swinging light test for
RAPD. Bilateral eye signs raise possibility
of cavernous sinus thrombosis
• Investigations – CT imaging of nose and
paranasal sinuses, orbits and brain with
contrast if:
1. Suspecting post-septal disease
(Chandler’s II to V)
2. No response to 24 hours of IV
antibiotics
3. Visual acuity cannot be assessed
adequately or routinely
• MR brain with contrast if intracranial
complication suspected.
• Treatment – IV antibiotic guided by

- 18 -
local protocol and intranasal steroid and
decongestants. Initially, hourly eye
observations and 4-hourly neuro-obs
until evidence of disease resolution.
• Thin medial subperiosteal collections
may be managed conservatively if
regular reliable acuity assessment is
available. All other collections must be
drained immediately.
• Drainage may be performed externally
to allow drain insertion or if expertise
available, endonasally.
• Maxillary antrum may be washed out
and aspirate sent for MC&S

Advanced Answer
question(s)

What if there is a • This is likely to represent a subperiosteal


forehead swelling collection of an osteomyelitic anterior
in this child? table of the frontal sinus or Potts Puffy
Tumour.
• This condition is associated with
intracranial complications in 33% of
cases and demands an MR brain with
contrast enhancement and repeat
assessment as symptoms dictate.
• IV antibiotic treatment should have
good bone penetrance and duration
suitable for treating an osteomyelitic
process.
• Surgical goals are to drain the pus –
direct incision may lead to fistulation

- 19 -
and frontal sinus surgery may be
hazardous. Frontal sinus trephine may
represent an effective compromise.
Bony sequestra occasionally require
open debridement.

1.2 HYPOVOLAEMIC SHOCK

SCENARIO You are asked to see a 3 yr old boy who


underwent adeno-tonsillectomy for OSA 2
(no clinical
hours previously. He is pale and aggitated
photograph)
but apyrexial with a heart rate of 165bpm,
respiratory rate 40/min and capillary refill
(CR) time of 5 seconds.

Introductory Answer
question

What is your • Hypovolaemic shock (uncompensated)


clinical diagnosis?

Competency Answer
question(s)

What is shock and • Shock refers to systemic organ


how would you dysfunction resulting from hypo-
manage this perfusion and/or hypo-oxygenation. In
child? this case it is from hypovolaemia due to

- 20 -
blood loss (cf. dehydration)
• When compensated blood flow to vital
organs is preserved but the child may be
agitated with raised HR and CR. Low BP is
a pre-terminal sign of decompensation
and circulatory collapse may quickly
follow.
• Seek input from an experienced
anaesthetist/paediatrician and
simultaneously assess and treat the child
using APLS protocol in an appropriate
setting eg RESUS bay, theatre recovery
or HDU with oxygen and fluids.
• A – clear clots and administer high flow
oxygen, B – assess effort, efficacy and
effect of breathing, C – reassess signs
and urine output and obtain 2 wide bore
IV access lines whilst obtaining blood for
cross-match, FBC, coagulopathy screen.
• Administer a 20ml/kg normal saline
(0.9%) bolus and check for response. If
bleeding continues and once 40ml/kg
had been administered, colloid/blood
products must be given next.
• Once stabilised the child should be taken
to theatre to formally control the bleed.

Advanced Answer
question(s)

How would your • Pyrexia might suggest an infective


management be component i.e. septic shock. If the child
changed if the was not penicillin allergic, I would
child was

- 21 -
pyrexial? administer cefotaxime at 80mg/kg.
How would it be • If no IV access was achievable
changed if IV immediately, obtain intra-osseous
access could not access. With a knee flexed at 30degree
be obtained? over a towel clean the skin over either
antero-medial surface of tibia 3cm below
the tibial tuberosity or antero-lateral
femur 3cm above the lateral condyle.
Rotate an 18G IO needle at 90 degrees to
the bone until a ‘give’ is felt. Aspirated
to confirm position and 20ml of
crystalloid pushed through the syringe
and line secured.

1.3 EPIGLOTITIS

SCENARIO You are asked to see a 3 yr old with a 12


hours history of sore throat and noisy
(no clinical
breathing. On examination she is pyrexial
photograph)
and drooling.

Introductory Answer
question

What is your • Epiglottitis


differential
• Laryngo-tracheo-bronchitis (croup)
diagnosis?
• Deep neck space abscess

- 22 -
• Airway foreign body
• Severe pharyngo-tonsillitis (bacterial or
viral aetiology)

Competency Answer
question(s)

How would you • History – Taken whilst making primary


manage this ABC assessment in line with APLS
patient? principles. Establish rapidity of symptom
onset or any history of choking.
Vaccination history. Social history ie
children at home. DH/allergies esp.
antibiotics.
• Examination – Without further upsetting
child by oral examination or cannulation,
assess effort and efficacy of breathing
including phase of stridor, use of
accessory muscles, tracheal tug,
grunting. Desaturation is a pre-terminal
event. Ask family to hold nebulised
adrenaline in oxygen close to child.
Further assessments should be made in
theatre.
• Investigations – With the assistance of a
senior anaesthetist the child should be
taken to theatre with age appropriate
and smaller oro and naso-tracheal tubes,
ventilating bronchoscope and paediatric
pharyngoscope/Hopkins rod endoscope
and tracheostomy tray. Intubation with
spontaneously ventilating inhalational
anaesthetic.
• Treatment – IV ceftriaxone administered

- 23 -
and child to remain intubated on
paediatric ITU until tube leak present or
reassessed with laryngoscope in
48hours. Sepsis treated in conjunction
with paediatric intensivists.

Advanced Answer
question(s)

What is Heliox? • It is a gaseous mixture of helium and


oxygen typically 79 percent and 21
percent, respectively. It is less dense
than either oxygen or room air and has
properties of low resistance laminar
airflow, despite the increased flow
velocity which occurs as a consequence
of airway narrowing.

What is its role in • It represents a temporising measure


upper airway which facilitates ventilation and may
obstruction? reduce the likelihood of exhaustion
before the airway is secured by
intubation, tracheostomy or other
therapies. Its function is to minimise the
risk of hypercapnia and CO2
narcosis/acidosis.

1.4 FOREIGN BODY WHEEZING

SCENARIO A&E referral: You are called by the


paediatricians to see a 2 year old boy with a 6
(followed by a
month history of intermittent wheezing,

- 24 -
radiological occasional cough and sneezing. His parents
image) became concerned after he developed a high
temperature over the weekend.

Introductory question Answer

What is your • Differential diagnosis: FB inhalation,


differential asthma exacerbation, severe allergy,
diagnosis and how croup, bronchiolitis, undiagnosed
would you assess subglottic stenosis
the child?
• Assessment: Start with ABC. Then
History: FB ingestion, history of URTI prior to
onset, asthma, eczema, food allergies,
previous history of pyrexia-induced
convulsions. Any voice disturbance. Problems
at birth – premature, SCBU, intubation and
duration.
Examination: Recessions, listen to chest (is it
wheezing from LRT, a stertorous noise or
stridor); examine nose, FNE may be difficult
in this age group
Investigations: CXR and FBC and differential,
based on the history

Competency Answer
question(s)

Here is the CXR. CXR shows evidence of hyperinflation of the


Describe what right lung with mediastinal shift to the left.
you see This is most likely to be due to a foreign body
in the right main bronchus having a ball valve

- 25 -
effect

How would you With optical forceps and a ventilating


remove it? bronchoscope. The use of 1:10,000
adrenaline injected through the suction port
is very helpful to aid visualisation (by
vasoconstricting the blood vessels).

Advanced Answer
question(s)

What would you • Enlist help of respiratory colleagues for


do if you cannot bronchoscopy, failing that, option of last
remove it? resort is to consult with thoracic team
for possible thoracotomy, usually
reserved for patients with serious/life
threatening complications such as airway
obstruction or empyema.

- 26 -
1.5 ACUTE OTITIS MEDIA (AOM)

CLINICAL
PHOTOGRAPH

Introductory Answer
question

A 5yr old presents • An otoscopic photograph of a right


with a 3 day tympanic membrane with evidence of
history of otalgia bulging and erythema consistent with
and pyrexia. You acute otitis media.
examine the ear
which looks like
the clinical
photograph
above. Please
describe what you
see?

- 27 -
Competency Answer
question(s)

How would you • Management guided by an


manage this acknowledgement that AOM is typically
child? self-limiting simply requiring supportive
treatment. Treatment chosen in
partnership with parent. History and
examination used to determine the
appropriateness of supportive therapy vs
delayed antibiotics vs immediate
antibiotics.(SIGN Guidelines for AOM,
2003)
• Supportive treatment may be considered
in those over 2 yr old and an explanation
of likely duration of symptoms should be
given (4days) and an option given to take
antibiotic therapy if symptoms persisting
beyond 2-3days or symptoms worsening.
• Children with or at risk of complications
should be given immediate antibiotic
therapy. These include severely ill
children, those with a history of
complications, the immunodeficient,
craniofacial dysmorphism predisposing
to Eustachian tube dysfunction (Down
syndrome or cleft palate), children under
2 yrs with bilateral AOM and those with
a cochlear implant.
• First line antibiotic may be 5 days of
amoxicillin. Second line therapy is co-
amoxiclav if child not responding to 3
days of first line treatment or recently
treatment with antibiotics, high local

- 28 -
prevalence of beta-lactamase producing
organisms or at higher risk of
complications. Ensure Prevenar
(Pneumococcal Conjugate Vaccine) has
been considered.

Advanced Answer
question(s)

What is the role • No randomised studies have


of myringotomy in demonstrated the benefit of
the treatment of tympanocentesis in the routine
AOM? treatment of AOM.
• A firm indication may be children with a
complication of AOM or impending
complication. A relative indication may
be the severely ill child not responding to
antibiotic therapy.
• Tympanocentesis may be performed
under GA or LA using a 23g needle
mounted on a 2cm3 syringe.
• Elective myringotomy and
tympanostomy tube insertion has been
shown to reduce the rate of AOM in
children with recurrent AOM.

- 29 -
1.6 ACUTE MASTOIDITIS

CLINICAL
PHOTOGRAPH

Introductory Answer
question

What are the This is a clinical photograph showing a right


differential post auricular swelling with pinna proptosis.
diagnoses? The differential diagnoses are:
• Subperisoteal collection complicating
acute mastoiditis
• Post-auricular lymphadenopathy
• Post-auricular soft-tissue infection
• Pinna/mastoid trauma

- 30 -
Competency Answer
question(s)

How would you • History – duration of otalgia, otorhoea,


investigate and type and duration of antibiotic therapy.
manage this History of AOM complications.
patient? DH/allergies.
• Examination – assess for signs of
mastoiditis including dull TM, posterior
canal wall sag, and tenderness of antrum
on palpating the cymba concha.
Fluctuant post-aural collection. Assess
for intra- and extra-temporal
complications including AVPU,
meningism and facial nerve function.
• Investigations – CT imaging with
contrast of the temporal bone and brain
to help exclude co-existing intra-cranial
complications.
• Treatment – Trial of IV ceftriaxone if
child not grossly unwell or if no
collection. If otherwise or not responding
to 24hrs trial of therapy then incision
and drainage and cortical
mastoidectomy with drain and
myringotomy +/- grommet insertion.
Care not to extend post-aural incision
below level of floor of ear canal due to
risk of superficial facial nerve. Send pus
for MC+S.

- 31 -
Advanced Answer
question(s)

• The scan in figure 1.6a demonstrates the


Despite the above
treatment the enhancement of the wall of the sagittal
child continues to sinus with a contrast void in its lumen.
experience picket This is the ‘delta sign’ and suggests a
fence spikes of cerebral venous sinus thrombosis.
pyrexia. A CT scan
is performed. • Liaise with a microbiologist, to ensure
Please describe its adequacy of antibiotic therapy with
findings and intra-cranial penetrance, and a senior
explain how would otologist.
you manage this? • This thrombosis may result in raised ICP
causing ‘otitic hydrocephalus’. The child
should be managed with a paediatrician
and nursed with neurological
observation. Anti-coagulation may be
indicated in the presence of an
extending or symptomatic thrombosis.
The presence of local cerebritis may
Figure 1.6a. Delta increase the risk of anticoagulation
sign induced haemorrhage.

1.7 NEONATAL NASAL OBSTRUCTION

Whilst in the outpatient clinic you received a


SCENARIO
call for the Obstetrician who just delivered a
(no clinical baby. The newborn appears blue but
photograph) becomes pinks on crying. The Obstetrician is
concerned that the baby may have an airway

- 32 -
problem and would like you to come
urgently.

Introductory Answer
question

What is your
• Bilateral choanal atresia,
differential
diagnosis? • Nasal mass (glioma, encephalocele,
meningoencephalocele, nasolacrimal
duct tumours etc)
• Piriform aperture stenosis
• Severe neonatal rhinitis

Competency Answer
question(s)

How would you • History –brief neonatal history whilst


manage the case? inserting and securing a oropharyngeal
airway and orogastric tube for feeding.
• Examination – try to pass a fine bore
catheter through the nose, listen for
sounds on stethoscope held under nose.
Check for misting on a cold Lack spatula
placed under the nose.
• Investigations – Axial CT scan through
the post nasal space after decongesting
and suctioning of nose to confirm
diagnosis.
• If bilateral choanal atresia is present I
would think of CHARGE Syndrome and

- 33 -
request an ECHO to rule out cardiac
abnormalities, Ultrasound scan of
kidneys to rule out renal problems.
Later, slit lamp examination of eyes
(coloboma) as well as OAEs and ABR
• Treatment – surgery ASAP to create and
maintain patent posterior choanae. Can
be done endonasally under direct vision
by using a 120 degrees rigid Hopkins rod
in the mouth. The alternative is the
trans-palatine approach, which involves
removal the posterior part of the vomer.
A soft tube splint for up to 6 weeks is
necessary.

Advanced Answer
question(s)

What is CHARGE? • CHARGE is an acronym for Coloboma,


Heart abnormality, Atresia of choana,
Is CHARGE an Retardation of growth, Genital/urinary
association, a abnormality and Ear abnormality.
syndrome or a
• CHARGE is a syndrome, rather than an
sequence?
association, because the cause has been
identified (mutations of CHD7 gene on
chromosome 8).
• An association is when the cause is
unknown whilst a sequence is when one
abnormality leads to another eg Pierre
Robin sequence.

- 34 -
1.8 RETROPHARYNGEAL ABSCESS

SCENARIO You are asked to see a 3yrs-old child with a


24 hour history of worsening snoring,
temperature and reduced oral intake. She
exhibits a reduced range of neck movement.

Introductory Answer
question

What is your • Retropharyngeal collection


differential
• Prevertebral collection
diagnosis?
• Parapharyngeal collection
• Neck trauma

Competency Answer
question(s)

The A+E • The film demonstrates AP thickness of


department the pre-vertebral space in excess of a
requested a lateral vertebral body width with loss of cervical
cervical spine plain lordosis (normal prevertebral thickness
x-ray in extension. C1-C4 = 7mm, C4-C8 = 17mm). Suggests
retro-pharyngeal or pre-vertebral space
What is the likely
collection. Often secondary to
pathophysiology
suppurative lymphadenopathy of the
for this condition
retropharyngeal Rouviere node in
and how would
children under 4yrs of age.
you manage this

- 35 -
patient? • History – Blunt trauma, foreign body
ingestion, URTI, tuberculosis contact,
DH/allergy.
• Examination – Effort and efficacy of
breathing noting stertor/stridor and
administration of high flow humidified
oxygen by parent. Careful oral
assessment for parapharyngeal &
retropharyngeal swelling. Associated
neurology or chest signs.
• Investigation – cross-sectional imaging if
symptoms do not settle within 24 hours
of IV antibiotics or evidence of frank
collection. Allows planning of route of
drainage and identification of
complications.
• Treatment – analgesia and IV antibiotics.
Manage sepsis with paediatrician.
Drainage if signs of airway compromise
or no response to antibiotics. Liaise with
senior anaesthetist for naso-tracheal
intubation following gaseous induction
taking care not to rupture collection.
Oro-tracheal intubation over a rigid
endoscope may be required. Trans-oral
drainage is ideal first line route. Monitor
for signs of recurrence of complications.

Advanced Answer
question(s)

What are the • An extensive collection is best drained


principles of externally in order to allow insertion of a
draining a retro- drain, obtain tissue for histology and
pharyngeal thoroughly irrigate the cavity. Trans-

- 36 -
collection cervical incision at hyoid level retracting
extending into the SCM laterally and rotating larynx and
thorax? pharynx medially following ligation of
middle thyroid vein.
• Collections above T4 will drain through a
trans-cervical incision. Those below will
require discussion with a paediatric
surgeon regarding need for trans-
thoracic drainage.
• Repeat imaging is essential to ensure
resolution and management with a
paediatrician in a level 2 or 3
environment.

1.9 JUVENILE RESPIRATORY PAPILLOMATOSIS

SCENARIO You are referred a 4 year old boy in whom


the parents are concerned about persistent
hoarseness.

Introductory Answer
question

What is your 1. Vocal cord nodules


differential 2. Respiratory papilloma
diagnosis?
3. Extra-esophageal reflux
4. Vocal cord palsy

- 37 -
5. Chronic laryngitis

Competency Answer
question(s)

What is recurrent • An acquired condition of viral aetiology


respiratory chiefly HPV 6 and HPV 11 serotypes and
papillomatosis? typified by the presence of papillomata
within the laryngo-tracheal tract and
How would you beyond. Juvenile onset RRP tends to be
manage this more aggressive than adult onset
child? disease, but may spontaneously resolve
in adolescence. The presence of
papillomas beyond the vocal folds
suggests metaplasia of the respiratory
epithelium to squamous epithelium.
• The primary goal is making the diagnosis
with flexible and ultimately rigid airway
endoscopy to stage the disease. Given
that there is no cure for the condition
the management goal is to reduce
morbidity by reducing papilloma bulk to
optimise voice and airway whilst
minimising collateral damage to normal
structures.
• Close cooperation with the anaesthetist
to manage the shared airway with
techniques including: spontaneous
ventilation, intermittent apnoea and jet
ventilation techniques.
• Debulking techniques include: Cold steel,
CO2 laser ablation (risk stenosis or
webbing with overuse as well as spread
of virus to surgeon from the laser smoke)
and microdebrider (the preferred

- 38 -
method of most units).
• Adjuvant therapy may include alpha-
interferon for aggressive disease.
Cidofovir, a cytosine nucleoside
analogue, has been topically intra-
operatively but concerns have been
raised about carcinogenic potential.
• Tracheostomy is sometimes employed in
children whose disease presents a risk of
airway loss, but should be regarded as a
last resort as a tracheostomy has
precipitate distal airway involvement of
papillomata.

Advanced Answer
question(s)

Describe the role • National HPV vaccination strategies have


of HPV been established in the UK. The first
vaccination in the vaccine Cervarix was designed to
management of vaccinate against cervical cancer causing
serotypes 16 and 18.
RRP
• The present vaccine Gardasil is
quadrivalent covering the additional RRP
serotypes 6, 11. It is offered to all girls
aged 12-13yrs and administered as 0.5ml
intramuscularly at a 0, 2 and 6 month
schedule. All three must be given within
12months with the second at least 1
month after the first and the third at
least 3 months after the second.
• It is not licenced for the treatment for
active HPV disease i.e. pre-existing RRP.
• With the apparent rise in HPV-related

- 39 -
oropharyngeal cancer, the vaccine may
be given to boys in the future.

1.10 PRE-AURICULAR SINUS

CLINICAL
PHOTOGRAPH

Introductory Answer
question

What is the • Infected pre-auricular sinus


diagnosis?

Competency Answer
question(s)

Describe the • Pre-auricular sinus arises from failure of


embryology of fusion of the Hillocks of His from the first
this anomaly and branchial arch with attachment to the

- 40 -
how you manage cartilage of the root of helix. 25-50% are
this. bilateral and associated with Syndromes
e.g. Branchio-oto-renal (BOR) syndrome.
Describe the
surgical • Acute infections treated with directed
techniques used antibiotics or I+D if abscess.
to electively treat • Elective assessment of EAC for
this condition connecting pits suggesting first branchial
arch anomaly. Inspect contralateral ear
and neck for second arch cyst/sinus.
• Consider renal tract us/s for BOR
syndrome if pre-auricular sinus and one
of the following:
1. FH of hearing loss, renal anomaly or BOR
syndrome
2. A second dysmorphic feature
3. Maternal gestational diabetes
• Non-infected pits typically remain un-
infected and do not require further
assessment or surgery.
• The 3 techniques include:
1. Simple sinusectomy
2. Wide local excision by a supra-auricular
approach
3. ‘Inside-out’ technique
• Sinsuectomy involves elliptical skin
incision around the punctum and
excision of tract using probe +/-
methylene blue. 20-40% risk of
recurrence
• Wide local excision (Emery) includes an

- 41 -
elliptical incision extended in a curvilear
fashion above the pinna. An en-bloc
specimen is removed with a cuff of
perichondrium from helical root and
tissue removed to the depth of the
temporalis fascia remaining posterior to
the temporal artery to minimise injury to
the facial nerve branches. 5% risk of
recurrence.
• The inside-out technique (Jensma)
combines the wide-local incision
approach and surgical magnification with
opening of the tract to follow each
branch of the sinus.

Advanced Answer
question(s)

What is the • First arch not a first cleft abnormality.


embryology of a
• The ear is formed by 6 hillocks of His
preauricular derived from the first and second arches
sinus?
• Failure of complete closure of the
hillocks of His results in sinus formation

- 42 -
1.11 MICROTIA

CLINICAL
PHOTOGRAPH

Introductory Answer
question

What is the name Microtia (grade 3)


of this condition?

Competency Answer
question(s)

What are the Functional issues –hearing loss usually CHL


issues related to up to 60dB due to canal atresia +/- ossicular
this condition and

- 43 -
how would you dysfunction. It is important to determine the
manage them? status of the other ear and the presence of
other abnormalities, for example is the
patient syndromic (Golderhar, CHARGE). For
rehabilitation of hearing loss, consider a trial
of bone conduction hearing aid on soft band
and later BAHA
Cosmetic issues –surgical reconstruction or
prosthesis. Only a few centres achieve good
results with surgery, which involves multiple
staged procedures. Bone anchored
prosthesis is probably the preferred option.

Advanced Answer
question(s)

What factors Timing of surgery is crucial. Ideally the


should be patient should be fully involved in the
considered if decision making. In the case of a child around
surgical age 6-7 when the rib is matured enough to
harvest cartilage. The size of the normal
reconstruction is
pinna may be used as a ‘template’
required?
Imaging of the ossicles and facial nerve to
determine possible abnormal anatomy which
may make surgery very challenging.
Multidiscipilinary team involvement

- 44 -
1.12 OTITIS MEDIA WITH EFFUSION (GLUE EAR)

SCENARIO GP referral
(no clinical Please could you see this 4 year old girl
photograph) whose parents have concerns about her
hearing.

Introductory Answer
question

How would you History: Length of history (>3months),


initially assess this speech delay, balance disturbance, pain,
child? history of AOM, nasal problems/nasal block,
snoring/OSA, sore throats, chest infections,
birth history, other infections / immune
deficits.
Examination: otoscopy, nasal airflow, oral
cavity.
Investigations: PTA and tymps since she is 4
years old.

Competency Answer
question(s)

This is what the This is a clinical photograph of the left


ear looks like (on tympanic membrane showing otitis media
both sides). Tell with effusion because of the retracted
me what you see. tympanic membrane, cloudy appearance and
neoannulus; the hyperaemia of the tympanic

- 45 -
membrane probably indicates a recent URTI.
Watch and wait (at least 3months).
Medications: No evidence that
decongestants, antibiotics or antihistamines
What are the non- are beneficial. Steroids may be helpful but
surgical options has significant side effects.
for treating glue
Autoinflation such as with the Otovent
ear?
balloon has been shown to improve effusions
over a 2 week to 3 month period but
evidence for longer term benefit is lacking.
Bearing in mind the complications associated
with ventilation tube insertion, many
clinicians consider autoinflation techniques
particularly useful in cases of unilateral
middle ear effusions.
Hearing aids should also be considered.
Adherence to the 2008 NICE guidelines are
often required.

Advanced Answer
question(s)

How would you Reassess with particular attention to nasal


manage a patient function and pathology, also the patient’s
who has had 3 immune status should be considered.
sets of Examine the ears carefully for the presence
conventional of retraction pockets.
grommets in the
Options: optimise nasal function, medical
past, and a t-tube
(hearing aid), surgical – another t-tube, extra-
which has fallen
annular t-tube.
out, who develops
a further middle
ear effusion?

- 46 -
1.13 BRANCHIAL CLEFT SINUS

SCENARIO You are referred a 6year-old boy with a


discharging sinus on the lateral aspect of the
(no clinical
neck behind the angle of the mandible.
photograph)

Introductory Answer
question

What is your Branchial cleft sinus


differential
Lateral thyroglossal duct anomaly
diagnosis?
Mycobacterial disease
Actinomycosis

Competency Answer
question(s)

How would you History – Pre-existing punctum or cyst. Prior


manage this local surgery. FH of Branchio-Oto-Renal
patient? syndrome.
Examination – inspect EAC for
communication and contra-lateral neck for
sinus/cyst.
Investigation – fistulogram of tract. MRI
considered. Barium swallow if type 3 or 4
cleft anamoly is suspected.
Treatment – definitive treatment is excision

- 47 -
during quiescence. Type 1 sinus is EAC
duplication and approached through
parotidectomy incision with facial nerve
monitoring. Type 2 sinus traced between
internal/external carotid above CN12 into
tonsil in older children via step-ladder
incision.

Advanced Answer
question(s)

Describe the Type 3 and 4 sinuses both communicate with


presentation of the pyriform fossa and often the ipsilateral
type 3 and 4 cleft thyroid gland and may present with recurrent
sinus and its suppurative thyroiditis.
operative
Type 3 sinus extends between CN10 and
management
common carotid piercing the thyro-hyoid
membrane.
Type 4 sinuses are very uncommon and run a
course parallel to the recurrent laryngeal
nerve by pierce the cricothyroid membrane.
Surgical options include excision of tract +/-
hemi-thyroidectomy or endoscopic cautery
of the sinus opening into piriform fossa in
young children.

- 48 -
1.14 DROOLING

SCENARIO You are asked to see a 4 yrs-old child. His


mother is concerned about his face and
(and clinical clothes being regularly soiled by saliva
photo)

Introductory Answer
question

What is the Drooling/sialorrhoea


clinical name for
Drooling may be physiological up to 5 years
this condition and old. Due to overflow, not over-production.
at what age is this Associated with poorer oromotor
condition coordination in younger children.
considered
abnormal?

- 49 -
Competency Answer
question(s)

History and examination: number of bibs,


How would you
clothing changes per day. Oromotor
examine him
coordination/ control
specifically?
Physical and mental development: ?age
appropriate. Dental problems (?has the
dentist been involved)
Impact on family and quality of life.

Which groups of
patients have Chronic drooling: Cerebral palsy, syndromic
drooling? (e.g. Trisomy-21), children on anti-
convulsants.
Acute drooling: foreign body, infections e.g.
epiglotitis, tonsilitis, gingivitis.
How would you
manage this
child? Management should be guided by the cause
and severity of drooling and an appreciation
of the psychosocial impact. Management of a
drooling child is challenging and requires a
multi-disciplinary approach. Involve the
community paediatrician, paediatric
neurologist, dentist, physiotherapist etc.
Severity may be graded by the presence of
peri-oral skin maceration and the number of
clothes changes per day for the soiling.
Investigation includes MDT assessment of
head control, dental malocclusion and
swallowing dysfunction. Assess for causes of
hypersecretion eg dental carries and causes
of incomplete lip seal eg macroglossia.

- 50 -
Conservative: sitting positions, changing food
texture, oromotor exercises, cognitive
behavioural therapy
Medical: Hyoscine, glycopyrolate
Botulinum toxin injection. This is normally
available at tertiary paediatric units. Can be
injected under ultrasound guidance and can
be repeated. Effect wears off after 3 - 4
months.
Surgery: tonsillectomy, salivary gland
surgery.

Advanced Answer
question(s)

How would this Surgical options include submandibular duct


child’s co- re-positioning, submandibular +/- sublingual
morbidity affect gland excision and parotid duct ligation.
your surgical
Duct re-routing has low morbidity but may
options?
increase the tendency for salivary aspiration
if pre-existing dysphagia.
Associated risks of re-routing include ranula
formation.
Bilateral SMG removal may increase
likelihood of dental caries because of
reduced protective effect of saliva. Parotid
duct tie off carries risk of sialocele formation.
Aspiration needs to be excluded before
embarking on surgery.

- 51 -
1.15 LARYNGOMALACIA

SCENARIO You are asked to see a 10-week-old boy who


mother is concerned with persistent noisy
(no clinical
breathing.
photograph)

Introductory Answer
question

What is your Stertor may be due nasal obstruction caused


differential by piriform aperture stenosis, choanal atresia,
diagnosis for nasal and post-nasal mass, Pharyngeal
noisy breathing? obstruction due to large tonsils, poor muscle
tone, glossoptosis +/- mandibular hypoplasia
Stridor may be due to supraglottic pathology,
including laryngomalacia, cysts or
papillomata, Glottic pathology including vocal
cord palsy, glottis web, cysts, papillomata,
Subglottic pathology including stenosis, cysts,
haemangioma, Tracheal including vascular
ring/sling, congenital anomalies,
tracheobronchomalacia.

Competency Answer
question(s)

What is the most Laryngomalacia is the most common cause of


likely diagnosis chronic stridor in infancy.
and how would
History – Age at onset, rate of progression,
you manage this

- 52 -
patient? impact on feeding, choking, apnoea, and
cyanosis. Birth history as well as number and
When would you
duration intubations. Neuromuscular
perform direct
disorders.
laryngoscopy?
Examination – effort and efficacy of
ventilation including nasal flare, tracheal tug,
chest recession. Phase and type of noise:
pharyngeal stertor improved with jaw thrust
or laryngotracheal stridor. Nasal cold spatula
testing. Oral examination – cleft palate and
glossoptosis. Neck – mass. Top to toe exam
for cutaneous haemangioma.
Fa

Growth chart from ‘red book’.


st

A child with a history consistent with simple


-P roo

laryngomalacia with inspiratory stridor,


rin f

thriving without prior tracheal intubation or


P

t P Co

haemangioma may be managed with regular


outpatient review, an outpatient supraglottic
ub py

examination and treatment of co-existing


extra-oesophageal reflux.
lis
hi

Direct laryngoscopy should be performed if


n

history of intubation, haemangioma,


g

diagnostic uncertainty, biphasic stridor or


failure to thrive.

Advanced Answer
question(s)

The child Secondary airway lesions are present in over


undergoes rigid 50% of children with severe laryngomalacia
laryngo-tracheo- who require aryepiglottoplasty.
bronchoscopy. A
I would assess for any dynamic component of
mid-tracheal
the narrowing including deterioration on
compression is
expiration i.e. tracheomalacia, pulsatility or

- 53 -
noted. What is fixed narrowing which may result from a
the significance of complete tracheal ring.
this and how
Any possibility of extrinsic compression
would you
should be investigated with CT thorax which
manage this
in young infants may be performed by ‘feed
child?
and wrap’ techniques or in older children may
require sedation using local protocol eg oral
chloral hydrate +/- rectal paraldehyde. Rarely
infants may decompensate with sedatives
and may need imaging under general
anaesthetic with intubation.
Vascular compression due to complete rings
include double aortic arch causing
posterolateral compression of the distal
trachea, right aortic arch with left
ligamentum arteriosum. Incomplete rings
may be caused by aberrant innominate artery
compression of the anterior wall of the upper
trachea. Compression may also be caused by
anomalous left or right pulmonary artery
(pulmonary artery sling).

- 54 -
1.16 DOWN SYNDROME

SCENARIO
(clinical
photograph)

Introductory Answer
question

This young boy • This young boy has classical features of


just moved into Down syndrome; these include
the area. What upslanting palpebral fissures with
syndrome is he epicanthic folds, small low set ears,
affected by? wide nasal bridge and open mouth
because of macroglossia.

