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SNHL Saisuree Nivatwongs ENT-PMK Sensorineural hearing loss Introduction • • • • • • History Physical examination Auditory testing Vestibular testing Laboratory testing Radiographic testing Sensorineural hearing loss Etiology • Development & Hereditary disorder • Infectious disorder • Pharmacologic disorder • Trauma • Neurologic disorder • Vascular & Hematologic disorder • • • • Immune disorder Bone disorder Neoplasms Endocrine & Metabolic disorder • Disorder of unknown etiology **Sudden Sensorineural hearing loss** History • • • • • • Unilateral / Bilateral Chronicity Tinnitus / Vertigo Otalgia / Otorrhea Headache Eye symptoms • • • • • Underlying disease Ototoxic drugs Hx of trauma Noise exposure Family Hx Physical Examination • • • • • Weber / Rinne test Otoscopy Cranial nerve Stigmata of associated disease Generally no abnormality** Auditory Testing • Conventional audiometry • Tympanometry • Acoustic reflex threshold • Auditory brainstem response • Electrocochleography • Otoacoustic emission Clinical evaluation Laboratory testing • Fluorescent treponemal antibody absorption test : FTA-ABS • Microhemagglutination test for Treponema pallidum : MHA-TP • Venereal disease research laboratory : VDRL • Routine hematologic studies • Routine metabolic studies Vestibular testing • Adjunct in selected patients Radiographic testing • MRI with Gadolinium Retrocochlear hearing loss? • Computed tomography Labyrinthine abnormality? Etiology • • • • • • • • • • Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology Development & Hereditary disorder • Waardenburg syndrome • Large vestibular aqueduct syndrome • Usher syndrome • Alport syndrome Etiology • • • • • • • • • • Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology Infectious disorder Labyrinthitis • • • • • • Serous labyrinthitis Abnormal process within the labyrinth Endolymphatic hydrops Hearing loss and vestibular dysfunction Permanent or transient Sudden onset of sensorineural hearing loss and acute vertigo Viral labyrinthitis is common • • • • • Suppurative labyrinthitis Bacterial invasion of the inner ear Profound hearing loss and acute vertigo Caused by a fistula between the middle ear and the labyrinth Alternatively, the route of invasion can be meningogenic Most common etiology of deafness associated with meningitis Infectious disorder Herpes zoster oticus • Varicella-zoster infection • Most commonly associated with facial paralysis • HL and vertigo can occur Infectious disorder Measles • Not uncommon cause of deafness in children • Bilateral HL • Moderate-to-profound HL • Vestibular function can be similarly affected Mumps • Paramyxovirus infection • Unilateral SNHL • Unilateral deafness in otherwise healthy children • Sudden deafness in adult Subclinical mumps infection in those without previous immunity Infectious disorder Cytomegalovirus • Common cause of congenital and progressive HL in children • Sudden SNHL in adults • Hearing loss associated with AIDS may represent reactivation of latent CMV infections Infectious disorder Syphilis • • • • Congenital or acquired syphilis 80% Symptomatic neurosyphilis HL in syphilis Meningolabyrinthitis Syphilitic HL Indistinguishable from Ménière’s disease • Hennebert’s sign (a positive fistula test without middle ear disease) • Tullio’s phenomenon (vertigo or nystagmus on exposure to highintensity sound) Etiology • • • • • • • • • • Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology Pharmacologic disorder Aminoglycosides Streptomycin, Kanamycin, Neomycin, Amikacin, Gentamicin, Tobramycin, and Netilmycin Death of the hair cell Different patterns of ototoxicity with different aminoglycosides Unilateral or asymmetric Reversibility of the HL Risk factors (1) presence of renal disease (2) longer duration of therapy (3) increased serum levels (4) advanced age (5) concomitant administration of other ototoxic drugs Ototopical preparations Neomycin, Gentamicin, and Tobramycin-containing Cochlear or vestibular ototoxicity Avoid the use of