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SNHL Diagnosis and Treatment Dr Mandana Amiri Otolaryngologist KUMS Why is hearing very important? Means to recognize the world Foundation to develop speech Affection sharing, life enjoying Important means of communication Level of Hearing loss Level dB HL Normal ≤25dBHL Mild 26~40dBHL Moderate 41~70dBHL Severe 71~90dBHL Profound ﹥90dBHL Ear Anatomy It’s a Miracle How We Hear Semi Circular Canals Ossicles Nerve Ear Drum Cochlea Developed by HLA-Wake County, Raleigh, NC Mechanism of Hearing Mechanism of Hearing Mechanism of Hearing Sensorineural Hearing Loss Clinical Evaluation Of The Patient With Hearing loss 1) History 2) Physical Examination 3) Audiometric Testing: Audiometry ABR ECG OAE 4) Vestibular Testing 5) Laboratory Testing 6) Radiologic Testing Etiology Of SNHL 1) Developmental And hereditary 2) Infectious disorder 3) Pharmacologic Toxicity 4) Renal disorders 5) Trauma 6) Irradiation 7) Neurologic Disorders & Neoplaasms 8) Vascular and Hematologic 9) Immune disorder 10) Bone disorder 11) Endocrine and Metabolic 12) Unknown Developmental And hereditary 1) 2) 3) 4) 5) 6) Non syndrome hereditary hearing loss Warrensburg Syn Alport Syn Ushler Syn Inner Ear Anomalies Large Vestibular Aqueduct Syn LVA Synd Infectious disorders 1) 2) 3) 4) 5) 6) Labyrinthitis Otitis Media Viral Infection Syphilis Rocky mountain Spotted fever Lyme Disease Clinical characteristics of ototoxic deafness Bilateral hearing loss Hearing loss happens at high frequency Reversible or progressive With tinitus, vertigo Pharmacologic Toxicity 1) Aminoglycosides 15) Dwferoxamine 2) Ototo[pical preparation 16) Lipid Lowering 3) Loop Diuretics 4) Antimalaria 5) Salicylates 6) NSAIDs 7) Analgesic and Anal/Narco 8) PDEs Inhibitors 9) Vancomycin 10) Erythromycin 11) Cisplatin And Carboplatin 12) Nitrogen Mustards 13) Vincristin and Vinblastin 14) Eflornithine ANTIBIOTICS WITH GOOD EVIDENCE FOR OTOTOXICITY Drug Vestibulotoxicity Erythromycin Hearing Toxicity Toxic Level yes High IV doses only Usually 2 weeks Gentamicin 8.6% minor Streptomycin very toxic minor dihydrostreptomicin minor toxic very toxic Tobramycin Yes minor in 6% Netilmicin 2.4% Amikacin not toxic 13.9% Neomycin minor very toxic Kanamycin minor very toxic Etiomycin moderate Vancomycin nontoxic none to moderate Metronidizole toxic (rarely) unknown Capreomycin Less toxic than Gentamicin In topical ear drops synergistic with gentamicin yes Table from: http://www.tchain.com/otoneurology/disorders/bilat/ototoxins.html See other classes of ototoxic drugs on the same web site. Ototoxic drug (more than 90) Aminoglycoside antibiotics Antitumor drug- cisplatin, carboplatin Diuretic- furosemide, ethacrynic acid Salicylate-aspirin antimalarial drug- quinin Renal disorders 1) 2) 3) 4) 5) Genetic cause (Alport) CRF Hemodialysis Renal Transplantation Electrolyte and Metabolic Abn Trauma 1) Head Injury : Fracture (L&T) Blunt 2) NIHL 3) Acoustic Trauma 4) Barotraumas: Flying , Diving ,….. 