Competency Answer
question(s)

Why might he • Patients with Down syndrome may have

- 55 -
come to see you disorders pertaining to the Ear, Nose,
as an ENT Throat and Neck.
surgeon?
• Ears: Small low set pinnae, Ear canal
stenosis, Conductive hearing loss, Wax
impaction, Otitis Media with Effusion
(OME) (decreased immunity, hypotonic
palate, Eustachian tube dysfunction),
Cholesteatoma, Ossicular abnormalities
(deformed stapes, thickened malleus),
Up to 80% facial nerve dehiscence (wide
angle 2nd genu), Sensorineural hearing
loss in 21% of patients under 20 years
of age, 55% of patients over 20 years
(labyrinthine hypoplasia, small bony
island LSCC)
• Nose: Structure (cosmesis) and
function, Flat, Nasal congestion
(increased URTI), drooling, Small narrow
nasopharynx (OME), Sinus hypoplasia
(increased sinonasal disease),
hypotonicity of pharynx
• Throat (inc Airway): macroglossia,
Obstructive Sleep Apnoea, no lip seal,
drooling, subglottic narrowing,
recurrent croup (reduced
immunity/immature T and B cell
function), smaller endotracheal tube
needed
• Neck: Atlantoaxial instability (10%); this
is relevant to Tonsillectomy, Ear
Surgery. Pre-op x-ray neck +/-
neurology opinion may be needed.
Patients should be operated in the
neutral position.

- 56 -
Advanced Answer
question(s)

Why is there an • May be related to subglottic narrowing


increased (Steeple’s sign on X-ray) and/or
incidence of dysfunctional immune system with
CROUP in children reduced/immature T and B cell
with Down function.
syndrome?

1.17 HAEMANGIOMAS

SCENARIO You are asked to see a 2 month old infant


with noisy breathing worse when crying and
(no clinical
associated with feeding difficulty. There is a
photograph)
quiet biphasic stridor at rest. You notice a red
lesion on his chin.

Introductory Answer
question

What is your • The nature of stridor suggests subglottic


differential pathology.
diagnosis? • The association with a red facial lesion
suggest a haemangioma or a vascular
malformation.
• The differential includes subglottic

- 57 -
stenosis (primary or acquired), subglottic
web/cyst and foreign body

Competency Answer
question(s)

Assuming you • History – exclude associated diagnosis eg


suspect a choking episodes and previous
subglottic intubations. Birth and developmental
haemangioma history. Assess impact on feeding.
how would you
• Examination – Note any dysmorphism
manage this
and degree of respiratory effort i.e.
patient?
tug/recession and efficacy i.e. oxygen
What are the saturation. Assess nasal patency, oral
side-effects of examination and fibre optic supraglottic
propanolol? assessment in clinic if available. Top to
toe exam for haemangioma of infancy.
Growth chart for failure to thrive.
• Investigations – Will require rigid airway
examination under GA for diagnosis
whilst spontaneous ventilating with
inhalational anaesthesia via
nasopharyngeal airway.
• Treatment – the pathology may grow
over the first few months of life. Most
patients with haemangioma do not
require treatment but functionally
impairing lesions (especially subglottic)
may be treated with propranolol,
systemic or intra-lesional steroids.
Open/laser resection, tracheostomy and
historically intra-lesional vincristine.
• Propranolol is a non-selective beta-
antagonist, therefore bradycardia and

- 58 -
potential heart failure, hypoglycaemia,
bronchospasm and hypotension are
possible side effects.

Advanced Answer
question(s)

What are the 1. Vasoconstriction leads to colour change


proposed and softening of the haemangioma
mechanisms of
2. Down regulation of RAF-mitogen-
action of
activated protein kinase pathway
propanolol.
3. Decreased expression of VEGF and b-FGF
What dose of
genes
propranolol
would you 4. Induction of apoptosis of capillary
prescribe? endothelial cells.
• Start with 0.16mg/kg/day to max
2mg/kg/day; initial treatment should be
in hospital as cardiac monitoring is
required as well as paediatrician input for
possible hypoglycaemia
• Note that 10% of haemangiomas may not
respond.

1.18 CYSTIC HYGROMA

SCENARIO You are asked to see an 18 month-old child


with a history of neck swelling since birth.
(no clinical
photograph)

- 59 -
Introductory Answer
question

What is your • Cervical cystic hygroma


differential
• Haemangioma
diagnosis?
• Teratoma
• Simple cyst
• Branchial cyst
• Cervical lymphadenopathy secondary to
e.g. atypical mycobacteria

Competency Answer
question(s)

How might they • Typically present with soft, smooth,


present and how transilluminating, compressible, painless
would you swelling in the posterior triangle of the
manage this neck.
child? • Airway obstruction is possible

- 60 -
• Microcystic oral disease may present
with acute infection and bleeding
Management is led by whether the disease is
microcystic and macrocystic, whether it
impacts or will impact on function and
whether a staged approach is appropriate.
MR imaging can provide a radiological
diagnosis and stage the disease.
Only 5% of such lesions spontaneously
involve. The two treatment options are
1. Intra-lesional sclerotherapy – no
consensus on best therapy. Requires
ultrasound guided aspiration and
injection under GA with acute
inflammatory response needing to be
covered with anti-inflammatories or
occasionally elective intubation.
2. Open excision – aim to debulk
macrocystic disease. Oral, pharyngeal or
laryngeal disease may be treated with
surface coblation.

Advanced Answer
question(s)

Describe a staging De Seres Classification (STAGE – LOCATION)


system for I Unilateral infrahyoid
cervico-facial
II Unilateral suprahyoid
cystic hygroma.
III Unilateral, supra and infrahyoid
IV Bilateral suprahyoid
V Bilateral supra and infrahyoid

- 61 -
Giguere Classification
Give an example • Macrocystic – cystic spaces at least 2cm
of a sclerosant or more
used for intra- • Microcystic – cystic spaces less than 2cm
lesional • Mixed
sclerotherpy and
All sclerosants work by the same principle of
give alternatives.
provoking an inflammatory thus causing
fibrosis and obliterating cystic spaces
OK-432, ( Picibanil )
• Inactive strain of Group A Streptococcus
pyogenes. Unique challenge is highly
variable availability.
• This is not licensed in the UK.
Doxycycline
• 10mg/ml by ultrasound guided injection
under GA. Maximum suggested dose of
100mg for term children 12months and
200mg if >12months. Unique side-effects
include possible hypoglycaemia and
macrolide hypersensitivity.

- 62 -
1.19 THYROGLOSSAL DUCT CYST

Clinical
photograph

Introductory Answer
question

What is the • Thyroglossal duct cyst (TGDC)


differential
• Midline dermoid cyst
diagnosis?
• Sebaceous cyst
• Midline branchial cleft cyst
• Lymph node

Competency Answer
question(s)

What clinical test • TGDC will elevate on both swallowing


might assist in and tongue protrusion.
diagnosis?
• TGDC represents the failure of

- 63 -
Explain how the obliteration of the developmental tract
embryology of that the thyroid tissue makes from its
thyroglossal duct origin at the foramen caecum of the
cyst informs the tongue (junction of the anterior 2/3 and
operative posterior 1/3).
management.
• Complete excision of the TGDC should
include the descending tract from the
tongue base across the midline of the
hyoid bone. This forms the embryological
basis of the Sistrunk procedure.
• Thyroid tissue within the TGDC may
represent the only functioning thyroid
tissue. This may be established by pre-
operative cervical us/s to minimise the
risk of removing the only functioning
thyroid tissue.

Advanced Answer
question(s)

What are the • Procedure specific complications are


main accidental pharyngostomy, hypoglossal
complications of nerve injury and TGDC recurrence.
this procedure • Pharyngostomy may be minimised by
and how might careful dissection on the deep surface of
these be the TGDC. CN12 injury is minimised by
minimised? avoiding the lateral cornu of the hyoid.
TDGC recurrence may be further
improved by performing an en-bloc
resection of the central compartment
tissue either side of the TGDC tract up to
the hyoid bone and tongue base.

- 64 -
1.20 LINGUAL THYROID

CLINICAL
PHOTOGRAPH

Introductory Answer
question

What is the • Lingual thyroid


differential
• Thyroglossal duct cyst
diagnosis?
• Midline branchial cleft cyst
• Lymphangioma
• Dermoid cyst
• Minor salivary gland tumour
• Tongue base tumour

Competency Answer
question(s)

What • Technetium Tc-99m sodium thyroid scan.


investigations This will confirm the nature of the

- 65 -
could you swelling and establish the presence of
perform to functioning thyroid tissue elsewhere. In
confirm the 75% cases the ectopic thyroid tissue
diagnosis? represents the only functioning thyroid
tissue in the body.
• Most ectopic tissue is asymptomatic but
How would you
may increase in size and become
manage this
symptomatic during puberty and
patient?
pregnancy. It is four times more common
in females. They may bleed, and
malignant transformation has been
reported.
• In symptomatic older patients unfit for
complete resection partial trans-oral
ablation is suggested. Radioactive iodine
ablation has also been used.
• In younger patients with a consequently
greater risk of malignant transformation
complete resection is advised. If possible
trans-orally or otherwise by trans-cervical
pharyngostomy and a covering
tracheostomy. Post-operative T4
replacement may be given to suppress
ectopic thyroid tissue recurrence.

Advanced Answer
question(s)

What are the • Lingual


main sites of
• Sublingual
ectopic thyroid
tissue? • Pre-tracheal
• Mediastinal

- 66 -
1.21 CONGENITAL MIDLINE NASAL MASS

SCENARIO
(clinical
photograph)

Introductory Answer
question

Describe what • T1 weighted image showing a midline


you see in the MR line nasal lesion communicating with the
Images. third ventricle of the brain and T2
weighted image of the same lesion; the
most likely diagnosis is a third ventricular
encephalocele.
What is the • Nasal glioma
differential
diagnosis for • Nasal dermoid
midline nasal • Encepahlocele
mass?
• Meningoencephalocele

Competency Answer
question(s)

How would • History – Increase in size of the lesion on

- 67 -
manage this crying or straining. Previous meningitis.
patient? Vaccination history.
• Examination – nasal endoscopic
examination. Furstenberg sign -
compression of the ipsilateral jugular
vein leads to an increase in size of the
mass.
• Investigation – combined CT and MR
imaging to determine the presence and
nature of intra-cranial extension.
• Treatment – early planned surgery is
advised due to risk of meningitis in the
presence of cyst infection and intra-
cranial tract extension.

Advanced Answer
question(s)

A MRI was • No biopsy to be performed


performed
• I would use a transverse or lateral
and
rhinotomy incision. If the lesion extended
demonstrate
into the lower half of the nose an open
s a cystic
rhinoplasty approach may be used.
lesion with
no • If intra-nasal components are present
intracranial they may be removed with a combined
extension. endoscopic resection.
Describe your
surgical • Any intra-cranial extension necessitates
approach. the assistance of an anterior skull-base
surgeon who may use a sub-frontal
craniotomy for access and dural repair.

- 68 -
1.22 CLEFT LIP AND PALATE

CLINICAL PHOTO

Introductory Answer
question

What is the • Unilateral cleft lip


diagnosis?
• Would need to perform oral examination
to determine extent and palatal integrity.

Competency Answer
question(s)

Describe a • Clefting may be of the lip, palate or


classification combined (CLAP).
system for clefts
• Primary palatal clefting is anterior to the
and a schedule of
incisive foramen and can include the
treatment
alveolus. Secondary palatal cleft are
justifying your
posterior to incisive foramen and may
answer.

- 69 -
involve the soft palate.
• Veau classification of palatal cleft:
Class 1 – isolated soft palate
Class 2 – isolated hard palate
Class 3 - unilateral CLAP
Class 4 – bilateral CLAP
• Management requires a multidisciplinary
approach including speech and language
therapist, orthodontist, facial plastic and
cleft surgeon.
• Infancy – cleft lip repair once 10weeks or
10 pounds, to aid oral feeding
• Toddler – cleft palate repair at 10months
to aid phonation
• Late childhood – alveolar bone grafting at
12years +/- dental implant
• Early adulthood – midfacial advancement
and rhinoplasty options.

Advanced Answer
question(s)

Describe the • Failure of midline fusion of soft palate


clinical features musculature. Typically associated with a
of submucosal bifid uvula and midline notching of the
clefting and its free border of the hard palate.
relevance to the
• Resulting weakness in the Eustachian
ENT surgeon.
tube dilators increases the tendency to
recurrent OME.
• Adenoidectomy may result in

- 70 -
velopharyngeal insufficiency (VPI)
• Children with suspected submucosal cleft
are best assessed prior to adenoidectomy
by the cleft MDT. Alternatively suction
diathermy adenoidectomy may be
undertaken leaving a band of tissue
against which the soft palate may
approximate to minimise the risk of
permanent VPI.

1.23 CONGENITAL CHOLESTEATOMA

SCENARIO You are asked to see a 10yrs-old child who is


complaining of unilateral hearing loss. On
(and clinical
Otoscopy a white mass is seen behind the
photograph)
tympanic membrane.

- 71 -
Introductory Answer
question

What is the most Congenital cholesteatoma.


likely clinical
diagnosis?

Competency Answer
question(s)

What are the • The Derlacki and Clemis criteria require:


requirements for 1. White mass medial to an intact TM
a diagnosis of
2. Normal pars tensa and pars flacida
congenital
cholesteatoma? 3. No previous ear surgery, otorrhoea or
perforation
How does the
location of • Congenital cholesteatoma typically
begins in the antero-superior
congenital
mesotympanum and is described to be
cholesteatoma more extensive on presentation but no
differ from more likely to recur if completely excised.
primary acquired
cholesteatoma.

Advanced Answer
question(s)

Describe how the Several theories have been proposed to


aetiology of explain the congenital cholesteatoma.
congenital These include:
cholesteatoma 1. Persistence of epidermal rest cells in the

- 72 -
differs from middle ear.
primary acquired 2. Epithelial cells present in the amniotic
cholesteatoma. fluid may enter the middle ear cleft and
subsequently grow into a cholesteatoma.
Theories for acquired cholesteatoma include:
1. Immigration
2. Retraction
3. hyperplasia due to chronic inflammation
4. Metaplasian due to vit deficiency
5. Implantation
For further details see Persaud et al 2007 “An
evidence-based review of the
aetiopathogenic theories of congenital
and acquired cholesteatoma.” Journal
Laryngology Otology. 121 (11):1013-
1019.

1.24 WAARDENBURG SYNDROME

Clinical
photograph

- 73 -
Introductory Answer
question

What is the • Waardenburg syndrome – auditory-


diagnosis? pigmentary syndrome.

Competency Answer
question(s)

List the syndromic • Clinical diagnosis requires 2 major criteria


features of or 1 major and 2 minor
Waardenburg
• Major – dystopia canthorum, white
syndrome.
forelock, heterochromia iridium,
congenital SNHL, affected first degree
relative
• Minor – high and broad nasal root,
hypoplastic nasal alae, medial eyebrow
flare, skin hypopigmentation, premature
greying of hair
• Type 1 – with dystopia canthorum
• Type 2 – without dystopia canthorum
• Type 3 – as for type 1 plus
musculoskeletal abnormality
• Type 4 – as for type 2 plus Hirschprung’s
disease.

Advanced Answer
question(s)

What is the • Type 1/2/3 are inherited in an autosomal


pattern of dominant fashion.
inheritance of the

- 74 -
various subtypes? • Type 4 is inherited in an autosomal
recessive fashion.
• Type 1/3 – heterozygous
haploinsufficiency from PAX3 mutation
• Type 2 – MITF mutation.
• Type 4 – homozygous mutation in EDN3
(endothelin 3) or endothelin receptor B
(EDNRB).

1.25 RANULA

SCENARIO

(clinical
photograph)

Introductory Answer
question

What is the • Extravasation mucocele (ranula)


differential
• Lymphangioma

- 75 -
diagnosis? • Dermoid cyst
• Supra-hyoid thyroglossal duct cyst
• Ectopic thyroid

Competency Answer
question(s)

How would you • The diagnosis is clinical but may be aided


manage this by aspiration of mucus from the ranula
patient? and a staging MRI if there is significant
cervical extension.
• If symptomatic the pseudo-cyst can be
treated by intra-lesional sclerosant but
the most effective therapy is complete
excision of the cyst and associated
causative salivary gland – typically the
sublingual.
• Large ranula may ‘descend’ through the
floor of mouth musculature into the
neck. Such lesions often require intra-
operative decompression to facilitate
excision.

Advanced Answer
question(s)

Please explain • The sublingual gland empties into several


why ranula small ducts (ducts of Rivinus) which unite
formation may to form the duct of Bartholin which in
complicate turn drains onto the submandibular duct.
Wharton’s duct Repositioning surgery may injure
repositioning. Bartholin’s duct leading to ranula
formation.

- 76 -
• The risk of ranula formation may be
minimised by resecting the sublingual
gland when repositioning Wharton’s
duct.

1.26 CERVICAL LYMPHADENOPATHY

SCENARIO GP referral: Please could you see this 4 year


old boy with a lump behind right angle of
jaw/upper neck

Introductory Answer
question

Describe the • This is a clinical photograph showing a

- 77 -
clinical swelling in level II of the neck of a child.
photograph.

Competency Answer
question(s)

What points are • History: duration, change in size, URTI,


you going to Child well, skin conditions scalp, weight
cover in the loss, night sweats
history and what
• Examination: Size, site, shape, skin over
are you going to
lump, temperature, tenderness,
look for on
transillumination, consistency,
examining him?
attachment, mobility, pulsatility, fixed,
What would reducible, irreducible, additional lumps.
make you Ears nose & throat examination, Axillae
reassure and or groin lymphadenopathy ,
discharge a child hepatosplenomegally.
with
lymphadenopathy • Discharge: History of association URTI,
at first Fluctuating in size, Soft, small (<2cm),
appointment? mobile nodes, Instruct to return if nodes
get progressively bigger
What would
make you list • List for excision: Supraclavicular LN, Node
such a child for >3cm, History of malignancy, Generalised
excision biopsy at lymphadenopathy, Hepatosplenomegally
first • Fever, weight loss night sweats
appointment? Are
there any tests • Tests pre-op FBC, CXR (to exclude
you would want mediastinial lymph nodes)
pre-op? • Neither discharge nor excise; CXR, FBC,
If you are not Serology for Bartonella, EBV, CMV,
discharging or toxoplasma and Brucellosis and HIV
listing the child testings. Ultrasound Neck
what are you • Then next appointment…

- 78 -
going to do? – If serology positive (10%)– Discharge
And what do you – If negative and lump same or bigger – List
do at the next for excision
appointment? – If negative and lump smaller - Discharge

Advanced Answer
question(s)

What do you • Malignant proliferation of the lymphoid


know about system associated with B-symptoms:
lymphoma? weight loss, night sweats and fevers.
• May be Hodgkin’s or non-Hodgkin’s
lymphoma depending on the presence of
Reed-Sternberg cells.
• 4 subtypes of Hodgkin’s lymphoma (i)
Nodular sclerosing (ii) Mixed Cellularity
(iii) Lymphocyte predominant and (iv)
Lymphocyte depleted
• Diagnosis by lymph node biopsy.
• Staging: CT Chest and abdomen, bone
marrow biopsy, Stage 1 - Confined to
single lymph node region. Stage 2 –
Involvement of 2 or more regions on
same side of diaphragm. Stage 3 –
Involvement of nodes on both sides of
the diaphragm. Stage 4 – Spread beyond
the lymph nodes.

- 79 -
1.27 TRAUMATIC PERFORATION OF TYMPANIC MEMBRANE AND
NON-ACCIDENTAL INJURY (NAI)

SCENARIO A 5 year old boy is brought to the Emergency


Department (ED) by his parents with a
bleeding, bruised and swollen right ear. His
Mother stated that he fell onto that side 5
days ago and it has only now started bleeding
and become swollen. When the ED registrar
assessed the child, his Father stated that he
had been hit in the ear at school yesterday.
On examination, you notice a fresh bruise
over his right pinna and mastoid area and an
older bruise around his left eye. There is
dried blood at his meatus. Dad states that
the eye bruise had happened at school. The
child looks scared and does not talk to you.

Introductory Answer
question

What is your • Blunt ear trauma


differential
• Pinna Haematoma
diagnosis?
• Underlying coagulopathy
• Balance disorder leading to falls
• Otitis Media with perforation

- 80 -
• Non-accidental injury (NAI)

Competency Answer
question(s)

This is what is • This an otoscopic image showing a right


seen on tympanic membrane with an anterior
Otoscopy. perforation. There is a haematoma of
Describe what the posterior wall of the ear canal and
you see and blood crusts in the canal. This would be
explain your consistent with a spontaneous or
diagnosis. traumatic perforation. In the absence of
any history of infection, and in the
presence of external bruising, a traumatic
perforation by non-accidental injury
would be the most likely diagnosis.
• A fall onto this ear five days ago, causing
this amount of trauma would be unlikely.
The bleeding would have occurred
immediately after the injury and the
bruising and swelling would have come
on within an hour – not five days later.
• A blow to that ear the day previously may
be a plausible explanation, however it
would have needed to be a blow of
considerable force to cause a
perforation, and the school are likely to
have a record of this.
• The major alarming factor is the presence
of multiple injuries of different ages.
• The inconsistency and implausibility of
the stories concerning the injuries are
also an alerting factor, as are delayed
presentations.

- 81 -
• A quiet child who refuses to speak, or the
so called “frozen watchfulness” in a post-
lingual child are also signs to be aware of.

Advanced Answer
question(s)

How would you • In line with the GMC document “Good


manage this Medical Practice”, it is the duty of all
case? doctors to protect Children and Young
People and act on any concerns they
have about the safety or welfare of a
child or young person.
• You must read the GMC guidance on
protecting Children and Young people.
• It is the duty of every doctor working
with, or coming into contact with
children, to be aware of their local child
protection procedures.
• If I am concerned about NAI, I would
inform the the Responsible Officer of the
Child Protection Team at the hospital;
this is usually a paediatric doctor. The
team will carry out the necessary
assessment and then liaise with the
appropriate agency, such as your local
authority children’s services, the NSPCC
or the police.
• If there is serious concern, admit the
child to hospital to allow time to contact
local child protection services and enlist
their expertise.
• It is important to explore all possibilities
of diagnosis including NAI, as rare causes

- 82 -
can sometimes be missed.
• It is important to make sure that the
clinical needs of the child are met and
not overshadowed by the child
protection concerns. In this case consider
topical antibiotics and aural toilet to
remove crusts.

1.28 CAUSTIC INGESTION AND NON-ACCIDENTAL INJURY

SCENARIO You are asked to see a 10month old in A+E


who was brought by her mother with
(no clinical
reluctance to drink having been alone in the
photograph)
kitchen. She is apyrexial but drooling with
ulceration to chin and lips.

Introductory Answer
question

What is your • Caustic ingestion +/- NAI neglect


differential
• Herpes stomatitis
diagnosis?
• Kawasaki disease

Competency Answer
question(s)

How would you • History – What may have been ingested,

- 83 -
manage this when, volume and has the bottle been
patient? recovered. Alkali results in a deeper
liquefactive necrosis than the coagulative
necrosis of acids.
• Examination – Evidence of airway
compromise and staging of injury.
Inspect for signs of NAI eg injury out of
keeping with a child of that age.
• Investigations – In conjunction with a
paediatrician blood taken for
paracetamol and salicylate levels.
• Treatment – If airway compromise
present child will likely need to be
intubated in theatre. If not emergent an
upper endoscopy should be carried out
within 48hrs to stage extent and depth of
injury. Any ulcerative processes require
prophylactic antibiotics, PPI +/-
corticosteroids. Nasogastric tube
insertion and parenteral nutrition should
be considered.
• Follow-up – be observant for
complications including bleeding,
perforation/sepsis and stricture
formation.

Advanced Answer
question(s)

What would you • These are signs consistent with physical


do? abuse or potential neglect.
• I would liaise with a paediatrician
specialising in child protection who in
turn should liaise with the statutory child

- 84 -
protection authority. I would ensure that
the child remains in a place of safety until
this is achieved and that the nursing staff
are aware of these concerns.
• I would take a full social history and
consider the need to protect any siblings
at home from abuse.
• Consider the need for support and
protection of other family members
within a multi-agency team framework.

1.29 JUVENILE NASOPHARYNGEAL ANGIOFIBROMA (JNA)

SCENARIO A 14 year old boy presents to your outpatient


clinic with progressively worsening
(no clinical
intermittent right-sided epistaxis.
photograph)

Introductory Answer
question

What would you • Easily bruises


specifically look
• Family history of bleeding disorders
for in the history
and on • Intranasal prominent vessel or old
examination, and bleeding point
what is your
differential • Unilateral intranasal lesion
diagnosis?

- 85 -
• Idiopathic epistaxis
• Digital trauma
• Allergic rhinitis
• Bleeding disorder
• Pyogenic granuloma
• Juvenile nasopharyngeal angiofibroma
(JNA)

Competency Answer
question(s)

After seeing a DO NOT BIOPSY!


right-sided
Differential
intranasal
polypoidal lesion, • JNA
how would you go
• Antrochoanal polyp
on to investigate
this case? • Pyogenic granuloma
CT imaging Investigations
reports a likely
• CT/CTA, MRI/MRA or Carotid
JNA with anterior angiography (looking specifically at
bowing of the ascending pharyngeal or sphenopalatine
posterior arteries)
maxillary sinus
• Holman-Miller sign – also includes
wall. What is the posterior bowing of the pterygoid plate.
name of this sign
and its other
feature?

- 86 -
Advanced Answer
question(s)

How would you Fisch Classification


classify a JNA and
I – limited to nasal cavity
what are the
treatment II – into sinuses or pterygomaxillary fossa
options? (bony destruction)
III – into infratemporal fossa or parasellar
IV – into cavernous sinus, optic chiasm,
pituitary
Treatment
• Embolise prior to surgery
• Surgery
1) Endoscopic (if small)
2) Transpalatal (junction of hard/soft palate –
rarely due to disruption of the growth
plate)
3) Transantral (Caldwell-Luc)
4) Combined lateral rhinotomy and
transnasal
5) Maxillary swing
6) Craniofacial resection (if cribiform plate
involved)
• Recurrence reduced by drilling basi-
sphenoid

- 87 -
1.30 AIRWAY FOREIGN BODY

An otherwise healthy 4 year old boy has been


SCENARIO
brought to Accident and Emergency by his
(FOLLOWED BY A anxious parents. He had been playing in the
CLINICAL loft when they found him choking and
PHOTOGRAPH) coughing. He now appears to be well and
does not have any acute airway problems.

Introductory Answer
question

What is going
• Based on the history and clinical
through your presentation, this is an emergency
mind and what situation with a possible foreign body in
would you do? the aerodigestive tract

Competency Answer
question(s)

What would you • I would go to see the child and family,


do? staying calm and in control of the
situation.
• Quickly assess Airway, Breathing and
Circulation
• Take a targeted history – focusing of the
incident, ie witnessed choking episode,
vomiting, cyanosis neck trauma etc.

- 88 -
Ok. Here is the • I would check for stridor, stertor,
chest xray. drooling, dysphagia and dyspnoea
Describe what you • Then I would consider requesting a chest
see. x-ray
• This is a portable erect AP chest x-ray
showing a radiolucent object resembling
a coin in the upper aerodigestive tract
• I would also like to see a lateral view to
assess if the FB is in the oesophagus,
airway or in-between (intra-thoracic)
• Inform the senior anaesthetist and
What will you do paediatrician. Consider informing the
next? senior emergency theatre coordinator
about the case and to identify
appropriate surgical instruments
required (e.g. forceps, rigid endoscopes,
paediatric tracehoestomy tubes etc.)
• Do the above but ask for the senior
anaesthetist, A&E consultant to be
present.
• Consider transferring the child to Majors
or Resus.
• Consider giving the child some Heliox
(Mixture of helium and oxygen. The
composition has a lower density than
room air, thus making breathing easier in
cases or airway obstruction.)
• This child needs to go to theatre to have
this foreign body removed.
• Speak to the family and seek informed,
signed consent.

- 89 -
• If you had informed the other health-
care professionals earlier, transfer to
theatre may be more expeditious.

Advanced Answer
question(s)

What would you • This is a clinical problem and the child


do if you were in a should be kept in a calm, reassuring
district general environment with his parents. You
hospital and there should contact your hub-and-spoke
was no paediatric paediatric ENT unit urgently to discuss.
anaesthetic They may either send out a retrieval
cover? team or a complete surgical team to
remove the foreign body in theatre.
Name and
describe the • Ventilating bronchoscope, locking
components in mechanism for endoscope, suction
this picture. portal, adaptor for ventilation circuit,
extension collar on the bronchoscope (to
fit with the endoscope) light prism

- 90 -
1.31 SLEEP DISORDERED BREATHING IN A CHILD

SCENARIO A GP refers you a 3 year old boy with snoring


and intermittent episodes of cessation of
(no clinical
breathing. The GP is concerned about the
photograph)
child sleep disordered breathing, in particular
obstructive sleep apnoea.

Introductory Answer
question

What do you A spectrum of conditions ranging from


understand by
1. Snoring (normal blood gases)
sleep disordered
breathing? 2. Upper airway resistant syndrome
(paradoxical and out of phase breathing,
increased pulse transit time)
3. Obstructive hypoventilation (mild
hypercapnia)
4. Obstructive sleep apnoa (hypercapnia
and hypoxia)

Competency Answer
question(s)

How would you History


assess the child in 1. Nocturnal: snoring, number of episodes
clinic? of cessations of breathing, sweating, bed
wetting, sword swallowing position,

- 91 -
When would you restless sleep, frequent awaking.
refer a child for 2. Diurnal: hyperactive, poor concentration,
respiratory swallowing issues, mouth breathing.
investigations
Full ENT Examination (syndromic, nasal
such as obstruction, mouth breathing, large tongue,
polysomnography adenoidal face, OME).
or to a tertiary
Investigations (consider overnight pulse
paediatric centre?
oximetry or full sleep study if child is
syndromic or failure to thrive is present.
• Diagnosis of OSA is unclear
• Age under 2 yrs
• Weight under 15kg
• Down
• Cerebral palsy
• Hypotonia/Neuromuscular disorders
• Craniofacial abnormalities
• Mucopolysaccharidosis
• Obesity
• Significant co-morbidity
• Residual symptoms after adequate
adenotonsillectomy

Advanced Answer
question(s)

Why might you • Pulmonary oedema may occur post


need a HDU bed operatively, when the cause for the
in a tertiary obstruction (eg adenotonsillar
hypertrophy) has been removed.

- 92 -
referral centre? o PEEP may be required in a HDU setting.

- 93 -
Rhinology and Facial Plastics

2.1 ANOSMIA

SCENARIO A 55 year old male chef complains of


inability to detect odours. He has no
(followed by significant past medical history and is
radiological images) otherwise fit and well.

Introductory Answer
question

How would you • This patient may be suffering from


manage this anosmia and therefore I would start by
patient in clinic? taking a targeted history on the aetiology
of the condition, i.e. conductive and
sensorineural causes of Anosmia
• I would then proceed to examine the
nose with a rigid nasendoscope paying
particular attention to the olfactory cleft
areas.
• If no obvious cause is found, I would
proceed to investigate with a University
of Pennsylvania Smell Investigation Test
(UPSIT) or other smell test and maybe a
MRI brain.

- 94 -
Competency Answer
question(s)

Here are the • These are T1 contrast-enhanced MRI


patient’s MRI coronal and saggital sections showing a
brain scans. Tell lesion in the olfactory region of the brain.
me what they
• The lesion has a broad base and bilateral
show?
dural tails; these are features consistent
with a meningioma.
• Such lesion can account for the patient
inability to detect odours (anosmia).
• Refer to a neurosurgeon for management
of the meningioma
• Fit smoke alarms
• Change gas cooker to electric as patient
is unable to smell gas leak
• Document and be aware of sell-by and
use-by date on foods
How would you
counsel this
patient?