aminoglycosidecontaining topical preparations in uninflamed ears with tympanic membrane perforations Ingredients of ototopical preparations also have ototoxic potential Polymyxin B, Propylene glycol, Acetic acid, Antifungal agents Pharmacologic disorder Loop diuretics Effect by blocking sodium and water reabsorption in the proximal loop of Henle Reversible SNHL Bilateral and symmetric Sudden in onset Alteration of endolymphatic ion concentration and endocochlear potential Risk factors (1) Renal failure (2) Rapid infusion (3) Aminoglycoside administration Antimalarials Quinine Tinnitus, SNHL, & Visual disturbances Chincinonism Tinnitus, headache, nausea, and disturbed vision Quinine appears to be primarily on hearing and usually is transient Permanent hearing loss may occur with large doses or in sensitive patients Pharmacologic disorder Salicylates Aspirin Tinnitus and reversible SNHL HL Dose-dependent Moderate-to-severe range SNHL, loss of otoacoustic emissions, reduced cochlear action potentials Alteration of the “tips” of auditory nerve fiber tuning curves Alteration in turgidity and motility of outer hair cells Nonsteroidal antiinflammatory drugs Naproxen, Ketoralac & Piroxicam Ototoxicity resulting from use of NSAIDs is rare Only reversible physiologic changes, without major morphologic changes Pharmacologic disorder Vancomycin Almost received Vancomycin & loop diuretics or aminoglycosides Ototoxicity Intravenously Permanent or transient SNHL Excreted by the kidney Renal failure Vancomycin half-life Increase ototoxicity Itself ototoxic Unclear Erythromycin Uncommon Partially Intravenously Reversible on discontinuation No reports - Newer macrolide - Clarithromycin - Azithromycin Pharmacologic disorder Cisplatinum Nitrogen mustards Antineoplastic agents Mechlorethamine has Serious ototoxicity Limited use Severe toxic Shrinkage of the organ of Corti Loss of inner and outer hair cells Cell-cycle nonspecific cancer chemotherapeutic agent Dose-limiting SNHL Adults (25% to 86%) Children (84% to 100%) Bilateral / Irreversible Tinnitus or vertigo HL Dose-related Progressive outer hair cell loss Inner hair cells, neural structures and the stria vascularis are affected Pharmacologic disorder Vincristine and vinblastine The vinca alkaloids Potent neurotoxicity Peripheral neuropathy Cranial neuropathies, ataxia, and hearing loss Loss of hair cells and primary auditory neurons Eflornithine Drug treatment of trypanosomiasis Some Pneumocystis carinii pneumonia, Cryptosporidiosis, Leishmaniasis, and Malaria Cause major and doserelated SNHL Pharmacologic disorder Deferoxamine Iron-chelating agent Auditory and visual neurotoxicity Particularly with larger doses in younger patients The SNHL is reversible in some patients when the dosage is reduced Lipid-lowering drugs Wallerian-like degeneration High doses of HMG-CoA reductase inhibitors Optic& vestibulocochlear nerve degeneration No clinically significant effect on vision or hearing Etiology • • • • • • • • • • Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology Trauma Head injury Blunt head injury alone Concussive injury of the labyrinth Labyrinthine injury SNHL Temporal bone fracture Labyrinthine concussion Longitudinal fractures Similar to acoustic trauma Limited to the high F Worse at 4 kilohertz Transverse fractures Complete loss of auditory & vestibular function Penetrating injuries Subluxation ofthe stapes into the vestibule Profound SNHL Trauma Noise-induced HL & Acoustic trauma First published in the 1930s Common occupationallyinduced disabilities Common in industry Caused by excessive noise exposure Temporary SNHL that recovers over the next 24 to 48 hours High intensity & repeated Permanent Outer hair cell Most effect More damage in - High-frequency sound - Continuous sound - Pure tones Symmetric & Bilateral HL Limited to 3 kHz, 4 kHz, and 6 kHz Greatest loss 4 kHz Progress rapidly in first 10 to 15 years of exposure Trauma Noise-induced HL & Acoustic trauma Common patterns Flat & downsloping losses Acoustic trauma Unilateral or asymmetric OSHA does not