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Sudden Deafness
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Definition
30 decibel (dB) loss over threecontiguous frequencies occurringwithin 3 days
Abrupt and rapidly progressivelosses
Awakening with it in the morningor developing a progressive lossover 12 hours or less
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Epidemiology
Incidence: 5 to 20
cases /100.000
Male = Female
More on left ear???
Bilateral loss: 1%
- 2% Age at
presentation: 40-
54 years
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Etiology
Defined Cause
Idiopathic: >>>
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Defined Cause of SSNHL
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Idiopathic SSNHL
Viral infection
Vascular compromise
Intracochlear membrane rupture Immune inner ear disease
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Viral infection
History of recent viral infection
28% report a viral-like upperrespiratory 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
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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
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Vascular Compromise
AICA
No collateralvasculature
Cochlearfunction:sensitive tochanges in blood
supply Thrombosis,
Embolus,Reduced blood
flow, Vasospasm
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Intracochlear Membrane
Rupture
Rupture of intracochlear
membranes would allow mixing of
perilymph and endolymph,
effectively altering theendocochlear potential
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Immune inner ear disease
Progressive hearing loss
Cogan's syndrome, SLE, Temporal
arteritis, Polyarteritis nodosa
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Diagnosis
History
Onset, time course, associated
symptoms
Risk factor, past medical history
Medication
PE
Complete H & N examination
Pneumaotoscopy: find for fistula
sign
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Diagnosis 2
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)
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Diagnosis 3
Imaging study
MRI with contrast
(Acoustic
Neuroma)
CT Scan (Mondini,
LVA)
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Lab Test
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Treatment
90% of cases will be Idiopathic
Treat known causes by
addressing the underlying
condition
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Treatment
Therapy for ISSNHL is
controversial
Difficult to study
High spontaneous recovery rate
Low incidence
Makes validation of empiric
treatment modalities difficult
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Treatment
Vasodilators
Rheologic agents
Antiinflammatory agents Antiviral agents
Diuretics
Triiodobenzoic acid derivatives Surgery
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Vasodilators
Improve blood supply to cochlea
Reversing hypoxia
Histamine, Nicotinic acid,Papaverine, Procaine, Niacin
Carbogen inhalation(5% carbon
dioxide and 95% oxygen)
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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
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Antiinflammatory Agents
Corticosteroids
The mechanism of action of
corticosteroids is unknown
Reduction of cochlea and auditory
nerve inflammation is the
presumed pathway
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Antiviral Agents
Acyclovir, Amantadine,
Famciclovir, Valacyclovir
Viral etiology
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Diuretics
Cochlear endolymphatic hydrops
The mechanism of action is not
understood.
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Triiodobenzoic Acid
Derivatives
Diatrizoate meglumine
(angiographic contrast agent)
Affect the stria vascularis and
assist in maintaining the
endocochlear potential
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Surgery
Repair of oval and round window
perilymph fistulae
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Results
Recovery rates: 47% - 63% Mattox & Simmons
complete recovery: PTA < 10 dB orequaling the uninvolved ear
good recovery: PTA < 40 dB or > 50 dBimprovement from the initial audiogram
Wilson complete recovery: Recovery to within 10
dB of the prehearing loss speech receptionthreshold (SRT) or PTA
Partial recovery was defined as recovery towithin 50% of the prehearing loss SRT orPTA
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Evidence Based 1
Vasodilator:
Several studies using vasodilator
therapy as a component of treatment
failed to show significant differencesfrom placebo
Based on controlled studies, littledata support vasodilator therapy
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Evidence Based 2
Rheologic agents
LMW dextrans or Pentoxifylline did
not demonstrate recovery rates
better than placebo
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Evidence Based 3
Steroid
61% (oral steroids) vs 32% (placebo)
Transtympanic steroid: high delivery
concentration to the inner ear andlow 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
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Evidence Based 4
Antiviral
Multicenter, randomized, prospective,
double-blind trial comparing prednisolone
against prednisolone and acyclovir did not
show a significant beneficial effect ofacyclovir - Stokroos 98
No significant benefit from the addition of
valacyclovir to concurrent oral prednisonetherapy in a larger multicenter,
randomized, prospective trial - Tucci 2002
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Evidence Based 5
Triiodobenzoic acid derivatives
No significant difference in recovery
using diatrizoate in a multidrug
regimen, compared withspontaneous recovery rates
Wilkins 87
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Evidence Based 5
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
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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 torespond to steroid therapy
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Wilson (1980)
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Cinamon (2001)
Low frequency loss improved more
High frequency loss improved less
Patients without vertigo have better
outcome
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Four prognostic variables
Time since onset
Audiogram type
Vertigo
Age