Advanced Answer
question(s)

What score on Due to lack of air flow, laryngectomy patients


UPSIT would you become anosmic and therefore would score
expect from a around 10/40. This is because the probability
laryngectomy of getting a correct answer is 1 in 4 and
patient and do patients are told to guess if they don’t know.
you think that it is

- 95 -
different in a A malingerer will score approximately 0-5/40.
malingerer?

2.2 CSF RHINORHOEA

SCENARIO (no You are asked to see a 45year-old overweight


clinical woman with persistent clear unilateral nasal
photograph) discharge.

Introductory Answer
question

What is your • Cerebrospinal Fluid (CSF) rhinorrhoea


differential
• Vasomotor rhinitis
diagnosis?
• Gustatory rhinitis
• Allergic rhinitis

Competency Answer
question(s)

Assuming you • History –focused history for head


suspect CSF trauma, nasal surgery and symptoms of
rhinorrhoea, how meningism.
would you
• Examination – observe leak with head in
manage the case?
dependent position or by placement of
pledgets to collect sample

- 96 -
• Investigations – Tau protein analysis of
>0.5ml of fluid to confirm diagnosis; if
positive and atraumatic - localise source
of leak by CT of skull-base to include
temporal bone. (Tegmen or petrous apex
leak with fluid filling middle ear and
tracking down the Eustachian tube can
present with rhinorrhoea). Also T2
weighted MRI (where CSF signal is high).
Pre-operative intra-thecal fluorosceine
may be used to further localise.
• Treatment – advise to seek medical
attention if develop signs of meningeal
irritation. If following head injury – head
up bed rest, laxatives for 1/52. Surgery
and lumbar drain if not settling. If
atraumatic then surgical repair following
localisation by endoscopic or open
technique with neurosurgeon.

Advanced Answer
question(s)

What is Idiopathic • A syndrome of raised intracranial


Intracranial pressure (ICP) in the absence of
Hypertension and hydrocephalus most often seen in the
what is its obese. Chronic raised ICP is suspected to
relationship with lead to skull base remodelling which
CSF rhinorrhoea predisposes to CSF leak. May also lead to
optic nerve atrophy.
• Diagnosis is based on history, slit
ventricles on CT and raised ICP on LP
• May require temporary lumbar drain
post-op to reduce risk of leak repair

- 97 -
failure.

2.3 FUNGAL RHINOSINUSITIS

SCENARIO GP referral:
(and IMAGING) Please could you kindly see this 42 year old
lady with a history of recurrent episodes of
sinusitis, occurring 4 times each winter,
requiring antibiotics on each occasion and
which is now causing her significant
symptoms. A CT scan was organised and here
is a section.

Introductory Answer
question

Describe the • Coronal CT scan of the nose and


imaging. paranasal sinuses, showing unilateral
opacification of the right maxillary,
ethmoid sinuses. Some irregularities and
heterogeneity noted within the

- 98 -
substance of the maxillary sinus. There is
a large expansive mass within the right
side of the nose leading to extensive
bony remodelling. There appears to be
an extension of the lesion into the right
orbit with a possible mucocele.

Competency Answer
question(s)

What is the Differential:


differential
• Acute on chronic rhinosinusitis
diagnosis?
• Recurrent acute rhinosinusitis
• Fungal rhinosinusitis
• Antrochoanal polyp/Nasal polyposis
• Inverted papilloma
• Dental abscess
• Neoplastic lesion (although in this scan
the opacification extends to involve the
anterior and posterior ethmoid sinuses as
well)

How do you Diagnosis based on Bent & Kuhn diagnostic.


diagnose fungal criteria (5 major)
sinusitis? 1. Atopy (on history or serology to fungal
antigen or allergy testing)
2. Nasal polyposis
3. Characteristic CT scan findings
4. Positive fungal smear
5. Allergic mucin

- 99 -
Do you know of NB. In addition there are 6 minor criteria
any classification (asthma, unilateral predominance, bone
of fungal erosion on radiography, fungal culture,
sinusitis? charcot leiden crystals, eosinophilia). In
practice, you may also add MRI scan findings
to this list owing to the well described
ferromagnetic properties of fungi. Some of
these criteria are only diagnosed
postoperatively however, hence their
limitations for clinical diagnosis.
Classification
• Invasive (acute, such as mucormycosis vs
chronic)
• Non-invasive (fungal ball, Allegic fungal
rhinosinusitis)
OR
• Fungal Ball – non-invasive, usually
Aspergillus, typically unilateral maxillary
sinus, may have unilateral
proptosis/facial hypesthesia, Histo – Y-
shaped hyphae, endoscopic removal
• Allergic – fungal infection (Aspergillus,
molds) leading to antigen for allergic
response, rhinorrhoea/nasal congestion,
RAST for fungus/molds, no invasion,
endoscopic debridement, topical steroids
+/- antifungals
• Acute Invasive (Mucormycosis) – rapidly
invades bone/soft tissue
(necrosis)/vessels (thombus/infacts),
Aspergillus/Mucor/Rhizopus,
immunocompromised (DM, chemo, HIV,
bone marrow transplant), 50% mortality

- 100 -
with CNS/cavernous sinus involvement,
Histo – T-shaped hyphae, Surgical
debridement and Amphotericin B
Chronic Invasive – Rare, indolent invasion of
soft tissues (but otherwise similar to acute),
surgical debridement and long term
Amphotericin B and Itraconazole for 1 year

Advanced Answer
question(s)

What is the • Fungi have been shown to be almost


evidence that ubiquitous in nasal samples – up to 97%
fungi are in fact (Ponikau, Mayo Clinic). However the type
causal to the of sample (eg. Surface smear versus a
symptoms of washing) or whether PCR applied causes
chronic wide variations in yield. Also allergy
rhinosinusitis? identified to fungal antigen not always
the type of fungus identified in the nasal
sample, evidence collectively suggesting
therefore that the fungus may be a
commensal rather than a pathogen.
Do you treat with
antifungals and Antifungals: Double-blind placebo and
what is the randomised controlled trials exist for both
evidence that oral and topical antifungal agents compared
they are against placebo controls. Some evidence
effective? exists that they are effective even in CRS
patients.

- 101 -
2.4 HEREDITARY HAEMORRHAGIC TELANGECTASIA (HHT)

SCENARIO
(clinical
photographs of
tongue and face
of the same
patient)

Introductory Answer
question

Look at the • Hereditary haemorrhagic telangiectasia


images and tell (HHT) (aka Osler-Weber-Rendu syndrome)
me is the Differentials would include:

- 102 -
diagnosis. • Capillary malformation-AVM syndrome
• CREST syndrome
• Hereditary benign telangiectasia
• Bleeding disorders (ie. Von Willebrand
disease)
Diagnosis is based on 4 criteria:
• Epistaxis (spontaneous and recurrent)
• Telangiectasias (lips, oral cavity, fingers,
nasal septum)
• Internal Lesions (GI telangiectasia,
pulmonary/hepatic/brain AVMs)
• Family History (first degree relative with
HHT)
>2 – Definite
>1 – Possible
0-1 - Unlikely
Competency Answer
question(s)

- 103 -
What is HHT? • HHT is a genetic disorder of the blood
vessel wall, characterized by abnormal
blood vessel unable to contract.
• Autosomal dominant (5 genetic types)
Tell me about the
genetics of HHT. • HHT1 (Chromosome 9q33-34)
• HHT2 (Chromosome 12q11-14)
• HHT3 (Chromosome 5q31 (unidentified
gene))
• HHT4 (Chromosome 7p14 (unidentified
gene))
• HHT and juvenile polyposis – MADH4
How would you (Chromosome 18q21)
manage this • HHT1 – pulmonary and cerebral AVMs
patient? higher than HHT2 and oral/nasal mucosal
telangectasias present earlier.
• HHT2 – Hepatic AVMs higher than in HHT1
• Take great care when examining such
patients in clinic as even rigid
nasendoscopy can trigger a significant
epistaxis. Avoid unnecessary
instrumentation where possible.
Figure 2.4a.Nasal
mucosa of a HHT • Epistaxis is most common presentation,
patient followed by bleeds anywhere in the GI
tract, AVMs (pulmonary, liver,
intracranial). Telangiectasia on finger tips.
History:
• spontaneous recurrent epistaxis. FH.
Frequency of blood transfusions to assess
severity.

- 104 -
Examination:
What are the • Oral mucosal telangectasia
treatment options
• Nasal mucosal telangectasia
for a HHT patient
presenting with • Conjunctival or retinal telangectasia
recurrent chronic
troublesome • Focal neurology in brain AVM
epistaxis. • Tachypnoea/cyanosis/clubbing/pulmonary
bruit in pulmonary AVM
• Jaundice/hepatosplenomegaly/right upper
quadrant bruit or thrill
• Signs of anaemia
Figure 2.4b. Clots • nasal, oral and digital examination.
in the nose of a
Investigations:
HHT patient
• FBC, Clotting
• ABGs – low pO2 in right-to-left shunt
• Imaging – CXR then CT or Transthoracic
contrast echocardiogram (pulmonary
AVM), MRI or CT (Brain AVM), MRI (Spinal
AVM), Doppler ultrasound
• Diagnose by FH, muco-cutaneous
telangiectasia and screen for visceral AVM
so genetic testing will identify causative
gene in the proband in 80% of cases.
Family risk may be assessed by linkage
analysis.
Acute Treatment
• Avoid traumatic packing in acute episodes.
Kaltostat soaked in adrenaline or
dissolvable nasopore is preferred to
abrasive merocel. Transanexnic acid is

- 105 -
helpful in prevent fibrinolysis.
Chronic Treatment
• Medical Treatment: Coagulation therapy
is often the quickest way to stop epistaxis,
but there is some controversy as to the
best way to do this. Many experts believe
that laser coagulation therapy (KTP laser)
is preferable to electric and chemical
cautery because it causes less damage to
the inside of the nose. However, some
experts have reported good success with
bipolar electric cautery. Regardless of the
method used, it is important to have
coagulation therapy by someone who has
expertise in treatment of HHT patients.
Most patients who undergo coagulation
therapy see significant improvement for a
period of time, but it usually needs to be
repeated periodically.
• Sprays and ointments containing
oestrogen, bevacizumab (Avastin), and
tranexamic acid have been used in small
numbers of patients and are currently
being studied in clinical trials.
• Several small research studies have
suggested that various oral therapies can
help some patients for whom the local
therapies (i.e. home moisturizing care and
laser therapy) have not been successful.
Oral contraceptive pills have been used
the most, and while they do seem to help
some patients, they have significant side
effects. Drugs that affect either the
formation of clots or vasculature are being
investigated (i.e., estrogens, tamoxifen, n-

- 106 -
acetylcysteine, bevzcizumab (Avastin), and
others) to examine the effectiveness and
safety of these drugs.
• Surgical Treatment: Septodermoplasty,
Youngs Procedure and KTP laser are
surgical treatment options, but are usually
only considered when epistaxis is severe,
significantly affecting quality of life and
coagulation therapy has repeatedly failed
to help. Septodermoplasty replaces the
thin lining of the nose with a split skin
graft. It can significantly reduce or stop
haemorrhages, often for two or more
years. Daily care of the nose is required
after septodermoplasty to keep the nose
moist and clean. In Youngs procedure, the
nostrils are surgically closed with local skin
flaps. This therapy is quite effective but
the downside is that taste and smell are
affected.
• Embolization: This procedure blocks an
artery which, in most cases, stops severe
epistaxes that have been unresponsive to
other treatments. This procedure is
usually only effective for 6-8 weeks as
collateral vessels enlarge and lead to
recurrence. This therapy for the nose
should be used only on an emergency
basis until more durable therapies can be
started.

- 107 -
Advanced Answer
question(s)

What does LASER • Light Amplification by the Stimulation


mean? Emission of Radiation

What are the • KTP laser: wavelength of 532nm puts it in


properties of the visible spectrum (Bright green laser light);
wavelength short enough so can go down
KTP laser that
a flexible tube; 532nm wavelength means
make it suitable that it is preferentially absorbed by red
for HHT blood cells and vessels. Acts by
treatment? coagulating vessels. Tends not to damage
normal mucosa/skin.
What is the key
surgical step in • Leave the perichondrial layer intact
septodermoplasty because it is the source of blood supply to
the skin graft.
• Because of the protective squamous
epithelial layer
• Visceral AVM may present with GI/liver
bleed, CNS/spinal CVA. Pulmonary AVM
There is usually may lead to pulmonary haemorrhage or
no bleeding from cerebral abscess.
telangiectasias in
• Uncontrolled pulmonary AVM carry a
the skin such as
mortality risk in pregnancy and should be
that seen on the referred for consideration of
nose. Why is that? embolisation.
What are the • CNS AVM may be embolised but the role
systemic of CNS screening in the absence of
complications of symptoms is contentious and best made
in an MDT.
this condition and
to manage?
how might they

- 108 -
2.5 ANTROCHOANAL POLYP

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Tell me about this


• Saggital MRI T2 weighted showing a mix
scan intensity lesion in the nasal cavity
extending towards the choana
• This is most likely to represent an
antrochoanal polyp

- 110 -
Competency Answer
question(s)

What are the Clinical features:


clinical features?
• Unilateral, usually adolescent, young
What is the adults
treatment? • Unilateral nasal obstruction
• Unilateral nasal discharge
• Headache (mostly unilateral)
• Epistaxis
• Sleep apnoea
• Blocked nose
• Difficulty in swallowing
• OME
• Endoscopic polypectomy with removal of
the uncinate process
• Recurrence may require a Caldwell Luc
approach

Advanced Answer
question(s)

What is the Theories:


pathogenesis of • May be due to faulty development of the
an antrochoanal maxillary sinus ostium (growth due to
polyp? impediment to the venous return from
the polyp)
• Pressure drop next to a constriction
causes a suction effect pulling the sinus

- 111 -
mucosa into the nose
• Mucopolysaccharide changes lead to
polyp formation

2.6 SARCOIDOSIS

SCENARIO A 50 year old lady is under the respiratory


team with worsening shortness of breath.
(no clinical
She is referred to you by them because they
photograph)
have diagnosed a ‘Bell’s palsy’. She is also
complaining of a painful red eye and cheek
swelling.

Introductory Answer
question

Given the • Sarcoidosis. The patient is demonstrating


collection of the signs of Uveoparotid fever
symptoms, what (Heerfordt’s disease)
is the most likely
diagnosis?

- 112 -
Competency Answer
question(s)

What is • Systemic granulomatous disease.


sarcoidosis?
• Type IV hypersensitivity reaction, but
What are the antigen/ aetiology unknown.
other presenting
symptoms and • Causes accumulation of mononuclear
complications of cells, leading to non-caseating
sarcoidosis? granuloma formation followed by organ
fibrosis (mainly affecting lungs), but is a
multisystem disease.
Symptoms/Complications
• Can often be asymptomatic, being an
incidental finding on a CXR (hilar
lymphadenopathy)
• Cough/SOB
• Weight loss
• Fever
• Uveoparotid fever (uveitis, facial palsy,
parotid swelling, fever, SNHL)
Sarcoidosis of the • Cervical lymphadenopathy
skin
• Nasal crusting/mass/perforation
• Supraglottic mass
• Erythema nodosum
• Subcutaneous nodules (Darier-Roussey
nodules)
• Liver dysfunction

- 113 -
• Renal dysfunction
• Splenomaegaly
• Arrhythmias
• Lytic or sclerotic bone lesions
• Neuropathy

Advanced Answer
question(s)

How would you • CXR – hilar lymphadenopathy


confirm the
• Raised serum ACE
diagnosis of
sarcoidosis? • Hypercalcaemia
What is the • Protein electrophoresis
histopathology (hypergammaglobulinaemia)
involved in this
condition? • Lung (or affected organ) biopsy

What are the • Accumulation of T-cells


interventions for • Accumulation of mononuclear cells
the ENT surgeon?
• Phagocytosis
• Non-caseating granuloma formation
• Fibrosis
• Excision biopsy of cervical lymph node
• Nasal septal biopsy
• Managing sequelae of nasal septal
perforation
• Managing facial palsy/parotid
involvement
• Diagnosing and managing vocal cord

- 114 -
palsy
• Diagnostic rigid laryngoscopy +/- biopsy
• Surgical excision of obstructing
supraglottic lesions

2.7 SINONASAL TUMOUR

A 55 year old man has been referred to you


SCENARIO
with a 6 months history of progressive left-
FOLLOWED BY A sided nasal obstruction. More recently, he
CLINICAL has been suffering with intermittent nasal
PHOTOGRAPH discharge, scabbing and mild epistaxis.

Introductory Answer
question

What is your • Benign: Antrochoanal polyp, inverting


differential papilloma, meningoencephaloceole
diagnosis?
• Malignant: Adenocarcinoma, squamous
How would you cell carcinoma, esthesioblastoma
assess this
patient? • History: Onset and severity of sinonasal
symptoms, occupational history (hard
word, toxic fumes)
• Examination: In addition heck for
cutaneous lesions, palpate for
lymphadenopathy

- 115 -
• Nasendoscopy

Competency Answer
question(s)

You were unable • Radiological assessment e.g. CT and/or


to pass the MRI of the paranasal sinuses, orbit, brain,
nasendoscope on neck.
the ipsilateral side
• It is unwise to biopsy the lesion when
which is
intracranial extension is unknown (risk of
completely
CSF leak if the lesion was a
occuded by a
meningoencephaloceole). Furthermore,
granular, fleshy,
the lesion may be highly vascular risking
firm mass. On the
torrential epistaxis in the outpatients
contralateral
department.
nasal cavity, the
septum was had a • Patients needs to have a biopsy of the
convex deviation, lesion under GA for histological diagnosis
although the and then discussion at the MDT.
mucosa appeared
healthy. • Axial MRI scan demonstrating a left sided
lesion occluding the entire nasal cavity
What would you with deviation of the septum to the right.
do next?
• Coronal MRI scan more posteriorly
Would you take a showing that the mass does not appear
biopsy of the to involve the skull base, posterior
lump in clinic? ethmoids and orbit.
How would you
proceed with the
management of
this patient?
Here are the MR
images. Describe
what you see.

- 116 -
Advanced Answer
question(s)

What do you • Major surgical excision, followed by post-


think will be the operative radiotherapy.
decision at the • Surgical approaches include external
MDT meeting? approach (e.g. lateral rhinotomy,
midfacial degloving) or endoscopic
What are the
resection (if this is the only option
surgical acceptable to the patient, he may need
approaches? to be referred to a specialist endoscopic
rhinologist).

2.8 OLFACTION

SCENARIO A 37 year old gentleman presents to your


specialist rhinology clinic saying he has had a
(no clinical
reduction in his sense of smell for the last 12
photograph)
months.

- 117 -
Introductory Answer
question

What medical • Hyposmia (reduced) Anosmia (complete)


term would you
• Rhinosinusitis with or without polyps
use to assign to a
reduced sense of • Idiopathic
smell, and what
are some of the • Pregnancy
likely causes to • Trauma
disrupt the sense
of smell? • Neoplasia – intranasal or intracranial
• Congential abnormality

Competency Answer
question(s)

What other • Anosmia – no sense. Seen in trauma or


medical terms are congenital abnormalities
you aware of to
• Hyposmia – reduced sense. Seen in
describe
smokers, increasing age, disease
disruption to
processes
one’s sense of
smell? • Hyperosmia – increased sense. Seen in
hunger, pregnancy or endocrine
disorders (Addison’s disease)
• Phantosmia – olfactory hallucinations
(intracerebral tumours)
• Dysosmia – disordered sense (during
neural degeneration/recovery)
• Parosmia – change in quality (dementia)
• Cacosmia – foul smell Seen in acute or
chronic rhinosinisitis, infections

- 118 -
How would you Smell Tests
investigate
• UPSIT (University of Pennsylvania Smell
hyposmia?
Identification Test)
Uses microencapsulated odours which are
released by scratching standardised odour-
impregnated test booklets (some smells
‘Americanised’)
• Sniffin’ Sticks
Pen-like odour dispensing devices which are
used to assess olfactory threshold,
discrimination and identification.
• Combined Olfactory Test
The test consists of an odour recognition test
of nine odours (i.e. coffee, engine oil, etc),
where an odour in a bottle is chosen from a
list of four possible odours in a forced choice
manner. This is followed by a threshold test
using a series of three-fold dilutions of 1-
butanol. The mean of the two scores is the
combined olfactory score.
• Smell Diskettes
Reusable diskettes as applicators of 8
different odorants (coffee, vanilla, smoke,
peach, pineapple, rose, coconut, vinegar).
Using a questionnaire with illustrations, the
test was designed as a triple forced multiple
choice test resulting in a score of 0 to 8
• Threshold (Doty) Bottles
• Olfactometers
Cross-sectional Radiology
• CT – exclude paranasal sinus disease,

- 119 -
trauma
• MRI – intracranial pathology
Blood Tests
• Thyroid Function Tests
• ACE/ANCA
• Zinc

Advanced Answer
question(s)

What is the Vomeronasal organ (VNO)


spherical
• The vomeronasal (or Jacobson’s) organ is
structure on the
an extra olfactory sense organ on the
septum of this
nasal septum found in animals, believed
patient?
to be involved in the detection of
pheromones.
• The receptors contained in this area arise
from those found in the olfactory system.
The VNO appears as a pit on either side
of the nasal septum. Unlike the olfactory
bulb, which sends neuronal signals to the
olfactory cortex, the VNO sends neuronal
signals to the accessory olfactory bulb
and ultimately to the hypothalamus.
Although chemical communication does
appear to occur in humans, it does not imply
that the human VNO is functional, and its role
remains controversial.

- 120 -
2.9 SEPTAL PERFORATION and SEPTAL BUTTON

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Describe the • Right and Left endoscopic views of the


photographs septum of the nose showing a septal
button in situ.
• The perforation appears to be quite
large, perhaps over 1 cm, which is
difficult to correct surgically.

- 121 -
Competency Answer
question(s)

Tell me about the • Trauma: nose picking, septoplasty, nasal


causes for septal cautery,
perforations.
• Infection: septal abscess, syphilis, TB
• Chemical irritants: cocaine, decongestant
nasal sprays, chemicals from button
batteries.
• Inflammation: sarcoidosis, Wegener’s
granulomatosis
• Neoplasia: SCC adenocarcinoma,
melanoma
How would you • Idiopathetic
attempt to close a
small (less than • Free graft: simple or composite
1cm) septal autografts, allografts,
perforaton • Rotation or advancement of
mucoperichondrial or mucoperiosteal
flap
• Pedicle flaps: local nasal mucosa, buccal
mucosa, composite septal cartilage

Advanced Answer
question(s)

What do you Secondary nasal speech. Rhinolalia clausa is


understand by hyponasal speech due to nasal
term rhinolalia? obstruction, eg. polyps, foreign body,
adenoid hypertrophy. Rhinolalia aperta is
hypernasal speech mainly due to
velopharyngeal insufficiency. Large

- 122 -
perforation of the septum may lead to
rhinolalia aperta.

2.10 NASAL TRAUMA

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Describe what • Clinical photograph showing gross C-


you see in the shape deviation of the nose to the left
clinical with significant distortions of the brow
photograph? tip aesthetic lines on both sides,
especially the upper and middle third of
The above patient
the nose. The nasal bones and the upper
was referred for a
lateral cartilages are all involved in the
septorhinoplasty
deformity.
procedure after
years of domestic • Premorbid shape of the nose, mechanism
abuse. What is of injury, whether physical abuse has
important in the been dealt with and situation resolved
history? before surgery.

- 123 -
Competency Answer
question(s)

You examine her • This may be due to previous trauma, due


nose and found a to autoimmune disease such as
septal perforation Wegeners, sarcoidosis, infection or
in the absence of secondary to drug abuse.
previous surgery.
• Ask the patient directly, write to GP, GP
What may be the
needs to carry out tests to ensure she is
cause?
drug-free for 1 year before
How would you reconstructive surgery.
ensure that the
patient is not • Urine, saliva, blood tests;
using cocaine?
What test would
the GP request
for cocaine?

Advanced Answer
question(s)

What other test • Cocaine can be tested for in hair for up to


can the GP 90 days.
undertake?

2.11 LIMITED WEGENER’S DISEASE

SCENARIO You are referred a 50-yr-old man with


persistent excessive nasal crusting and
(no clinical

- 124 -
photograph) epistaxis.

Introductory Answer
question

What is your • Infections


differential
• Traumatic perforation
diagnosis?
• Wegener’s granulomatosis (WG)
• Sarcoidosis
• Atrophic rhinitis
• Sino-nasal malignancy

Competency Answer
question(s)

How would you • History –nasal surgery, trauma (including


manage this digital), topical drugs, symptoms of
patient? connective tissue disease (especially
respiratory), and symptoms of local
spread including visual
impairment/diplopia, facial numbness.
• Examination – 3 pass nasendoscopy,
focused cranial nerve examination for
local extension, CXR and urinalysis for
WG and sarcoid.
• Investigations – nasal biopsy for
histology and culture with imaging
guided by clinical signs. Serology is
essential. cANCA, ANA, Rheumatoid
Factor plus serum ACE. CXR and urinalysis

- 125 -
obligatory.
• Treatment – Guided by underlying
diagnosis. WG/sarcoidosis managed in
conjunction with rheumatologists etc.
Basic hygiene with decrusting and nasal
douching +/- antibiotics guided by swabs.
Septal button as first line management of
symptomatic perforation.

Advanced Answer
question(s)

The cANCA • Wegener’s is a systemic autoimmune


returns positive. vasculitic condition affecting the upper
What is the role and lower airways and causing
of antibiotic necrotising glomerulonephritis.
therapy in
• cANCA immunofluorescent pattern is due
managing this
to antibody reactivity against the
patient?
leukocyte proteinase-3 protein (PR3).
This is also known as Myeloblastin.
• Most patients with WG are colonised
with Staph. aureus. Evidence suggests
that this may promote disease flares. Co-
trimoxazole may suppress these.
• Sino-nasal ‘mucosal failure’ manifesting
as atrophic rhinitis is associated with
Klebsiella ozaenae which may be
controlled by quinolone antibiotics.

- 126 -
2.12 SYSTEMIC WEGENER’S GRANULOMATOSIS
(GRANULOMATOSIS POLYANGIITIS, GPA)

SCENARIO You are asked to see a patient on the medical


ward with renal failure who is having
(no clinical
intermittent epistaxis. On examination there
photograph)
is nasal crusting with a friable mucosa.

Introductory Answer
question

What is your • Infections


differential
• Traumatic perforation
diagnosis?
• Wegener’s granulomatosis (WG)
• Sarcoidosis
• Atrophic rhinitis
• Sino-nasal malignancy

Competency Answer
question(s)

Background
Tell me about
• Unknown aetiology – possibly
Wegner’s
autoimmune
Granulomatosis?
• Idiopathic necrotising granulomatous
vasculitis
• Multinucleated giant cells which may
affect upper and lower respiratory tracts

- 127 -
and may also cause necrotising
glomerulonephritis
• Affects upper and lower respiratory
tracts
• Causes glomerulonephritis
• Limited – no renal involvement
Systemic – renal involvement
Symptoms
• Haemoptysis, cough, dyspnoea
• Haematuria
• Fever, night sweats
• Arthralgia/myalgia
• Cutaneous lesions
Signs
• Epistaxis, nasal obstruction,
rhinosinusitis, septal
ulceration/perforation
• Subglottic stenosis
• Uveitis
• Hearing loss (CHL/SNHL)
Investigations
• cANCA and PR3 (86% specific)
• Biopsy (lung – highest yield, renal – if
glomerulonephritis, nasal – poor yield)
Prognosis very poor if left untreated

- 128 -
Advanced Answer
question(s)

What are the If diagnosis equivocal despite cANCA/PR3 and


diagnostic options the nasal septal mucosa appearance shows
here, when would hypererythema or irregularity, then a nasal
you perform a septal biopsy could be performed. This often
nasal septal gives a poor diagnostic yield as it is often
biopsy, and what obscured by inflammatory cells. Pulmonary
are the treatment or renal biopsy are other possibilities but
options for this carry higher morbidity.
condition?
Treatment can be in the form of
administering:
• Steroids
• Cyclophosphamide
• Azathioprine
• Methotrexate

2.13 EMPTY NOSE SYNDROME

A 47-year old man has been referred back to


SCENARIO
your clinic 2 years after having had sinonasal
surgery for nasal obstruction. He complains
bitterly of nasal congestion, scabbing and
dryness.

- 129 -
Introductory Answer
question

Describe what
• Coronal and sagital sections showing loss
you see? of mucosal surfaces necessary to
humidify inhaled air
• This may result in empty nose syndrome
and atrophic rhinitis

Competency Answer
question(s)

What is empty • It is a recognised complication of


nose syndrome? sinonasal surgery, especially following
radical turbinectomy. The absence of

- 130 -
How would you normal nasal structures is universal in
manage this these patients, and the symptoms of
patient? atrophic rhinitis coupled with a
cavernous nasal airway lacking
identifiable turbinate tissue has been
termed the empty nose syndrome.
• Managing this condition is challenging
and the evidence base for most
treatment modalities remains low.
• Recommended conservative
management does not differ significantly
from atrophic rhinitis and includes a
combination nasal lavage, lubricant drops
and topical corticosteroids.
• Surgical intervention for ENS aims to
increase nasal airway resistance by
narrowing the nasal valve region or to
reconstruct the resected turbinate.

Advanced Answer
question(s)

How would you • Conservative: topical steroids.


manage
• Surgery: Numerous techniques, mostly
hypertrophy of technology driven, such as laser,
the inferior Coblation, turbinoplasty have been
turbinate? described. However, few studies have
evaluated clinical outcomes using
objective measures of nasal airflow such
as rhinomanometry. The randomised
clinical study undertaken by Passàli D et
al. (2003) reported that submucosal
resection and outfracture of the inferior
turbinate resulted in optimal long-term

- 131 -
normalization of nasal patency and in
restoration of mucociliary clearance at up
to 6 years follow-up.

2.14 FRONTAL MUCOCOELE

This 58-year old woman was referred from


SCENARIO
the ophthalmologist with gradually
(followed by a progressing diplopia, proptosis and a swelling
clinical over his left eye.
photograph)

Introductory Answer
question

How would you


• Full history focusing on her current
assess this sinonasal symptoms and visual problems,
patient? previous history of craniofacial trauma,
sinonasal surgery, sinus pathology (e.g.
nasal polyposis, Samster's, systemic
vasculitis), smoking, occupation (e.g. hard
wood worker, exposure to toxic fumes).
• Full ENT examination including
endoscopy of the nasal cavities.

Competency Answer
question(s)

This patient gives • Coronal CT scan showing a smooth,

- 132 -
a 6 months homogenous lesion in the left frontal
history of nasal sinus, extending into the left orbit. There
congestion and is bony erosion over the supraorbital rim.
headache. She The globe appears to be displaced
had endoscopic downwards compared to the opposite
sinus surgery side. There is a rim of thin bone at the
several years ago bottom of the lesion. There is evidence of
for chronic previous sinus surgery as the anterior
rhinosinusitis with ethmoid cells have been excised.
polyps, and had
• This is likely a frontal mucocele,
been on topical
secondary to scarring/ stenosis of the
treatment since.
frontal sinus outflow tract. Pressure
Describe what
effect of the mucocele causes bony
you see in this
erosion and remodelling. There may even
scan.
be reversible palsy of the trochlear nerve
supplying superior oblique muscle,
resulting in diplopia and difficulty walking
down stairs.
• She will require surgery to drain the
mucocele. This can be undertaken as an
What do you external approach (e.g. Lynch-Howarth
think has incision) or endoscopically (e.g. Draf IIb,
happened to this III). Prophylactic antibiotics remains
patient? contentious and should be withheld if the
How would you patient is asymptomatic Consider
manage this referring the patient to a rhinologist. A
patient? MRI scan may be required to determine
the entent of the mucocele and there
should be discussion with the
ophthalmologist to discuss
reconstruction of the defect on the outer
table, if necessary

- 133 -
Advanced Answer
question(s)

What would you


• I would be concerned that the mucocele
do if whilst may become infected i.e mucopyocele.
waiting for
surgery, she • This patient requires admission,
intravenous antibiotics and urgent scans
presents with
(CT and MRI). Depending on the
acute swelling, radiological findings, I may be to confer
headache, pyrexia with my ophthalmologist and
and photophobia? neurosurgical colleagues. Nevertheless,
this patient would require emergent
surgery in the form of external drainage
(via a Lynch-Howarth incision).
• An endoscopic approach to re-establish
intra-nasal drainiage should be
considered, or seek help from a more
experienced colleague after the acute
infection has been drained.