allow unprotected exposures greater than 90 dBA based on an 8 hour/day time weighted average (TWA) Variability Age, gender, race, and coexisting vascular disease No known way to predict susceptibility to NIHL Protection Earplugs or earmuffs Many hazardous noise exposures are not occupational in origin Trauma Barotrauma Unequalized pressure differentials between the middle and external ears Occurs during flying or underwater diving Pain, hyperemia and possible perforation of the tympanic membrane Edema and ecchymosis of the middle ear mucosa Conductive HL may result Perilymphatic fistula Pathologic communication between the perilymphatic space of the inner ear and the middle ear Congenital or acquired Occur at either the round or oval windows Perilymphatic fistula Congenital Occur in the stapes footplate with labyrinthine anomalies Such as Mondini dysplasia Communicate with the subarachnoid space and result in cerebrospinal fluid leak and possible meningitis Profound hearing loss Acquired Result of - Barotrauma - Direct trauma of temporal - Indirect trauma of temporal - Complication of stapedectomy Sudden SNHL and vertigo after a head injury, barotrauma, or heavy lifting or straining May be spontaneously Diagnosis Middle ear exploration Trauma Irradiation Conventional fractionated irradiation of the temporal bone Fractionated irradiation Limited extent to treat vestibular schwannoma Difficult to determine because of the limited data available Stereotactic irradiation (“radiosurgery”) for vestibular schwannoma This modality Risk of SNHL High as with microsurgical removal Etiology • • • • • • • • • • Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology Neurologic disorders Multiple sclerosis Multiple areas of CNS demyelination, inflammation, and glial scarring Age 20 to 30 years More common in women Cause Unknown 4% to 10% of MS SNHL Progressive or sudden Bilateral, unilateral, symmetric, or asymmetric Speech discrimination Normal or reduced Abnormalities of the ABR MRI Periventricular white-matter plaques on T2weighted images Neurologic disorders Benign intracranial hypertension Pseudotumor cerebri Increased intracranial pressure Without evidence of mass lesion, obstructive hydrocephalus, intracranial infection, or hypertensive encephalopathy Headache and visual blurring Pulsatile tinnitus SNHL and vertigo More in young, obese women SNHL Fluctuating, low-F Unilateral or bilateral Vertigo and aural fullness Diagnosis Papilledema CSF pressure > 200 mmH2O ABR abnormalities Management - Weight loss - Acetazolamide - Furosemide - Lumbar-peritoneal shunting Etiology • • • • • • • • • • Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology Vascular and hematologic disorders • • • • • • Migraine Headache and visual aura Basilar migraine Vertigo, SNHL Tinnitus, aural fullness Distortion & recruitment 46% Bilateral, low-F-SNHL Fluctuated HL Similarity, between basilar migraine and Ménière’s Dz. Drugs in basilar migraine No systematic study • • • • • • • • • • • Vertebrobasilar arterial occlusion Brainstem syndromes Anterior inferior cerebellar artery (AICA) Occlusion of AICA SNHL Thrombosis or embolism Area infarcted Inferior pons Acute AICA infarction Acute vertigo with N/V Facial paralysis, SNHL Tinnitus, gaze paralysis Loss of pain and temperature sensation on the face Ipsilateral Horner’s syndrome Vascular and hematologic disorders Waldenström’s macroglobulinemia • Abnormally large amounts of IgM in the plasma • Increased blood viscosity • Subsequent ischemic lesions • Progressive & sudden SNHL • SNHL responded to alkylating agents or plasmapheresis Vascular and hematologic disorders Sickle cell anemia • Incidence of SNHL • ~ 22% of sickle cell disease • Progressive or sudden • Associated with sickle cell crises Vascular and hematologic disorders Leukemias & Lymphomas • SNHL Leukemic infiltrates Inner ear hemorrhage Vascular occlusion Labyrinthine ischemia Etiology • • • • • • • • • • Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology Immune disorders Cogan’s syndrome o Attacks of acute