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Primary Care Update

Brief Summaries for Clinical Practice

Sudden Hearing Loss:


Guideline for Diagnosis and Management

udden hearing loss can be


frightening for patients
and challenging for clinicians. One of the many
causes is sensorineural
hearing loss (SNHL); early recognition and treatment may improve the
likelihood of hearing recovery.
Recently, the American Academy of OtolaryngologyHead and
Neck Surgery Foundation published new guidelines on the diagnosis and management of sudden
hearing loss in adults; the focus of
the recommendations is on sudden
SNHL.1 Highlights of these guidelines are presented here.
BACKGROUND
Working definition. The guide-

line panel defined sudden SNHL as


a hearing loss of at least 30 dB that
affects at least 3 contiguous frequencies and occurs over a 72-hour
period.1 An abnormality of the cochlea, auditory nerve, or higher aspects of central auditory perception
or processing is involved.
Causes. Up to 90% of cases of
sudden SNHL are idiopathic at initial
presentation (Table); however, vascular, viral, or multiple causes are
typically implicated.2 Among the
causes of sudden SNHL that need to
be recognized and addressed early
are vestibular schwannoma (acoustic
neuroma), stroke, and malignancy.3
Epidemiology. The incidence
of sudden SNHL is reported to be
from 5 to 20 per 100,000 population;
some estimates are as high as 160
per 100,000.4,5 In the United States,
about 4000 cases occur each year.
EVALUATION
History. The first step is to dis-

tinguish SNHL from conductive


hearing loss (CHL) in a patient who
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april 2012

presents with sudden hearing loss.


Ask the patient about recent trauma, external ear and canal pain, ear
drainage, fever, and other systemic
symptoms.1 Patients with sudden
SNHL often report tinnitus, ear fullness or pressure, and vertigo; however, these symptoms may also be
associated with CHL.
Physical examination. Inspect
the ear canals and visualize the tympanic membranes. Causes of CHL include cerumen impaction, middle ear
fluid, otitis media, foreign bodies, perforated tympanic membrane, canal
edema from otitis externa, otosclerosis, trauma, and cholesteatoma.1 The
otoscopic examination typically reveals abnormalities in patients with
CHL, while otoscopic findings are
usually normal in those with SNHL.
Traditionally, the Weber and
Rinne tuning fork tests have been
used to distinguish SNHL from CHL.
Although the results of these tests
can be misleading,6,7 the guideline
panel recommends that tuning fork
tests be used to confirm audiometric
findings.
A Weber test is performed by
placing the fork on the patients
forehead; look for lateralization of

sound to one side. If the sound


lateralizes to the affected side, it is
likely that the hearing loss is conductive. If the sound lateralizes to
the opposite ear, the loss is probably sensorineural.
The Rinne test is done to compare bone conduction with air conduction for both ears. An abnormal
result of a Rinne test indicates a conductive hearing loss of at least 20 dB.
Patients with presumptive sudden SNHL should be assessed for
bilateral hearing loss, recurrent
episodes of sudden hearing loss,
and focal neurologic findings.
These clinical features can point to
an underlying cause of the hearing
loss, such as autoimmune conditions, metabolic disorders, bilateral
Meniere disease, and certain neurological disorders.
Diagnostic studies. A CT scan
of the head is not warranted during
the initial evaluation of patients with
sudden SNHL. The scan provides
no useful information that would affect initial management, and it poses
significant risks, including radiation
exposure and adverse effects of intravenous contrast. Reserve CT for
patients with focal neurological find-

Table Causes of sensorineural hearing loss


Cause

Examples

Infectious

Viral and bacterial infections (including Lyme disease)

Vascular

Stroke, other thromboembolic phenomena,


hypercoagulable states

Neoplastic

Vestibular schwannoma, malignancy

Inflammatory

Immune-mediated or in conjunction with other factors

Idiopathic

Data from Stachler RJ et al. Otolaryngol Head Neck Surg. 2012.1

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Primary Care Update


Sudden Hearing Loss:
Guideline for Diagnosis and Management

ings, a history of trauma, or chronic


ear disease.
The guideline panel also advises against routine laboratory tests
in patients with idiopathic sudden
SNHL. A specific test may be considered if the history indicates it
might be useful in identifying a potential cause of the hearing loss,
such as drawing Lyme titers in endemic regions.
Further workup. For patients
with idiopathic sudden SNHL, order
an MRI scan, auditory brainstem response (ABR), or audiometric follow-up to detect retrocochlear pathology. A small but significant percentage of these patients have an
underlying lesion, most often a vestibular schwannoma.1 In addition,
MRI can help identify other causes
of sudden SNHL, such as cochlear
inflammation or multiple sclerosis,
or it may reveal evidence of an underlying cause of the sudden SNHL,
such as small vessel cerebral ischemia. The overall percentage of MRI
abnormalities directly related to sudden SNHL ranges from 7% to 14%.8,9
Thus, MRI has the highest yield of
any diagnostic test in the setting of
sudden SNHL.1 If MRI is contraindicated (eg, in patients with pacemakers or other metallic implants), an alternative is a fine-cut CT scan of the
temporal bones with contrast.
The ABR test is highly sensitive
for vestibular schwannomas that are
larger than 1 cm; however, its reported sensitivity for smaller schwannomas varies from 8% to 42%.10-12 Consider the ABR test for the initial evaluation of older patients in whom the
consequences of a missed diagnosis
of a small tumor may be less worrisome than in younger patients.
While the guideline panel generally recommends MRI and ABR to
detect any underlying retrocochlear
pathology in patients with sudden
SNHL, serial audiometry is an option
in selected patients. For those who
have some residual hearing after the
initial episode of sudden SNHL, pro296