2.15 INVERTED PAPILLOMA

SCENARIO A 45 year old man is referred from his GP.


FOLLOWED BY A In the clinic letter, the GP has diagnosed a
CLINICAL "unilateral nasal polyp" in the left nasal
PHOTOGRAPH cavity and asks for further management.
The patient has been suffering with
unilateral nasal obstruction, cacosmia

- 134 -
and occasional epistaxis.

Introductory question Answer

What is your
• Benign: Simple inflammatory polyps
differential (e.g large antrochoanal polyp),
diagnosis? inverted papilloma, angiofibroma,
granulomatous lesion, tuberculous
• Malignant (primary or secondary):
squamous cell carcinoma,
adenocarcinoma, met from kidney or
elsewhere in the body)
• The history should concentrate on
How would you
sinonasal symptoms: rhinorhoea,
assess this obstruction, pain, pressure, epistaxis,
patient? reduction in sense of smell, excessive
itching and sneezing. Visual
disturbance and previous sinus
surgery as well as significant past
medical history are also relevant.
• Full ENT Examination including
endoscopy of the nasal and
nasopharyngeal regions.

Competency Answer
question(s)

Describe what
• This is an endoscopic view of the right
you see. nasal cavity; the septum is on the
right and the middle turbinate is on

- 135 -
the left.
• There is a fleshy/polypoidal mass
originating from the lateral wall,
blocking the middle meatus. It does
How would you
not appear to be vascular and the
investigate this surface appears to be lined with
patient? squamous epithelium.

Why MRI scan? • At this stage, my working diagnosis is


either inverted papilloma or a
Biopsies have malignant sinonasal tumour and
confirmed therefore I would request a CT and
MRI of the nose and paranasal sinus
inverted
and then a biopsy of the lesion under
papilloma and a general anaesthesia in theatre, if I
imaging shows am convinced it is not a juvenile
no invasion of angiofibroma.
the orbit or skull • It is good for defining soft tissue
base. Where is lesion and inverted papilloma has a
the most characteristic cerebriform (brain-like)
common site of appearance.
origin for • Lateral nasal wall, followed by
inverted maxillary and ethmoid sinuses. True
papilloma? frontal sinus inverted papilloma is
uncommon.
How would you
• Endoscopic surgical resection is
manage this appropriate for most lesions (e.g
patient? medial maxillectomy, full-house FESS,
Draf III), external approach (e.g
lateral rhinotomy, craniofacial
resection for frontal sinus disease or
extensive skull base involvement). A

- 136 -
Caldwell - Luc and/or septotomy
could also be undertaken to improve
exposure to facilitate resection. The
site of origin may be identified on CT
scans (an area suggestive of osteitis)
and should be drilled to prevent
recurrence.
• Follow-up: You would recommend
long-term review for recurrence. The
European position paper suggest 3
years follow-up.

Advanced Answer
question(s)

What
• Krause system
classification
• Yes, the literature states that the
system do you
incidence of synchronous and
know for
metachronous carcinoma to be
inverted approximately 5-10 percent.
papilloma? However, it should be remembered
that this data is based in a review of
Is there tertiary departments and the
malignant incidence could be biased.
potential with • The evidence for using radiotherapy
inverted in the primary treatment of inverted
papilloma? papilloma. The issue of radiotherapy
in extensive disease following surgical
What is the role resection remains contentious. This
of radiotherapy decision is predicated on the severity
in treating of metaplasia and dysplasia in the
inverted histological samples, and should be

- 137 -
papilloma? duly discussed in a multidisciplinary
setting.

2.16 ATROPHIC RHINITIS

SCENARIO A 45 year old man from the middle east


presents with nasal obstruction epistaxis,
anosmia, foul smelling green, yellow crusts
and occasional choking episodes. He
previously had FESS, reduction of concha
bullosa and resection of his inferior
turbinates.

Introductory Answer
question

What is going
• Atrophic rhinitis would be my main
through your concern
mind with the
above history? • I would also be thinking of
granulomatous diseases such as
Why is he having Wegeners, sarcoidosis, syphilis, leprosy
and TB.
choking
episodes? • Crust from the nose obstructing the
larynx

- 138 -
Competency Answer
question(s)

What is the cause • Primary cases may arise denovo and is


of atrophic common in India and Middle East. Role of
rhinitis? coccobacillus and Klebsiella unclear but
these organisms are frequently present
Tell me about
in atrophic rhinitis
treatment of
atrophic rhinitis. • Secondary cases are seen in developed
countries and may be iatrogenic – due to
excessive destruction of turbinates by
surgery or after irradiation therapy.
• Nasal douche, antibiotics, vitamin A,
glucose in glycerine
• Submucous injections of paraffin
• teflon strips, and autogenous cartilages
grafting
• Submucosal injection of 50% Teflon in
glycerin paste
• Repeated stellate ganglion blocks (causes
imbalance in favour of parasympathetic
activity in nose)
• Young's operation (reopen 6-9 months)
(Nb many believe steroids to be
contraindicated)

Advanced Answer
question(s)

What are the CT • Thickening of sinuses


findings in

- 139 -
atrophic rhinitis? • Resorption of bulla and uncinate
• poorly defined OMC
• Hypoplastic maxilla
• Erosion of lat nasal wall and atrophy of
turbinates.

2.17 CAVERNOUS SINUS THROMBOSIS

SCENARIO A 23 year old university student presents with


FOLLOWED BY A progressively increasing sharp headache in
CLINICAL the regions of the eyes and upper face over
the last 48 hours. She suffers with severe
PHOTOGRAPH
acne and recently developed a furuncle on
the cheek. She denies any symptoms of
rhinosinusitis. Rhinological and dental
examinations were completely normal but
examination of the eyes reveals evidence of
bilateral chemosis.

Introductory Answer
question

What do you
• The history is typical of someone with
think is the cause potential cavernous sinus thrombosis.
for her
symptoms?

- 140 -
Competency Answer
question(s)

Which organism • 70% Staph aureus is responsible for up to


is most commonly 70% of cavernous sinus thrombosis,
associated with other organisms include s.pneumoniae,
cavernous sinus anaerobes and fungi.
thrombosis?
• sinuses (sphenoid, ethmoid, or frontal)
Apart from the
face, which other • dental abscess, nares, tonsils, soft palate,
regions be the middle ear, or orbit (orbital cellulitis)
source of • obstruction of the ophthalmic veins
infections?
• sixth nerve palsy leading to double vision
What is the
mechanism for • 3rd nerve palsy leading to ptosis,
the chemosis? mydriasis, alterned eye movements

If left untreated • Raised IOP (exopthalmos,


what other ophthalmoplegia, sluggish pupils,
symptoms might decreased acuity)
she develop? • Hypoaesthesia of V1 and V2
• Meningeal and septic signs

Advanced Answer
question(s)

Which • CT with contrast as well as MR (MRV) to


radiological check for flow voids
investigation • High dose broad spectrum antibiotics
would you such as ceftrixone (3-4/52), steroid and
request? heparin.

What is the

- 141 -
treatment?

2.18 POTT’S PUFFY TUMOUR

GP Referral:
SCENARIO
FOLLOWED BY A Dear Dr, Please could you see this gentleman
CLINICAL with a forehead lump which appears to be
PHOTOGRAPH increasing in size over the last 48 hours. The
lump is associated with a swinging pyrexia and
nasal discharge. He has a history of
rhinosinusitis.

Introductory Answer
question

Based on the
• I would be considering an infective or
history given and neoplastic process.
the appearance of

- 142 -
the lump, what is • For example, frontal sinusitis, Pott’s
your differential puffy tumour, benign sinus tumour such
diagnosis? as a polyp, primary malignancy or
metastatic disease.

Competency Answer
question(s)

How would you • After taking a full history and performing


reach a a full ENT examination I would request:
diagnosis? blood profile such as FBC, CRP, ESR and
blood culture.
• I would take a nasal swab for MC+S
• I would also request a contrast CT head
and para-nasal sinuses and MRI with
contrast brain.

What is Pott’s • Pott’s Puffy tumour is a subperiosteal


collection of pus and an osteomyelitic
Puffy tumour?
anterior table of the frontal sinus.
• This condition is associated with
intracranial complications in 30% of cases
and demands an MR brain with contrast
enhancement and repeat assessment as
symptoms dictate.
• Intracranial complication is indicated by
How is the focal neurological symptoms/reduced
condition GCS/raised intracranial pressure.
treated?
• Prolonged course of IV antibiotics
typically for 6 weeks followed by oral
step down.
• Surgical drainage of pus [via external
incision and/or functional endoscopic

- 143 -
sinus surgery (FESS)].
• Direct incision may lead to fistulation and
frontal sinus surgery may be hazardous.
• Frontal sinus trephine may represent an
effective compromise.
• Bony sequestra occasionally require open
debridement.
• When there are co-existing intracranial
complications (30% of cases) treatment is
best decided by a multi-professional
team involving surgeons, radiologist and
microbiologist.

Advanced Answer
question(s)

What is the • Polymicrobial organisms: strep,


typical bacteroides and staphylococci
microbiological • If intracranial complications are present
organisms then the more likely organisms are
associated with fusobacter and bacteroides
Pott’s Puffy
tumour?

- 144 -
2.19 ALLERGIC RHINITIS

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Describe what
• Clinical photograph of the right nostril
you see in the showing enlarged inferior turbinate and

- 145 -
photograph watery rhinorrhoea
• This may represent rhinitis (allergic or
infective) or CSF rhinorrhoea

Competency Answer
question(s)

How would you • I would take a full history and perform a


attempt to full ENT examination
differentiate?
• In the history I would like to know about
the nature of symptoms colour of d/c,
ocular or respiratory symptoms); increase
on bending forward, timing
(seasons/perennial,
outdoor/school/home,
exacerbation/alleviation); Duration, FH,
Related history (asthma, eczema); Social
history (pillows, pets etc.)
• On examination I would look for Allergic
shiners, Morgan Dennie lines, Allergic
salute; Conjuctival erythema; OME, Pale
blue enlarged inferior turbinates,
Rhinorrhoea, dried blood); high arched
palate & discoloured palate = mouth
breather; cobblestoning of posterior
pharyngeal wall = PND)
• Skin Prick Test or RAST (or beta 2
transferin if I am concerned about CSF
rhinirrhoea)

- 146 -
Advanced Answer
question(s)

What is the • Type 1 hypersensitivity reaction: IgE


immunopathology binds allergen – cross linking causes mast
of allergic rhinitis? cell degranulation (Fc bound to mast cell)
Release of mediators – histamine,
prostaglandin D2, tryptase, heparin, PAF,
leukotrienes.
What is the
treatment Stepwise treatment
recommended by All patients allergen avoidance based on SPT
ARIA? or RAST
• Mild intermittent Oral H1 blocker,
intranasal H1 blocker, consider
decongestant
• Mod/severe intermittent & mild
persistent As above + Intranasal
Corticosteroid (CS), if no improvement
4/52 step up
• Mod/severe persistent Intranasal CS. If no
improvement 4/52 (increase IN CS dose,
add H1 blocker for itch/sneeze, oral CS,
ipratropium for rhinorrhoea)
Pharmacology:
Neoclarityn has additional effect on nasal
blockage
Metanalaysis: Corticosteroid is better than H1
antagonists
Beclomethasone has effects on growth
Mometasone/fluticasone low bioavailability

- 147 -
Leucotrienes antagonists + H1 blocker not as
good as intranasal corticosteroids.

2.20 RHINITIS MEDICAMENTOSA

CLINICAL
PHOTOGRAPH

Introductory Answer
question

What is the
• The patient will develop rhinitis
problem medicamentosa
associated with
long term usage
of the nasal drops
shown in the

- 148 -
photo?

Competency Answer
question(s)

Tell me more • It is essentially rebound nasal congestion


about that secondary to vasodilatation after topical
condition. sympatheticomimetic treatment
This may be due to a number of factors:
• longer beta effect than alpha effect
• prolonged vasoconstriction causes tissue
hypoxia and thus reactive hyperaemia
• alpha 2 agonists stimulate negative
feedback loop at presynaptic terminals
• alters vasomotor tone and increase
parasympathetic activity
How is the • Patient education
condition
• Stop topical decongestants
treated?
• Sedating anti-histamine at night to help
sleep
• Nasal douching
• Topical nasal corticosteroid
• Systemic decongestants in first 1-2 weeks
or a short course of oral steroids or im
corticosteroid injection eg Deprofos.

- 149 -
Advanced Answer
question(s)

What is the nasal • This cycle refers to the turbinate and


cycle? other nasal mucosa undergoes regular
periodic side-to-side or alternate
engorgement with blood (note that the
turbinates are made of erectile tissue,
which also explains honey moon rhinitis).
• Cyclical alternate variation of blood flow
in the turbinates is present in
approximately 80% of people.
• Frequency of cycling is 30mins to 5 hours
and is specific for an individual
• Most active in adolescence and under
hypothalamic control
• The nasal cycle is linked to the ultradian
rhythm of alternating cerebral
hemispheric activity.
• The sympathetic system regulates blood
flow to the nasal mucosa by regulating
resistance vessels. Increased resistance
and thus less flow into the nasal mucosa,
leading to decongestion.
• The parasympathetic system regulates
blood volume of the nasal mucosa by
regulating capacitance vessels. Leads to
relaxation of capacitance vessels,
allowing congestion and even edema
formation in tissues.

- 150 -
2.21 DISORDER OF SCARRING

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Describe what
• A keloid scar because the scar tissue
you see in the extends beyond the incision
photographs
• A hypertrohic scar because the scar
tissue is confined to the site of the

- 151 -
incision

Competency Answer
question(s)

What is Keloid? • Keloids are the result of an overgrowth of


dense fibrous tissue
Who tends to get
keloid? • Usually develops after healing of a skin
Tell me about the injury
treatment for • Does not usually regress spontaneously
keloid? • Tends to recur after excision
• Variation in normal healing. At 6-8weeks
catabolic and anabolic processes in
equilibrium. If more anabolic (ie more
collagen deposition) will lead to disorder
of scarring (hypertrophic/keloid scar).
• 16% of Blacks. Highest incidence 10-
20yrs. F>M (due to earlobe piercing)
• Prevention is better than cure so if
possible avoid surgery, unnecessary
piercing etc, apply minimal tension in
sutures during surgery and try not cross
joints (contractures)
In general, large unsightly keloid is managed
by surgical excision and one of the following:
• Compression/occlusive dressings
• Triamcinolone 10-40mg every 6/52
• Silicone gel
• Interferon
• Radiotherapy

- 152 -
Advanced Answer
question(s)

Any other • Verapamil (Blocks synthesis and secretion


treatment of extracellular matrix molecules)
available? • Bleomycin (Injections cause necrosis of
keratinocytes )
• 5FU (inhibits fibroblastic proliferation)
• Retinoic acid derivatives, immune
modulators

- 153 -
2.22 SEPTORHINOPLASTY

CLINICAL
PHOTOGRAPHS

Introductory Answer
question

Talk me through
This patient appear to have a left sided
the assessment of deviated nasal pyramid with a dorsal hump;
the nose for the nose also appear to be long although the
septorhinoplasty nasolabial angle is about 90 degrees. I would
with reference to assess further in terms of:
the patient seen Horizontal Thirds
in the
• Upper – trichion to glabella

- 154 -
photographs. • Middle – glabella to sub-nasale
• Lower – sub-nasale to menton
Vertical Fifths
• Width of nose = width of eyes = width of
lateral canthus to ear
Lines and angles
Tell me about the
lines and angles • Frankfort horizontal line = Superior
point of auditory canal to most inferior
relevant to
point of infra-orbital rim; this is
septorhinoplasty important for clinical photograph before
and after surgery
• Facial line is a vertical line from the
glabella perpendicular through Frankfort
horizontal and ideally should touch the
anterior chin; hence indicating if there is
excessive protrusion or retraction of the
chin.
• Nasofrontal Angle = 115-130 degrees
(angle defined by glabella-nasion line
intersecting nasion-tip line)
• Nasofacial Angle = 30-40 degrees
(angle defined by glabella-pagonion line
intersecting tip-pagonion line)
• Nasomental Angle =120-132 degrees
(angle defined by nasion-tip line
intersecting tip-pagonion line)
• Nasolabial Angle = 90-110 degrees
(95-110 for females)
• Columullar show 2-4mm on profile
view

- 155 -
• Nasal Projection - Goode’s Method: ratio
of (nasal tip to alar line) to (nasion to
nasal tip line); normal is 0.55-0.60

Competency Answer
question(s)

How would • Open approach via inverted V and


correct the marginal incisions
deformities
• Nasal laparotomy
mentioned?
• Isolate the septal cartilage (and perform
septoplasty if necessary)
• Division of upper lateral cartilages (ULCs)
from septum bilaterally
• The cartilage of the hump is incised with
a scalpel; this creates a joint at the
junction of the bone and cartilage
• An osteotome is used on the bony dorsal
hump
• The bone cartilage hump is removed in
one piece.
• Rasp if necessary
• Trim upper lateral cartilage as required
• Medial and lateral osteotomies to close
open roof deformity
• Secure ULC to septum with or without
spreader grafts

- 156 -
Advanced Answer
question(s)

What are the Advantages of endonasal (closed) approach


advantages and • less dissection
disadvantages of
the endonasal • less swelling
(closed) vs open • less internal and external scarring or flap
septorhinoplasty? necrosis
• more immediate and predictable ability
to feel changes made
• shorter operating time
• decreased morbidity
• quicker return to normal appearance
• no post-operative nasal tip numbness
Advantages of open approach
• direct exposure, inspection and
assessment of nasal framework
• precise modification and stabilisation of
the abnormality
• better results if patient for thick nasal
skill
• can deal with difficult nasal tip, eg shield
graft option
• easier to insert and secure spreader
grafts
• can address internal nasal valve
dysfunction

- 157 -
• revision surgery
• good tool for training

2.23 SADDLE NOSE DEFORMITY

CLINICAL
PHOTOGRAPHS

- 158 -
Introductory Answer
question

Describe the
• Saddle nose deformity
abnormality
present in the
photographs

Competency Answer
question(s)

What are the The causes may be surgical or medical


caused for this
• Rhinoplasty or SMR (0.4%)
deformity
• Wegeners – necrotising granulomas and
How would you
vasculitis of upper and lower respiratory
investigate this
tracts.
patient?
• Relapsing polychondritis – recurrent
What operation episodes of AI catrilage inflammation and
would you do to destruction,
correct the
• Substance abuse such a s cocaine
deformity
• Syphilis – intranasal ulcerative lesions
that lead to osteo-chondritis
• Leprosy
• Ectodermal dysplasia
• Photographs, C-ANCA, autoimmune
screen, VDRL, leprosy serology
• Augmentation septorhinoplasty

- 159 -
Advanced Answer
question(s)

What would be • Chronic autoimmune disease


contraindications
• ongoing cocaine usage
for surgical
correction? • poor peri-operative risk
• relative = multiple previous rhinoplasties
and boxers)

2.24 NASAL RECONSTRUCTION AFTER TRAUMA

CLINICAL
PHOTOGRAPH

- 160 -
Introductory Answer
question

This man has


• Grossly deviated nose to the right.
been referred to
you following a • Depressed nasal bone on the left.
drug-related • Bilateral distortion of the brow-tip
assault. Describe aesthetic lines
what you see in • Soft tissue bruising, lacerations etc.
this picture.

Competency Answer
question(s)

How would you


• History – focusing on the trauma event,
manage this
clear rhinorrhoea (CSF, skull base
patient?
fracture), visual problems e.g. diplopia
(blow out fracture), sinonasal symptoms
(septal hematoma, perforation),
• Examination – septum, skin over nasal
dorsum (thick, sebaceous, mobile, scars),
alar cartilages (keystone area), nasal tip.
• Clinical photographs - remember to
obtain consent. Standard views from the
front, profile, oblique, basilar and from
the top.
• Empathetic discussion of issues which
concern the patient (aesthetics,
functional) and realistic expectations of
nasal surgery.
• Given that this was an assault, enquire
about psychological needs and refer if

- 161 -
necessary
• It may be necessary to have any
psychological assessments complete
before proceeding with surgery. There
may be need to apply for commissioners
for funding; so explain that surgery
should not be rushed. Furthermore, the
nasal reconstruction required by this
patient is complex and will require time
to plan or even refer on to a more
experienced facial plastics colleague.

Advanced Answer
question(s)

How would you • Given the complexity of the nasal


surgically manage deformity, an external approach would
this patient? be more appropriate. This would give
opportunity to deal with the septum,
bony pyramid and also the nasal tip.
• Autologous cartilage grafts can be
obtained from the nasal septum, pinna or
even costochondral rib.
• The nasal tip may require a spreader
graft to open up the nasal valve region,
or may require inter/intra-domal sutures
to improve definition of the bulbous tip,
or may require more support with a strut
graft.
• Shield grafts can be used to smooth out
depressions of the nasal dorsum.
• Osteotomies will be required to reset the
bony pyramid.

- 162 -
2.25 ALAR BASAL CELL CARCINOMA

CLINICAL
PHOTOGRAPH

Introductory Answer
question

A 55 year old man • Lateral/oblique view of patient with


has noticed an lesion on left alar side wall. Well
area of ulceration circumscribed, ulcerated centre.
on the side of his
• Comment also on any other relevant
nose. Describe
abnormal features or pathology seen as
the clinical
at this stage you do not consider MOHs
photograph.
first for complete excision. Know what it
is that the examiner wants to ask you
about, hence cover your bases. Focus in
however on the most obvious
abnormality.

- 163 -
Competency Answer
question(s)

What is the • Differential: infective (eg. TB), traumatic,


differential neoplastic (BCC, SCC, amelanotic
diagnosis? melanoma)
How would you • Diagnosis: The discussion is between an
reach a incision vs excision biopsy. Given this is a
diagnosis? complex reconstructive area, incision
Assuming biopsy biopsy may be preferable so that
confirms a BCC definitive therapy can be planned once
with a histology is known. Alar rim lesion / BCC
morpheaform • Treatment partly depends on the extent
component, what and depth of the lesion. It will need to be
would your excised, then the defect reconstructed.
treatment be? This will in turn depend on the depth of
What are your the lesion as a multiple layer
reconstructive reconstruction may be required, with
options in this each layer having several options
area? available.
• Reconstruction
- Discuss reconstructive ladder for outer
(skin) layer – secondary intention (not
good here due to size and location),
primary closure (not possible), grafting
(SSG or FTSG), local flap (discuss options
further – bilobed or nasolabial), distant
flap (not necessary).
- Inner lining: usually septal mucosa is
used, although an inner lining can also be
created by fashioning a flap immediately
adjacent to, and similar in size as, the
excised segment (ideally within the same

- 164 -
subunit) and inverting it such that the
outer skin becomes the inner lining of the
defect. This effectively doubles the size of
the outer (lateral) defect which can then
be reconstructed as above.
- Middle layer: usually cartilage either
from septum or ear.
• Alternatively a composite graft such as
from the pinna can be used for two of
the layers. Also, if the middle and inner
linings have been taken care of, a
forehead flap can be used if the skin
defect is larger than can be
accommodated by a local nasal or facial
flap.

Advanced Answer
question(s)

What about if it • Skin MDT


was an SCC?
• Offer RXT to avoid complex
reconstruction.

- 165 -
2.26 PINNAPLASTY

SCENARIO GP referral: Many thanks for seeing this 8 year


old boy who has prominent ears and whose
(and clinical
parents are seeking advice regarding surgical
photographs)
correction.

Introductory Answer
question

Described what • Clinical photographs of a young man


you see in the showing prominent pinnae with
photographs. underdevelopment of antihelical fold and
overdeveloped conchal bowls. Lobules
are normal.
• Although a lateral view of the ear may
appear normal, it is likely that the
concha-scaphoid angle will be greater

- 166 -
than 90 degrees and this suggests a
protruding ear.

Competency Answer
question(s)

What methods of • At birth – banding can be attempted but


correction do you success is dependent on early
know? intervention.
• Malleable splints, such as Ear Buddies™
can be taped to the pinna to refashion
the antihelical fold. These are usually
applied for 2 weeks and can be repeated
as necessary. With persistence, splintage
can still be effective up to the age of two
years, but the process is much more
difficult as the infant is by then able to
reach up to the ears and potentially
interfere with the splints.
• Later in childhood – Surgery: There are
numerous techniques and modifications
in current practice to correct prominent
ears. Nevertheless, these fall into either
What are the Mustade or Scoring.
complications of Mustarde technique (posteriorly placed
pinnaplasty? sutures to create the antihelical fold)
Anterior cartilage scoring (described by
Chongchet, Stenstrom) technique.
• These procedures can be carried out
What else is
under local anaesthetic, but in young
important to children a general anaesthetic is usually
consider pre- required. Other methods to weaken
operatively? cartilage can be employed (eg. Drilling).

- 167 -
• Complications – bleeding/infection/scar,
telephone ear, over or under-correction,
late failure, hypertrophic/keloid scar,
tattooing of skin, extrusion of suture
material.
• Explore concerns of the child and parents
e.g. bullying.self awareness.

Advanced Answer
question(s)

What do you do • Prominent conchal bowl:


about a
1. Furnas conchomastoid suture – the success
prominent
of this depends on being able to site the
conchal bowl and
mastoid end of the suture as posteriorly
lobular
as possible. Also a deep bite of the
protrusion?
mastoid periostium is important for good
anchorage;
2. Conchal reduction: this is often required
along with a Furnas suture for
hypertrophy of the conchal bowl as
Furnas suturing alone will not always
correct this.
• Lobular protrusion
1. lobulomastoid setback suture (analogous
to the Furnas suture described above);
2. excision of the cauda helicis (the most
inferiorly situated piece of conchal
cartilage just superior to the soft lobule).

- 168 -
2.27 RHOMBOID FLAP

SCENARIO (and Following discussion at the skin MDT meeting,


surgical surgery was recommended for the BCC lesion
photographs) on the right temple of this patient.

Introductory Answer
question

How would you • Generally, excised lesions on the temple


excise the lesion are best reconstructed using a rhomboid
and reconstruct transposition flap.
the defect?
• This involves excising the lesion in a
rhomboid shape with 60 and 120 degree
angles and then using one of the 4
options with the least tension and
cosmetic disruption.

- 169 -
Competency Answer
question(s)

Please draw me • The following drawings need to be


how you would converted to line diagrams
perform the
excision and use
a rhomboid flap.

- 170 -
An easy way to remember how to draw it
quickly is illustrated in the diagram below.

- 171 -
Advanced Answer
question(s)

Who first • Limberg 1946


described the
rhomboid flap?

2.28 RHINOPHYMA

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Describe what • This is Rhinophyma which is due to


you see in this hypertrophy and hyperplasia of
picture. sebaceous glands in the skin and
associated with the face mite demodex
folliculorum

- 172 -
• There is evidence of scars suggesting
previous surgical intervention.
• Also be aware that other cutaneous
malignancies e.g. SCC/ BCC could be
concealed underneath.

Competency Answer
question(s)

What causes • Unknown.


rhinophyma? • May be seen in patients with acne
rosacea
• Not linked with alcoholism
How would you
diagnose • Clinical diagnosis
rhinophyma? • Punch biopsy (to rule out other
pathologies e.g. BCC/ SCC)

Advanced Answer
question(s)

- 173 -
How would you • Consider referring this patient to the
manage a patient Dermatologist
with • Consider referring to a clinical
rhinophyma? psychologist for support
• Varied treatment options but the
principles are paring down the bulky
tissue to either allow re-epithelialisation
or split-skin grafting.
• A variety of lasers have been used e.g.
Nd:YAG, Er:YAG, Argon, carbon dioxide in
a resurfacing mode (Sharplan 4 - 7 mm
spot at 20 - 40W or continuous 10 - 20 W
using a defocused 2 - 3mm beam).
• Debulking can also be done using the
microdebrider and FloSeal™ applied
topically for haemostasis.

- 174 -
- 175 -
2.29 LOWER LIP SCC AND KARAPANDZIC FLAP

CLINICAL
PHOTOGRAPH

Introductory Answer
question

What is your • BENIGN OR MALIGNANT LESION


differential
• TRAUMA, INFECTION, INFLAMATION
diagnosis of the
image seen • BCC, SCC MALIGNANT MELANOMA
above?

Competency Answer
question(s)

How would you • History and ENT examination


manage this
• If cause is not clear, I would arrange an
patient? incision or punch biopsy, which can be
Biopsy comes done under a local anaesthesia.

- 176 -
back as SCC. • Skin MDT
What would you
• MOH surgery or excision with at least
do? 4mm margins
How would you • Reconstruction ladder
treat this lesion
surgically and
reconstruct the
defect?

Advanced Answer
question(s)

What is a • Karapandzic flap is rotation advancement


Karapandzic flap? lip flap to restore circumoral sphincter

• the main disadvantage is small mouth


opening

- 177 -
2.30 Z-PLASTY

CLINICAL
PHOTOGRAPH

Introductory Answer
question

This 35 year old • The scar needs revising; however, one


man is unhappy should always consider if camouflaging
about the scar with makeup as a option in managing
next to his eyes scar disorder.
How would you • A Z-plasty would be the best technique to
deal with this revised such as scar because lengthening
problem? is required as well as changing the
direction of the scar.
• A Z-plasty is an example of a
transposition flap.

- 178 -
Competency Answer
question(s)

Show me how
you would do a Z-
plasty

Diagram illustrating z-plasty after removal of


scar tissue (An easy way to draw before
and after is to remember Z becomes S)

By how much is
the scar
lengthened?

- 179 -
Note that the direction of the scar and the
length of the scar increase depending on the
angles used; the rule is every 15 degrees there
is an increase in length of 25 percent (so 30
degress/25 %; 45 degrees/50% ; 60
degrees/75% etc)

Advanced Answer
question(s)

What is your • Level 1 – healing by 2nd intention eg


understanding of concave surfaces on nose eye ears and
the temple (NEET vs NOCH*), primary
reconstruction closure, delayed primary closure
ladder? • Level 2 – graft (split skin, full thickness,
composite)
• Level 3 – flaps (local and regional)
• Level 4 - Free flaps
• Level 5 – Prosthesis
*Convex surfaces heal poorly by secondary
intension, nose, oral, cheek helix

- 180 -
CHAPTER 3

Head and Neck Surgery

3.1 SALIVARY GLAND NEOPLASM

CLINICAL IMAGES

Introductory Answer
question

You have been • This is a T1-weighted MRI scan (axial and


referred a 60 year coronal).
old woman with a
• There is around, well defined mass within
slowly growing
the left parotid gland on axial image.
mass in her neck.
This mass is bright on the coronal image
She is otherwise
post-gadolinium contrast.
completely
asymptomatic. • There is no lymphadenopathy, erosion of
Describe what the mandible or invasion of the

- 181 -
you see. sternocleidomastoid muscle.

Competency Answer
question(s)

Based on these • Parotid neoplasm (benign or malignant).


scans, what is Overall, benign parotid lesions are most
your differential common e.g. pleomorphic adenoma,
diagnosis? Warthin's.
What clinical Intra-parotid lymph node. This would likely
features are you be pathological and a thorough diagnostic
aware of which workup is necessary, including otoscopy, and
suggests parotid rule out any cutaneous lesions.
gland
• Pain, rapid growth, fixity to surrounding
malignancy?
structures, nerve involvement or neck
How would you metastasis.
manage a patient
with a parotid • Conservative. Not unreasonable option,
pleomorphic especially if the patient is medically unfit
adenoma? for surgery. Patients should be
appropriately counselled on the risks of
malignant change (carcinoma ex
pleomorphic) and continued growth of
the tumour (large tumours place the
facial nerve at risk during surgery).
• Surgery

Advanced Answer
question(s)

You decide to • It is well documented that tumour


operate and spillage pertains to a higher rate of
during the recurrence. Revision parotid surgery is
procedure, there challenging with a higher risk of trauma
is tumour to the facial nerve.

