1

38
Tumors of Ear

High Yield Facts

• Hitzelberger sign is characterized by hypoesthesia of posterior canal wall, is a sign of involvement of facial
nerve.
• Most common site of origin of acoustic neuroma is inferior vestibular nerve.
• Most common true neoplasm of middle ear is glomus tumors.
• Rising sun appearance as tumor arises from floor of middle ear in glomus tumor.
• Brown’s sign: Glomus pulsates and blanches on increasing ear canal pressure.
• Aquino’s sign: Glomus tumor blanches on compression of carotids.
• Schwartzes sign is seen in active Otosclerosis.
• Loss of corneal reflex earliest sign in acoustic neuroma due to V nerve involvement.
• Phelps sign is destruction of bone between carotid canal and jugular foramen.
• Magnetic resonance Imaging (MRI) with Gadolinium is gold standard for diagnosing of acoustic neuroma.

zz Cochlear nerve in anteroinferior quadrant

Anatomy of Internal Auditory Canal zz Inferior vestibular nerve in posteroinferior quadrant
(IAC)
Anatomy of Cerebellopontine Angle (CPA)
Contents of Internal Auditory Canal zz It is a triangular area bounded anterolaterally by petrous
temporal bane, medially by pons and brainstem,
In addition to internal auditory vessels, following nerves posteriorly by cerebellum and flocculus.
enter the IAC. zz Contents of the angle are: Anterior Inferior cerebellar
zz Facial nerve in anterosuperior quadrant artery and VII, VIII cranial nerve
zz Superior vestibular nerve in postsuperior quadrant zz Immediately superior is the cranial nerve and III, IV, VI
are further up.
zz Inferiorly lies IX, X, XI cranial nerve: Thus in lesions of
CPA all these nerves can be involved.

Lesions of CP angle
zz M/C Acoustic neuroma = 80%
zz Meningoma = 10%
zz Congenital Cholesteatoma = 5%
zz Others = 5%
Fig. 1: Contents of internal auditory canal
 Triple O

Acoustic Neuroma/Vestibular  Webers → towards normal side

686 Schwannoma/Neurilemmoma  Schwabach → shortened
 PTA → shows sensorineural hearing loss
(Nutshell)  Tone decay > 30dB
zz 80% of all CP angle tumors  Recruitment negative
zz Benign encapsulated, extremely slow growing tumors  Speech discrimination score poor (speech discrim-
zz Bilateral tumors seen in neurofibromatosis ination score becomes worse at higher speech in-
zz Tumors almost always arise from the Schwann cells of tensity and this phenomenon is called as roll over
the vestibular division of VIII nerve phenomenon)
zz Most commonly arises from inferior vestibular nerve  BERA – Delay of > 0.2 m sec in Wave V between the
zz 40-60 years, no sex predilection 2 sides. The best test for acoustic neuroma is BERA
zz Cochleo vestibular symptoms: Earliest, progressive zz Acoustic reflex: Shows stapedial reflex decay
unilateral SNHL, tinnitus, difficulty in understanding zz Vestibular test: Caloric test usually show diminished
speech out of proportion of pure tone hearing loss (char- or absent response but may be normal if tumor is very
acteristic of AN) small.
zz Cranial nerves: 5th nerve earliest to be involved zz This is because caloric test is mainly a test for lateral
zz Hitzelberger sign (hypoaesthesia of posterior meatal semicircular canal which is innervated by superior
wall), loss of taste, decreased lacrimation are due to vestibular nerve. Thus, a small tumor arising from
facial nerve involvement inferior vestibular nerve may not lead to any change in
zz Poor speech discrimination and Roll over phenomenon caloric response. Later when superior vestibular nerve is
are seen compressed the caloric response is reduced.
zz Short increment Sensitivity Index (SISI) shows a score of zz Investigation of choice: Gadolinium enhanced MRI =
0-20% 100% diagnostic yield
zz Diminished or no response to calorie tests
zz Gold standard for diagnosis: MRI with gadolinium en- Treatment
hancement
zz Surgery is the treatment of choice
zz Treatment: Surgical removal, gamma knife surgery
Surgical Approach Indication
Histopathology Hearing preservation • Patient has good hearing
zz Histopathological examination shows two morphological • Retro sigmoidal approach and tumor is large >3cm size
tissue patterns. • Middle cranial fossa • Patient has good hearing
 Antoni A Pattern: It has closely packed cells with approach and tumor size is < 1.5cm
small spindle–shaped and densely stained nuclei • Retro labyrinthine (i.e. small tumors)
Hearing ablation • Small CPA tumor not
called as Verocay Body
Translabyrinthine approach extending into lateral part of
 Antoni B Pattern: It has loose cellular aggregation
internal auditory canal
of vacuolated pleomorphic cells.
• Suitable for tumors <3cm
In any particular VS, one type of cellular pattern may
but disadvantage is SNHL
predominate or both types can be completely admixed. • M/C approach
TABLE 1: Classification of VS according to Size
OTORHINOLARYNGOLOGY

