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CLINICAL REVIEW

Sudden Sensorineural Hearing Loss: A Diagnostic

and Therapeutic Emergency
Andrew D. P. Prince, BA and Emily Z. Stucken, MD

The family physician’s role in recognizing and managing sudden sensorineural hearing loss (SSNHL) is
crucial. A recently updated otolaryngologic clinical practice guideline has been released for this emer-
gency syndrome, but dissemination is limited to a specialty journal. As a result, the guidelines may not
be widely available in the primary care setting where patients often present. We provide this focused
review to clarify and disseminate SSNHL guidelines for the frontline family physician. ( J Am Board Fam
Med 2021;34:216–223.)

Keywords: Audiology, Clinical Decision-Making, Conductive Hearing Loss, Family Medicine, Family Physicians,
Otolaryngology, Primary Health Care, Sudden Hearing Loss

Introduction The CPG for SSNHL is intended for all clini-

Sudden sensorineural hearing loss (SSNHL) is a cians who diagnose or manage patients aged 18 years
medical emergency in which patients experience a and older that present with sudden hearing loss. The
sudden (within 72 hours) drop in hearing that is CPG is focused specifically on idiopathic SSNHL—
sensorineural in nature, not the result of mechanical hearing loss that is rapid in onset, not due to conduc-
blockage from fluid or wax buildup. Prompt recog- tive pathology, and not explained by an identifiable
nition is critical as there is a window of time in cause. Despite intense efforts to disseminate CPGs,
which medical interventions may be successful in previous studies have shown that uptake of recom-
restoring hearing and/or reducing tinnitus, thus mendations in CPGs is limited and adherence to cer-
providing significant improvement in patient qual- tain key action statements is low.9–11 The need to
ity of life.1 Although SSNHL can result from a variety disseminate the guidelines beyond the otolaryngol-
of identifiable causes (neoplastic, infectious, autoim- ogy community is crucial, as the majority of patients
mune, neurologic, ototoxicity), 90% of cases are idio- with SSNHL are first evaluated by nonotolaryngolo-
pathic.2–4 SSNHL affects 5 to 27 per 100,000 people gists, with 15,000 to 66,000 patients visiting family
annually, with about 66,000 new cases per year in the medicine clinics, urgent care centers, and emergency
United States.4–7 The incidence, debilitating conse- rooms for assessment of this complaint annually.7 To
quences of missed diagnosis and management, and our knowledge, this is the first report specifically
the scarcity of randomized controlled trials led the aimed at disseminating the guidelines for SSNHL
American Academy of Otolaryngology–Head and into the family medicine literature, with a goal of
Neck Surgery to develop a clinical practice guideline achieving broader adherence to the CPG.
(CPG) that was recently updated in 2019 to assist pro- The family physician’s role in recognizing and
viders in appropriately managing this condition.7,8 managing SSNHL is pivotal. In their role at the
frontline, family physicians can provide the highest
quality assessment, management, and patient cen-
This article was externally peer reviewed.
Submitted 6 May 2020; revised 25 July 2020; accepted 30 tered care. Timely recognition of SSNHL by the
July 2020. initial evaluating physician affects the availability of
From the University of Michigan Medical School, Ann
Arbor, MI (ADPP); Department of Otolaryngology–Head treatment options, as well as the success rates of
and Neck Surgery, University of Michigan, Ann Arbor, MI treatment. Improved awareness, clarity, and dis-
(EZS).
Funding: None. semination of CPGs is critical to improve adher-
Conflict of interest: None. ence and the quality of clinical care.7,10 We
Corresponding author: Andrew D. P. Prince, BA, University
of Michigan Medical School, 1301 Catherine St, Ann Arbor, describe a case report of a typical patient presenting
MI 48109 (E-mail: [email protected]). with SSNHL, followed by a summary of the

216 JABFM January–February 2021 Vol. 34 No. 1 http://www.jabfm.org

 J Am Board Fam Med: first published as 10.3122/jabfm.2021.01.200199 on 15 January 2021. Downloaded from http://www.jabfm.org/ on 13 July 2022 by guest. Protected by copyright.
current CPG to equip the frontline family physi- vol. 161, no. 2, pp. 195 to 210. Copyright © 2020
cians with tools for managing sudden hearing loss. by Andrew Prince. Reprinted by permission of
SAGE Publications, Inc.