- 182 -
spillage. Given • Continue with the operation. There is no
that the evidence that any intra-operative
pathology is manoeuvre decreases the risk of
benign, is this recurrence. Most surgeons would cover
occurrence the breached tumour surface with a swab
significant? and wash the surrounding surgical site
with copious amounts of saline. If the
breach is small or limited to the tumour
What would you capsule, some surgeons even repair it to
do in the event of prevent actual spillage of neoplastic
tumour spillage? tissue into the surrounding wound. The
parotid bed is again washed out at the
end of the operation.
• Importantly, the case should be
discussed at the Head and Neck MDT to
decide if post-operative radiotherapy is
warranted.
• These patients will require long-term
follow-up.

- 183 -
3.2 NECK LUMP

SCENARIO A GP has referred a 30 year old man with a


left neck mass, which appeared following a
(no clinical
viral upper respiratory tract infection.
photograph)

Introductory Answer
question

How would you • History


clinically assess this
• Examination
patient?
• Think central versus lateral neck masses.

• Branchial cyst

What is the likely • Lymphadenopathy (reactive, infective)


differential • Neoplastic (lymphoma, metastasis)
diagnosis in this
patient?
• USS +/- FNA (first line)

How would you • CT/MRI Neck (adjuncts to diagnosis and


investigate this planning of surgery)
patient?

• Straw/ cream-colored, cloudy, low


What is the typical cellularity
description given to

- 184 -
the FNA aspirate for
a brachial cyst?

Competency Answer
question(s)

What is the • Theories include branchial cleft remnant


pathogenesis of or trapped embryonic cells within a
branchial cyst? lymph node.

Assuming this • Watchful wait – whilst it is not entirely


patient has a unreasonable, the patient should be
branchial cyst, how counselled that the cyst may persist or
would you manage acutely enlarge following another
this patient? infection causing potential airway
compromise.
• Surgical excision

Describe the
images you see.
CT scans in axial,
coronal and saggital
planes respectively.
There is a low
intensity mass on
the left side of the
neck (Level 2/3),
beneath the
sternocleidomastoid
muscle. The mass
appears to be well
defined and there is
no evidence of

- 185 -
lymphadenopathy.

Advanced Answer
question(s)

Would your • Yes! An older patient presenting with a


management be any lump which appears to represent a
different in an older branchial cyst on ultrasound should be
patient, say for regarded as having a malignancy until
example, aged 55 proven otherwise. The 'branchial cyst'
years old? could represent a cystic metastatic lymph
node.

How would you • Further imaging of the neck and chest


manage such a should be undertaken (if not already
patient? previously performed). Ideally the scans
and FNA cytology should be discussed at
a Head and Neck MDT to facilitate
targeted biopsies of the upper
aerodigestive tract .
• If the scans remain equivocal, some units
would consider a PET-CT scan which may
be part of their diagnostic protocol for an
unknown primary.
• Otherwise, an examination under
anaesthetic, multiple blind biopsies (PNS,
base of tongue, pyriform fossa) and
excision of the cystic mass may be
reasonable. Should such an undertaking
be considered, it may be worth placing
the skin incision in a line for a possible
neck dissection in the future.

- 186 -
3.3 HOARSE VOICE

SCENARIO A 25 year old man presents with a hoarse


voice which is getting worse. He is otherwise
(followed by a fit and well. However, he smokes 10
clinical cigarettes a day and drinks socially.
photograph)

Introductory Answer
question

What is your Benign or malignant cause


differential
• Benign: vocal cord nodules or cysts,
diagnosis and
nerve palsy (vagus or RLN), Papilloma.
what procedure
would you • Malignant in view of smoking and alcohol
perform in clinic history
to make a
diagnosis? • Fine Nasal Endoscopy examination (FNE).

Competency Answer
question(s)

This is what you • This is the typical appearance of laryngeal


see on FNE, how Papilloma.
would you
• The aetiological agent is Human
manage the case
Papilloma Virus (HPV).
and what is the
cause for this • After taking a full history and examining
condition? the patient I would list the patient for

- 187 -
microlaryngoscopy and excision biopsy of
the lesions using cold steel or a
microdebrider. Care must be taken to
avoid webbing in the region of the
anterior commissure.
• In cases of frequent reoccurrences, I
would consider further surgical
debridement along with adjuvant therapy
such as intra-lesional cidofovir or antiviral
agents eg ribavirin.
• The histology is likely to show HPV
subtypes HPV 6 or 11 (the latter is more
aggressive).

Advanced Answer
question(s)

Why might the • Vaccination: Gardasil offers some


incidence of this protection against HPV 6,11, 16 and 18
condition while Cervirix offers protection against
decrease in the subtypes 16 and 18 only.
future?

3.4 CARCINOMA OF UNKNOWN PRIMARY (CUP)

SCENARIO A 56 year old male presents with a 2 month


history of left neck swelling. He is otherwise
(with clinical
fit and well. He is a smoker (15
photograph)
cigarettes/day) and drinks 20 units per week.

- 188 -
Introductory Answer
question

What is your Malignant causes:


differential • Secondary (metastatic neck disease)
diagnosis?
• Primary: E.g. lymphoma,
submandibular/parotid gland tumour
Benign causes:
• Paraganglioma
• Nerve sheath tumour
• Laryngocele
• Reactive/infected node i.e.TB

Competency Answer
question(s)

Assuming it is a • After a full history pertaining to red flag


metastatic node, symptoms and complete
how would you otolaryngological examination including
investigate the FNE, I will proceed to requesting an

- 189 -
case? ultrasound-guided Fine Needle Aspiration
for Cytology (FNAC).
• I will also request a staging MRI scan of
the head and neck as well as a CT scan of
chest and upper-abdomen
• If a primary is obvious on examination, I
will proceed to doing a pharyngo-
laryngo-oesphagoscopy with a view to
obtaining a tissue diagnosis from the
primary and also rule out synchronous
primary disease within the upper-
aerodigestive tract.
• If no primary disease is detected, I will
request a half-body CT-PET scan to look
for a source particularly within the
oropharynx and then perform
panendoscopy with bilateral
tonsillectomies and targeted biopsies of
any site of increased activity seen on the
scan.

Advanced Answer
question(s)

If it turns out to • Multi-disciplinary team involvement


be an unknown
• This can be treated either by selective
primary with a
neck dissection or neck irradiation of
single 2.5 cm level
ipsilateral levels II-IV. Since 40-55% of all
II node, how
metastatic neck disease arise from
would you treat?
oropharyngeal primaries, this would
provide adequate control. If
extracapsular spread (ECS) is seen in the
neck dissection specimen, adjuvant RT is
indicated.

- 190 -
• There is no evidence to suggest that
combined modality treatment (unless
evident ECS) or mucosal irradiation
increases either overall survival or loco-
regional control.

3.5 TONSILLAR CARCINOMA

Clinical
photographs

- 191 -
Introductory Answer
question

What do you see • Considering this is an elderly gentleman,


and what is your this is most likely a right tonsillar
most likely malignancy with metastatic neck disease.
diagnosis?

Competency Answer
question(s)

How would you • MDT setting


treat it assuming
• T2N2b puts this disease as a stage 3 and
it is is a T2N2bM0
therefore advanced malignancy. Early
SCC and patient is
malignancy (stage1-2) is treated with
willing to undergo
unimodality treatment and advanced
treatment?
(stage 3-4) with multimodality treatment.
• Therefore, this case needs multi-modality
treatment with curative intent i.e.

- 192 -
i) Surgery followed by chemoradiotherapy
(CRT) /RT alone (if chemotherapy is
contra-indicated i.e age > 70 years/poor
renal function)
ii) CRT followed by ipsilateral neck
dissection.
• Surgical options include:
a) ipsilateral selective neck dissection
followed by chemoradiotherapy
b) traditional lip split mandibulotomy with
wide excision plus free flap
reconstruction with bilateral selective
neck dissection
c) transoral laser/robotic-assisted resection of
primary disease plus bilateral selective
neck dissection.

Advanced Answer
question(s)

How might the • HPV +ve tumours have a better prognosis


HPV status affect than if HPV-ve.
management and
• Ang et al NEJM 2010, demonstrated
outcomes?
distinct stratification in prognosis based
on HPV and smoking status into high risk
(HPV-ve smokers), intermediate risk
(HPV+ve smokers) and low-risk (HPV+ve
non-smokers) tumours.
• While it is not yet proven, research
through De-ESCALaTE, RTOG 1016 and
ECOG de-intensification studies hope to
determine whether treatment for HPV
+ve tumours could be de-escalated.

- 193 -
Options could include RT in combination
with Cetuximab (instead of standard CRT)
or de-intensification of RT dose.

3.6 NASOPHARYNGEAL CARCINOMA

CLINICAL
PHOTOGRAPH

Introductory Answer
question

This is an
• There is a fleshy lesion in the posterior
endoscopic view part of the nose consistent with either a
of the nose of a benign process (such as adenoid
55 year old hypertrophy) or a malignant lesion (such
Chinese man as a nasopharyngeal carcinoma)
complaining of • Considering the patient is from Asia, I

- 194 -
nasal blockage, would be very concerned that this is a
hearing loss and possible nasopharyngeal carcinoma.
epistaxis.
Describe what
you see and tell
me the most
likely diagnosis.

Competency Answer
question(s)

What is the • 25/100,000 in SE Asia compared to


incidence of NPC 1/100,000 in Europe; Bimodal pattern
in Asia compared (adolescent, 50-60yrs), M:F = 2:1
to Europe?

• Multifactorial: genetics, salted fish


Why is there a (nitrosamines) and EBV
higher incidence
of NPC in Asia? • EBV is particularly associated with WHO
type 2 (non-keratinising SCC) and 3
(undifferentiated NPC)
• WHO Type 1 NPC is keratinizing SCC

How would you • Audiogram and tympanogram


investigate this
patient? • FBC, U&E, LFTs; IgA and IgG to EBV Viral
capsid antigen (correlate tumour burden)
• USS and FNAC neck masses
• CT head neck and chest
• CSF microscopy if skull base is breached
• MRI if intracranial extension suspected

- 195 -
• Consider PET

Advanced Answer
question(s)

Explain T2N2M0 • T2 means that there is parapharyngeal


classification for extension of the cancer;
NPC • N2 means that there is either bilateral
metastases in lymph nodes or 6cm or less
in greatest dimension above the
supraclavicular fossa.
• M0 means there is no distant metastasis

What treatment
modality would • In adult cases: Concurrent cisplatin or
the MDT decide 5FU with RT
to use? • In Paediatric cases: Neoadjuvant chemo
followed RT (IMRT if possible to avoid
cord damage).

- 196 -
3.7 LARYNGEAL CARCINOMA AND NECK DISSECTION

CLINICAL
PHOTOGRAPHS

Introductory Answer
question

What is your • Benign lesion eg granuloma, amyloid


differential
• Malignant lesion eg SCC
diagnosis?

- 197 -
Competency Answer
question(s)

How would you • History, examination including flexible


manage this nasendoscopy
patient? • CT neck and chest
• Microlaryngoscopy and biopsy

What are the • Treatment options: cold steel, laser,


treatment radiotherapy
options for a T1a
No SCC lesion?

• Larynx because of the greater risk of


What if the CT airway obstruction
scan shows a
synchronous
lesion? Which
one takes
priority?
• radical neck dissection – removal of all
cervical nodes, SMG, IJV, SCM and XI
nerve
What are the
different types of • modified radical neck dissection –
neck dissections? removal of levels I-V cervical nodes with
preservation of one or more of 3 non-
lymphatic structures (IJV, SCM and XI)
• selective neck dissection (usually for N0
neck)
1. levels I-III for oral lesions

- 198 -
2. levels II-IV for larynx and hypopharynx
3. levels II-V usually for skin tumours eg
melanoma
4. level VI – for thyroid cancer
• Extended neck dissection

Advanced Answer
question(s)

Why is it possible • Emissary veins dilate and allow venous


to take both blood flow from the intracranial to
IJVs during extracranial systems.
bilateral neck
dissection?

3.8 SUPRACLAVICULAR METASTATIC SQUAMOUS CELL


CARCINOMA

SCENARIO GP referral (2WW):


(no clinical Many thanks for urgently reviewing this 60
photograph) year old man with a 6 week history of an
increasing right sided neck lump

- 199 -
Introductory Answer
question

What is the • Benign – infective (bacterial, viral, TB),


differential lipoma, vascular, neural
diagnosis?
• Malignant- primary (lymphoma),
secondary (SCC from aerodigestive tract
or other site eg. Chest or abdomen,
bearing in mind the location of the
lesion).

Competency Answer
question(s)

What features in • History: Rate of growth of lump; pain;


the history and otalgia; hoarseness; dysphagia;
examination odynophagia; weight loss; smoking; night
findings would sweats; travel abroad; haemoptysis;
help you history of local infection of lump; history
distinguish of URTI
between a benign
• Examination: neck node features – size,
or malignant
site (this can give a clue as to the possible
cause?
primary site), fixity, skin involvement;
neural involvement; oral cavity
Assume there is a examination; flexible nasendoscopy of
5cm fixed lump upper aerodigestive tract.
found in the right
supraclavicular
fossa, with no • Bloods, CXR, CT scan neck and chest, USS
other abnormality guided FNAC.
found on ENT
examination
including FNE. • FNAC is first choice if suspect SCC,
What however in younger patients with risk of

- 200 -
investigations lymphoma, may prefer trucut core biopsy
would you as FNAC often not sufficient yield to
arrange? make diagnosis of lymphoma.

What about an • Incisional biopsy would be a diagnostic


incisional biopsy? option once SCC is excluded.

Advanced Answer
question(s)

FNAC confirms • Aim is to find the primary site and stage


SCC and FNE does the tumour. As primary site is likely to be
not demonstrate infraclavicular, consider PET scan before
a primary lesion. panendoscopy to identify primary. If the
What would you primary is ultimately within the upper
do? aerodigestive tract, the PET will also
better direct biopsies to likely sites. Also
need to undertake full staging
investigations including CT scan
chest/abdomen.

• Treatment: Always in conjunction with


Head & Neck MDT and following full
If the FNAC shows discussion with patient and relatives,
SCC, how would taking into account patient
you treat the comorbidities.
patient? - Management of the neck: Neck
dissection +/- RXT depending on
histological prognostic factors such as
extracapsular spread, neurovascular
invasion.
- Management of the primary if site

- 201 -
identified: ChemoRXT or surgery.

• Management of the occult primary: Wide


field RXT to likely sites or watch and wait,
balancing effectiveness of treatment
against morbidity from RXT.

3.9 BLACK HAIRY TONGUE

CLINICAL Dorsal surface of the tongue showing taste


PHOTOGRAPH buds

- 202 -
Introductory Answer
question

What are the • There are 4 different types of papillae


different types of present on the tongue: fungiform,
papillae found on filiform, foliate and circumvalate.
the tongue?
• Generally they contain the receptors for
taste (taste buds) except the filiform
papillae, which are long and thin and
most numerous.

Competency Answer
question(s)

Tell me what you • Clinical photograph of the dorsal surface


know about the of the tongue consistent with the
condition shown diagnosis of a black hairy tongue.
in this
• It is a condition of defective
photograph?
desquamation, hypertrophy and
elongation of the filiform papillae which
are present throughout the surface of the
tongue.
• It is called black hairy tongue because of
keratinisation but does not have to be
black in colour.
• May be due to poor oral hygiene, low
roughage diet, antibiotic usage or post-
Radiotherapy.
• It is more common in males, especially
those who smoke and drink lots of tea
and coffee. Also increased incidence in
HIV, IV drug usage. Higher in those

- 203 -
incarcerated.
• The condition is mostly asymptomatic
but when candidal overgrowth occurs it
may lead to burning tongue syndrome (or
Glossodynia).
• The condition may be treated with
brushing and or tongue scraping. Diet
changes i.e. more roughage, less caffeine
etc.
• Surgery is reserved for unresponsive
cases; Electrodessication, CO2 laser,
scissors.

Advanced Answer
question(s)

How would you • Oral hairy leukoplakia is a white patch on


distinguish this the side of the tongue with a hairy
condition from appearance and occurs mainly in patients
oral hairy who are immunocompromised (hence
leukoplakia? HIV-associated hairy leukoplakia).
• It is caused by Epstein-Barr Virus (EBV)
and therefore may be distintuished from
black hairy tongue by punch biopsy and
immunostaining for EBV.

- 204 -
3.10 BENIGN PAROTID LESION

SCENARIO GP referral: Thank you for seeing this 67 year


old man with a six month history of a swelling
(clinical on the side of the face which has recently
photograph) become painful.

Introductory Answer
question

What do you see • This is a swelling in the upper part of the


in the photograph neck in the region of the parotid gland. It
and what is your may represent a benign or malignant
differential salivary or non-salivary lesion.
diagnosis?

Competency Answer
question(s)

How would you • History: swelling constant or


manage this intermittent, relation to eating/drinking,
pain, facial nerve palsy, smoking, weight

- 205 -
patient? loss, medical comorbidities (dehydration,
diabetes, sarcoid).
• Examination: swelling – firm or soft;
location – clench teeth (parotid or
masseter); intraoral examination (Deep
lobe; also Stensens and Whartons ducts,
stones); neck nodes; FNE of upper
aerodigestive tract (UADT).
• Investigation – Ultrasound plus FNAC,
MRI scan.

Assuming FNAC is • To preserve the facial nerve (CNVII):


suggestive of - CNVII nerve monitor
pleomorphic
adenoma and you - Surgical landmarks - tympanomastoid
are performing a suture, posterior belly digastric; tragal
parotidectomy – pointer
describe how you - Measures to minimise bleeding:
would preserve Hypotensive anaesthesia, local
the facial nerve in anaesthetic with adrenaline, head up
the surgery? position, gentle dissection above plane of
nerve.

What would you


do if the patient • Postop CNVII palsy:
woke up from
surgery with a VII - Wait for LA to wear off.
nerve palsy? - Is the palsy expected (i.e. if the tumour
was involved with the nerve) or
unexpected. If unexpected: manage any
compression (eg. haematoma), consider
steroids; monitor closely.
- If palsy does not recover, consider nerve
conduction studies for prognostic

- 206 -
purposes.
- May need facial reanimation techniques
(CNVII grafting or repair if known to be
transected, otherwise static or dynamic
reanimation methods).

Advanced Answer
question(s)

Assume histology • Pleomorphic adenoma v ACC: both are


confirms adenoid from the same cell type (from the
cystic carcinoma intercalated duct) and are generally well-
(ACC) – explain differentiated, hence similarities in the
how the cytology FNAC cytology profile.
and histology can
be so apparently
incongruent?

Does this change • Management – ACC needs very close and


management, long term follow up. Due to them being
compared with if slow growing and well-differentiated,
histology showed distant metastases can appear years
a pleomorphic later; ensure margins clear; wider
adenoma? scanning including chest and brain.

- 207 -
3.11 MALIGNANT PAROTID LESION

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Describe what • Fungating lesion in the right tail of


you see. parotid region/ level 2 neck.
• Further skin changes in right pre-
auricular region.
• Patient is under GA, on operating table
site of operation marked.

- 208 -
Competency Answer
question(s)

What is your • Primary cutaneous malignancy.


differential
• Fungating malignancy (lymph node,
diagnosis?
parotid).

What clinical
features are you • Pain, rapid growth, fixed to surrounding
aware of which structures, nerve involvement or neck
suggests parotid metastasis.
gland
malignancy?

• Ultrasound guided FNAC is


How would you recommended for all salivary tumours
assess this patient and cytology should be reported by an
in clinic and what experienced expert histopathologist.
investigations
would you have • Some units undertake MRI neck and CT
requested for? chest to stage the disease.
• It is imperative to discuss the patient at a
head and neck MDT.
What is the role
of frozen section • Accurate diagnosis is often difficult and
in diagnosing false negative rates are significant; hence
parotid frozen section should not be relied on
neoplasms? except for establishing excision margins.

Apart from • Neck dissection is recommended in all


parotidectomy, cases of malignant parotid tumours
how will you except for low grade small tumours.
manage a patient Therapeutic neck dissection is indicated
as seen in the for lymph node involvement. Elective

- 209 -
picture? neck dissection is indicated for high-stage
and clinically high-grade disease such as
high-grade adenocarcinoma, carcinoma
ex-pleomorphic adenoma, squamous cell
carcinoma, high-grade mucoepidermoid
and undifferentiated carcinoma.
The following factors are indications for post
operative radiotherapy:
• microscopic residual disease.
• adenoid cystic tumours.
• aggressive undifferentiated tumours.
• large tumours "4cm rule".

Advanced Answer
question(s)

How would you • As a general principle, if the facial nerve


manage the facial function is normal pre-operatively then
nerve in this every attempt to preserve facial nerve
patient? function should be made during
Parotidectomy. If the facial nerve is
divided intra-operatively, then
immediate microsurgical repair (with an
interposition graft if required) should be
considered.
• See ENTUK Head and Neck Cancer:
Multidisciplinary Management
Guidelines, 2011.

- 210 -
3.12 VOCAL CORD DYSPLASIA/EARLY GLOTTIC SCC

SCENARIO GP referral (2 week wait): Thank you for


seeing this 50 year old patient with a two
(no clinical
month history of hoarseness.
photograph)

Introductory Answer
question

How would you • History: hoarseness – questions primarily


manage this focussed at assessing risk factors
patient? (smoking & alcohol, weight loss,
occupation, history of thyroid problems,
acid reflux); also is hoarseness persistent
or intermittent; referred otalgia;
haemoptysis; aspiration; associated
dysphagia; odynophagia.
• Examination: full neck examination for
cervical lymphadenopathy; examination
of oral cavity; FNE of larynx and pharynx.
• Investigations: baseline blood tests
including FBC; TFTs; CXR; further
investigations to be guided following
clinical assessment.

Competency Answer
question(s)

What is your • Benign: chronic laryngitis,

- 211 -
differential laryngopharyngeal reflux, benign growths
diagnosis? (nodule, polyp, cyst, sulcus, papillomata).
• malignant: dysplasia; malignant lesion
(SCC).

How would you


make a diagnosis? • Microlaryngoscopy + Biopsy

Biopsy shows • Re-biopsy if biopsy and clinical suspicion


dysplasia – what do not correlate well (i.e. do not trust a
do you do? negative histology). If representative –
lifestyle advice (stopping smoking, reduce
alcohol), regular follow up to ensure no
progression.

If SCC is • TOLS v Radiotherapy:


confirmed, what
are the relative Local control: similar survival rates for T1,
merits of evidence suggests TOLS associated with
Transoral worse results in T1b rather than T1a lesions
Laryngeal Surgery but rates similar for radiotherapy. For T2 –
(TOLS) versus poor data available for TOLS so far, RXT 70-
radiotherapy 75% 5 year survival. Overall impression:
(RXT)? probably finely balanced decision.
Surgery one off and more convenient,
however consider perioperative risks in
patients with comorbidities.
Voice: radiotherapy affects whole larynx
regardless of tumour size, whereas effects on
voice with surgery are dependent on size and
depth of lesion. Consensus is that surgery is
worse especially if anterior commissure
involved and with larger tumours. Results on

- 212 -
voice are equal for small T1a lesions.

Chance of requiring eventual total


laryngectomy (TL): Similar for T1a lesions
however treated; for T1b marginal increased
chance of eventual TL if radiotherapy was the
primary modality of treatment rather than
TOLS; for T2 eventual TL rates lower with
TOLS due to possibility for multiple TOLS
salvage.

Advanced Answer
question(s)

If radiotherapy is • Initial assessment as above with


given and there is particular attention paid to excluding a
a biopsy-proven synchronous second (usually lung)
recurrence after 2 primary.
years, what
• Treatment: TOLS if lesion amenable
would your
depending on staging; RXT if TOLS
management be?
performed first; consider TL if fixed cords
or if lesion not suitable for TOLS.

- 213 -
3.13 HPV AND OROPHARYNGEAL LESION

CLINICAL
PHOTOGRAPHS

Introductory Answer
question

Describe the On the left is a clinical photograph showing a


clinical unilateral lesion in the right upper pole of the
photographs. tonsillar fossa. The lesion is approximately
twice the size of the uvula and has a
mulberry-like appearance. The specimen is
shown in the right photograph. Part of the
left tonsil is visible and appears to be normal.

- 214 -
Competency Answer
question(s)

What is the most • Oral papilloma caused by HPV.


likely diagnosis of
• HPV 16 and 18 as associated with
the lesion and tell
oropharyngeal carcinoma (tonsil and
me about the
tongue base)
possible
aetiology?
• Small double stranded DNA virus
Tell me about • Most common sexually transmitted virus
HPV virus
• Wide range of sequelae (mild disease to
high-grade squamous lesions)
• Mild Disease
• Genital warts
• Anal warts
• Cervical dysplasia
• Severe Disease:
• Carcinomas of the cervix, vagina, vulva,
anus and penis.
• Non-genital types associated with warts
on hands, feet etc
• Over 120 subtypes

How is the virus


detected? • Method not Standardised
• Direct Detection of HPV Subtype DNA is
possible with PCR or in-situ hybridisation
but this is very expensive

- 215 -
• Immunohistochemistry for P16, a
surrogate marker,, is cheaper 100%
Sensitive, 79% specific.
• 7% discordant rate between PCR and P16

Advanced Answer
question(s)

Do you think boys In view of the apparent increase in incidence


should also be of HPV related oropharyngeal carcinoma in
vaccinated with young adults, the answer is probably, Yes.
HPV VACCINE?
Gardasil offers some protection against HPV
6,11, 16 and 18 while Cervirix offers
protection against subtypes 16 and 18 only.

- 216 -
3.14 FACIAL PAIN

SCENARIO A 45 year old business woman attends your


clinic complaining of facial pain.
(no clinical
photograph)

Introductory Answer
question

What is your • Pain may be arising from any of the


differential multiple systems in the head and neck
diagnosis? region such as the nervous system,
rhinological system, otological system,
dentition and TMJ, vascular system or
ophthamological system.

Competency Answer
question(s)

Tell me about • The four main types of neurological head


neurological pain are migraine, cluster headache,
pain? tension headache and trigeminal
neuralgia.
• Migraine affects about 10-15% of
population and is thought to involve a
trigeminovascular mechanism. It may be
triggered by stress, menstruation, dietary
intake (tyramine). 10% display aura

- 217 -
(visual, limb or face dysaesthesia, mood
change). Aura precedes pain by 10-60
mins. Throbbing unilateral pain that
spreads. Photophobia, phonophobia and
nausea may last 4-72 hours. Patients are
often pain free between attacks.
Treatment: Antiemetics, Analgesics,
Ergotamine – can abort impending
attack, Sumatriptan (5-HT1 agonist) given
subcutaneously can abort attack,
Prevention by avoiding precipitants, or
trial use of beta blocker, pizotifen or
methysergide.
• Cluster headaches occur 1-8 times/day,
each attack lasts 15 mins – 3 hours and
may go on for 3-12 weeks. Patients can
be symptom-free for up to 1.5 years. The
deep throbbing severe headache often
wakes the patient up. The headache is
associated with parasympathetic
overdrive such as nasal congestion,
injected conjunctiva and ptosis and may
be confused with allergic rhinitis.
Exercise with increased sympathetic
activity (eg pacing up and down) is
helpful in relieving symptoms, other
forms of treatment includes oxygen
therapy (cerebral vasoconstriction),
ergotamine, sphenopalatine ganglion LA
block.
• Tension headache is usually a dull aching
pain which may be unilateral or bilateral.
There are associated with specific trigger
points in muscles such as
sternocleidomastoid, trapezius,
temporalis and pterygoids. Tension

- 218 -
headache is linked to stress and
therefore relaxation is therapeutic.
• Trigeminal neuralgia is a sharp short
stabbing pain in the distribution of one or
more of the three branches of the
trigeminal nerve. The pain occurs in
response to innocuous stimuli. It is due
to local demyelination of trigeminal root
entry zone. This may be caused by
compression by a small vein or artery in
posterior cranial fossa. Treatment
includes carbamazepine, gabapentin
alcohol/Glycerin/Radiofrequency
ablation to the trigeminal ganglion.
Surgical decompression of vascular loop
via a posterior fossa craniotomy is rarely
required.

Advanced Answer
question(s)

Have you heard • This refers to neuropathic pain following


about post- acute herpes virus infection.
herpetic
• It is usually a burning pain, occasionally
neuralgia?
throbbing
• The mechanism involves a modulating
effect of large calibre nerve fibres
(proprioceptive) at the dorsal root entry
zone.
• It can be treated with neuropathic agents
Amitriptylline, Carbamazepine or
Gabapentin. TENS, Acupuncture and
Capsaicin ointment have been used.

- 219 -
3.15 PHARYNGEAL POUCH

SCENARIO A 74 year old man has been referred to you


by his GP with a 6 month history of
(followed by
progressive dysphagia.
clinical images)

Introductory Answer
question

How would you • History focusing on the onset of


assess him? dysphagia, type (solids, fluids), weight
loss, associated symptoms (pain,
hoarseness, neck lumps, odynophagia).
• Clinical examination, including fiberoptic
endoscopy to rule out neoplasm.

Competency Answer
question(s)

On further • Zenker's diverticulum or pharyngeal


questioning, the pouch.
patient also
• Differential: Oesophageal stricture,
describes post-
severe reflux disease/ hiatus hernia.
prandial
regurgitation of
food and weight
loss. Apart from
endoscopic

- 220 -
evidence of reflux,
clinical
examination was
unremarkable. • Water soluble contrast, or Barium
What is your swallow.
differential
diagnosis?
• Barium swallow image demonstrating a
pharyngeal pouch. There is no evidence
How would you
of a filling defect within the pouch or
investigate this
aspiration on this image. A full series of
patient?
images would be required for
confirmation.
Describe what you
see.

What is a • Also known as Zenker's diverticulum, it is


pharyngeal a pulsion diverticula herniation through
pouch? Killian’s dehiscence (or Killian’s triangle)
above the cricopharyngeus muscle,
between the oblique and transverse
constrictor muscles of the pharynx.

How would you


manage this • Appreciating that many patients with
pharyngeal pouch are elderly, an

- 221 -
patient? anaesthetic opinion is needed to ensure
that they are medically fit for a general
anaesthetic. Endoscopic stapling is now
an established technique in the UK. NICE
guidance (2003) also recommend that
sub-specialisation within ENT
departments should occur for this
procedure.
• A recent literature review (Leong et al.,
2012) of outcomes from UK departments
reported that 92.3% (540 out of 585)
were successfully stapled. Forty-five
(7.7%) procedures were abandoned
intra-operatively. The most common
reason was difficulty assessing a small
pouch. The majority of patients (92%)
had resumed oral intake by the second
post-operative day. Most patients (87%)
were discharged by the second post-
operative day. Outcomes were good with
over 90% reporting resolved or
significantly improved symptoms.

Advanced Answer
question(s)

Assuming you • Day case pharyngeal pouch stapling is not


have currently routine UK practice. Minor
successfully complications included dental trauma,
stapled the transient hoarseness and sore throat may
pouch, what cause delayed resumption of oral intake.
are your post- Given that many of these patients are
operative elderly, they will require in-patient care
instructions? and close observation in the immediate
post-operative period.

- 222 -
• The most feared complication following
this procedure is perforation. The UK
review (Leong et al., 2012) reported an
overall perforation rate of 4.8% The
patient needs to be regularly monitored
for retrosternal pain, tachypnoea,
tachycardia, pyrexia and odynophagia.

What do you see


on this lateral • A linear gas shadow with pre-vertebral
swelling (normally C2-4 soft tissue
neck x-ray?
thickness 7mm max) and straightening of
the normal lordosis is typical of a
perforation of the oesophagus.