Intrameatal Tm Extrameatal Tm M
Grade I Small 1 – 10
Grade II Medium 11 – 20
Grade III Moderately large 21 – 30
Grade IV Large 31 – 40
Grade V Giant > 40

Investigations
zz Audiological test: Show features of retrocochlear hearing
loss
 Rinne +ve
Fig. 2: MRI brain – shows a lesion/mass In cerebellopontine angle
 Chapter 38  |  Tumors of Ear

Glomus Tumor (Nut Shell) zz Highly vascular – Main Blood supply: ascending pha-

zz Most common benign neoplasm of middle ear

ryngeal artery 687
zz Commonly affect middle aged females (typically in 4th
zz Rule of 10:10% familial; 10% multicentric: 10% functional or 5th decade of life)
(secrete catecholamine’s) zz Malignant transformation and metastasis are rare
zz Middle age; Females affected 5 times more zz Less than 10% tumor are associated with catecholamine
zz Benign encapsulated, extremely vascular, very slow secretion
growing, locally invasive
zz Abundant thin walled blood sinusoids with no contractile
muscle coat: Profuse bleeding
Pathologically
zz Types: Glomus jugulare (from jugular bulb), Glomus They originate from the ‘chief cell’ which contains acetylcho-
tympanicum (from promontory) line, catecholamine and serotonin
zz Classic findings are clusters of chief cells k/a Zellballen,
Symptoms with a rich vascular plexus throughout the entire Tumor.
Therefore, they are highly vascular and may bleed
zz Conductive deafness substantially during surgical excision
zz Pulsatile tinnitus, swishing character, temporarily stopped  Bilateral tumors occur in 1 – 2 %
by carotid pressure  Can be hereditary also
 Also associated with pharamatoses (neurologic
Otoscopy disease with cutaneous manifestations like von
Recklinghausen neurofibromatosis, Sturge-Weber
zz Red reflex
syndrome, tuberous sclerosis and von Hippel –
zz Rising sun appearance when the tumor arises from the
Lindau disease)
floor of middle ear
zz Also associated with MEN Type ! syndrome
zz Brown’s sign (pulsation sign) (blanching sign) when
Rule of Ten: For Glomus tumors – 10% tumors are familial,
ear canal pressure is increased, the tumor pulsates
10% secrete catecholamines and 10% are multicentric
vigorously.
zz Aquino sign: Tumour blanches on compression of
ipsilateral carotid artery Spread of Tumor
zz CT Scan-Phelp’s sign: The absence of normal crest of TABLE 2: Spread of Glomus tumor and its presentation
bone between the carotid canal and jugular fossa on
lateral tomography is virtually diagnostic of glomus Site of Spread Presentation
jugulare • Tympanic membrane • Vascular polyp
• Labyrinth petrous, pyramid • Hearing loss
Glomus Tumor and mastoid
• Jugular foramen and base • Cranial nerve palsies VII, VIII,
zz Glomus tumor is the most common benign tumors of of skull IX to XII
middle ear • Eustachian tube
zz Resemble carotid body therefore also called as • Intracranially spreads • Mass on nasopharynx
chemodectoma • Lung, liver lymph nodes
zz Consists of paraganglionic cells derived from neural