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april 2012

gression of hearing loss detected on


repeated hearing tests is suggestive
of retrocochlear pathology.
MANAGEMENT
Spontaneous recovery. Some

patients recover completely without


medical intervention, often within the
first 3 days; others regain their hearing slowly over a 1- to 2-week period.
The greatest spontaneous improvement in hearing occurs during the
first 2 weeks; late recovery is rare.
Corticosteroids. For patients
with idiopathic sudden SNHL, corticosteroids may be offered as initial
therapy. These agents have sites of
action in the inner ear, and they are
effective in the treatment of viral,
vascular, syphilitic, autoimmune, endolymphatic hydrops (Meniere disease), and other causes of hearing
loss. The recommended regimen
consists of oral prednisone given at
1 mg/kg/d in a single (not divided)
dose, with the usual maximum dose
of 60 mg/d, and treatment duration
of 10 to 14 days. Corticosteroid
therapy seems to be most effective
during the first 2 weeks after the episode of sudden hearing loss; little
benefit is seen after 4 to 6 weeks.
Consider intratympanic corticosteroid perfusion for patients in
whom systemic corticosteroids are
ineffective. The corticosteroid may
be delivered by a needle through
the tympanic membrane, or it may
be placed into the middle ear
through a tympanostomy tube or a
myringotomy. The intratympanic
route avoids the significant adverse
effects of additional systemic corticosteroid therapy.
Other treatments. Hyperbaric
oxygen therapy may be beneficial as
an adjuvant intervention if this treatment is started within 3 months of
the onset of idiopathic sudden
SNHL. The greatest benefit has
been seen in patients with severe to
profound hearing loss.1
Antivirals, thrombolytics, vasodilators, vasoactive substances, and

antioxidants have no role in the treatment of idiopathic sudden SNHL


because their effectiveness has not
been demonstrated in this setting.1
FOLLOW-UP

Within 6 months of the diagnosis of idiopathic sudden SNHL, order


follow-up audiometric evaluation. If
the patients hearing loss is permanent, auditory rehabilitation may be
required. Counsel patients who have
residual hearing loss about the potential benefits of hearing aids and
assistive listening devices.
A list of organizations that
provide information and support
for patients with hearing loss can
be found on the web site of the
NIH National Institute on Deafness
and Other Communication Disorders: http://www.nidcd.nih.gov/
directory/.
n
REFERENCES:

1. Stachler RJ, Chandrasekhar SS, Archer SM,


et al. Clinical practice guideline: sudden hearing
loss. Otolaryngol Head Neck Surg. 2012;146:S1.
DOI: 10.1177/0194599812436449
2. Rauch SD. Clinical practice: idiopathic sudden
sensorineural hearing loss. N Engl J Med. 2008;
359(8):833-840.
3. Saunders JE, Luxford WM, Devgan KK,
Fetterman BL. Sudden hearing loss in acoustic
neuroma patients. Otolaryngol Head Neck Surg.
1995;113(1):23-31.
4. Byl FM Jr. Sudden hearing loss: eight years
experience and suggested prognostic table.
Laryngoscope. 1984;94(5, pt 1):647-661.
5. Klemm E, Deutscher A, Mosges R. A present
investigation of the epidemiology in idiopathic
sudden sensorineural hearing loss [in German].
Laryngorhinootologie. 2009;88(8):524-527.
6. Miltenburg DM. The validity of tuning fork tests
in diagnosing hearing loss. J Otolaryngol. 1994;23
(4):254-259.
7. Browning GG, Swan IR, Chew KK. Clinical role
of informal tests of hearing. J Laryngol Otol.
1989;103(1):7-11.
8. Schick B, Brors D, Koch O, Schafers M, Kahle
G. Magnetic resonance imaging in patients with
sudden hearing loss, tinnitus and vertigo. Otol
Neurotol. 2001;22(6):808-812.
9. Cadoni G, Cianfoni A, Agostino S, et al. Magnetic
resonance imaging findings in sudden sensorineural hearing loss. J Otolaryngol. 2006;35(5):310-316.
10. Chandrasekhar SS, Brackmann DE, Devgan
KK. Utility of auditory brainstem response audiometry in diagnosis of acoustic neuromas. Am J Otol.
1995;16(1):63-67.
11. El-Kashlan HK, Eisenmann D, Kileny PR.
Auditory brain stem response in small acoustic neuromas. Ear Hear. 2000;21(3):257-262.
12. Schmidt RJ, Sataloff RT, Newman J, Spiegel JR,
Myers DL. The sensitivity of auditory brainstem
response testing for the diagnosis of acoustic neuromas. Arch Otolaryngol Head Neck Surg. 2001;127
(1):19-22.

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