nonsyphilitic interstitial keratitis o Auditory and vestibular dysfunction o Unilateral or bilateral SNHL o Severe vertigo, nausea, vomiting, and tinnitus o Progresses to a profound loss over months o Ophthalmologic findings o If treated SNHL is responsive o Aggressive treatment with steroids Immune disorders Polyarteritis nodosa o Necrotizing vasculitis of small- and medium-sized arteries o Myriad of findings, including weight loss, fatigue, fever, anorexia, arthritis, neuropathy, hypertension, renal failure, abdominal pain, and SNHL o Biopsy Necrotizing vasculitis o Unilateral or bilateral o Facial paralysis also may be seen o Management - Aggressive doses of steroids - Immunosuppressive drugs Immune disorders Relapsing polychondritis o An inflammatory reaction in multiple cartilages o The auricles 1st affected o Arthritis and eye findings o HL Conductive Sensorineural Mixed HL o SNHL Sudden or progressive o May be associated with vestibular disturbances o Rx Steroids Immunosuppresive Dapsone Immune disorders Wegener’s granulomatosis o Necrotizing granulomatous vasculitis involving principally the lungs, airway, and kidneys o Usually Conductive HL o CHL Involvement of the eustachian tube or middle ear o SNHL If extends into the inner ear Immune disorders Primary autoimmune inner ear disease o McCabe Bilateral SNHL responsive to immunosuppressive drugs o Sudden or progressive HL o Involves both ears o Associated with vestibular symptoms o Strongly mimic Ménière’s disease o Humoral autoimmunity Abnormal o Responsiveness of the HL to steroids or cytotoxic drugs The hallmark o Used Methotrexate Reduce the need for continued high-dose steroids Etiology • • • • • • • • • • Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology Bone disorders Otosclerosis Primarily causes CHL Uncommonly Progressive SNHL Especially in late disease CT images Radiolucent area surrounding the cochlea Advanced otosclerosis Bilateral profound mixed hearing loss Bone disorders Paget’s disease Osteitis deformans Most common in older ~ 50% of Paget’s disease Conductive, SNHL or mixed Rarely fixed stapes footplate RX Calcitonin Eidronate disodium Etiology • • • • • • • • • • Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology Neoplasms Vestibular schwannoma Most common neoplasm SNHL Originate from 8th CN Within the CPA or the IAC Approximately 80% of all CPA neoplasms Progressive unilateral SNHL Principally the high frequencies Neoplasms Vestibular schwannoma Speech discrimination is reduced out of proportion to the pure tone thresholds Sudden SNHL 10% of patient Unilateral or asymmetric tinnitus With or without hearing loss Mild or severe vestibular symptoms or may have none Disorders of unknown etiology Presbycusis Aging process Without other apparent etiology Age-related change Stiffness of the basilar membrane 30% of aged > 65 years Worse for high frequencies More severe in men Schuknecht 4 types Presbycusis Sensory presbycusis - Progressively hair cells loss - Steeply sloping HFSNHL Strial presbycusis - Atrophy of the stria vascularis - Flat audiograms Neural presbycusis - Loss of auditory nerve fibers - Reduced speech discrimination out of proportion to their pure tone thresholds Cochlear presbycusis - Mechanical CHL Disorders of unknown etiology Ménière’s disease Fluctuant SNHL Tinnitus, episodic vertigo, and aural fullness Progresses, gradually or quickly HL Tinnitus “Buzzing” or “Roaring” Aural fullness Typically fluctuates Vertigo Several hours After attacks Fatigued for 24 hours or more Profound loss is rare Low F Commonly Bilateral in 30% to 50% Endolymphatic spaces dilatation of the inner ear Disorders of unknown etiology Ménière’s disease Vestibular destructive therapy No effective No therapy Effective in HL Medical therapy - Sodium-restricted diet - Diuretic administration Lack of an objective diagnostic test Idiopathic endolymphatic hydrops Other pathologic endolymphatic hydrops processes - Syphilis - Temporal bone trauma - Serous labyrinthitis - Stapedectomy - Autoimmune disease Thank you for your attention