OTORHINOLARYNGOLOGY
crest (Paragangliomas)
zz It usually arises from dome of jugular bulb as glomus
jugulare or from promontory along the course of
tympanic branch of IXth cranial nerve (Jacobson’s M/C cranial nerve involved = Facial nerve followed by the last four
nerve) and along the course of branch Xth cranial nerve cranial nerves.
(Arnold’s nerve) as glomus tympanicum
zz Sometimes it may be multicentric (10% cases) i.e.
originates from more than 1 site Clinical Features
zz Most common site in middle ear: hypotympanum When tumor is intratympanic:
zz Earliest symptoms are deafness (conductive) and tinnitus
Features (pulsatile and of swishing character, synchronous
with pulse and can be temporarily stopped by carotid
zz Slow growing locally invasive, noncapsulated tumor pressure). This is because jugular bulb is related to floor
which causes destruction of the bone and facial nerve of middle ear.
 Triple O

zz Otoscopy shows red reflex; rising sun appearance, Phelps sign: absence of normal crest between the carotid
688 tympanic membrane appears bluish and bulging. canal and jugular fossa on lateral tomography, in case of
zz Pulsation sign/ Brown sign/ Blanching sign is positive glomus jugulare.
(when ear canal pressure is raised with Siegel’s speculum,
tumor pulsates vigorously and then blanches; reverse zz HRCT and gadolinium enhanced MRI is used to
happens with release pressure). delineate the intracranial extent of tumor.
zz Aquino sign: It is blanching of the mass with manual A combination of CT scan and contrast MRI is the imaging
compression of ipsilateral carotid artery. regimen of choice for glomus jugular tumor.

When Tumor present as polyp zz Audiogram will show conductive deafness if the middle
ear space is invaded with tumor. If inner ear is invaded
zz History of profuse bleeding from the ear either SNHL is seen
spontaneously or on attempts to clear it. zz Angiography: It is necessary when CT scan shows
zz Dizziness, vertigo, facial paralysis, earache otorrhea. involve­ment of jugular bulb, carotid artery or intracranial
 Audible bruit: Heard by stethoscope over mastoid at extension.
all stages. Following procedures are done:
 Some glomus tumor secrete catecholamines and  For carotid artery: Carotid arteriography
produce headache, sweating flushing,etc.  For jugular bulb: Jugular venography
 Patient may show features of cranial nerve IX and X,  For intracranial extension: Vertebral arteriography
involvement viz. Dysphagia or hoarseness.

Pulsatile tinnitus: Pulsatile tinnitus is characteristic of glomus Biopsy is contraindicated in glomus tumors since they are very
tumor but can also be seen in other conditions. vascular
zz Other Conditions Causing Pulsatile Tinnitus
Arterial – Glomus tumor, AV malformation of temporal bone,
aberrant internal carotid artery, carotid/subclavian atherosclerosis Staging System
Venous – High jugular bulb, benign ICT. zz Fisch
zz Glasscock jackson staging
Investigations
Fisch Staging in ENT
zz Examination under microscope: Pulsatile mass seen.
zz Glomus Tumour
zz Catecholamines levels: Check the levels of serum zz Juvenile Nasopharyngeal Angiofibroma
catecholamines and their breakdown product VMA in
urine before surgery for glomus jugulare
zz CT scan: Investigation of choice. Helps to distinguish Treatment
glomus jugulare from glomus tympanicum with the help
of. Surgery: Microsurgical total tumor removal is the treatment of
choice for most patients. Patients with functionally secreting
OTORHINOLARYNGOLOGY

tumors need to be alpha blocked with phentolamine before

surgery as alpha adrenergic hormones are released during
surgery.
Embolization: In inoperable patients who have received
radiation.
Radiation- In inoperable lesions, old age patients.