Case Report
A 64-year-old man with hypertension presents to his Discussion
family medicine physician complaining of blocked Based on the CPG for SSNHL, the following
hearing in his left ear for 1 day. He noticed a loud approach is recommended, described in stepwise
ringing sound and the feeling of the left ear being fashion from the moment of patient presentation.
plugged. He has not had any dizziness, pain in his Figure 1 depicts the management schema.
ear, or drainage from his ear. He denies any recent
trauma, noise exposures, respiratory illness, change History and Physical
in medications, or other neurologic symptoms. When a patient presents with sudden hearing loss,
On physical examination, the left external ear and the CPG recommends (Grade C) a detailed history
ear canal are unremarkable. The left tympanic mem- to look for clinical features in patients with idiopathic
brane is normal in appearance and no definite middle SSNHL that may be associated with an underlying
ear effusion can be seen. A vibrating 512-Hz tuning disease such as vestibular schwannomas, stroke, noise,
fork placed on the midline of the forehead is per- and ototoxic medications (Table 1).12–15 Typical clini-
ceived louder in the right ear. Cranial nerve exami- cal features of SSNHL include the rapid development
nation is otherwise intact, and there are no other of unilateral hearing loss, a normal ear examination,
abnormalities noted on a complete head and neck ex- and associated clinical symptoms of a stuffy or full
amination nor focal neurological examination. ear, tinnitus, and dizziness.2,3 Patients may notice dif-
The family physician is concerned that the ficulty hearing on awakening, when hearing had been
patient may be experiencing SSNHL and discusses normal or at baseline the evening prior. Ipsilateral
the diagnosis, natural history, treatment options, tinnitus is very common, and dizziness is present in
and current evidence regarding this condition. The 30% to 60% of cases of SSNHL. If persistent and
patient and physician jointly decide to try a course bothersome both may portend a poorer prognosis,
of oral steroids as initial therapy, and the patient is and significant economic and psychological bur-
prescribed 60 mg prednisone daily for 10 days, fol- den.16–18
lowed by a 7-day taper. The family physician also There is a strong recommendation (Grade B) to
orders magnetic resonance imaging (MRI) brain/ distinguish between SSNHL and sudden conductive
internal auditory canal protocol with gadolinium or mixed hearing loss, as this will define potential
and places an urgent referral to an otolaryngologist treatments and prognosis.7 Delay in treatment of
for a hearing test and further evaluation. SSNHL often results when a clinician assumes that a
The otolaryngology office schedules the patient patient has conductive hearing loss (CHL) without
for an urgent audiogram and evaluation by an otolar- considering a diagnosis of SSNHL.2 Collecting a
yngologist the following day. The audiogram demon- good history as discussed above can help accurately
strates a significant drop in sensorineural hearing distinguish patients with SSNHL; however, some of
levels throughout the frequency spectrum. After dis- the associated symptoms including tinnitus, ear full-
cussion, the patient elects to complete his oral steroid ness, and vertigo may also be present in CHL.2,19,20
therapy before any further intervention is taken. He Patients with SSNHL often present with a chief
has a partial response to therapy 2 weeks after onset complaint of a “blocked ear,” which may bias the ex-
of symptoms, and he elects to undergo an intratym- aminer toward a perception of fluid in the middle
panic (IT) injection at this time. At followup 1 week ear. A focused head and neck physical examination
later, his hearing has returned to his baseline level. with tuning fork examination is required to differen-
tiate CHL from SSNHL.
CPGs for SSNHL Examination should include inspection of the
The guidelines discussed below were adapted from ear canals, visualization of the tympanic membrane,
S. Chandrasekhar, B. Tsai Do, S. Schwartz et al, and cranial nerve testing. Most causes of CHL can
Clinical Practice Guideline: Sudden Hearing Loss be diagnosed by otoscopy including cerumen
(Update), Otolaryngology–Head and Neck Surgery. impaction, foreign body impaction, middle ear