• This is an ENT emergency as delayed


How would you
management may lead to serious
manage a
sequelae such as mediastinitis. The
perforated
patient needs to be kept nil by mouth
pharyngngeal
and resuscitated as necessary. Expedient
pouch?
return to theatre should be planned and
involvement of another senior head and
neck surgeon should also be considered.
• Repair of the perforation is undertaken
as an external approach (left side of
neck). The patient will require
intravenous antibiotics, a nasogastric
feeding tube and prophylactic reflux
treatment.

- 223 -
3.16 PARAPHARYNGEAL MASS

SCENARIO This 21-year old girl presents with a history of


right neck swelling of 2 years, progressively
(with a clinical
increasing in size. Otherwise fit and well.
photograph)

Introductory Answer
question

What are your • Branchial cyst


differentials?
• Lymphadenopathy
• Parapharyngeal mass

How is the • The parapharyngeal space is divided into


parapharyngeal 2 parts by the fascial condensation called
space divided? aponeurosis of Zuckerkandl and Testut,
joining the styloid process and tensor veli
palatine muscle which arises by a flat
lamella from the scaphoid fossa (of the
medial pterygoid plate) from the
sphenoid spine and from the lateral wall

- 224 -
of the cartilage of the Eustachian tube.
Assuming that this
is a • Schwannoma
parapharyngeal
• Paraganglioma
space tumour
arising from the • Neurofibroma
post-styloid
compartment,
what would be
your differentials?

Competency Answer
question(s)

How would you History – duration, mass effect, local


manage this neurology, FH, symptoms of excess
patient? catecholamines
Examination - inspect mass, palpation,
pulsation, bruit, inspect oral cavity for
medialization
Investigation
CT scan of neck to assess mass, then proceed
for FNAC if it is not vascular in origin
MRI may be able to give a radiological
diagnosis but may not be able to show skull-
base erosion.
Proceed to surgical excision if required
following discussion at appropriate MDT. If
paraganglioma suspected will need endocrine
review +/- genetics referral.

- 225 -
Advanced Answer
question(s)

What are the


suprahyoid spaces
marked out on
the MR images?

• Parotid space

• Masticater space

• Pharyngeal mucosal space

- 226 -
• retropharyngeal space

• peri-vertebral space

3.17 HEMI-TONGUE ATROPHY

CLINICAL
PHOTOGRAPH

- 227 -
Introductory Answer
question

This 65-year old


• Obvious: Hemitongue (right) atrophy
lady appears at with loss of bulk and natural contours,
the head and smooth appearance (loss of papillae).
neck clinic for
• Less obvious: tongue fasciculation,
routine follow-up.
deviation towards the side affected.
Describe what Patient's own teeth!
you see.
• Not obvious: visible scars, facial palsy,
lesions on the face

What other • Dysarthria


clinical signs • Oral/ tongue ulcers due to poor
might you expect deglutition
in this patient? • Evidence of worn dentition (contralateral
molars)
• Neck scar, free flap etc.
• Palpable neck lump

Competency Answer
question(s)

What is the • Hypoglossal nerve palsy (cranial nerve


pathophysiology XII)
of this condition?

• Excision of CN XII was part of a wider


What may have oncologic resection e.g. adenoid cystic
caused the carcinoma of the submandibular gland
hemitongue
atrophy in this • Iatrogenic e.g. submandibular gland
excision for sialolithiasis

- 228 -
patient? • Hypoglossal canal: schwannoma,
meningioma, metastasis, spread of
nasopharyngeal carcinoma, large glomus
jugulare (grown into the hypoglossal
canal)
• Central causes: vascular e.g. thrombosis
of the vertebral artery, motor neurone
disease, syringobulbia

Advanced Answer
question(s)

How would you • Medial to the posterior belly of the


locate the digastric muscle. The nerve becomes
hypoglossal nerve exposed when the digastric is retracted
intra-operatively? superiorly.
• Superiorly, the nerve emerges from the
hypoglossal canal at the skull base,
between the internal jugular vein and
internal carotid artery.
• The nerve turns anteriorly over the
If this patient was carotid and as it does, gives off the ansa
cervicalis.
being seen at the
skull base clinic • The patient may have suffered facial
following excision nerve palsy as a consequence of acoustic
of an acoustic neuroma excision.
neuroma, how • A hypoglossal - facial nerve anastamosis
would you is a recognised method of reanimating
account for the facial paralysis. A House-Brackman grade
IV can be expected as best outcome.
hemitongue
atrophy seen
here?

- 229 -
3.18 CERVICAL MYCOBACTERIAL LYMPHADENITIS (SCROFULA)

SCENARIO (and This 27 year old HIV positive patient presents


Clinical with a painless neck lump that has be getting
photograph) bigger over the last 6 months. It does not
move with swallowing or tongue protrusion.

Figure 3.18 Clinical photo showing a painless


neck lump

Introductory Answer
question

What is your • This patient may be


differential immunocompromised and therefore at
diagnosis? an increased risk of infectious, so an
infection would be high on my list of
differential diagnosis. I would also
consider a neoplastic lesion.

- 230 -
• So my differential diagnosis would be:
1. infections, including Tuberculous
mycobacteria
2. Non-tuberculous (atypical) mycobacteria
(NTM)
3. Metastatic SCC or NPC
4. Papillary thyroid carcinoma
5. Lymphoma

Competency Answer
question(s)

How would you • Ultrasound scan and FNAC


attempt to make
• FNAC including Ziehl Neelsen stain for
a definitive acid fast bacteria in tuberculous
diagnosis? mycobacteria
• Culture of NTM using specific growth and
staining techniques

Advanced Answer
question(s)

Where is the • The neck is the commonest site of


commonest site extrapulmonary manifestation of
for TB other than tuberculosis.
the lungs? • Furthermore cervical mycobacterial
lymphadenitis tends to be unilateral, with
a predilection for nodes in the posterior
triangle (51percent) and deep upper
cervical (48 percent).
• In the case of mycobacteria tuberculosis:

- 231 -
Multi-drug therapy for mycobacteria
tuberculosis infection (this may include
Tell How would pyrazinamide, isoniazid, ethambutol,
you treat this rifampicin and streptomycin (‘PIERS’).
patient? Note that during therapy new or existing
cervical nodes may enlarge but this is not
indicative of failure as it is only transient.
• In the case of non-tuberculous
mycobacteria: Surgical excision.

3.19 PSEUDOMEMBRANOUS COLITIS (C.Diff infection)

SCENARIO A 66 year old patient underwent an


uneventful neck dissection. He was
(no clinical
discharged 3 days later with 7 day course of
photograph)
oral Co-amoxiclav because of aspiration at
the time of induction of anaesthesia. Four
days later, he developed non-bloody foul
smelling diarrhoea, fever, fatigue, loss of
appetite and severe abdominal pain.

Introductory Answer
question

What do you • A infective process affecting the gastro-


think is the cause intestinal system

- 232 -
of his symptoms? • In view of the antibiotic, I would consider
Pseudomembranous Colitis.
• MRSA is also a possibility

Competency Answer
question(s)

How would you • Full history and examination


manage this
• Admit the patient under joint care with
patient? Infectious Disease team
• Send bloods for blood culture,
haematological and biochemical profiles
and resusitate patient as required
• Stool sample for C. Difficile toxin
• Stop co-amoxiclav
• Rx Metronidazole +/- oral vancomycin (
risk of vancomycin resistant
Enterococcus)
• Faecal transplant for refractory cases

Advanced Answer
question(s)

Tell me about • Isolation eg side room with own toilet


infection control
• Barrier nursing
in your hospital
with regards to • Hand washing
C.Difficile • Own toilet in room
infection.

- 233 -
3.20 REINKE’s OEDEMA

A 45 year old woman smoker complains that


SCENARIO
her voice is becoming masculine and her
friends can’t recognised her on the phone
anymore.

Introductory Answer
question

What is going
• The history suggests Reinke’s oedema of
through your the vocal folds.
mind?
• I would also be thinking of a neoplastic
process since she is a smoker.

Competency Answer
question(s)

Which other • Voice Abuse


conditions are
• Reflux
associated with
Reinke’s • Hypothyroidism
oedema?

• After a taking a full history, I would


What would you proceed to examine the larynx with a
do next? flexible nasendoscope.

- 234 -
Draw a cross
section of the
vocal fold
showing the
various layers
and Reinke’s
space

What treatment • Surgery only if suspicion of CANCER or


would you offer? AIRWAY compromise
• Lateral cordotomy and suction or laser
vaporization
• Stop smoking
• SALT

Advanced Answer
question(s)

Is the surgery • Difficult (arm chair surgery)


easy or difficult?

Have you ever • No


seen Reinke’s
oedema in a
man?

- 235 -
3.21 RADIOTHERAPY AND SIDE EFFECTS

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Describe the
• Clinical photograph of the neck showing
clinical photo. erythema anteriorly in the region of the
larynx.
• The may represent radiotherapy changes
or thyroiditis

Competency Answer
question(s)

What are the Acute:


other side effects

- 236 -
of radiotherapy • Skin: erythema; desquamation; tanning;
loss of hair follicles; sweat/sebaceous
gland dysfuntion
• GI: Loss of taste; salivary dysfunction;
mucositis; N+D+V
• Bone marrow suppression
• Lung pneumonitis
Long term:
• Neck: fibrosis
• Jaw: fibrosis, reduced mouth opening
• Lymphatic: Lymphoedema
• Wounds: delayed healing
• Skin: telangectasias, ulceration leading to
ORN
• Salivary: xerostomia (amifostine through
treatment, pilocarpine after); dental
disease
• CNS: transient radiation myelopathy;
transverse myelitis (Lhermittes)
• Endocrine: hypothyroisim, GH deficiency
• Eye: cataracts, retinitis, keratitis(dry eye)
• Ears: OME; SNHL
• 2nd malignancy

- 237 -
Advanced Answer
question(s)

How does • Ionizing radiation interacts with


radiotherapy water/oxygen to form free radicals which
works? breaks dsDNA with the loss of cells
reproductive capacity
• Cancer cells have reduced ability to
repair damage – thus selective
• Normal cells produce p53 prolong ‘S’
stage and allow more time for DNA
repair).

3.22 SCC TONGUE

SCENARIO

(followed by a
clinical
photograph)

- 238 -
Introductory Answer
question

Tell me about this


• Clinical photograph showing an exophytic
clinical photo? lesion of the left lateral surface of the
tongue.
• The appearance is most consistent with a
malignant lesion such as squamous cell
carcinoma (the 2nd commonest oral
cancer); histological confirmation would
be required after full history and
examination.

Competency Answer
question(s)

What are the risk • Alcohol, smoking, Human Papilloma Virus


factors associated (HPV) infection (for tonsil and tongue
with SCC tongue? base mainly), previous radiotherapy,
betel nut chewing, sharp teeth and spicy
food.

What are the


• Conservative (if patient terminally ill with
treatment
other co-morbidity)
options?
• Surgical excision with neck dissection if
indicated
• Chemoradiotherapy
• Palliative treatment
• Oral cancer is mainly treated with
surgery, occasionally radiotherapy may
be used. Defect may be reconstructed

- 239 -
with a free flap such as radial forearm,
anterior thigh or rectus abdominis if bulk
is needed. Work up usually involves
OPG, MRI oral cavity and neck, CT chest
as well as EUA and biopsy.

Which staging • Panendoscopy and CT chest are


system is used for important for possible synchronous
tongue cancer? lesions.

• The tumour is staged by the TNM


classification:
T1: tumour 2cm

T2: 2cm < tumour 4cm

T3: tumour > 4cm


T4: tumour invading into adjacent structures

Advanced Answer
question(s)

How would you • Basic rule is to treat any patient whose


deal with a N0 risk of occult mets is greater than 20
neck? percent: Metastatic risk for floor of
mouth (25%), Tongue (60%), Base of
tongue (55%), Buccal mucosa /
Retromolar region (20%), Tonsil (36%),
Aryepiglottic Fold (30%); Piriform sinus
(65%).
• In general cavity tumours with depth
>3mm have higher risk of occult mets
(>20%)
• If surgery is proceeding for the primary

- 240 -
lesion then a neck dissection should be
done as it allows for histopathological
staging.
• Otherwise RT to primary lesion and neck
• Regional recurrence of pathological N0
neck is 1.9 percent.

3.23 VOCAL PROCESS GRANULOMA

This is an endoscopic view of the larynx in an


CLINICAL
intubated patient.
PHOTOGRAPH

- 241 -
Introductory Answer
question

Describe what
• A fleshy polypoidal lesion of the right
you see and tell hemilarynx. It is difficult to see where it is
me your attached.
differientials.
• This may represent a polyp or vocal
process granuloma. Other less likely
differientials includes SCC, TB,
hsitoplasmosis, coccidiomycos,
blastomycosis, Wegeners, syphilis,
leprosy, Crohns

Competency Answer
question(s)

Tell me more • Uncommon benign lesions commonly


about vocal centered on the vocal process of the
process arytenoid
granuloma.
• Evolve from repeated insults to mucosa
overlying fragile thin perichondrium (can
be mehanical, inflammatory or both).
• Initial ulceration leads to necrosis.
• Granulomatous reaction to repaeted
trauma or acid reflux
• Tends to occur in male professional voice
users or in females post intubation
• Patients may present with chronic cough,
throat clearing, hoarseness, Globus, sore
throat, dyspnoea, haemoptysis

- 242 -
Advanced Answer
question(s)

How is the • Surgery: laser or cold steel – no


condition randomised control trials regarding
treated? removal or leaving associated
cartilageleave cartilage
• Voice therapy – target breath control,
elevation of pitch, elimination of hard
glottic attacks
• PPI, limit intake alcohol, smoking,
caffeine, chocolate (all stimulate acid)
• If resistant consider botox therapy (to
decrease forceful adduction of
arytenoids).

- 243 -
3.24 PARAPHARYNGEAL SPACE ABSCESS

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Describe the scan


• Axial T1 weighted MRI without contrast
above. in the region of the suprahyoid neck
(through Parapharyngeal space)
• The most strike abnormality is ring
enhancing lesion with central low
intensity signal in the right
parapharyngeal space

- 244 -
• The most likely diagnosis is a
parapharyngeal abscess (DD branchial
cyst).

Competency Answer
question(s)

What signs and • Trismus, Parotid oedema, Dysphagia,


symptoms may Retromandibular neck, mass, medial
be present? dispalcement of tonsil

What is the • Tonsil, Dental (3rd molar), Pharynx,


source of Petrous temporal bone, Parotid, lymph
infection in such node
an abscess?

• From the neck: the lateral aspect of the


How would you parapharyngeal space is bordered by the
access the fascia of the posterior belly of the
parapharyngeal digastric muscle. Therefore cervical
space to drain access for abscess drainage involves
this abscess? identifying and sectioning of the
digastric muscle.

What are the


boundaries of the • Superior - base of skull
parapharyngeal
space? • Inferior - hyoid bone
• Anterior - pterygomandibular raphe
• Posterior – Fascia of tensor veli palatini
• Medial - superior constrictor
• Lateral - digastric muscle, Parotid,

- 245 -
mandible, lat pterygoid

Advanced Answer
question(s)

What are the • Septic thrombosis


potential
• Carotid blowout
complications of a
parapharyngeal • Cranial nerve involvement (horners, X,
XII)
abscess?
• Mediastinitis
• Pyopneumonithis
• purulent pericarditis
• bronchial erosion
• aspiration pneumonia

- 246 -
3.25 GOITRE

CLINICAL
PHOTOGRAPH

Introductory Answer
question

What is the
• This is an axial CT scan through the neck
abnormality at the level of the trachea showing a
shown in the huge goitre
image
• At the level shown there is no
compromise to the tracheal airway.

Competency Answer
question(s)

What are the Goitre aetiology may be divided into

- 247 -
causes a goitre? subgroups as shown below:
• Physiological: Puberty, increased
metabolic demand pregnancy
• Autoimmune: Graves’ disease,
Hashimoto’s thyroiditis
• Thyroiditis: Subacute granulomatous/de
Quervain’s, subacute lymphocytic, silent
Riedel’s thyroiditis, acute infective
(transient)
• Granulomatous diseases: Sarcoidosis,
tuberculosis, Iodine deficiency, Idiopathic

During • The recurrent laryngeal nerve is located


thyroidectomy in Beahr’s triangle, which is formed by (1)
how would you the common carotid artery, (2) trachea
find the recurrent and (3) inferior thyroid artery
laryngeal nerve
and the superior • The superior laryngeal nerve is found in
laryngeal nerve Joll’s triangle, which is formed by (1) the
midline, (2) superior thyroid pedicle and
(3) strap muscle.

- 248 -
Advanced Answer
question(s)

Tell me about the • The thyroid is the 1st endocrine gland to


embryology of develop
the thyroid gland.
• At 24th day gestation there is
proliferation of endodermal epithelial
cells on median surface of pharyngeal
floor Between Tuberulum Impar
(ant) and copula (post), known as
foramen caecum
• Thyroid precursor (thyroid primordium)
starts as thickening and develops into
thryoid diverticulum. Initially hollow,
later solidifies and becomes bilobed.

- 249 -
3.26 NASOLABIAL CYST

CLINICAL IMAGE

Introductory Answer
question

What is the
• Axial CT soft tissue window image
abnormality in this showing a lesion consistent with a
CT scan? nasolabial cyst (also known as a
Klestadt’s cyst, Non-odontogenic cyst,
epithelial inclusion cyst, fissural cyst).
• Less typical differentials include a
neoplastic lesion (minor salivary gland
tumour); skin appendage lesion,
developmental abnormalities,
odontogenic lesion.

- 250 -
Competency Answer
question(s)

What are the • Generally asymptomatic swelling under


typical features of a alar nasi,unilat nasal blockage; painful
nasolabial cyst? only if infected

How would you • Surgery via a sublabial approach is gold


treat this problem? standard

Advanced Answer
question(s)

What is the • Thought to arise from entrapped


aetiopathogenesis? nasolacrimal tissue or entrapped
nasolabial tissue (hence fissural cyst)

- 251 -
4.27 FREY’S SYNDROME AND BOTOX

CLINICAL
PHOTOGRAPHS

Introductory Answer
question

The patient is
• Starch-iodine test for Frey’s syndrome
awake and asked
• There is a small positive reaction near the

- 252 -
to eat a few right ear lobe suggestive of gustatory
grapes after the sweating.
white material is
applied. What
test is shown in
the diagram?

Competency Answer
question(s)

What is Frey’s • Frey’s syndrome is also known as


syndrome? ‘Gustatory sweating’ and is related to
parotid surgery or injury.
• During surgery, parasympathetic
secretomotor fibres innervate the parotid
gland may be transected. The fibres
regenerate in the skin where they
assume control of sweat gland activity
because of the common
neurotransmitter acetylcholine. This
inappropriate innervation results in facial
sweating in response to salivatory
stimuli.

How is the
condition • Aluminium based deodorant
treated?
• Topical glycopyrrolate
• Botox injection
• Neuronectomy (Jacobson’s nerve section
in the middle ear)

Tell me about • Botulinum toxin (Botox) injection is now

- 253 -
Botox therapy? the gold standard treatment for Frey’s
syndrome. Botox is a protease exotoxin,
which works by blocking the release of
acetylcholine from the cholinergic nerve
end plates leading to inactivity of the
glands or muscle innervated.

Advanced Answer
question(s)

Do you know of • Yes, Botox has a role in multiple non-


any other non- cosmetic head and neck conditions
cosmetic pertaining to larynx (spasmodic
therapeutic use dysphonia), mouth (drooling),
oesophagus (achalasia), face (hemifacial
for Botox, apart
spasm), autonomic nervous system (first
from Frey’s bite syndrome), pain (migraine) and nose
syndrome? (rhinitis)
For an evidence-based review of the use
of Botox in non-cosmetic head and neck
conditions, see Persaud et al. JRSM short
report 2013; Feb 4 (2):10).

- 254 -
3.28 GIANT PLEXIFORM NEUROFIBROMA OF SCALP

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Describe what
• Clinical photograph of a massive lesion
you see in the on the head of a patient most likely to be
photograph benign or a benign tumour that has
become malignant
• Such lesion is consistent with
neurofibroma, most likely plexiform
neurofibroma (involving multiple
peripheriphal nerves) rather than the
dermal type which affects on one
peripheral nerve.

- 255 -
Competency Answer
question(s)

Which condition is • NF1 and NF2, both autosomal dominant


neurofibromas conditions.
associated with?

After having the


• Joint case with a plastic surgeon to cover
lesion for 40 years
the resulting defect with a split skin graft
the patient
decides to have it Surgical Steps
removed. How
would you • The consented patient is anaesthesied
perform the and placed in a supine position with the
procedure? lesion hanging over the northern edge of
the operating table to allow easy access
to the undersurface of the tumour. A
circumferential incision line is drawn
around the lesion and then Local
anaesthesia is injected subcutaneously
(zylocaine (1%) and adrenaline
(1:100,000) .
• The skin is incised with a scalpel initially
and then monopolar diathermy is used.
Haemostasis is achieved with diathermy
and suture ties. Care is taken to locate
and ligate branches of the superficial
temporal arteries, which are the main
blood supply to the tumour.
• Dissection then proceeds in the plane
between the periosteal layer and the
capsule of the tumour. This results in
bloodless dissection and separation of
the tumour from the scalp.

- 256 -
• The scalp is then prepared for the split-
thickness skin graft by checking for any
breach in the periosteal layer. Any
denuded bony area is saucerised with a
cutting bur to create a vascular bed so
that the overlying split skin graft will
remain viable.
• Two separate large split-thickness skin
grafts are harvested from the thigh and
carefully prepared to cover the entire
surgical defect on the scalp.
• The skin grafts are carefully placed on the
scalp, trimmed and secured with clips to
the free skin edge; the two skin grafts
were joined using 4.0 vicryl rapide .
• Gelonet gauze is then applied over the
split skin graft followed by copious
amounts of chloramphenicol ointment .
• Finally blue gauze, mefix adhesive
dressing and a head hooded cap are
added sequentially to further enhance
the pressure dressing over the split-
thickness skin graft.

Advanced Answer
question(s)

Do plexiform • Yes
neurofibromas
• Yes, a parotid tumour weighting 2.5kg
have malignant (reported in ENTNews from Doncaster
potential? ENT Team)

- 257 -
The weight of the
resected
plexiform
neurofibroma was
exactly 3.4576
Kgs. Do you know
the weight of the
largest head and
neck lesion
previously
resected in the
UK?

RESECTED
SPECIMEN

- 258 -
CHAPTER 4

OTOLOGY and NEUROTOLOGY

4.1 ACUTE OTITIS EXTERNA

SCENARIO You are asked to see an 82 year old man with


a 1 month history of an intermittently painful
(no clinical
discharging right ear. The pain has recently
photograph)
become more persistent.

Introductory Answer
question

What is your • Simple Otitis Externa (OE)


differential
• CSOM with perforation
diagnosis?
• Cholesteatoma
• Necrotising Otitis Externa
• Malignancy

Competency Answer
question(s)

How would you • History –Essential to identify if there is a


history of diabetes and treatment

- 259 -
proceed? compliance; water exposure and cotton
bud use; previous ear disease or surgery.
• Examination – Careful otomicroscopy
with microsuction plus take a swab for
culture.
• Investigations – Swab and Pure Tone
Audiogram (PTA) initially. Consider high
resolution CT (HRCT) temporal bones if
suspicious of Necrotising OE (NOE).
• Treatment – Meticulous aural toilet with
regular microsuction is the mainstay of
treatment. Topical antibiotic drops such
as Ciprofloxacin, Sofradex or Gentisone is
the primary treatment, guided by culture
sensitivities. Oral or IV antibiotics are
reserved for cases of NOE.

What is • NOE is skull base osteitis secondary to


Necrotising Otitis uncontrolled otitis externa usually caused
Externa (NOE)? by pseudomonas infection. Typically in
the elderly, diabetic or
immunocompromised patient. A high
level of suspicion is required for prompt
diagnosis and treatment to avoid
complications such as cranial nerve
involvement. It is associated with a
mortality of up to 50%

How can it be • A HRCT temporal bones looking for bony


further erosion or skull base involvement is the
investigated and investigation of choice. Any suspicion of
treated? skull base osteomyelitis can be further
investigated with a Gallium or Nuclear

- 260 -
medicine white cell scan. HBA1C can
delineate poor diabetic control.
• Long term, high dose IV antibiotics are
required, plus very regular aural toilet.
Meticulous diabetic control is essential.
• Surgical debridement of necrotic bone
may be indicated, but is not routine.
• Hyperbaric Oxygen treatment may have a
role, and is currently undergoing trials.

Advanced Answer
question(s)

What are the • Locorten-Vioform = Flumetasone 0.02%,


constituents Clioquinol 1%
Locorten Vioform
• Like ciprofloxacin ear drops, Locorten
and is it safe to
vioform is safe to use if there is a
use when there is
perforation in the tympanic membrane.
a perforation of
the tympanic
membrane?

Do you know the


constituents of • Gentisone HC = Hydrocortisone 1%,
any other ear Gentamicin 0.3%
drops? • Otomize = Dexamethasone 0.1%,
Neomycin 3250 units/mL, Acetic acid 2%
• Sofradex = Dexamethasone 0.05%,
Framycetin 0.5%, Gramicidin 0.005%

- 261 -
4.2 EXOSTOSES AND OSTEOMAS

SCENARIO A 35 year old man presents with history of


recurrent episodes of bilateral otitis externa.
(followed by a
He works as a gardener and enjoys rowing
photograph)
and swimming. He is otherwise fit and well.

Introductory Answer
question

What are the • Water exposure


causes of
• Cotton bud use
recurrent otitis
externa? • Foreign body
• Exostoses

- 262 -
• Skin conditions affecting the canal or
meatus i.e. eczema, psoriasis
• immunosuppression

Competency Answer
question(s)

This is what you • These are typical of ear canal exostoses.


see on otoscopy. It occurs in patients who are regularly
What is this and exposed to cold water and wind. This
how can it be patient’s outdoor occupation and
managed? hobbies put him at risk.
• The exostoses are benign bony
outgrowths of the tympanic ring. They
occlude the canal and may lead to
trapped wax and water becoming
infected. In extreme cases they can even
lead to conductive hearing loss.
• Management includes advising ear
protection from water and wind in mild
cases, to surgical excision in extreme
cases. The exostoses may be removed by
drill or gauge, with careful preservation
of the canal skin.
• Risks to TMJ and facial nerve so pre-op
CT important.

What are the • Exostoses occur medially, along the


differences tympanic ring and are often bilateral.
between They are benign sessile bony outgrowths
exostoses and of cortical bone.
osteoma?
• Osteomata occur at the bony-
cartilagenous junction along suture lines

- 263 -
and are usually unilateral. They are true
neoplasms consisting of cancellous bone.

Advanced Answer
question(s)

Can osteomata of • Yes. Gardner’s syndrome, or familial


the ear canal be colorectal polyposis is an autosomal
genetic? dominant condition associated with
formation of skull osteomata and is a
genetic cause of ear canal osteoma.
Patients may also present with multiple
sebaceous cysts, fibromas and thyroid
cancer. It is caused by a mutation in the
APC gene on Chromosome 5q21.

4.3 PERFORATION OF TYMPANIC MEMBRANE

CLINICAL
PHOTOGRAPH

- 264 -
Introductory Answer
question

Describe the This is an endoscopic photograph of a left


clinical tympanic membrane with an anterior inferior
photograph. central perforation with creamy discharge
(pus) and blood in the deep ear canal.

Competency Answer
question(s)

What are the • The main issue is the increased risk of


issues related to recurrent infection and discharge.
this condition and
• Recurrent discharge can be socially
how would you
embarrassing and problematic.
manage them?
• Mainstay of treatment is protecting the
ear from water to reduce the risk of
infection.
• Water precautions involve avid use of ear
plugs or cotton wool with Vaseline and
avoiding direct water contact to the ear.
• It may be asymptomatic, in which case
nothing other than water precautions are
required.

Advanced Answer
question(s)

In problematic • Careful discussion with the patient


cases, how can should involve the options of
this be treated? conservative versus surgical management
in order to facilitate an informed choice.
• Recurrent infection can lead to fibrosis

- 265 -
and fixation of the ossicles resulting in a
conductive hearing loss and
sensorineural hearing loss can occur long
term, secondary to ototoxic toxins
secreted from the infecting organisms.
• The risks and complications of
Myringoplasty surgery should be
discussed, including hearing loss, taste
disturbance, graft failure, bleeding and
infection.
• This clinical picture may well represent a
complication of grommet insertion, in
view of the position of the perforation.
In this case, myringoplasty may be
complicated by recurrence of effusion,
and this should be addressed.

4.4 TYMPANIC MEMBRANE RETRACTION

SCENARIO A 28 year old man presents with hearing loss.


There is no pain. He had a history of
(clinical
grommet insertion as a child but is otherwise
photograph)
fit and well. A pure tone audiogram reveals
conductive hearing loss with an air-bone gap
of 30dBHL on the left. Tympanometry reveals
a “C-type” trace in the left ear.

- 266 -
Introductory Answer
question

Describe this • This is a clinical otoscopic photograph of


image? a left tympanic membrane with
retractions of both the pars tensa and
pars flaccida. The incudostapedial joint is
clearly visible.

Competency Answer
question(s)

How would you • Retractions of the tympanic membrane


grade this? are graded accordingly with the Sade
system for pars tensa retractions and the
Tos classification for pars flaccida
retractions.
• Sade Classification:
Stage 1 – stretched annulus with a mild

- 267 -
retraction
Stage 2 – Retraction onto incudostapedial
joint
Stage 3 – Retraction onto promontory (lifts on
valsalva)
Stage 4 – Adherent to promontory

What is the risk of


• Tos Classification:
a retraction
pocket? Grade 1 – dimple or mild retraction
Grade 2 – draping over neck of malleus
Grade 3 – distruction of scutum
Grade 4 – deep retraction with keratin
(synonymous with cholesteatoma)

How would you


• Progression with erosion of ossicles and
manage this? development of cholesteatoma.
• This is controversial and it is very
important to know the status of the
other ear.
• The mainstay is close observation,
scrupulous cleaning when necessary and
protection from water/moisture to avoid
infection.
• Debris and keratin build up must be
removed regularly to prevent the
development of cholesteatoma.
• Improve middle ear ventilation to
prevent progression.

- 268 -
- Regular valsalva manoeuvres
- Use of inflation devices such as Otovent
balloon.
- Nasal treatment if signs of
rhinitis/postnasal space oedema
- Grommet insertion in selected patients.
If symptomatic with recurrent
infection/discharge consider reinforcement
Tympanoplasty.

Advanced Answer
question(s)

Describe the • There are 3 layers to the tympanic


embryology and membrane held on a fibrous annulus
structure of the attached to the tympanic ring of the
tympanic temporal bone.
membrane.
• The outer layer is the epidermal
squamous layer (skin). It is formed, along
with the external auditory canal, from
ectoderm of the first branchial cleft.
• The middle fibrous lamina propria layer is
formed from mesoderm. Its structure is
of concentric circles of type II collagen
with radial strands adding reinforcement.
On either side of this stiff layer are loose-
connective tissue layers containing nerve
fibres and a vascular plexus.
• The inner layer is mucosal and is formed,
along with the mucosa of the middle ear,
from the endoderm of the first branchial
pouch.

- 269 -
• The pars flaccida has some fibrous tissue
but it is not as well organised as the layer
in the pars tensa and this results in a lack
of rigidity.

4.5 CHOLESTEATOMA OF THE MIDDLE EAR

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Describe the • This is a clinical otoscopic photograph of


photograph? a left tympanic membrane with a frank
attic cholesteatoma. (The dark
discolouration is due to oxidised keratin).

- 270 -
Competency Answer
question(s)

What is • Cholesteatoma is a collection of


cholesteatoma KERATINISING squamous epithelium
and how is it leading to bone and soft tissue erosion as
caused? well as infection. It is most commonly
found in the middle ear cleft, but can
occur elsewhere such as the external ear
canal.
• There are 4 general types:
- Primary acquired = occurs in a retraction
pocket secondary to negative middle ear
pressure.
- Hyperplasia and migration following
chronic inflammation
- Secondary acquired = migration of
epithelial cells through a perforation, or
implanted via trauma or iatrogenically.
- Congenital = embryonic epithelial rests in
the middle ear with an intact tympanic
membrane (also know as epidermoid
formation or Michael’s body – after the
Otopathologist Leslie Michael who re-
discovered it).