Fig. 3: Rising sun sign – glomus tumor

 Chapter 38  |  Tumors of Ear

Image-Based Questions 689

1. The following is a features of BERA findings of Vestibular 12. Hitzelberger sign is seen in: (AI 2008)
schwannomma  (JIPMER 2018) A. Bell’s palsy B. Vestibular schwannoma
A. Flat amplitude C. Cholesteatoma D. Mastoiditis
B. Increased amplitude in 5, 6 13. Pulsatile tinnitus in ear is due to: (Recent Pattern 2016)
C. Decreased amplitude in 5, 6 A. Malignant otitis media B. Meniere’s disease
D. Increased inter-wave latency between 1, 5 and 1 and C. Osteoma D. Glomus tumor
3 waves 14. Brown sign is seen in: (AI 2007)
2. Glomus tumor invading the vertical part of carotid canal A. Glomus tumor B. Meniere’s disease
is? (Recent Pattern 2015) C. Acoustic neuroma D. Otosclerosis
A. Type B B. Type C2 15. In acoustic neuroma cranial nerve to be involved
C. Type C3 D. Type C1 earliest is:
3. Most common presentation of CP angle tumor is? (AI 2007)
(Recent Pattern 2016) A. 10 B. 7
A. Loss of corneal reflex B. Headache C. 5 D. 3
C. Dysphagia D. Raised ICP 16. Acoustic neuroma commonly affects the:
4. Which intervention is best in patient operated for  (Recent Pattern 2007)
bilateral acoustic neuroma for hearing rehabilitation: A. 5th cranial nerve B. 6th cranial nerve
A. Bilateral cochlear implant  (PGI Nov 2012) C. 7th cranial nerve D. 8th cranial nerve
B. Unilateral cochlear implant 17. Mass in ear on touch bleeding heavily, cause is:
C. Brainstem hearing implant  (Recent Pattern 2005)
D. High power hearing aid A. Glomus Jugulare B. Ca mastoid
E. Myringoplasty C. Acoustic neuroma D. Angiofibroma
5. Phelp sign is seen in? (Recent Pattern 2016)
18. Cerebellopontine angle tumor produces: (PGI 2005)
A. Otosclerosis B. Glomus tumor
A. Absent corneal reflex
C. Meniere's disease D. Lateral sinus thrombosis
B. Deafness
6. Fisch classification is used for? (Recent Pattern 2015)
C. Tinnitus
A. Paravertebral tumors
D. Trigeminal neuralgia
B. Synovial sarcomas
19. A patient is suspected to have vestibular schwannoma
C. Glomus tumor
the investigation of choice for its diagnosis is: 
D. Retroperitoneal tumors
A. Gadolinium enhanced MRI  (AIIMS 2004)
7. Glomus jugulare is seen in? (Recent Pattern 2016)
B. Contrast enhanced CT Scan
A. Promontory B. Hypotympanum
C. PET scan
C. Epitympanum D. None of these
8. True about vestibular schwannoma: D. SPECT
 (Recent Pattern Jun 2011) 20. True about Acoustic neuroma is: (PGI June 2004)
A. U/I hearing loss is common presentation A. Arises from vestibular nerve
B. Mostly malignant B. Malignant tumor
C. Most common tumor of CP angle C. Lower pole displaces trigeminal cranial nerve

OTORHINOLARYNGOLOGY
D. Sensorineural deafness D. Upper pole displaces IX, X, XI
E. Uncapsulated 21. A patient with bleeding from ear, tinnitus and
9. All are true about glomus jugular except  progressive deafness. On examination red swelling
A. Rising sun sign is seen  (Recent Pattern Jun 2011) behind intact TM, blanches on pressure. Management
B. Involves 9th and 10th cranial nerve includes all except (AIIMS Nov 2001)
C. Pulsatile tinnitus is seen A. Pre op embolization B. Surgery
D. Malignant C. Radiotherapy D. Interferons
10. True about glomus all except (AI 2010) 22. Mass in ear bleeds heavily on touch  (2016)
A. Pulsatile tinnitus B. Rising sun sign seen A. Angiofibroma B. Acoustic neuroma
C. Invades epitympanum D. Involves 9th and 10th nerve C. Glomus jugular D. Carcinoma mastoid
11. Acoustic neuroma commonly arise from: (AI 2011) 23. Earliest ocular finding in acoustic neuroma is: 
A. Inferior vestibular nerve A. Loss of corneal sensation (PGI 2000)
B. Superior vestibular nerve B. Diplopia
C. Cochlear nerve C. Papilledema
D. Facial nerve D. Ptosis
 Triple O