doi: 10.3122/jabfm.2021.01.200199 Sudden Sensorineural Hearing Loss 217

 J Am Board Fam Med: first published as 10.3122/jabfm.2021.01.200199 on 15 January 2021. Downloaded from http://www.jabfm.org/ on 13 July 2022 by guest. Protected by copyright.
Figure 1. Sudden sensory hearing loss management schematic. Abbreviations: TM, tympanic membrane; CT, com-
puted tomography; MRI, magnetic resonance imaging; ABR, auditory brainstem response; OD, once daily; Dex,
dexamethasone; Methylpred, methylprednisolone; HBOT, hyperbaric oxygen therapy; IT, intratympanic.

fluid, otitis externa, tympanic membrane abnor- is likely CHL in that ear. A complete history and tar-
malities, trauma, and cholesteatoma. Unlike CHL, geted physical examination with tuning fork testing
patients with SSNHL will almost always have a nor- are essential but do not supplant formal audiometric
mal otoscopic examination.7 The authors acknowl- testing.
edge, however, that definitive otoscopic diagnosis of
a middle-ear effusion may be challenging in some sit-
uations and encourage the addition of a tuning fork Labs, Imaging, and Audiometry
test to distinguish between CHL and SNHL when Though most SSNHL is idiopathic, there are
audiometric data are not readily available. In general, several other possible etiologies to consider. CPG
the tuning fork evaluations provide a reliable method guidelines strongly recommend against (Grade B)
to acutely assess the type of hearing loss and are sup- routine, “shotgun,” laboratory workup as the evi-
ported by the CPG.7,8 Figure 2 demonstrates proper dence supporting their use is limited to observatio-
tuning fork technique and result interpretation. nal and case-control studies.7 Within the literature
Ahmed et al,21 proposed the hum test as an alterna- most studies are underpowered and none support
tive to the Weber tuning fork test with similar sensi- that a laboratory test improves management or out-
tivity and specificity. This test can be used without a comes.8,22–24 Furthermore, labs have associated
tuning fork by asking the patient to hum; if he or she cost and potential harms related to false-positive or
hears one’s own hum louder in the affected ear, there false-negative results. Laboratory studies should be

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Table 1. Nonidiopathic Causes of Sudden Sensorineural Hearing Loss
Causes Diagnosis

Infections Herpesviruses, influenza, measles, mumps, rubella, human immunodeficiency virus, Lyme disease,
meningitis
Ototoxic drugs Aminoglycosides, vancomycin, erythromycin, loop diuretics, antimalarials, cisplatin, sildenafil, cocaine
Neoplasms Vestibular schwannoma, meningioma, intracranial metastases, lymphoma, leukemia, plasma cell dyscrasia
Trauma Head injury, barotraumas, noise exposure
Autoimmune disease Cogan’s syndrome, susac syndrome, systemic lupus erythematosus; rheumatoid arthritis, sjögren’s syndrome,
vasculitides
Vascular disorder Vertebrobasilar cerebrovascular accident or transient ischemic attack, cerebellar infarction, inner ear
hemorrhage
Various causes Meniere’s disease, otosclerosis, Paget’s disease, multiple sclerosis, sarcoidosis, hypothyroidism

limited to specific tests cued by pertinent findings address this, most, otolaryngology practices have
in the history or physical examination. a specific workflow to triage referrals for sudden
It is recommended (Grade C) that an audiomet- hearing loss and assure that these patients are
ric evaluation is obtained for SSNHL, or a referral seen promptly.
made to a physician that can obtain one, within Imaging is an important adjunct to SSNHL
14 days of presentation and for serial evaluations.7 workup and choosing the most effective modality is
Audiometry is the most reliable evaluation for dif- key. Routine computed tomography of the head is
ferentiating CHL from sensorineural hearing loss strongly recommended against (Grade B) in the initial
and establishes frequency-specific hearing and word assessment of patients with presumptive idiopathic
recognition ability. There may be concern regarding SSNHL without focal neurologic findings.7 A routine
the logistic ability for a patient to undergo an audio- head computed tomography scan is a very low-yield
metric evaluation within the specified time frame. To study for examination of the inner ear, and yet is often

Figure 2. Recommended technique and result interpretation for Weber and Rinne testing.

