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Sudden Deafness

Definition
30 decibel (dB) loss over three contiguous
frequencies occurring within 3 days
Abrupt and rapidly progressive losses
Awakening with it in the morning or developing a
progressive loss over 12 hours or less

Vascular Compromise
AICA
No collateral vasculature
Cochlear function: sensitive to changes in blood
supply
Thrombosis, Embolus, Reduced blood flow,
Vasospasm

Epidemiology
Incidence: 5 to 20 cases /100.000
Male = Female
More on left ear ???
Bilateral loss: 1% - 2%
Age at presentation: 40-54 years
Etiology
Defined Cause
Idiopathic: >>>

Intracochlear Membrane Rupture


Rupture of intracochlear membranes would allow
mixing of perilymph and endolymph, effectively
altering the endocochlear potential
Immune inner ear disease
Progressive hearing loss
Cogan's syndrome, SLE,
Polyarteritis nodosa

Idiopathic SSNHL
Viral infection
Vascular compromise
Intracochlear membrane rupture
Immune inner ear disease
Viral infection
History of recent viral infection
28% report a viral-like upper respiratory infection
within 1 month
Recent viral seroconversion
Increased viral titers
Pathologic changes:
Loss of hair cells, supporting cells, atrophy of the
tectorial membrane, atrophy of the stria vascularis,
and neuronal loss
Viral infection can be implicated as a cause of
ISSHL, but it cannot as yet be proved
Mumps, Arenavirus, Measles, Rubella, Herpes
Zoster oticus, Mononucleosis

Temporal

arteritis,

Diagnosis
History
Onset, time course, associated symptoms
Risk factor, past medical history
Medication
PE
Complete H & N examination
Pneumaotoscopy: find for fistula sign
Ancillary Procedure
Audiometric testing (PTA, Speech Audiometry,
OAE, ABR, Tympanometry)
VNG (if vestibular symptoms and/or signs are
present)
Lab
Imaging study
MRI with contrast (Acoustic Neuroma)
CT Scan (Mondini, LVA)
Imaging study
MRI with contrast (Acoustic Neuroma)
CT Scan (Mondini, LVA)

Corticosteroids
The mechanism of action of corticosteroids is
unknown
Reduction of cochlea and auditory nerve
inflammation is the presumed pathway

Antiviral Agents
Acyclovir, Amantadine, Famciclovir, Valacyclovir
Viral etiology

Lab

Diuretics
Cochlear endolymphatic hydrops
The mechanism of action is not understood.
Triiodobenzoic Acid Derivatives
Diatrizoate meglumine (angiographic contrast
agent)
Affect the stria vascularis and assist in maintaining
the endocochlear potential
Surgery
Repair of oval and round window perilymph fistulae

Treatment
90% of cases will be Idiopathic
Treat known causes by addressing the underlying
condition
Therapy for ISSNHL is controversial
Difficult to study
High spontaneous recovery rate
Low incidence
Makes validation of empiric treatment modalities
difficult
Vasodilators
Rheologic agents
Antiinflammatory agents
Antiviral agents
Diuretics
Triiodobenzoic acid derivatives
Surgery
Vasodilators
Improve blood supply to cochlea
Reversing hypoxia
Histamine, Nicotinic acid, Papaverine, Procaine,
Niacin
Carbogen inhalation(5% carbon dioxide and 95%
oxygen)
Rheologic Agents
Altering blood viscosity to improve blood flow and
oxygen delivery
LMW Dextrans, Pentoxifylline
Heparin, Warfarin
Dextrans
hyper-volemic hemodilution and affect Factor VIII
Antiinflammatory Agents

Results
Recovery rates: 47% - 63%
Mattox & Simmons
complete recovery: PTA < 10 dB or equaling the
uninvolved ear
good recovery: PTA < 40 dB or > 50 dB
improvement from the initial audiogram
Wilson
complete recovery: Recovery to within 10 dB of
the prehearing loss speech reception threshold
(SRT) or PTA
Partial recovery was defined as recovery to
within 50% of the prehearing loss SRT or PTA
Evidence Based
Vasodilator
Several studies using vasodilator therapy as a
component of treatment failed to show significant
differences from placebo
Based on controlled studies, little data support
vasodilator therapy
Rheologic agents
LMW dextrans or Pentoxifylline did not demonstrate
recovery rates better than placebo
Steroid
61% (oral steroids) vs 32% (placebo)
Transtympanic steroid: high delivery concentration
to the inner ear and low systemic concentrations
Differences in delivery technique, corticosteroid,
dose, and dosing schedule, direct comparisons
are difficult
large, randomizied, prospective, blinded study is
warranted for this treatment
Antiviral
Multicenter, randomized, prospective, doubleblind trial comparing prednisolone against
prednisolone and acyclovir did not show a

significant beneficial effect of acyclovir


Stokroos 98
No significant benefit from the addition of
valacyclovir to concurrent oral prednisone
therapy in a larger multicenter, randomized,
prospective trial - Tucci 2002
Triiodobenzoic acid derivatives
No significant difference in recovery using
diatrizoate in a multidrug regimen, compared with
spontaneous recovery rates
Wilkins 87
Repair of perilymphatic fistulae
A universal standard for positive identification of a
fistula has not been achieved
Without uniform standards, outcomes of surgical
repair are difficult to compare
Prognostic factor
Wilson (1980)
Vertigo not statistically significant
Age less than 40 years favorable for recovery
Type of audiogram
Midfrequency loss with best recovery
Profound loss less likely to have recovery
Loss between 40 dB 85 dB more likely to respond
to steroid therapy
Cinamon (2001)

Low frequency loss improved more


High frequency loss improved less
Patients without vertigo have better outcome

Four prognostic variables


Time since onset
Audiogram type
Vertigo
Age