24. Acoustic neuroma causes: (PGI June 99) 33. Vestibular neuroma does not correct: 
690 A. Conductive deafness A. Absence of caloric reflex  (Recent Pattern 15)
B. Retrocochlear deafness B. Nystagmus
C. Cochlear deafness C. Normal corneal reflex
D. Any of the above D. High frequency sensorineural deafness
25. Schwannoma involves the: (AI 99) 34. Progressive loss of hearing, tinnitus and ataxia are
A. Cochlear part of VIIIth nerve commonly seen in a case of: (Recent Pattern 15)
B. Vestibular part of VIIIth nerve A. Acoustic neuroma
C. Hypoglossal nerve B. Cerebral glioma
D. Vagus nerve C. Otitis media
26. Which is the most Pulsatile tumor in External Auditory D. Ependymoma
Canal which bleeds on touch (AIIMS 95) 35. Acoustic neuroma of few mm diameter, the investigation
A. Adenoma of choice: (Recent Pattern 14)
B. Glomus A. Plain X – ray skull
C. Squamous cell carcinoma B. MRI scan
D. Basal cell carcinoma C. CT Scan
27. The earliest symptom of acoustic nerve tumor is: (AI 95) D. Air encephalography
A. Vertigo 36. The glomus tumor invasion of jugular bulb is diagnosed
B. Tinnitus by  (Recent Pattern 15)
C. Otorrhea
A. X-Ray
D. Sensorineural hearing loss
B. Jugular venography
28. A 70-year – old male presents with loss of sensation in
C. Carotid angiography
external auditory meatus (Hitselberger sign positive).
D. PET Scan
A. Bell’s palsy
37. Which most common bony tumor of middle ear
B. Mastoiditis
(Recent Pattern 14)
C. Vestibular Schwannoma
A. Adenocarcinoma B. Squamous cell carcinoma
D. Cholesteatoma
C. Acoustic neuroma D. Glomus
29. Earliest sign seen in Acoustic neuroma is:
38. Pulsatile tinnitus is seen in: (Recent Pattern 15)
(Recent Pattern 16)
A. Reduced corneal reflex B. Facial weakness A. Osteoma B. Otitis externa
C. Cerebellar signs D. Unilateral deafness C. Mastoiditis D. Glomus
30. Most common cerebellopontine angle tumor is: 39. Treatment of middle ear malignancy are all except:
(Recent Pattern 15) A. Total or sub total petrousectomy  (Recent Pattern 14)
A. Meningioma B. Cholesteatoma B. Radical mastoidectomy
C. Acoustic neuroma D. All of the above C. Type 1 tympanoplasty
31. In acoustic neuroma all are seen except: (Recent D. Radiotherapy
Pattern 15) 40. The location of jugular tumor is glomus
A. Facial palsy B. Tinnitus (Recent Pattern 15)
C. Diplopia D. Loss of corneal reflex A. Promontory B. Epitympanum
32. In a patient with acoustic neuroma all are seen except: C. Hypotympanum D. Mastoid air cells
(Recent Pattern 15) 41. True about glomus tumor are all except
A. Reduced corneal reflex A. Pulsatile tinnitus  (Recent Pattern 14)
OTORHINOLARYNGOLOGY

B. Acute episode of vertigo B. Arise from non chromaffin cells

C. Cerebellar signs C. Lymph node metastasis
D. Facial nerve may be involved unilateral deafness D. Multicentric
 Chapter 38  |  Tumors of Ear