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 J Am Board Fam Med: first published as 10.3122/jabfm.2021.01.200199 on 15 January 2021. Downloaded from http://www.jabfm.org/ on 13 July 2022 by guest. Protected by copyright.
ordered.11 It is recommended (Grade B) to obtain an side effects.29 IT injections may be offered either as
MRI to evaluate for alternate pathologies involving primary therapy for SSNHL, in combination with
the pathway from the cochlea through the brainstem systemic steroids as primary therapy, or as salvage
commonly referred to as “retrocochlear pathology.”7 therapy after a patient has failed an initial course of
Vestibular schwannomas are the most frequent mass systemic steroids.7 The 2019 CPG does not make a
lesion discovered in cases of SSNHL, with an inci- specific recommendation on route of administration
dence of 2.5%.15 An MRI is the standard and we (systemic, IT, or combination therapy) for primary
advise the sensitive and widely available internal audi- treatment.7 This differs from salvage therapy, which
tory canal protocol MRI with gadolinium enhance- will be discussed separately.
ment.5,8,25,26 Auditory brainstem response (Grade C) If an IT steroid injection is selected, this proce-
may be offered for patients with SSNHL who do not dure is performed in the clinic setting by an otolo-
wish to have an MRI. However, a normal auditory gist or general otolaryngologist comfortable with
brainstem response is less sensitive than MRI and may the procedure. It can be performed at the initial
miss small tumors.8,27–29 visit to an otolaryngologist’s office, without the
need for special scheduling. This procedure is per-
Management formed under otomicroscopic guidance and entails
A shared decision-making approach to management direct topical anesthesia of the tympanic membrane
and developing a plan of care for patients with (no systemic sedation or anesthesia is administered),
SSNHL is important. There are a myriad of incon- followed by a controlled puncture of the tympanic
clusive or modestly beneficial treatment options membrane to instill a liquid steroid formulation to
ranging from observation to combination modality the middle ear space. IT injections provide a high
steroid therapy. The CPG strongly recommends concentration of steroid to the inner ear by diffusion
(Grade B) educating patients about the favorable through the round window membrane, while mini-
natural history, benefits, and risks of interventions, mizing systemic absorption.30,31 Multiple trials,
and the limitations of existing evidence.7 Despite reviews, and meta-analyses have yet to show any sig-
the intuitiveness of this recommendation, it is one of nificant difference in therapeutic outcomes between
the most frequently skipped by otolaryngologists and systemic therapy, IT, and combination therapy (with
nonotolaryngologists alike.11 We encourage practi- the exception of the Battaglia study,32 which sug-
tioners to reference the CPG, which provides a use- gested that initial therapy with IT steroids, either
ful patient education handout titled, “Frequently alone or in combination with oral steroids, conferred
Asked Questions/Patient Education.”7 benefit over systemic steroids alone).32–40 Ultimately,
A widely used treatment of idiopathic SSNHL is when you consider the devastating effects of SSNHL
administration of corticosteroids, either oral or IT. to patients, even a small possibility of hearing
The CPG provides the option (Grade C) to use corti- improvement makes corticosteroids a reasonable
costeroids as initial therapy to patients with SSNHL treatment to offer patients.
within 2 weeks of symptom onset.7 Historically, sys- If systemic steroids are chosen for initial treat-
temic steroids have been utilized by clinicians because ment, they should be given within 14 days. The rec-
steroid administration is one of the few treatment ommended dose of oral prednisone is 1 mg/kg/day
options that has shown efficacy, despite mostly equiv- in a single (not divided) dose, with the usual maxi-
ocal data.5,7,8,30,31 Systemic steroid therapy may be mum dose of 60 mg daily prescribed for 7 to 14 days,
contraindicated in cases of insulin-dependent or then tapered over a similar period of time.8,32,40 The
poorly controlled diabetes, tuberculosis, or peptic equivalent dose of prednisone (60 mg) is 48 mg for
ulcer disease. Treatment with corticosteroids seems to methylprednisolone and 10 mg for dexamethasone.7
offer the greatest recovery when initiated in the first Early treatment is of the utmost importance, and
2 weeks following SSNHL, with little benefit after 4 adequate initial dosing is important, with avoidance
to 6 weeks; this is similar to the timeline of spontane- of the methylprednisolone and prednisone dose
ous improvement in hearing, making it difficult to packs, which do not provide adequate steroid dose
conclude corticosteroids offer greater benefit than ob- for this indication.7 Serious side effects should
servation alone.2,5,6 IT steroid administration was first be closely monitored including blurred vision, glau-
described for SSNHL in 1996.28 IT steroid use is coma, weight gain, muscle weakness, osteoporosis,
increasing in popularity due to a reduction in systemic susceptibility to infections, worsening of diabetes,

