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

Epidemiology
Incidence: 5 to 20 cases /100.000 Male = Female More on left ear ??? Bilateral loss: 1% - 2% Age at presentation: 4054 years

Etiology
Defined Cause Idiopathic: >>>

Defined Cause of SSNHL

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

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

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, Temporal arteritis, Polyarteritis nodosa

Diagnosis
History
Onset, time course, associated symptoms Risk factor, past medical history Medication

PE
Complete H & N examination Pneumaotoscopy: find for fistula sign

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)

Diagnosis 3
Imaging study
MRI with contrast (Acoustic Neuroma) CT Scan (Mondini, LVA)

Lab Test

Treatment
90% of cases will be Idiopathic Treat known causes by addressing the underlying condition

Treatment
Therapy for ISSNHL is controversial Difficult to study
High spontaneous recovery rate Low incidence Makes validation of empiric treatment modalities difficult

Treatment
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
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

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

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 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

Wilson

Evidence Based 1
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

Evidence Based 2
Rheologic agents
LMW dextrans or Pentoxifylline did not demonstrate recovery rates better than placebo

Evidence Based 3
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

Evidence Based 4
Antiviral Multicenter, randomized, prospective, double-blind 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

Evidence Based 5
Triiodobenzoic acid derivatives
No significant difference in recovery using diatrizoate in a multidrug regimen, compared with spontaneous recovery rates Wilkins 87

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

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

Wilson (1980)

 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