Answers with Explanations 691

1. D.  Increased inter-wave latency between 1,5 and 1 and IX and Xth nerve- there is dysphagia, pharyngeal and
3 wave (PL Dhingra 7/e, Chap 4, p 28) laryngeal palsies.
XI, XII, III, IV, VI are affected when the tumor is large.
Brainstem Evoked Response Audiometry (BERA)
4. C. Brain stem hearing implant
•• Has waves 1-7
(Dhingra's 6e/p 127, 5e/ p 141)
•• EE COLI
The condition of bilateral acoustic neuroma post operated
•• Distal part of 8th nerve
hearing rehabilitation is done by brain stem hearing
•• Proximal part of eight nerve
implant. This implant is designed to stimulate cochlear
•• Cochlear nucleus
nuclear complex in the brain stem directly by placing
•• Superior olivary complex
implant in the lateral recess of fourth ventricle. In these
•• Lateral lemniscus
case cochlear implant are of no use as the 8th nerve is
•• 6 & 7 – inferior colliculus
resected. In unilateral vestibular schwannoma is not
Vestibular schwannoma – lesion which is retrocochlear performed as the other ear is functional. Myringoplasty is
Features closure of tympanic membrane perforation.
Interaural wave 5 latency difference greater than 0.2 msec 5. B. Glomus tumor (Scott Brown Otorhinolaryngology 6th
Complete absence of wave 5  e/3/23/4, Dhingra's 6e/p 109, 5e/ p 120)
Prolonged 1- 5 interwave time interval Phelp’s Sign
2. B. Type C2 (Internet emedicine medscape) The absence of normal crest of bone between the carotid
The Glasscock-Jackson and Fisch classifications of glomus canal and jugular fossa on lateral tomography is virtually
tumors are widely used. The Fisch classification of glomus diagnostic of glomus jugulare
tumors is based on extension of the tumor to surrounding 6. C. Glomus tumor (Scott Brown Otorhinolaryngology
anatomic structures and is closely related to mortality and 6th e/3/23/3)
morbidity. Refer question 1 for explanation.
•• Type A tumor: Tumor limited to the middle ear cleft 7. B. Hypotympanum (Dhingra's 6e/p 109, 5e/ p 123)
(glomus tympanicum) Glomus tympanum arise from dome of jugular bulb
•• Type B tumor: Tumor limited to the tympanomastoid and invades Hypotympanum and jugular foramen
area with no translabyrinthine compartment involve- causing neurological signs of IX and XIIth cranial nerve
ment involvement.
•• Type C tumor: Tumor involving the in translabyrinthine 8. A. U/I hearing loss is common presentation, C. Most
compartment of the temporal bone and extending into common tumor of CP angle, D. Sensorineural deafness
the petrous apex (Dhingra's 6e/p 112, 5e/ p 124)
•• Type C1 tumor: Tumor with limited involvement of the Refer theory for explanation.
vertical portion of the carotid canal 9. D. Malignant (Dhingra's 6e/p 109, 5e/ p 120)
•• Type C2 tumor: Tumor invading the vertical portion of Glomus tumor is the most common benign neoplasm of
the carotid canal middle ear. It has its origin from glomus bodies, found in
•• Type C3 tumor: Tumor invasion of the horizontal the dome of jugular bulb or on the promontory along the
portion of the carotid canal course of tympanic branch of IXth cranial nerve (jacobson’s

OTORHINOLARYNGOLOGY
•• Type D1 tumor: Tumor with an intracranial extension nerve).
less than 2 cm in diameter
10. C. Invades epitympanum (Dhingra's 6e/p 109, 5e/ p 120)
•• Type D2 tumor: Tumor with an intracranial extension
The glomus arises from the floor of the middle ear and
greater than 2 cm in diameter invades into the hypotympanum.
3. A. Loss of corneal reflex (Dhingra's 6e/p 112, 5e/ p 124) 11. A. Inferior vestibular nerve (Glasscock –Shambaugh,
Cranial nerve involvement in Cerebellopontine angle Surgery of the ear, 6/e, p 644)
tumors More recently acoustic neuroma arises from inferior
Vth nerve- This is the earliest nerve to be involved, division of vestibular nerve while previously it was thought
where there is reduced corneal sensitivity, numbness or superior vestibular nerve was the site of origin of acoustic
parathesia of face. neuroma.
VIIth nerve- Sensory fibers are affected early. There hy- 12. B. Vestibular schwannoma (Dhingra's 6e/p 112, 5e/ p 124)
poaesthesia of posterior meatal wall (Hitzelberger’s sign), Hitzelberger sign (hypoesthesia of posterior meatal
loss of taste in electrogustometry, loss of lacrimation in wall), loss of taste, decreased lacrimation are due to facial
Schirmer’s test). Delayed blink reflex may be an early man-
ifestation.
 Triple O

nerve involvement in vestibular schwannoma (acoustic 23. A. Loss of corneal sensation

