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SENSORINEURAL HEARING LOSS IN ADULTS นพ.อัครรัตน์ ศาสตร์สูงเนิน อาจารย์ที่ปรึ กษา รศ.สมชาย ศรี ร่มโพธิ์ทอง 28 ธันวาคม 2549 Sensorineural hearing loss Introduction Clinical evaluation • • • • • • 28-Dec-2006 History Physical examination Auditory testing Vestibular testing Laboratory testing Radiographic testing 2 Sensorineural hearing loss Etiology • Development & Hereditary disorder • Infectious disorder • Pharmacologic disorder • Trauma • Neurologic disorder • Vascular & Hematologic disorder 28-Dec-2006 • • • • Immune disorder Bone disorder Neoplasms Endocrine & Metabolic disorder • Disorder of unknown etiology **Sudden Sensorineural hearing loss** 3 Clinical evaluation History • • • • • • Unilateral / Bilateral Chronicity Tinnitus / Vertigo Otalgia / Otorrhea Headache Eye symptoms 28-Dec-2006 • • • • • Underlying disease Ototoxic drugs Hx of trauma Noise exposure Family Hx 4 Clinical evaluation Physical examination • • • • • 28-Dec-2006 Weber / Rinne test Otoscopy Cranial nerve Stigmata of associated disease Generally no abnormality** 5 Clinical evaluation Auditory testing • • • • • • 28-Dec-2006 Conventional audiometry Tympanometry Acoustic reflex threshold Auditory brainstem response Electrocochleography Otoacoustic emission 6 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 28-Dec-2006 Vestibular testing • Adjunct in selected patients Radiographic testing • MRI with Gadolinium Retrocochlear hearing loss? • Computed tomography Labyrinthine abnormality? 7 Etiology • • • • • • • • • • 28-Dec-2006 Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology 8 Development & Hereditary disorder Large vestibular aqueduct syndrome Nonsymdromic hereditary HL • Not associated with hereditory abnormalities • Genetic factor • ~ 90% Recessive fashion 28-Dec-2006 • • • • • • • Inner ear dysplasia Enlarged vestibular aqueduct Normal to profound HL Asymmetric Anacusis with fluctuation Progressive HL Well demonstrated on CT imaging of temporal bone 9 Development & Hereditary disorder Waardenburg syndrome • Autosomal-dominant 1) Dystopia canthrum (Lateral displacement of the media canthi) 2) Broad nasal root 3) Confluence of the medialportions of the eyebrows 4) Partial or total heterochromia iridis 5) A white forelock 6) Sensorinearal hearing loss • Vary hearing loss • Unilateral / Bilateral 28-Dec-2006 10 Development & Hereditary disorder Alport syndrome • Autosomal-dominant • More common in woman • Much severe in men (55-75% ESRD) • Interstitial nephritis • SNHL • Ocular manifestations • Progressive hearing loss 28-Dec-2006 11 Development & Hereditary disorder Usher syndrome • Autosomal-recessive • Retinitis pigmentosa & SNHL • Type I ~85% Profound HL Absent vestibular response Retinitis pigmentosa 28-Dec-2006 Stereocilia are arranged in three tiers atop a hair cell. Tip links connecting shorter stereocilia to their taller neighbors. 12 Etiology • • • • • • • • • • 28-Dec-2006 Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology 13 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 28-Dec-2006 • • • • • 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 14 Infectious disorder Herpes zoster oticus • Varicella-zoster infection • Most commonly associated with facial paralysis • HL and vertigo can occur 28-Dec-2006 15 Infectious disorder Measles • Not uncommon cause of deafness in children • Bilateral HL • Moderate-to-profound HL • Vestibular function can be similarly affected 28-Dec-2006 16 Infectious disorder Mumps • Paramyxovirus infection • Unilateral SNHL • Unilateral deafness in otherwise healthy children • Sudden deafness in adult Subclinical mumps infection in those without previous immunity 28-Dec-2006 17 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 28-Dec-2006 18 Infectious disorder Rocky Mountain spotted fever • Tick-borne infection • Rickettsia rickettsii • Headache, fever, myalgias, and petechial rash • Systemic vasculitis • Progressive SNHL • Vasculitis involving the auditory system 28-Dec-2006 19 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) 28-Dec-2006 20 Infectious disorder Lyme disease • • • • Tick-borne infection Borrelia burgdorferi Facial paralysis Possible etiology of SNHL in endemic areas 28-Dec-2006 21 Etiology • • • • • • • • • • 28-Dec-2006 Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology 22 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 28-Dec-2006 Ototopical preparations Neomycin, Gentamicin, and Tobramycin-containing Cochlear or vestibular ototoxicity Avoid the use of aminoglycoside-containing 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 23 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 28-Dec-2006 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 24 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 28-Dec-2006 Nonsteroidal antiinflammatory drugs Naproxen, Ketoralac & Piroxicam Ototoxicity resulting from use of NSAIDs is rare Only reversible physiologic changes, without major morphologic changes 25 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 