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and hypertension. The CPG does not specify which measures.7 There are a variety of further manage-
provider should initiate steroid therapy. If the history ment options for SSNHL sequela that start with
and physical examination strongly suggest SSNHL, appropriate patient centered counseling and deci-
and there are no contraindications present, it is most sion making. Besides counseling, some patients
prudent for the initial provider to begin the treat- require therapeutic interventions such as speech
ment regimen, given the time-sensitive nature of reading, and auditory training.7 Further data-sup-
response-to-therapy. ported measures include hearing aids, hearing-
Among the other initial treatment options avail- assist devices, and cochlear implantation, which
able, the CPG strongly recommends against (Grade may rehabilitate hearing and quality of life.7,8,49,50
B) routine prescription of antivirals, thrombolytics,
vasodilators, or vasoactive substances to patients
with SSNHL.7 Studies examining antiviral, vasoac-
Conclusion
The family physician is often the initial provider to
tive, vasodilatory, and rheologic agents have been
interface with patients who present with SSNHL.
inconclusive, revealed no significant benefit, or pose
The family medicine provider plays a crucial role in
significant side effects.8, 41,42 Less frequently imple-
the early recognition and initial management of
mented in the United States is the option (Grade B)
to refer to a clinician who can provide hyperbaric SSNHL. A thorough history and physical examina-
oxygen therapy (HBOT) combined with steroid tion are the cornerstones of evaluation to differenti-
therapy within 2 weeks of onset, or as salvage ther- ate between sudden CHL and SNHL, and to assess
apy within 1 month of the onset of SSNHL.7 The for important neurologic diagnosis such as stroke.
therapy is not currently approved by the U.S. Food Prompt initiation of steroid therapy is recom-
and Drug Administration, but there have been mul- mended for patients with SSNHL, along with MRI
tiple trials and reviews that demonstrated potential imaging, audiology evaluation, and consultation
benefit.7,43,44 with an otolaryngologist. We encourage the family
When initial therapy fails, guidelines recom- physician to feel empowered to begin initial therapy
mend (Grade B) IT steroid therapy for incomplete with oral corticosteroids if their suspicion for
recovery from SSNHL 2 to 6 weeks after the onset SSNHL is high, even while awaiting formal audio-
of symptoms.7 Salvage therapy refers to failure of metric confirmation or referral to a specialist.
systemic or topical steroids, HBOT, or observation. To see this article online, please go to: http://jabfm.org/content/
Data suggests that IT steroids are an effective mo- 34/1/216.full.
dality for salvage therapy. Conflicting and weak
data exists for the use of systemic steroids and References
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doi: 10.3122/jabfm.2021.01.200199 Sudden Sensorineural Hearing Loss 223