What are the • Cholesteatoma is associated with chronic


issues related to infection and discharge.
this condition? • If left untreated, a cholesteatoma will
slowly progress. This occurs through a
combination of persistent low middle ear
pressure, further migration of epithelial

- 271 -
cells into the cholesteatoma,
proliferation and the release of osteolytic
enzymes.
• Progressive expansion and infection of
the cholesteatoma can lead to
complications. These are subdivided into
otological complications, extra and
intracranial complications.

• Otologic (Intra-temporal) complications;


- Destruction of the ossicular chain
- Hearing loss (conductive and
sensorineural)
- Perilymph fistula (Vertigo)
- Facial nerve damage (Palsy)
- Acute mastoiditis
- Petrositis (Gradenigo syndrome)

• Extra-cranial complications
- External abcesses
- Subperiosteal mastoid abscess
- Bezold Abscess (sternomastoid)
- Citelli Abscess (digastric)
- Lucs Abscess (temporalis)

• Intra-cranial Complications
- Meningitis

- 272 -
- Extradural, Subdural abscesses
- Intracerebral abscess
- Cerebritis/encephalitis
- Lateral/sigmoid sinus thrombosis
- Hydrocephalus

Advanced Answer
question(s)

What do you see Coronal CT showing a small lesion in the


in this CT scan? middle ear. I may represent a
cholesteatoma, granulation tissue or
glomus tympanicum

Describe this
Otoscopic view of the right tympanic
photograph
membrane should an intact drum with a
pearly white lesion in the anterior
superior quadrant. This is likely to
represent a congenital cholesteatoma as
it is the typical position to find such a
lesion.

What do
DWI is Diffusion Weighted Imaging. This
understand by
refers to Diffusion-weighted echo-planar
DWI? Magnetic Resonance Imaging. This can
be used as a pre-operative scanning
modality or as an alternative to second
look in combined approach
tympanoplasty patients. It can
distinguish between inflammatory tissue
and cholesteatoma, where other imaging

- 273 -
modalities cannot.

4.6 OTOSCLEROSIS

SCENARIO GP referral: A 40 year old woman is referred


(followed by an to the ENT clinic with a left sided hearing loss.
audiogram) Examination confirms the tympanic
membranes to be normal in appearance,
whilst audiometry demonstrates a mixed
pattern bilateral loss, worse on the left, with
the predominant component being
conductive.

Introductory Answer
question

What other History: Length of history (any childhood


questions would history); history of previous ear infections;
you ask in the previous ear surgery; history of trauma to the
history to further ear (direct / barotrauma); any noise exposure
determine the history; family history of ear problems; did
possible cause of hearing get worse during any previous
the conductive pregnancy; tinnitus, balance disturbance.
hearing loss?

- 274 -
Competency Answer
question(s)

Look at the • The audiogram shows a conductive


audiogram. hearing in the right ear with a Cahart’s
Considering this notch. In the presence of a type A
and the clinical tympanogram, the most likely diagnosis
history, what is would be otosclerosis.
the likely
diagnosis?

How would you


• Management – history/examination.
manage the
Investigations – stapedial reflexes,
patient?
speech audiogram, high resolution CT
scan temporal bones looking at stapes
footplate. Treatment – conservative with
monitoring, hearing aid, stapedectomy,
fluoride treatment (Not currently
recommended. Fluoride incorporates
into hydroxyapatite. Treatment
monitored by absence of Schwartze’s
(Flamingo blush) sign if present and serial
audiometry. Discuss negatives – stomach
ulceration, arthritis, osteopenia).

• How do you • Footplate classification (Ludman,


classify

- 275 -
variations in originally postulated by Belluci):
the footplate
Type I - stapes minimally fixed in oval
and what is
window
the surgical
significance Type 2 - thin footplate, resists perforation
of this? more than I
Type 3 - thick footplate
Type 4 - fixed to oval window, can only be
perforated by drilling.
Relevance relates to degree of ease of
stapedotomy and possibility of floating
stapes footplate.

Advanced Answer
question(s)

What are the pre- • Contraindications to stapedectomy:


operative and
- Preoperative: only hearing ear;
intra-operative
predominant cochlear component or
contraindications
minimal air bone gap; tinnitus or vertigo
to stapedectomy?
as predominant symptoms; active foci as
judged by positive Schwartz sign; minimal
likelihood of significant improvement in
hearing impairment as judged by
Glasgow Benefit Plot.
- Intraoperative: Incorrect diagnosis;
floating footplate (could try to elevate
but dangerous as risk of permanent
SNHL); low stapedial artery crossing
footplate; perilymph gusher; overhanging
facial nerve
Severe dizziness post stapedectomy: Clinical
assessment first (is it fluctuant),

- 276 -
examination, audiometry, CT scan if
persists, to check position of piston. May
not be related to stapedectomy. If
hearing deficit or fluctuant consider re-
exploration.

4.7 MENIERE’S DISEASE

SCENARIO (no You have been referred a 45 year old lady


clinical with episodic dizziness. She has also
photograph) developed right sided tinnitus. On further
questioning she states that she has been
having difficulty using the telephone with her
right ear recently. She has a history of
migraine. She is otherwise fit and well with
no regular medication.

Introductory Answer
question

What is your • Right-sided Meniere’s disease.


differential
• Right Vestibulopathy.
diagnosis?
• Vestibular Neuronitis.
• Vestibular Migraine.

- 277 -
Competency Answer
question(s)

How would you • History – The most important questions


proceed? are;
- Is this Vertigo?
- What is the duration of the Vertigo?
- Are there any associated symptoms?
• Vertigo is a hallucination of movement
and is the cardinal symptom of vestibular
dysfunction. Other non-specific dizziness
symptoms point to a cardiac or
neurological cause.
• The duration of the vertigo is key;
- Secs to mins = likely Benign Paroxysmal
Positional Vertigo (BPPV).
- Hours = likely Meniere’s, Migraine or
Vestibulopathy
- Days = Vestibular Neuronitis.
- Continuous = central or psychogenic
• Associated hearing loss, increase in
tinnitus intensity and/or an aural fullness
during attack suggests Meniere’s.
Headache associated with the attack
suggests vestibular migraine, as does aura
or parasymathetic symptoms. There is a
significant overlap with Meniere’s and
Migraine with up to 40% of patients
having both conditions simultaneously.

- 278 -
• Examination – Otoscopy for disease,
Cranial nerve examination in central
causes, Romberg and Unterberger tests
looking for proprioceptive and peripheral
vestibular signs, and Dix-Hallpike test for
BPPV. Hamalgi Head-thrust test to elicit
vestibulo-occular reflex (VOR)
abnormalities. Look carefully for
spontaneous and provoked nystagmus.
How would you Examination often normal unless there
treat a diagnosis are concurrent symptoms.
of Meniere’s
disease?
• Investigations –Hearing testing with
tuning fork tests and pure tone
audiogram. MRI of Internal Acoustic
Meati looking for vestibular schwannoma
indicated by unilateral hearing loss and
tinnitus. In equivocal cases or suspected
vestibulopathy, Video or
Electronystagmography (VNG, ENG) and
Caloric testing.

• A stepwise approach is used.


• Simple lifestyle and dietary changes help
in most cases. Reducing Salt, caffeine and
alcohol can help in a large number of
cases.
• Drug therapy with Betahistine and/or
Bendroflumethazide is used effectively in
many patients. Prochlorperazine can be
used when required for symptomatic
relief during the vertigo.
• For those patients uncontrolled with the

- 279 -
above, intratympanic steroid has been
shown to provide some improvement.
• Endolymphatic sac decompression
surgery is effective in some.
• Ultimately, for those resistant or
debilitating cases, labyrinthectomy is
used. This can be either chemical via
intratympanic gentamycin or surgical.
• When Meniere’s is stable, vestibular
rehabilitation is very useful.

Advanced Answer
question(s)

Do you know of • Vestibular-Evoked Myogenic Potentials


any other tests (VEMPs) and Electrocochleography
for Meniere’s (ECochG)
disease?

• VEMP is a test whereby an auditory


Can you explain stimulation (95bD click) passes via the
what these are saccule and inferior vestibular nerve (IVN)
and draw an to the vestibular nucleus. A reflex arc
ECochG trace? then stimulates the ipsilateral
sternocleidomastoid (SCM) muscle via the
vestibulospinal tract. This, detected by
an action potential in the SCM is delayed
in Meniere’s Disease, along with any IVN
lesion such as vestibular schwannoma, or
saccular dysfunction. In superior
semicircular canal dehiscence the
potential is increased.
• ECochG is the recording of electrical
potentials in the auditory nerve and

- 280 -
cochlea in response to auditory clicks via
air conduction. A probe is placed on or
near the tympanic membrane with a
reference electrode on the mastoid and
forehead. It can detect Endolymphatic
hydrops in Meniere’s by an increase in
the summation potential/Compound
Action Potential ratio.

It is essentially an ‘ECG’ of inner ear function


(Outer Hair Cells (OHCs), Basilar membrane &
Vestibulocochlear nerve (CN8))
Abnormal in hydrops.
Transtympanic TT vs extratympanic ET active
electode (TT response 10 times larger)
Click stimulus (traditional) but short tone
bursts give better results; 1kHz tone burst
provides the best indication of hydrops.
The three section of the ECochG are:
1. Cochlear microphonics - reflects OHCs

- 281 -
function.
2. Summation Potential (SP) - basilar
membrane ‘set point’.
3. Action Potential (AP) - reflects CN8.

The ratio of amplitude of SP/AP is used for


measurement in disease;
Generally abnormal if ratio >40% (range 34-
47%) (published studies).

4.8 BENIGN PAROXSYMAL POSITIONAL VERTIGO

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Describe what is This is a clinical photograph of a patient

- 282 -
being done in the undergoing a particle repositioning
photograph. manoeuvre such as Epley.

Competency Answer
question(s)

What is Dix- • The Dix-Hallpike test is diagnostic of


Hallpike test? Benign Positional Paroxysmal Vertigo
(BPPV). There is usually a short latency
of around 2-10 secs after which a positive
test shows a torsional nystagmus, usually
towards the test ear, or the floor
(Geotropic) (or other nystagmus types in
rare cases – see below)
• BPPV is the most common peripheral
vestibular condition presenting in
balance clinics.
• It is characterised by short-lived episodes
of vertigo lasting seconds to minutes at a
time and provoked by changes in head
position. The most common provoking
positions are lying down and turning in
bed, looking up and bending down.
• In pure BPPV there are no other
otological symptoms such as hearing loss
or tinnitus, although they may co-exist.
What is the • It often occurs spontaneously, but other
pathophysiology aetiological factors include head injury,
of BPPV? post-viral (vestibular neuronitis), post-
surgical.
• It is often self-limiting.

• There are two theories of

- 283 -
pathophysiology.
- Canalithiasis. Dislodged otoconia from
the saccule/utricle float freely in the
endolymph of the semicircular canal. The
inertial effect of their movement in the
fluid causes cupula displacement and
short-lived vertigo which resolves when
the otoconia come to rest.
- Cupulolithiasis. Loose otoconia become
adherent to the cupula causing
stimulation due to gravity on movement.
Treatment of BPPV following a positive Dix-
Hallpike test is with a particle repositioning
manoeuvre such as the Epley.

Advanced Answer
question(s)

Are you aware of • Yes, there are 3 types. The most


the different common, accounting for up to 90% is
types of BPPV? Posterior canal BPPV. Lateral, or
Horizontal BPPV accounts for over 8%
and Superior canal BPPV is extremely
rare, accounting for less than 1% of
cases.
• Lateral canal BPPV tends to present with
more severe and prolonged symptoms.

How do these
types differ • Lateral canal BPPV elicits a horizontal
clinically? nystagmus on Dix-Hallpike test rather
than a torsional nystagmus. It may be
Geotropic (75%) or Ageotropic (25%).

- 284 -
The supine roll test (patient supine with
head rolled to side) is an alternative to
Dix-Hallpike for this. Treatment requires
a Barbecue 360° roll manoeuvre.
• Superior canal BPPV is very rare due to
the position of the canal.
• Superior canal BPPV exhibits a
downbeating nystagmus on Dix-Hallpike
testing without a torsional element.
• All episodes of downbeating nystagmus
should be investigated as a central cause
for this is more common than a superior
canal BPPV.

Is there a surgical • Yes. Semicircular canal occlusion can be


treatment for performed in intractable or disabling
BPPV? cases. It involves access to the canals via
a cortical Mastoidectomy approach and
plugging the affected canal with bone
pate, muscle and fascia.

4.9 TEMPORAL BONE FRACTURE

SCENARIO A 35 year old man presents in the Emergency


Department (ED) having fallen from his
(no clinical
bicycle. He lost consciousness for 5 minutes.
photograph)
ED have cleared him from a head injury point
of view, but have referred him as he is

- 285 -
complaining of bleeding and hearing loss in
his left ear.

Introductory Answer
question

What is your There has clearly been some ear trauma here,
differential but it is important to determine if it is
diagnosis? simple blunt trauma or a bony fracture.

Competency Answer
question(s)

How can you • Hearing loss may be conductive


determine the secondary to haemotympanum or
difference? ossicular disruption, or sensorineural
secondary cochlea injury in fracture.
• Bleeding may be caused by a traumatic
perforation or a fracture that passes into
the ear canal.
• Careful examination should include facial
nerve assessment and looking for signs
such as nystagmus and CSF leak.
• Audiogram must be performed if there is
a history of hearing loss or it is identified
by tuning fork tests.
• Tympanogram is contraindicated as it
may provoke a pneumocephalus.
• A CT scan should be performed if
fracture is suspected. Angiogram if

- 286 -
carotid injury is suspected, or where the
fracture involves the foramen lacerum.

This image shows a longitudinal fracture as


This is the CT
well as a transverse fracture of the
scan. What does
petrous temporal bone with a
it show?
pneumolabyrinth. This patient will have
an irreversible dead ear.

Advanced Answer
question(s)

What are the • Longitudinal are more common than


differences transverse fractures by 75% to 25%
between They tend to occur by a lateral blow
longitudinal and whereas transverse fractures are caused
transverse by fronto-occipital force.
fractures?
• Longitudinal fractures rarely cause
cochlea injury whereas a transverse
fracture is associated with cochlea and
IAC injury.
• Longitudinal is associated with
conductive hearing loss due to
haemotympanum or ossicular
discontinuity. Transverse is associated
with sensorineural loss due to cochlea or
IAC injury.
• Both types of fracture can result in
haemotympanum, but longitudinal

- 287 -
fractures are more often associated with
laceration through the tympanic
membrane and external canal fracture
therefore bleeding from the ear.
• Facial nerve injury is far more likely from
transverse fractures.
• Delayed nerve palsy may occur due to
oedema and ischaemia of the nerve.

How else may


temporal bone • Temporal bone fracture with or without
fracture be immediate facial nerve palsy.
classified?

How can one • The presence of Beta-2-transferrin which


determine if a is ONLY found in CSF and endolymph.
clear fluid leak
from the ear is
CSF?

- 288 -
4.10 NOISE-INDUCED HEARING LOSS

SCENARIO You are asked to see a 32yrs-old who


complains of a blocked ear following years of
(and audiogram)
attending rock concerts. The pure tone
audiogram reveals the following:

Introductory Answer
question

Please describe Normal Left and right air conduction


what the PTA thresholds with a 60dB dip at 4000Hz
shows. followed by a partial recover.

- 289 -
Competency Answer
question(s)

What is the likely • This pattern of SNHL with loss at 3-6KHz


diagnosis and and recovery at 8KHz is in keeping with
how would you noise induced hearing loss. A formal
manage this diagnosis requires an awareness of noise
exposure from other sources reference
patient?
to age standardised normograms for
hearing thresholds.
• Management is largely preventative for
future noise exposure with ear plugs (10-
15dbl attenuation) and ear muffs (15-
20dbl attenuation).
• Co-existing tinnitus and hyperacusis may
need to be managed with counselling if
appropriate.

Advanced Answer
question(s)

What is the • Outer hair cells and then inner hair cell
mechanism of loss via apoptotic and necrotic
noise induced mechanisms resulting from oxidative
hearing loss? stress and excito-toxicity
• Loss is potentiated by concomitant use of
How could you
ototoxic medication or genetic
develop a predisposition.
strategy to treat
• 4KHz may be preferentially degraded as
this condition?
the resonant frequency of the Organ of
Corti.
If the patient presents early enough <72hrs
empirical laboratory data suggest that there

- 290 -
may be a role for drugs to prevent the
production of free radicals and the calcium
led apoptotic pathway via steroids and
calcineurin antagonists.

4.11 NON-ORGANIC HEARING LOSS

SCENARIO GP referral:
(Audiograms) A 55 year old patient is referred to the ENT
clinic from the direct access audiology service
with an asymmetric hearing loss

Introductory Answer
question

• Look at the • The right ear shows unmasked air


audiogram conduction thresholds which when
and describe averaged is just within the normal range.
the
• Unmasked bone conduction is reduced to
abnormalities
60dBHL average.
.
• The left ear shows a mixed hearing loss
with masked bone conduction at around
70dBHL and masked air conduction at
around 90dBHL.

- 291 -
Competency Answer
question(s)

What is the most • Reduced unmasked BC raises the


likely diagnosis? suspicion of a non organic hearing loss,
as even in the presence of a hearing loss
in one ear, unmasked BC thresholds
should be heard in the contralateral
(good) ear.

Advanced Answer
question(s)

Which other tests • Subjective:


would you
- Stengers tuning fork test: Based on the
consider to
Stenger’s phenomenon in which only the
confirm your
louder of two similar tones presented to
suspicion that the
both ears at the same time will be heard.
hearing loss is
non-organic? - Chimani-Moos: the patient does not
accept that they hear Weber’s better in
occluded ear; Lombards (raise voice level
in response to noisy environment);
delayed speech feedback test;
• Objective: Maintenance of cochlea-
auricular palpebral reflexes; ABRs, CERA
(gold standard).

• Also suspect if voice normal (only if


bilateral non-organic), if gross
What test is being discrepancy between pure tone and
speech audiometry.
carried out here?
• Stenger’s test is been demonstrated.

- 292 -
Table 5.11a Tuning fork tests for sorting out the malingerer

TUNING FORK DESCRIPTION


TESTS

Stenger test • Principle


(Stenger 1900)
– Sound presented in both ears will only be
heard in the ear where the sound is
louder
• Test
– 2 tuning forks of same frequency used
– Eyes closed/blindfolded
– 1st tuning fork to good ear at 15cm
(heard), then removed
– 2nd tuning fork to deaf ear at 5cm (not
heard), not removed
– Re-introduce 1st tuning fork to good ear
at 15cm (heard if not a malingerer)
• Malingerer Response
– Denys hearing any sound as can only hear
sound in feigned ‘deaf’ ear

Teal test • Principle


– Conductive hearing
• Test
– Vibrating tuning fork to mastoid of ‘deaf’
ear (heard)
– Patient blind-folded and advised test to
be repeated
– Non-vibrating tuning fork to ‘deaf’ ear

- 293 -
and simultaneously vibrating tuning fork
near EAC of same ear (not heard if not a
malingerer)
• Malingerer Response
– Will claim to hear sound as will assume
through bone conduction when it is
actually through air conduction

Chimani Moos • Principle


test
– Variation of Weber test
• Test
– Tuning fork place on vertex
– Heard in good ear
– EAC occluded in good ear (heard in good
ear still)
• Malingerer Response
– Deny hearing any sound at all

- 294 -
Table 5.11b Voice tests for sorting out the malingerer (by Julian
Gaskin, FRCS (ORL-HNS).

VOICE TESTS DESCRIPTION

Lombard • Principle
– Person will raise voice in speaking in
noisy environment
• Test
– Patient asked to read text aloud without
stopping
– Noise introduced into good ear (i.e.
Barany box)
– Patient’s voice will get louder
• Malingerer Response
– Will continue to read at even tone

Hummel double • Principle


conservation test
– 2 different voices introduced into either
ear will create confusion
• Test
– A question is asked into one ear and a
different question asked into the other
– If deaf in one ear there will be no
confusion
• Malingerer Response
– Confusion or no response

- 295 -
Erhardt test (loud • Principle
voice test)
– Occlusion of the EAC causes attenuation
of 30dB
• Test
– Good ear is occluded with a finger
– Sound projected into that ear
– Normally, the patient will be able to hear
the dampened sound
• Malingerer Response
– Denies hearing any sound at all, even
when sound increased

Delayed speech • Principle


feedback test
– Delayed sound introduced into the other
ear will create confusion
• Test
– Patient asked to read text aloud without
stopping
– His voice is recorded and played back via
headphones with 2ms delay into deaf ear
• Malingerer Response
– Confusion, change reading pattern
(stammer, slow, raise voice)

- 296 -
4.12 DRUG-INDUCED HEARING LOSS

SCENARIO A 64 year old man with pulmonary


(followed by tuberculosis (TB) had a relapse despite a four
PTAs) month course of high dose anti-tuberculous
drugs. He was started on Amikacin, an
aminoglycoside antibiotic which is used in
multidrug-resistant TB. Two months into
treatment, he complained of a mild
deterioration in the hearing in his left ear.
There are no other otological symptoms.

Introductory Answer
question

Describe this PTA • This is a pure tone audiogram with the X-


axis representing increasing frequency of
pure tones and the Y-axis representing
hearing level in Decibels (dB).
• Only unmasked air conduction is
recorded in the right ear (represented by
an open circle) and left ear (represented
by a cross).
• It shows hearing, which on average is
within normal limits, however, there is a
slight difference between the two sides
with a mild low-frequency loss in the
right ear and a mild high-frequency loss
in the left ear.

- 297 -
Competency Answer
question(s)

Describe this • This PTA records both air conduction in


change in PTA each ear and unmasked bone conduction
in the right ear.
• The traces recorded show a bilateral
high-frequency sensorineural hearing
loss.

What is the • Given the history of the prolonged use of


diagnosis? an aminoglycoside antibiotic, and the
PTA, a diagnosis of drug-induced ototoxic
hearing loss is most probable. Certain
aminoglycosides such as Amikacin have
narrow therapeutic windows and as such
too high a dose can lead to ototoxicity.
• Ototoxicity is defined as a greater than
10dB reduction in hearing at one or more
frequency bilaterally.
• It most commonly affects high
frequencies initially, progressing to
involve the lower frequencies.

How would you • Immediately stop the offending drug if


treat ototoxicity? the clinical condition permits. Consider
alternative medication. In the vast
majority of cases, once the ototoxic drug
is withdrawn, the hearing will return to
normal within a few days. However, in
some cases there may be a permanent
deficit.

- 298 -
• It is important to exclude other causes of
sensorineural hearing loss in these
patients, and investigate accordingly.
• Amplification may be required to
improve the hearing. When profound or
total loss occurs, cochlea implantation
can be considered.

Advanced Answer
question(s)

What other drugs • Ototoxicity is common with


can cause aminoglycoside antibiotics, along with
ototoxicity and other drugs such as macrolides, loop
how does it diuretics, salicylates, quinine, heavy
occur? metals and chemotherapeutic agents.
• The drugs leach into the endolymph of
the inner ear and cause direct damage to
the outer hair cells initially and later the
inner hair cells. Damage can also occur to
the vestibular apparatus.
• The hair cell damage is thought to occur
as the result of free-radical damage from
reactive oxygen species as a result of the
drug action.

- 299 -
4.13 BONE ANCHORED HEARING AID (BAHA)

CLINICAL
PHOTOGRAPH

Introductory Answer
question

What is the name • Trans-cutaneous abutment for a bone


of device? anchored hearing aid (BAHA).

Competency Answer
question(s)

What are the Two indications


main indications
• Primary group: patients unable to
for this device?
benefit from air conduction hearing aid
due to: persistently discharging ear, canal
stenosis, microtia. Best suited to
conductive loss with adequate bone
conduction thresholds. Processor power

- 300 -
prescribed according to bone conduction
thresholds (e.g. <35dBHL Divino,
<45dBHL Intenso, <55dBHL Cordelle).

What are the • Secondary group is patients with


main profound unilateral hearing loss with
complications of adequate contra-lateral bone conduction
the BAHA and thresholds e.g. following vestibular
how might they schwannoma resection. The device
be overcome? reduces the head shadow effect,
improves sound localisation and speech
in noise discrimination.

• NB Surgery in adults is typically


performed as a single stage procedure
with device loading 4 weeks post
implantation.

• Soft tissue overgrowth / local


inflammation and peri-implant pain.
• Historically they have been overcome by
complete soft tissue reduction allowing
skin to adhere to bone at the point of
implant interface.
• Recent advances include the
development of longer abutments which
do not require soft tissue reduction as
well as abutments coated with
osteoneogenic hydroxyapatite to
encourage tissue adherence.
• Skin punch implantation may also reduce
the likelihood for local chronic pain due

- 301 -
to sparing of occipital nerve branches
during surgery.

Advanced Answer
question(s)

What are the • DIVINO < 35dBHL


different types of
• INTENSO < 45dBHL
BAHA available
and their BC • CORDELLE < 55dBHL
thresholds?
• BODY WORN < 55 dBHL
• Speech Discrimination < 60% in general

How does the


saphano works? • Implantable magnets used instead of a
transcutaneous titanium screw.

- 302 -
4.14 COCHLEAR IMPLANT

CLINICAL
PHOTOGRAPHS

Introductory Answer
question

What is this • Cochlear implant (CI) receiver and


device? stimulator package.

- 303 -
Competency Answer
question(s)

Who are the • The traditional candidate groups are:


traditional
1. Pre-lingually deafened children who are
candidate groups
offered simultaneous bilateral implants
for this device
and what are 2. Post-lingually deafened adults are
their candidacy offered unilateral implants unless visually
criteria? impaired.
• Adult candidacy criteria is bilateral
severe to profound hearing loss
( 90dBHL at 2 and 4 KHz) who do not
receive adequate benefit from acoustic
hearing aids (<50% Bamford-Kowal-
Bench sentence scores at 70dBSPL)
• Child candidacy criteria is bilateral
severe to profound hearing loss
( 90dBHL at 2 and 4 KHz) whose speech,
language or listening skills have not
developed or are not maintained at a
level appropriate to developmental age
and cognitive ability of the child.
• Both groups should be assessed by CI
MDT groups and have a 3month trial of
acoustic hearing aids (unless contra-
indicated or inappropriate)
• Investigative work-up includes CT scan to
exclude cochlear obliteration and MR
scan to ensure the presence of an
acoustic nerve in children.

- 304 -
Advanced Answer
question(s)

What is electro- • It is the process of stimulating the


acoustic cochlear with both electrical stimulation
stimulation and via the implant electrode and also by an
what is its role in acoustic hearing aid to the same device,
cochlear in separate devices, or by a hybrid
implantation? processor.
• It is possible in patients with significant
residual low frequency hearing with
thresholds under 65dBHL at 250 and
500Hz. This residual capacity may be
preserved by ‘soft surgical techniques’
including round window electrode
insertion, topical corticosteroid and new
electrode design.
• Early studies suggest that patients obtain
greater benefit in the speech in noise
scenario and a greater appreciation for
music.

4.15 CHRONIC OTITIS EXTERNA

SCENARIO GP referral:
(no clinical Please could you see this 66 year old lady
photograph) with a long history of itchy ears, associated
with ear discharge and a reduction in hearing.
She has had a number of courses of

- 305 -
antibiotics which offer only temporary relief.

Introductory Answer
question

What are the risk • Risk factors – skin conditions (eczema,


factors for psoriasis), instrumentation (cotton buds),
developing otitis trauma, water contact, occupation (dust
externa? eg. building site, musicians wearing ear
plugs), secondary to middle ear disease
(CSOM, perforated TM), foreign body,
diabetes, malignancy.

Competency Answer
question(s)

On looking in the • Say at outset that the combination of OE


ear you notice a in a 66 year old with granulations in ear
very narrow ear canal make you somewhat suspicious and
canal with this merits careful attention.
infected debris.
• Complete the examination to include
On neck palpation for lymphadenopathy and
microsuctioning cranial nerve examination.
you see some
granulations in • Investigations: ear swab, possible CT
the outer half of scan +/- MRI skull base depending on full
the ear canal and clinical assessment.
you cannot see • Treatment:
the ear drum.
How would you Initiate topical treatment for OE (Keep ears
proceed? dry, consider antibiotic holiday if multiple
recent courses, consider fungal infection
and antifungal treatment. Regular follow
up to clean ear until canal is patent and

- 306 -
can see TM). However do not assume
that this is the only problem there.
- Consider malignant OE – admit,
CT/MRI/WCC scan, iv antibiotics,
hyperbaric oxygen, debridement, DM
control
- May need biopsy to exclude SCC

Advanced Answer
question(s)

Do biofilms play a • Biofilms: dynamic structured network of


role in chronic microorganisms suspended within an
OE? extracellular matrix. Promotes antibiotic
resistance and reduced antibiotic
penetration due to elevation of minimum
inhibitory concentrations to levels in
excess of what can be delivered
systemically. Topical antibiotic drops are
delivered at higher concentrations locally
but the polymeric external matrix of the
biofilm reduces to some extent (but does
not eliminate) the absorption of the
antibiotic. Also the presence of beta-
lactamase-producing strains of
Pseudomonas aeruginosa increases in the
biofilm state. Some biofilms also develop
drug efflux pumps and this has been
shown for ofloxacin. Super-resistant
strains of bacteria have also been
identified within the biofilm.

What is the role


for surgery in OE? Hence these factors predispose to the
persistence of chronic OE.

- 307 -
Surgery in OE:
For suction clearance under GA in children;
for biopsy of suspicious granulations in
ear canal to exclude SCC, or deeper lying
polyps arising from middle ear or attic;
consider bony and/or cartilaginous
meatoplasty if canal very narrow (in
some cases lining with a split skin graft
may be necessary); in malignant OE may
need surgical debridement if medical
therapies ineffective and/or
complications such as facial palsy exist.

4.16 VESTIBULAR SCHWANNOMA (ACOUSTIC NEUROMA)

SCENARIO A 64 year old man presents with 6


month history of unilateral RIGHT sided
(followed by a clinical
high frequency tinnitus of increasing
photograph)
intensity. Recently, he developed severe
hearing loss in his right ear. He is
otherwise fit and well. On examination
he complains of discomfort on insertion
of the otoscope into his right ear, but the
examination is normal.

Introductory Answer
question

- 308 -
Other than a Pure • Magnetic Resonance Imaging of his
Tone Audiogram Internal Acoustic Meatus (MRI CPA/
(PTA), what single IAM). This is required to exclude a
investigation possible Cerebellopontine angle (CPA)
would you lesion in any patient who presents with
organise this unilateral or asymmetric audiological
patient? symptoms, such as unilateral tinnitus.

Competency Answer
question(s)

This is a selected • This is a coronal T1 weighted MRI scan


image from his with contrast through the level of the
MRI IAM series. internal acoustic meati with gadolinium
What does it enhancement. On the right side is a
show? lesion with high and low signal in the
cerebellar pontine angle. The lesion is
compressing the pons. It is consistent
with a vestibular schwannoma, which
may have had a recent bleed into its
substance, hence the sudden nature of
the hearing loss.

What is a • It is a benign encapsulated tumour of the


vestibular Schwann cells of the vestibular nerve. It
schwannoma and accounts for 85% of all CPA lesions. They
can you tell me a grow very slowly but are associated with
little more about a very rare malignant potential of less
this condition? than 1% Vestibular schwannnomas may
be solid or cytic.
• 95% are sporadic and unilateral and 5%
are bilateral and associated with
Neurofibromatosis Type 2, an autosomal

- 309 -
dominant condition presenting with
other intracranial and spinal lesions.
• There are two histological subtypes;
Antoni types A and B based on the
uniformity of the cells.
• They present classically with an
asymmetric sensorineural hearing loss,
unilateral tinnitus, vertigo and
vestibulopathy, and facial weakness.
Rarely they can present with central
signs.

