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SSNHL By Saisuree Nivatwongs ENT PMK SSNHL • • • • • • • • Incidence: 5-20 per 100,000 4,000 new cases/year in US Idiopathic Hearing loss at least 30 dB over 3 contiguous frequency Onset of hearing loss occurs in less than 72 hours Recovery rate without treatment 32% - 79% – Usually within 2 weeks of onset – Only 36% with complete recovery No middle ear disease Otologic emergency! SSNHL • Clinical Presentation – Sudden onset hearing loss • Less than 3 days • • Left side possibly more common (55%) Bilateral 2% – Usually unilateral – – – – – – – Median age 40-54 Male = Female Awakening from sleep Hearing a “popping” prior to hearing loss Aural fullness Tinnitus Vertigo Etiology Viral infection • Association of SSNHL with viral URI in 25% - 63% • Serology confirming active viral infection – HSV, VZV, CMV, influenza, measles parainfluenza, rubeola, mumps, rubella – Immunoreactivity against virus • Histopathology of human temporal bones – Atrophy of organ of Corti, spiral ganglion, tectorial membrane – Hair cell loss – Unraveling of myelin Etiology Vascular injury • Sudden onset suggesting infarction – Perlman (1959) demonstrated loss of cochlear microphonic 60 seconds after occlusion of labyrinthine artery in guinea pig – Polycythemia ,Buerger’s, macroglobulinemia, sickle cell, fat embolism, DM Etiology Intracochlear membrane rupture • Loss of endocochlear potential due to mixing of endolymph and perilymph • Gussen (1981) histologic evidence • Fallen out of favor Autoimmune cross-reacting circulation Ab DDX – – – – – – – Infectious • Bacterial: meningitis, labyrinthitis, syphilis • Viral: Mumps, CMV Inflammatory • Autoimmune, Cogan syndrome, Lupus, MS Traumatic • Temporal bone fracture, acoustic trauma, perilymph fistula Neoplastic • CPA tumor, temporal bone metastasis Toxic • Aminoglycosides, aspirin Vascular • Thromboembolism, macroglobulinemia, sickle cell disease, cerebral infarct, TIA Congenital • Mondini malformation, enlarged vestibular aqueduct Clinical Evaluation • Hx • Complete ENT exam • Audiogram include PTA, SRT, SDS • Tympanogram • ABR Radiograph • MRI with Gd 0.8-2 %of pt with SSNHL have been diagnosed CPA /IAC tumors • Non-contrasted CT temporal bone : R/o congenital malformation Laboratory Evaluation – CBC with diff • Polycythemia, leukemia, thrombocytosis – FBS , Electrolytes – Erythrocyte sedimentation rate (ESR) – FTA-Abs (Syphilis) – Coagulation profile – Thyroid function testing – Lipid profile – BUN, Creatinine – UA – ANA , rheumatoid factor , viral study Prognosis • • • • Severity of HL Audiogram shape Presence of vertigo Age • Without Rx : 30-65 % will experience complete or partial recovery Medical Rx • • • • Bed rest Low salt diet < 2 gm/day Diuretics : HCTZ Steroids Steroids • Historical perspective: Reduce inner ear inflammation • Rx as early as posible • Oral, IV 10 days • Cannot be used for all patients – Diabetics, ulcers, TB, glaucoma – Intratympanic steroids Medical Rx – Antivirals – Volume expanders : Dextran , hypaque – Vasodilators : Histamine phosphate , Ca antagonist , nicotinic acid – Anticoagulants : heparin – Carbogen inhalation (95%O2,5%CO2) SSNHL • Cochrane Database of Systematic Reviews – Wei (2003, Updated 2006): Steroids for idiopathic sudden sensorineural hearing loss – Only 2 prospective, double-blind, randomized, controlled trials evaluating therapy of SSNHL Intratympanic steroid • • • • Administration of steroids to middle ear round window niche/membrane directly targeting the inner ear Very little systemic absorption – May benefit patients for whom systemic steroids are contraindicated Higher concentration to end organ May salvage hearing loss when nonresponsive to systemic steroids Advantage of IT steroids • • • • • May be used when systemic steroids are contraindicated or refused Greater concentration achieved at target end organ May be performed in outpatient setting Possible use for salvage of hearing Relatively low complication rate Take Home Messages: – SSNHL is an otologic emergency – Systemic steroids are mainstay of therapy – Better prognosis if treatment started early (within 4 weeks of onset) – IT steroids may be an alternative when systemic steroids are contraindicated – IT steroids is another option when oral steroids fail to restore hearing Thank you for your attention