28-Dec-2006 Erythromycin Uncommon Partially Intravenously Reversible on discontinuation No reports - Newer macrolide - Clarithromycin - Azithromycin 26 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 28-Dec-2006 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 27 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 28-Dec-2006 Eflornithine Drug treatment of trypanosomiasis Some Pneumocystis carinii pneumonia, Cryptosporidiosis, Leishmaniasis, and Malaria Cause major and doserelated SNHL 28 Pharmacologic disorder Lipid-lowering drugs 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 28-Dec-2006 Wallerian-like degeneration High doses of HMG-CoA reductase inhibitors Optic& vestibulocochlear nerve degeneration No clinically significant effect on vision or hearing 29 Etiology • • • • • • • • • • 28-Dec-2006 Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology 30 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 28-Dec-2006 Transverse fractures Complete loss of auditory & vestibular function Penetrating injuries Subluxation ofthe stapes into the vestibule Profound SNHL 31 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 28-Dec-2006 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 32 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) 28-Dec-2006 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 33 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 28-Dec-2006 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 34 Perilymphatic fistula Congenital Acquired 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 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 28-Dec-2006 35 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 28-Dec-2006 Stereotactic irradiation (“radiosurgery”) for vestibular schwannoma This modality Risk of SNHL High as with microsurgical removal 36 Etiology • • • • • • • • • • 28-Dec-2006 Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology 37 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 28-Dec-2006 Abnormalities of the ABR MRI Periventricular white-matter plaques on T2-weighted images 38 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 28-Dec-2006 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 39 Etiology • • • • • • • • • • 28-Dec-2006 Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology 40 Vascular and hematologic disorders Vertebrobasilar arterial occlusion 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 • • • • • • • • • • • 28-Dec-2006 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 41 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 28-Dec-2006 42 Vascular and hematologic disorders Sickle cell anemia • Incidence of SNHL • ~ 22% of sickle cell disease • Progressive or sudden • Associated with sickle cell crises 28-Dec-2006 43 Vascular and hematologic disorders Leukemias & Lymphomas • SNHL Leukemic infiltrates Inner ear hemorrhage Vascular occlusion Labyrinthine ischemia 28-Dec-2006 44 Etiology • • • • • • • • • • 28-Dec-2006 Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology 45 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 28-Dec-2006 46 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 28-Dec-2006 47 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 28-Dec-2006 o SNHL Sudden or progressive o May be associated with vestibular disturbances o Rx Steroids Immunosuppresive Dapsone 48 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 28-Dec-2006 49 Immune disorders Primary autoimmune inner ear disease o McCabe Bilateral o o o o SNHL responsive to immunosuppressive drugs Sudden or progressive HL Involves both ears Associated with vestibular symptoms Strongly mimic Ménière’s disease 28-Dec-2006 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 50 Etiology • • • • • • • • • • 28-Dec-2006 Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology 51 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 28-Dec-2006 52 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 28-Dec-2006 53 Etiology • • • • • • • • • • 28-Dec-2006 Development & Hereditary disorder Infectious disorder Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology 54 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 28-Dec-2006 55 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 28-Dec-2006 56 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 28-Dec-2006 58 Presbycusis Sensory presbycusis - Progressively hair cells loss - Steeply sloping HFSNHL Strial presbycusis - Atrophy of the stria vascularis - Flat audiograms 28-Dec-2006 Neural presbycusis - Loss of auditory nerve fibers - Reduced speech discrimination out of proportion to their pure tone thresholds Cochlear presbycusis - Mechanical CHL 59 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 28-Dec-2006 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 60 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 28-Dec-2006 Other pathologic endolymphatic hydrops processes - Syphilis - Temporal bone trauma - Serous labyrinthitis - Stapedectomy - Autoimmune disease 61 Thank You 28-Dec-2006 62