692 neuroma, eighth nerve tumor). (Dhingra's 6e/p 112, 5e/ p 124)
13. D. Glomus tumor (Dhingra's 6e/p 109, 5e/ p 120) Cranial nerve involvement in Cerebellopontine angle
Earliest symptom of glomus tumor is hearing loss, tumors
tinnitus which is pulsatile and of swishing in character, Vth nerve- This is the earliest nerve to be involved,
synchronous with pulse and can be temporarily stopped where there is reduced corneal sensitivity, numbness or
by carotid pressure. parathesia of face.
14. A. Glomus tumor (Dhingra's 6e/p 109, 5e/ p 121) 24. B. Retrocochlear deafness
Brown sign (Pulsation sign) in Glomus tumor where when (Mohan Bansal's, p 167)
ear canal pressure is raised with Siegle’s speculum, tumor Acoustic neuroma has retrocochlear hearing loss. Hearing
pulsates vigorously and the blanches, reverse happens loss is more in high frequencies, poor discrimination,
with release of pressure. absent recruitment, threshold decay test is positive, absent
15. C. 5 (Dhingra's 6e/p 112, 5e/ p 124) stapedial reflex, in BERA a delay of 0.2 m/sec in wave V
The earliest cranial nerve to be involved is Vth nerve between two ears is significant.
followed by VIIth nerve. 25. B. Vestibular part of VIII nerve
16. D. VIIIth nerve (Dhingra's 6e/p 112, 5e/ p 124) (Dhingra's 6e/p 112, 5e/ p 124)
Acoustic neuroma is also called as eight nerve tumor or The Schwannoma almost always arises from the Schwann
vestibular schwannoma. cells of vestibular nerve and rarely from cochlear division
17. A. Glomus jugular (Dhingra's 6e/p 109, 5e/ p 121) of the VIII th nerve.
A red mass which bleeds on touch indicates glomus 26. B. Glomus (Dhingra's 6e/p 109, 5e/ p 120)
tumor as it arises from jugular bulb. The other condition The glomus is a tumor of middle ear and if it perforates the
is external auditory canal malignancy. Angiofibroma arises tympanic membrane it may present in EAC as a vascular
in nose from sphenopalatine foramen. tumor which bleeds heavily on touch.
18. A,B,C,D (Current otolaryngology 3/e, p 792) 27. D. Sensory neural hearing loss
The common CP angle tumors are Acoustic neuroma and (Dhingra's 6e/p 112, 5e/ p 124)
Meningioma. Cochleo vestibular symptoms: Earliest, progressive unilat-
Acoustic neuroma: There is deafness, tinnitus, facial nerve eral SNHL, often accompanied by tinnitus, difficulty in un-
involvement therefore absent corneal reflex derstanding speech out of proportion of pure tone hearing
Meningiomas: Deafness, vertigo, tinnitus, trigeminal neu- loss (characteristic of AN).
ralgias, facial paresis, lower cranial nerve deficits, visual Facts of Acoustic Neuroma
disturbances.
Earliest symptom: Progressive unilateral SNHL, often ac-
19. A. Gadolinium enhanced MRI scan companied by tinnitus
(Dhingra's 6e/p 113, 5e/ p 126) Earliest nerve involvement and sign- VIIIth nerve followed
MRI with Gadolinium scan is the gold standard for by Vth and VIIth, losss of corneal reflex
diagnosis of acoustic neuroma, it can easily diagnose even Most common nerve to be involved- Inferior vestibular
intra canalicular tumor. nerve
20. A. Arises from vestibular nerve (Glasscock –Shambaugh, Surgery of the ear, 6/e, p 644
(Dhingra's 6e/p 112, 5e/ p 124) Explanation: More recently acoustic neuroma arises from
Options b,c,d are incorrect as it is a benign tumor, upper inferior division of vestibular nerve while previously it was
pole displaces 3, 4, 6th cranial nerves, while lower pole thought superior vestibular nerve was the site of origin of
OTORHINOLARYNGOLOGY

displaces 9,10,11th cranial nerves. acoustic neuroma.)