• They can be treated by watchful waiting


with serial scanning, stereotactic
radiotherapy or surgery. Each case
would usually be managed through a
skull base multidisciplinary team.

Advanced Answer
question(s)

The patient • This is a positive Hitselberger’s sign. This


complained of is an alteration of sensation to the
discomfort on posterior external auditory meatus or ear
otoscopy in the canal presenting with, discomfort, pain,
left ear. What numbness or paraesthesia of this area of
could this skin. It is caused by a CPA lesion
represent? compressing, but not impinging on the
facial nerve.

Aside from PTA • Speech audiogram showing rollover – a


and MRI, are you phenomenon where speech
aware of any discrimination worsens with increasing
other sound intensity.

- 310 -
investigating • Stapedial reflex decay – an inability to
modalities that maintain the stapedial reflex for 10
can be used for seconds in a sustained stimulus at 10dB
diagnosis (Historic HL.
examples
• Asymmetric caloric responses without
accepted)?
any symptoms of vertigo.
• Auditory Brainstem Response (ABR) –
abnormal wave V latancy between ears
or abnormal interaural wave I-V and III-V
latency difference.

4.17RAMSAY HUNT SYNDROME

SCENARIO GP referral:
(with a clinical Please could you kindly review this 69 year
photograph) old man who noticed a sudden weakness of
his face.

- 311 -
Introductory Answer
question

What are the key • History: sudden or gradually progressive;


points to be any other associated neurological
covered in the symptoms (speech impediment, arm/leg
assessment of a weakness); any associated vesicles;
patient with a recent URTI or trauma or surgery; past
facial weakness? history of facial weakness.
• Examination: Lower Motor Neurone
(LMN) or Upper Motor Neurone (UMN)
weakness (forehead sparing or not); brief
neurological assessment; full cranial
nerve examination; assessment of grade
of facial weakness; examination of neck
including parotid glands; examination of
ears including tympanic membranes.

Competency Answer
question(s)

Assuming you • Grading:


have diagnosed a
• House-Brackmann (I - VI) is probably the
lower motor
most commonly used
neurone facial
weakness, what • Sunderland, (1st - 5th degree), Seddon
grading systems (neuropraxia, axonotmesis, neurotmesis),
do you know? Sunnybrook, Yanahigara, Sydney,
Describe the Nottingham etc.
Sunderland • Sunderland System;
system.
- Conduction block only correlates with
Sunderland (1st degree) and Seddon
(neuropraxia)

- 312 -
- Transection with intact endoneurium:
Sunderland (2nd degree) and Seddon
(axonotmesis)
- Transection with intact perineurium:
Sunderland (3rd degree) and Seddon
(neurotmesis)
- Above + disruption of perineurium:
Sunderland (4th degree) and Seddon
(neurotmesis)
- Above + epineurium remains intact:
Sunderland (5th degree) and Seddon
(neurotmesis)

What
• Investigations: Audiogram; CT temporal
investigations
bones (either routinely or if abnormality
would you
on otoscopy); MRI neck/parotids if neck
undertake?
lump palpable; nerve conduction studies
(not universally available).

What is the • Evidence for treatments: Scottish Bells


evidence for the Palsy study (NEJM, 2007) – early
use of steroids treatment with prednisolone is effective,
and antivirals in whilst treatment with acyclovir is not.
the management Likewise Cochrane review showed no
of a LMN facial benefit (2008). Various studies suggest
palsy? that valciclovir may be more effective
than acyclovir in combination with
prednisolone.

- 313 -
Advanced Answer
question(s)

What surgical Surgery


treatment is
• If >90% axonal degeneration on nerve
available for facial
conduction studies.
paralysis and
when is it • Approach depends on which part of the
appropriate? facial nerve needs to be accessed and
decompressed. Most likely site of surgical
injury is 2nd genu but compressive effects
of oedema most likely to be seen in
labyrinthine and/or tympanic segments.
Clinical assessment can guide to some
extent the site of the injury – the
presence of taste disturbance (chorda
involvement), hyperacusis (stapedius
involvement) and/or lacrimation (Greater
Superficial Petrosal Nerve involvement)
may determine whether a middle fossa
approach is required for a predominant
labyrinthine injury (although beware as
hyperacusis is not thought to be a
universally reliable marker of stapedius
involvement as in some patients with
hyperacusis, the contralateral stapedial
reflex is still present). However Fisch
(1985) reported good results with a
combined middle fossa approach and
mastoid decompression of the facial
nerve in 11 out of 12 patients with Bells
Palsy.
Facial reanimation (static vs dynamic).
• Static – gold weights, tarsorraphy, brow

- 314 -
lifts, static slings.
• Dynamic – temporalis transfer, XII-VII
anastomosis.

4.18 MASTOIDECTOMY FISTULA

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Describe what • This is a clinical photograph of a patient


you see in the with a posterior auricular mastoid fistula.
photograph There is a clean, dry opening through the
skin of the left post-auricular region. This
is likely to communicate with the left
mastoid antrum.

- 315 -
Competency Answer
question(s)

How is this • This is a rare complication of mastoid


situation likely to surgery.
have occurred?
• When a cortical Mastoidectomy has been
performed and the skin wound closed, it
is likely that there has been a wound
dehiscence. This can be caused by
haematoma, infection, inflammation or
poor suturing technique.
• It is more likely to occur in diabetic
patients and smokers due to poor tissue
healing.
• Granulations growing through the skin
wound can encourage a sinus to form.

Scar, Bleeding, Infection, Ear Discharge,


What are the Packs, Hearing Loss, Dead Ear, Alteration
other of Taste sensation, Facial paralysis
complications of (Temporary or permanent),
mastoid surgery? Vertigo/dizziness, Tinnitus, Need for
regular cleaning and water precaution,
Recurrence.

Advanced Answer
question(s)

How would you • Fistulas/Sinuses in this region are


manage this? notoriously difficult to treat. In order to
close this, a simple primary closure is
unlikely to suffice. There is more likely to
be a successful closure with a full

- 316 -
thickness advancement or rotation flap
from the adjacent occipital skin.

4.19 HIGH JUGULAR BULB

SCENARIO A 38 year old man presents with a conductive


hearing loss in his right ear. He was seen by
(followed by a
the SHO in clinic and listed for a grommet
clinical
insertion. Your registrar calls you to theatre
photograph)
during the procedure as he thinks the
tympanic membrane looks abnormal. There
is a 40dB conductive hearing loss and a type B
tympanogram.

- 317 -
Introductory Answer
question

Describe this • This is a clinical otoscopic photograph of


image? a right tympanic membrane with the
appearance of dark mass in the middle
ear. This may represent a
haemotympanum.

Competency Answer
question(s)

There is no • This appearance of blood behind the


history of trauma. drum in the absence of trauma suggests
What else could a high jugular bulb or dehiscent jugular
this be? bulb.

What would your


advice be?
• Do not proceed with myringotomy and
grommet insertion. This patient requires
further investigation such as a contrast
high resolution CT scan of the temporal
bones. Treatment of the conductive
hearing loss should initially be offered
with a hearing aid.

Advanced Answer
question(s)

• What causes • A high-riding jugular bulb occurs when


this there is a congenital anomaly of the floor
appearance? of the middle ear cleft, or a dehiscence in
the floor.
• The jugular bulb is the confluence of the

- 318 -
lateral venous sinuses situated in the
jugular fossa. It ultimately drains into the
internal jugular vein passing through the
jugular foramen.
• At least half of cases are asymptomatic
and are diagnosed only on careful
otoscopy.
• Symptomatic cases may present with
conductive hearing loss, pulsatile tinnitus
or rarely with facial palsy or vertigo
secondary to erosion into the facial canal,
vestibular aqueduct or posterior
semicircular canal.

Had your registrar • Uncontrolled bleeding from a punctured


performed a jugular bulb is not uncommon as a
myringotomy and presenting feature. The first manoeuvre
called you for would be to raise the head of the bed
uncontrolled whilst applying direct pressure to the
bleeding, what bleeding tympanic membrane. The
would you affected ear should then be tightly
advise? packed with BIPP ribbon, adrenaline
soaked ribbon gauze or gelfoam. The
surgery should be abandoned.
• If the bleeding is not controlled enlist the
help of an interventional radiologist to
attempt intravascular therapeutic
embolisation.

- 319 -
4.20 GLOMUS TYMPANICUM

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Describe what • This is a clinical otoscopic photograph of


you see in the a left tympanic membrane with a pink
photograph. lesion occupying the lower middle ear
space.

Competency Answer
question(s)

The patient • This is likely to be a Glomus Tympanicum


presents with a
pulsatile tinnitus.
What is your • A Glomus tumour is a neuroendocrine
diagnosis? neoplasm arising from neural crest cells
of the parasympathetic nerves. They are
also known as Paragangliomas or
What is a Glomus chemodectomas. They include Glomus

- 320 -
tumour? Tympanicum, Glomus Jugulare, Glomus
Vagale and Carotid Body Tumour.
• They are highly vascular tumours
supplied by the ascending pharyngeal
artery – a branch of the external carotid
artery.
• Glomus tympanicum presents with
pulsatile tinnitus and if extensive, may
present with the effects of local erosion
from the tumour. Other symptoms may
include otalgia, hearing loss, vertigo and
facial palsy.

• In Glomus tympanicum, increased


What are the pressure on the tympanic membrane
clinical signs with pneumatic otoscopy will cause
found in Glomus blanching of the tumour (Brown’s sign).
tympanicum? Pressure over the ipsilateral carotid
artery will also cause blanching (Aquino’s
sign).
• A CT scan can be used to assess size and
bony erosion. An MRI scan is required to
assess any intracranial extent of the
lesion and classically shows a “salt and
pepper” appearance on T2-weighted
images secondary to the varying levels of
blood flow. Angiograms may also be
employed and can be both diagnostic and
therapeutic if embolisation is used.

How would you


investigate this • Urine and plasma catecholamines are
condition? measured to assess any concurrent
pheochromocytomas.

- 321 -
• 10% are multiple and are seen in Multiple
Endocrine Neoplasia (MEN) type 2
syndrome.

• Yes, there is the Fisch and the Glasscock-


Jackson systems.
• Fisch
- Type A – localised to middle ear
- Type B – into mastoid (no bone erosion)
- Type C – invades bone
Are you aware of - Type D – intracranial extension
any classification
systems for • Glasscock-Jackson (Tympanicum)
paragangliomas? - Type 1 – limited to promontary
- Type 2 – filling middle ear
- Type 3 – extending into mastoid
- Type 4 – extending into EAC or carotid
• Glasscock-Jackson (Jugulare)
- Type 1 – involves jugular bulb, middle ear
or mastoid
- Type 2 – extending under IAC
with/without intracranial extension
- Type 3 – extending into petrous apex
with/without intracranial extension
- Type 4 – into clivis, infratemporal fossa,
intracranial.

- 322 -
Advanced Answer
question(s)

How would you • Surgical excision can be achieved through


treat this patient? a Tympanotomy using a laser for small
lesions (Fisch Type A). Larger lesions may
require transmastoid or infratemporal
fossa approaches.
• ENT surgeons operate on Fisch A-C with
joint neuro-otologist for Fisch D. The
intra-temporal fossa and translabyrithine
approaches to the petrous apex do not
require neuro-surgical input buth
intracranial approaches do.
• Pre-operative embolisation is often
employed.

Why are glomus • Because they secrete neuroendocrine


tumours so pro-angiotic factors and enymes. They
vascular? arise from angiotic neuroendocrine
chemoreceptor cells which are associated
with very vascular endothelium.
• This is why pre-operative embolisation of
the ascending pharyngeal artery may be
appropriate in some cases.

Do they have a
malignant • YES, in general paraganglomias have a 3-
potential? 4 percent malignant potential. For
temporal bone glomus, the figure is
around 1 percent.

- 323 -
4.21 MENINGIOMA

Clinical
photograph

Introductory Answer
question

Describe the • This is a MRI of the brain and CP angle,


clinical image. coronal view. There is a huge broad-
based mass in the left CP angle that is
enhancing on T1 weighted image, post
What is your Gadolinium contrast. Dural tail sign is
present, some element of compression of
diagnosis
the cerebellar peduncle.
• This is consistent with a left CP angle
meningioma

Competency Answer
question(s)

What are the Patient factor :


factors taken into Age, hearing status, fitness for surgery
consideration in
Disease (tumour) factor :
managing these

- 324 -
patients Size, unilateral vs bilateral, compression
on brainstem, operability
Surgeon factor :
Surgical teams available and preference
(i.e. retrosig vs translab)

What are the 1. Retrosigmoid- preserves hearing, gives


surgical good exposure of CPA for large tumours,
however cerebellar retraction gives
approaches for
constant headache post-operatively.
this tumour and
the pro’s and 2. Middle fossa - preserves hearing, ideal
for small intracanalicular tumours,
con’s of each
however the temporal lobe retraction
approach? causes risks of fits especially in the
elderly population
3. Translabyrinthine – facial nerve can be
preserved, good exposure of the CPA
however hearing destructive

Advanced Answer
question(s)

What are the • Lateral


categories of
• Mid-petrosal
meningioma
based on their • IAM
sites of origin? • Petroclival
• Meckel’s cave

- 325 -
4.22 BELL’S PALSY

A GP has referred this 29 year old woman


SCENARIO
who noticed a sudden weakness of the face
(followed by a which shortly followed an upper respiratory
clinical tract infection.
photograph)

Introductory Answer
question

What are the key


• History: sudden vs gradual, associated
points to be neurological symptoms (speech
covered in the impediment, limb weakness), associated
assessment of a skin vesicles (?Ramsay Hunt), recent URTI
patient with a or surgery, previous history of facial
facial weakness? weakness
• Examination: LMN vs UMN weakness
(forehead sparing?), cranial nerve
examination, assessment of grade of
palsy, examination of the neck (?parotid
mass), examination of the ear

Competency Answer
question(s)

What clinical • Many grading systems described.


grading systems
• The House-Brackmann (I - VI) is probably
of facial palsy do
the most commonly used
you know?

- 326 -
• Other grading systems such as
Sunderland (1st - 5th degree), Seddon,
Sunnybrook, Yanahigara, Sydney,
Nottingham etc.

Are there other • Yes, computer assisted systems e.g. the


methods of Glasgow Facial Palsy Scale (GFPS). This
grading facial was developed by Mr. Brian O'Reilly as
palsy more an objective method of assessing and
objectively? presenting the house-brackmann and
regional grades of facial palsy by
production of a facogram.

What
investigations • Audiogram
would you • CT temporal bones
undertake for a
patient diagnosed • MRI neck
with Bell's palsy? • nerve conduction studies

Advanced Answer
question(s)

How would you • Scottish Bell's Palsy Study


manage a patient (http://www.scottishbellspalsy.info/)
with Bell's palsy? suggests that early treatment with
prednisolone significantly improves the
chances of complete recovery at three
and nine months. There was no evidence
of a benefit from acyclovir given alone or
of any additional benefit from acyclovir
given in combination with prednisolone.
• Cochrane review concluded that the
available evidence from randomised

- 327 -
controlled trials shows significant benefit
from treating Bell's palsy with
corticosteroids.

Would you • This is not conventional treatment and it


consider nerve would not be insensible to refer the
decompression in patient to a neurotologist who has
Bell's palsy? experience with this.A Cochrane review
found that there was only very low
quality evidence and that this was
insufficient to decide whether an
operation would be helpful or harmful
for people with Bell's palsy. The authors
suggest that there would unlikely to be
further research into the role of surgery
as Bell's palsy usually recovered without
treatment.

- 328 -
4.23 CHRONIC OTITIS MEDIA ACTIVE MUCOSAL DISEASE

CLINICAL
PHOTOGRAPH

Introductory Answer
question

This patient is a
• Otoscopic view of the left ear with a large
49 year old central wet perforation with possible
engineer with erosion of the long process of the incus
chronic discharge as the stapes head is visible.
from ear nearly
• The history and clinical findings are
12 months. Tell suggestive of chronic otitis media of the
me what you see. active mucosal type, although there may
be an underlying cholesteatoma.

- 329 -
Competency Answer
question(s)

What is the • Acute infection leads to inflammation of


pathogenesis of middle ear mucosa and oedema.
active mucosal Ulceration, granulations and polyp may
chronic otitis follow because of breakdown of the
media? epithelial reparative mechanisms.
• The causative organism is usually
pseudomonas (50-98% of cases); they
use pili to attach to the necrotic/inflamed
middle ear mucosa. Pseudomonas tends
to release enzymes which inhibits
effective immune response. Other
organism implicated include s. aureus,
proteus, klebsielly, diphteroids and fungi.

How would you Investigations


treat this patient?
• Culture swab, CT if fails simple treatment,
Audio.
• Consider MRI if complications are
suspicious.
• 1). Topical antibiotic treatment/steroids
• 2). Aural toilet
• 3). Control of granulation tissue with
silver nitrate cautery
• 4). Water precautions
• Associated nasal symptoms should be
treated, eg with a nasal steroid and
maybe a short course of anti-
inflammatory antibiotics such as

- 330 -
clarithromycin (500mg be for 7days).

Advanced Answer
question(s)

When would you Indications for Surgery


perform surgery? • Otorrhoea >6/52 despite appropriate
antibiotics
• Cholesteatoma
• Coalescent mastoiditis

4.24a OTOACOUSTIC EMMISSIONS

CLINICAL GRAPH

- 331 -
Introductory Answer
question

What is this
• This is a graph of a transient evoked
graph? otoacoustic emmission

Competency Answer
question(s)

What does it • I would like to highlight the stimulus,


show? probe stability and the response in terms
of the signal to noise ratio.
• Stimulus is good, ie, a broad band click
(1.0 – 4.0kHz) that is clean, clear and has
a positive and negative deflection, <1ms);
intensity 78-86 dBpk SPL
• Probe stability is good as it is >85%
• Response: signal to noise ratio in dB
across freq band is better than:
Freq (KHz): 0.8 1.6 2.4 3.2
4.0
S:N (dB): 3 3 6 6 6

Clear responses present (i.e. Normal cochlear


and middle ear function and Hearing
thresholds better than 30 dB HL

S:N in at least 3 of the 5 frequency bands


needs to be equal to or greater than the
levels shown above.

- 332 -
Advanced Answer
question(s)

What do you • Poor test conditions in terms of stimulus,


think about this probe stability and background noise.
graph? Therefore the it should be repeated.
• Background noise can completely
obliterate an OAE and interfere with
signal to noise averaging.
• Noise rejection
• Probe fit should to stable, ie, tight to
reduce background noise into the
microphone
• Baby state should be quiet and settled

4.24b OTOACOUSTIC EMMISSIONs

CLINICAL GRAPH

- 333 -
Introductory Answer
question

What is this
• The new format of otoacoustic
graph? emmissions (OAEs)
• It shows clear responses (Pass) because
the stimulus is good, the probe is stable
and at least 2 ‘signals’ are above 6dB
• In brief, 2 or more greens at the
frequency ‘traffic light’ indicate a pass.

Competency Answer
question(s)

What are • Small sounds generated by healthy outer


otoacoustic hair cells either spontaneously or after a
emmissions click stimulus (broad band 1-
4khz)produced by tiny earphone for click
stimulus. A tiny microphone detects
OAEs
• Patient should be still and quiet during
recording
• Impaired cochleae do not produce OAEs
• High correlation between OAEs and
normal audiometric thresholds (better
than 30dBHL)
• No correlations between size of OAEs and
threshold level
• Retrocochlear disorders not identified by
OAEs
• Important for Hearing screening, NOHL,

- 334 -
Objective test for very young

Advanced Answer
question(s)

What do you • A click stimulus is not frequency specific


understand about and consist of either a Narrow band (2-
a click and a pure 4kHz) or Broad or wide band (1-4kHz)
tone acoustic • A pure tone stimulus is frequency
stimuli? specific; Tone burst and tone pips are the
2 types of pure tone acoustic stimuli; the
former has an overall length >20ms
whilst the latter has an overall length
<20ms)
Additional info:
• Tone burst has a defined start (rise time),
duration (plateau) and end (fall time); the
plateau is longer than the rise/fall time.
• Tone pips usually has a plateau which is
the same or shorter than the rise/fall
time.

- 335 -
4.25 UNIVERSAL NEONATAL HEARING SCREENING

CLINICAL GRAPH

Introductory Answer
question

What do you
• Despite a good stimuli and satisfactory
think about the probe stability there is no clear responses
otoacoustic (Failed) (all reds at the frequency traffic
emission tracing? light)

What is the aim


• To identify PCHL>40dB in the better ear
of the Universal
against a background of 1:1000 live birth
neonatal hearing in UK has permanent congenital hearing
screening? loss

- 336 -
• 1st wave England 2000

Competency Answer
question(s)

Tell me about the


Screening tests
screening tests.
• Normal well baby auto OAE and if the
baby passes then screening is over.
• If the screen OAE fails then it is repeat
+/- with auto ABR ( so the baby may end
up having 3 tests: auto OAE, repeat auto
OAE and auto ABR)
• High risk babies: Auto OAE AND Auto
ABR (High risk: eg nicu >48hrs, CF abn,
infection, FH etc both OAE and ABR
because of high risk of auditory
neuropathy).

If the baby fails


the screening • Referred via the Care pathways for
process what DIAGNOSTIC assessment, Rehabilitation
happens next? and Support.

Advanced Answer
question(s)

What is Auditory Not a diagnosis but a name for a pattern of


Neuropathy test results with
Spectrum • OAE/CM present
disorder (ANSD)
• ABR absent or grossly abnormal at high
levels
And

- 337 -
• Behavioural thresholds vary from normal
to profound
• Speech perception worse than predicted
from PTA, especially in noisy
environment
Type 1-post synaptic ANSD
• Nerve neuropathy
• Dyssynchrony
• aplasia
Type 2-pre synaptic ANSD
• Inner hair cells
• Junction inner hair cell and auditory
nerve

- 338 -
4.26 AUDITORY BRAINSTEM RESPONSE

CLINICAL
PHOTOGRAPHS

- 339 -
Introductory Answer
question

What
• Diagnostic ABR (not automated ABR)
investigation is
being carried out
on this child?

Competency Answer
question(s)

What is ABR? • Electrical response


• Generated by 8th nerve and brainstem
• Recorded via surface electrodes
• Typical morphology shown in printout
with waves 1-5
• 1-10 m secs post stimulus
• Click stimuli are not good enough
• Tone pips @.5, 1,2,4 kHz used
• Small (50 nV) cf background noise
• Involves artefact rejection and Response
averaging

Tell me about
this graph • There is a clear response at 35dB
• There is also a clear response 5dB above
(ie 40dB) but no clear response 5dB
below (ie 30dB)
• The ABR threshold is therefore 35 dB

- 340 -
Advanced Answer
question(s)

Explain what is a
clear response?

With regard to
Threshold assessment
the age of the
child what are the • Less than equal to 6 months: ABR
various threshold • Between 6 and 30 months: Visual
assessments Reinforcement Audiometry
available?
• 30 months and above: conditioned sound
field response (performance test), Pure
Tone Audiometry

- 341 -
4.27 PRESBYACUSSIS

CLINICAL
AUDIOGRAM

Introductory Answer
question

Describe this
• Bilateral sensorineural hearing loss
audiogram
• The pattern of hearing loss and the
speech discrimination is consistent with
neural presbyacusis.

Competency Answer
question(s)

What is • Refers to the hearing loss experienced by

- 342 -
presbyacusis? elderly individuals. Important problem as
elderly rely on hearing to counter deficits
in sight & reaction time. In addition to
decreased memory/concentration affects
speech recognition (worse in noisy
environments)

What is the
4 histopathological types
treatment?
• Sensory Epithelial atrophy with loss of
HCs/supporting cells in organ of Corti
(basal turn) possibly due to accumulation
of lipofuscin pigment. Good speech
discrimination
• Neural Atrophy of nerve cells in cochlea
and CNS pathways (?2100/35000
neurons lost/decade). Effects noted after
90% loss – i.e old age; Clinically
disproportionate loss in speech
recognition
• Metabolic Atropy of stria. Flat HL with
good speech recognition. 30-60 yrs
• Cochlear conductive Thickening and
stiffening of basilar membrane (good
speech discrimination)

Lip reading, air conduction HA, Assistive


listening device

- 343 -
Advanced Answer
question(s)

What is • Recruitment is the abnormal


recruitment? perception of sound with small
increase in intensity occurs in sensory
presbyacusis (typically the patient
says ‘speak up doc I cannot hear you’
and then when you raise your voice
the patient says ‘don’t shout I am not
deaf.’

How might the • Clipping or compression


hearing aid
audiologist deal
with this
problem?

What would you • Assess for Cochlear implant


do if the patient
complains that
hearing aids are
inadequate?

- 344 -
4.28 SUDDEN SENSORINEURAL HEARING LOSS

CLINICAL
AUDIOGRAMS

Introductory Answer
question

What is the
• Unilateral (left) sensorineural hearing
diagnosis? loss
• Normal hearing on the right, except 50
dB threshold dip at 8000Hz only

Competency Answer
question(s)

How would you • Sudden 30db HL in 3 contiguous


defined sudden frequencies for at least 3 days
SNHL?

• FTA-Abs, ANA, RF, ESR, INR, aPTT, FBC –


How would you (infection), TFTs, Blood glucose, Lipid
investigate and profile
treat this patient?
• MRI as 1-2% will have acoustic neuroma

- 345 -
(3-12% acoustics present with SSNHL)
• Audiometry
If cause identified – treat otherwise
• Carbogen (5% CO2) – vasodilator.
• Increase blood flow (LMW dextrans,
anticoagulants) – no supportive evidence
• Steroids – systemic use supported by
studies. Intratympanic for
sever/profound
• Others (antivirals, diuretics, hyperbaric
oxygen)
• Note spontaneous recovery in 47-63
percent of cases
Negative prognostic Age <15 or >65;
Eleveted ESR, Verigo or abnormal ENG;
HL contralateral; Severe SSHL

Advanced Answer
question(s)

What is the 4 theoretical pathways


pathogenesis of • Viral infection 17-33% patients
sudden SNHL? recal preceding viral illness. Higher rates
of seroconversion to HSV. Histopath
evidence of viral induced damge to
cochlea.
• Vascular compromise Coclea = end
organ. Function sensitive to b/supply.
Time frame fits with vascular cause.
Likely due to
thrombosis/embolism/spasm/decresed

- 346 -
blood flow. Risk factors similar to cardiac
(hyperchol, low HDL,, high fibrinogen,
smoking)
• Intracochlear membrane rupture
• Immune mediated

4.29 TINNITUS

CLINICAL
PHOTOGRAPHS

- 347 -
Introductory Answer
question

This patient
• These clinical photographs show a
complains of a vascular malformation (rather than
pulsatile noise in haemangiomas) affecting the face and
her left ear. What therefore the cause for her tinnitus is
is the most likely likely to be vascular in origin, possibly
cause? arteriovenous malformation.

Competency Answer
question(s)

What is tinnitus? • Various definitions exist such as the


perception of noise in the absence of an
external stimuli or an aberrant auditory
phenomenon unrelated to an external
auditory source or simply the sensation

- 348 -
of sound arising within the head.

What • Audiogram and tympanograms


investigations
• FBC and TSH
would you order
for pulsatile • Carotid dopplers
tinnitus?
• MRI/MRA head and neck
• CT temporal bones

Is there any
• Masking therapy (eg white noise
effective maskers) appears to be effective
treatment for
• Tinnitus retraining therapy is also
subjective
effective but no more than direct
tinnitus? counseling.
• There are other treatments but their
effectiveness is controversial. However,
there is evidence for favourable
outcomes with benzodiazepines (eg
clonazepam or alprazolam),
antidepressants (eg amitriptyline, SSRI
and tricyclic) as well as dexamethasone,
but not for acupuncture, carbamazepine,
Ginkgo Biloba or gabapentin.

Advanced Answer
question(s)

Have you heard


• Yes, it started in 1987 as the study of how
of Tinnitology? the perception of an aberrant auditory
phenomenon can be translated into
sensation and effect.

- 349 -
Why do you think • Benzodiazepines enhance GABA effects
benzodiazepines and may be effective because one
may be effective proposed mechanism for tinnitus is the
in the treatment loss of GABAergic inhibitory neurons.
of subjective
tinnitus?

4.30 CLINICAL ANATOMY OF THE TEMPORAL BONE

CLINICAL
PHOTOGRAPH

Introductory Answer
question

Describe the
• Lateral surface: inferior temporal line,
clinical anatomy spine of Henle, root of zygoma, tympanic
of the lateral

- 350 -
surface of the ring, tympanomastoid suture,
temporal bone tympanosquamous suture, TMJ,
shown here. mandibular condyle and fossa, masoid
bone

Competency Answer
question(s)

What is • Macewen’s triangle is formed by the


Macewen’s inferior temporal line, posterior EAC
triangle and its canal wall and a tangential line joining
clinical the temporal line to post wall of EAC
importance?
• It is a safe area to start drilling a cortical
mastoidectomy as it is the lateral to the
mastoid antrum and immediate behind
the suprameatal spine of Henle – a
reliable landmark.
• In adults, the antrum is approximately
1.5 cm deep to Macewen triangle.
• The antrum connects the middle ear
cavity with mastoid and it is the first air
cell of the temporal bone, formed by
vacuolisation rather than
pneumatisation, which produces the
mastoid air cells.
• Macewen area is the location of
subperiosteal abscesses often seen in
children; this is perhaps due to the
porous-like cribiform plate of bone.

How might you • By not drilling near the ossicular chain,


prevent acoustic especially the short process of incus.
trauma whilst
• The short process of the incus and fossa

- 351 -
drilling the incudus may be visualised early by
temporal bone? utilizing the refractive index of water
(remember temporal bone drilling should
be sub-aquatic surgery).

Advanced Answer
question(s)

Where is • Trautman’s triangle is the area bounded


Trautman's by the bony labyrinth (hard angle)
triangle and what anteriorly, the sigmoid sinus posteriorly
is it significance? and the posterior fossa dura (containing
superior petrosal sinus) superiorly.
• It is a potentially weak area through
which an infection of temporal bone may
spread to cerebellum.
• A large sigmoid sinus may reduce the size
of this triangle and also increase the
angulation of the superior petrosal sinus.
This potentially can impede the venous
drainage and predispose to the
development of endolymphatic hydrops
(asymptomatic hydrops vs symptomatic
hydrops).
• The endolymphatic sac is located anterior
to Trautman’s triangle, medial and
inferior to posterior semicircular canal
(SCC).

How else might


• By using Donaldson’s line, which is an
the imaginary line from Lat SCC bisecting
endolymphatic (perpendicularly) the posterior SCC; it
sac be located identifies the superior aspect of the
during saccus endolymphatic sac.

- 352 -
decompression endolymphatic sac.
surgery?

- 353 -
patient? impact on feeding, choking, apnoea, and
cyanosis. Birth history as well as number and
When would you
duration intubations. Neuromuscular
perform direct
disorders.
laryngoscopy?
Examination – effort and efficacy of
ventilation including nasal flare, tracheal tug,
chest recession. Phase and type of noise:
pharyngeal stertor improved with jaw thrust
or laryngotracheal stridor. Nasal cold spatula
testing. Oral examination – cleft palate and
glossoptosis. Neck – mass. Top to toe exam
for cutaneous haemangioma.
Fa

Growth chart from ‘red book’.


st

A child with a history consistent with simple


-P roo

laryngomalacia with inspiratory stridor,


rin f

thriving without prior tracheal intubation or


P

t P Co

haemangioma may be managed with regular


outpatient review, an outpatient supraglottic
ub py

examination and treatment of co-existing


extra-oesophageal reflux.
lis
hi

Direct laryngoscopy should be performed if


n

history of intubation, haemangioma,


g

diagnostic uncertainty, biphasic stridor or


failure to thrive.

Advanced Answer
question(s)

The child Secondary airway lesions are present in over


undergoes rigid 50% of children with severe laryngomalacia
laryngo-tracheo- who require aryepiglottoplasty.
bronchoscopy. A
I would assess for any dynamic component of
mid-tracheal
the narrowing including deterioration on
compression is
expiration i.e. tracheomalacia, pulsatility or

- 53 -

You might also like