5) Perilymphatic Fistula Congenital : Mondini Synd Acquired : barotraumas, stapedectomy Causes of NIHL Continuous Noise exposure The extent of hearing loss increases with time of exposure, and also increases with increasing the intensity of sound levels to which an employee is exposed Greatest amount damage occurs in the first 10-15 years Most scientific evidence suggests that the hearing loss does not progress once exposure to noise has discontinued National Occupational Health and Safety Commission (NOHSC) standard identifies a continuous exposure level of 85dB(A) over 8 hrs and a maximum peak exposure level 140 dB(C) Effect of NIHL NIHLs commonly appear as a sloping loss that is most prominent in the higher frequencies (4k) Effects speech perception by reducing perception of consonant sounds (p,b,k,s,z etc) needed for speech clarity. Vowels usually remain intact as there is often good residual hearing throughout the lower frequencies. Result = clients report that they can hear people speaking but not understand them – the vowels come through clearly but the important consonants are distorted. Progression of NIHL ( 0 – 5 years) Normal Hearing Mild high frequency loss = 0% = 4.6% (40yo), 0% (70yo) Progression of NIHL (10 – 20 years) Moderate high frequency loss Moderate to severe high frequency loss = 16.8% (40yo), 11.9 (70yo) = 23.8% (40yo), 18.9 (70yo) Progression of NIHL (25 + years) Moderate to Profound high frequency loss = 67.4% (40yo), 62.5% (70yo) Ear Anatomy Clinical characteristics of acoustic neuroma Unilateral progressive hearing loss, tinnitus Acoustic Neuroma Irradiation Doses: >45 Gy Latancy can be 0.5 to 2 year Radiosurgery Risk Factor: Aging preexisting HL Adjuvant Ototoxic Neurologic Disorders 1) M.S 2) BIH (Pseudo tumor Cerebri ) Vascular and Hematologic disorders Migraine Vertebrobasilar arterial occlusion Rheological Disorders and blood Dyscrasia Presbycusis • Age related H.L • 30% of >65 • High Freq & Severe in men • Four Pathologic Types: Sensory Pr Neural Pr Strial Pr Mechanical Pr Normal Ear Ear w/ Endolymphatic Hydrops Note bulging of membranous labyrinth (www.tchain.com/otoneurology/disorders/menieres/men_eti.html) Age and Sex Distribution: MD is an Equal-Opportunity Disease Moral: The disease strikes all ages and both sexes. Meniere & Endolymphatic Hydrops • Unpredictability & Variability • Low Freq • 30% Bilateral Delayed E.H Synd Syphilis Trauma of Temp Bone Serous Laby Stapedectomy AID SSNH • • • • • • • • Awaking with it or at over 12hours 5 to 20 per 100000/year 2% to 3% of ENT visits Peak in Sixth decade Male=Female Bilateral is rare Aural fullness, Tinnitus, Vertigo 40% Prognosis: 1-Severiy of loss 2-Aduiogram shape 3-Vertigo 4-SDS 5-Age -Recovery is 30% to 65% Etiology of SSNHL • Infectious Dis Viral: mumps,measles,rubella,CMV,HZV,HSV Meningitis Syphilis: 2%> Lyme Dis Aids • Neoplasms • Trauma Acustic N: 10.2% Mild & high Freq All patients should MRI Head Inj: Hemorrhage, mild loss of H.C Prilymphatic Fistula: congenital,stapedctomy, barotrauma Intracochlear M.B Etiology of SSNHL • Pharmacologic Toxicity • Immunologic Dis • Vascular Dis: PCA occlusion Migraine Sickle Dis Macroglobulinemia Buerger Dis Cardiac Surg Spinal Surg Treatment of SSNHL • Apparent Etiology: AB Withdrawal of drugs • Idiopathic etiology: Steroids: oral ,Intrathympanic Improve blood & oxygen histamine infusion papaverin, nicotinic acid carbogen Dextran, Manitol pentoxify line heparin Iodinated contrast Treatment of SSNHL • 10 days couse of prednisolone • 1mg/kg/day then audiometry • Valacyclovir 1000mg/TDS/10 days • Sodium Restriction (2 gr) • Diuretics • carbogen