21. D. Interferons (Dhingra's 6e/p 110, 5e/ p 121) Earliest present of VIIth nerve involvement- Hypoesthesia
The management of glomus tumors are by surgery, of posterior meatal wall (Hitzelberger sign)
radiation, embolization. Surgery – Microsurgical total 28. C. Vestibular Schwannoma (Dhingra's 6e/p 112, 5e/ p 124)
tumor removal is the treatment of choice for most patients. The case scenario given is that of Vestibular Schwannoma.
Patients with functionally secreting tumors need to be Hitzelberger’s sign (hypoaesthesia of posterior meatal
alpha blocked with phentolamine before surgery as alpha wall), loss of taste, decreased lacrimation are due to facial
adrenergic hormones are released during surgery. nerve involvement in vestibular schwannoma (acoustic
Embolization- In inoperable patients who have received neuroma, eighth nerve tumor).
radiation
Radiation- In inoperable lesions, old age patients. 29. A. Reduced corneal reflex (Dhingra's 6e/p 112, 5e/ p 124)
(Refer explanation 23)
22. C. Glomus jugular (Dhingra's 6e/p 109, 5e/ p 120)
The mass which bleeds heavily on touch in ear is glomus 30. C. Acoustic neuroma (Dhingra's 6e/p 112, 5e/ p 124)
jugular. Acoustic neuroma constitutes 80% of all Cerebellopontine
angle tumors and 10% of all the brain tumors.
 Chapter 38  |  Tumors of Ear

31. A. Facial palsy (Dhingra's 6e/p 112, 5e/ p 124) 35. B. MRI Scan (Dhingra's 6e/p 113, 5e/ p 125)
The cranial nerves involved in acoustic neuroma are, MRI Gadolinium contrast scan is superior to CT Scan in 693
8th,5th, 7th, 9,11, 3,4,6.In 7th nerve the sensory component is diagnosis of acoustic neuroma where it can diagnose few
affected leading to hypoesthesia of posterior meatal wall, millimetre size intracanalicular tumors.
delayed blink reflex which may be an early manifestation, 36. B. Jugular venography (Dhingra's 6e/p 110, 5e/ p 121)
but motar fibers are resistant so facial palsy is a very rare MRI gives soft tissue extent of the tumor. MRI angiography
presentation in acoustic neuroma. and venography further help in delineating invasion of
Facial weakness is very rare, as 90% of facial neurones jugular bulb and vein or compression of carotid artery.
can be non-functioning owing to compression, whilst the
37. B. Squamous cell carcinoma
facial function is clinically normal. (Logan Turner 11/e, p
(Dhingra's 6e/p 110, 5e/ p 122)
474)
In carcinoma primarily arising from middle ear or
32. B. Acute episodes of vertigo deep meatus, squamous cell variety is most common.
(Dhingra's 6e/p 112, 5e/ p 124) Adenocarcinoma may arise from glandular elements
Options a, c, d are true. In acoustic neuroma there is occasionally.
imbalance or unsteadiness are the vestibular symptoms,
38. D. Glomus (Dhingra's 6e/p 109, 5e/ p 120)
but true vertigo is seldom seen.
The earliest sign of glomus is hearing loss and tinnitus. The
The majority of patients will have experienced vertigo,
tinnitus is Pulsatile and swishing in character, synchronous
though this is not persistent Owing to the slow progression
with pulse which can be temporarily stopped with carotid
of the tumors, there is central compensation, that the
pressure.
patient may not appreciate his or her deterioration in
balance. 39. C. Type 1 tympanoplasty (Dhingra's 6e/p 111, 5e/ p 123)
 (Logan Turner 11/e, p 474) The treatment of middle ear carcinoma are a combination
of surgery and radiotherapy.
33. C. Normal corneal reflex
Surgery: Radical mastoidectomy, Total or subtotal petrou-
(Dhingra's 6e/p 112,113, 5e/ p 124,125)
sectomy depending on the extent of the tumor
In acoustic neuroma there is reduced or absent corneal
Radiotherapy: As a palliative measure when tumor involves
reflex.
IX, Xth cranial nerves, or spreads into cranial cavity or the
In Vestibular testing there is caloric test will show
nasopharynx.
diminished or absent response in 96% of the patients
and spontaneous nystagmus with eyes closed in 40. C. Hypotympanum (Dhingra's 6e/p 109, 5e/ p 120)
electronystagmography without history of disequilibrium. The glomus arises from the floor of the middle ear and
invades into the hypotympanum.
34. A. Acoustic neuroma (Dhingra's 6e/p 112, 5e/ p 124)
Progressive hearing loss, tinnitus, ataxia points out the 41. C. Lymphnode metastasis (Dhingra's 6e/p 109, 5e/ p 120)
diagnosis of acoustic neuroma. Glomus tumor is a benign tumor, so lymph node metastasis
is not seen.

OTORHINOLARYNGOLOGY