SENSORINEURAL HEARING LOSS IN ADULTS

Name: SENSORINEURAL HEARING LOSS IN ADULTS
Uploaded: 2017-07-29T19:30:12+00:00
Duration: PTM35S18
Channel: Nigel Simon
Description: SENSORINEURAL HEARING LOSS IN ADULTS

SENSORINEURAL HEARING LOSS IN ADULTS
นพ.อัครรัตน์ ศาสตร์สูงเนิน อาจารย์ที่ปรึกษา รศ.สมชาย ศรีร่มโพธิ์ทอง 28 ธันวาคม 2549

Sensorineural hearing loss
Introduction Clinical evaluation History Physical examination Auditory testing Vestibular testing Laboratory testing Radiographic testing 28-Dec-2006

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** 28-Dec-2006

Clinical evaluation History Unilateral / Bilateral Underlying disease
Chronicity Tinnitus / Vertigo Otalgia / Otorrhea Headache Eye symptoms Underlying disease Ototoxic drugs Hx of trauma Noise exposure Family Hx 28-Dec-2006

Clinical evaluation Physical examination Weber / Rinne test Otoscopy
Cranial nerve Stigmata of associated disease Generally no abnormality** 28-Dec-2006

Clinical evaluation Auditory testing Conventional audiometry
Tympanometry Acoustic reflex threshold Auditory brainstem response Electrocochleography Otoacoustic emission 28-Dec-2006

Clinical evaluation Laboratory testing Vestibular 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? 28-Dec-2006

Etiology Development & Hereditary disorder Infectious disorder
Pharmacologic disorder Trauma Neurologic disorder Vascular & Hematologic disorder Immune disorder Bone disorder Neoplasms Disorder of unknown etiology 28-Dec-2006

Development & Hereditary disorder
Large vestibular aqueduct syndrome Inner ear dysplasia Enlarged vestibular aqueduct Normal to profound HL Asymmetric Anacusis with fluctuation Progressive HL Well demonstrated on CT imaging of temporal bone Nonsymdromic hereditary HL Not associated with hereditory abnormalities Genetic factor ~ 90%  Recessive fashion 28-Dec-2006

Waardenburg syndrome Autosomal-dominant Dystopia canthrum (Lateral displacement of the media canthi) Broad nasal root Confluence of the medialportions of the eyebrows Partial or total heterochromia iridis A white forelock Sensorinearal hearing loss Vary hearing loss Unilateral / Bilateral 28-Dec-2006

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

Usher syndrome Autosomal-recessive Retinitis pigmentosa & SNHL Type I ~85% Profound HL Absent vestibular response Retinitis pigmentosa Stereocilia are arranged in three tiers atop a hair cell. Tip links connecting shorter stereocilia to their taller neighbors. 28-Dec-2006

Suppurative labyrinthitis
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 28-Dec-2006

Infectious disorder Herpes zoster oticus Varicella-zoster infection
Most commonly associated with facial paralysis HL and vertigo can occur 28-Dec-2006

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

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

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

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

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 high-intensity sound) 28-Dec-2006

Infectious disorder Lyme disease Tick-borne infection
Borrelia burgdorferi Facial paralysis Possible etiology of SNHL in endemic areas 28-Dec-2006

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 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 28-Dec-2006

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 28-Dec-2006

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 28-Dec-2006

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 28-Dec-2006

Cisplatinum 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 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

Eflornithine Drug treatment of trypanosomiasis Some Pneumocystis carinii pneumonia, Cryptosporidiosis, Leishmaniasis, and Malaria Cause major and dose-related SNHL 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

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 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

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 28-Dec-2006

Noise-induced HL & Acoustic trauma
First published in the 1930s Common occupationally-induced 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 28-Dec-2006

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 28-Dec-2006

Perilymphatic fistula
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 28-Dec-2006

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 28-Dec-2006

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 28-Dec-2006

Neurologic disorders Multiple sclerosis Abnormalities of the ABR
MRI  Periventricular white-matter plaques on T2-weighted images 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

Benign intracranial hypertension
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 28-Dec-2006

Vascular and hematologic disorders
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 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

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

Sickle cell anemia Incidence of SNHL ~ 22% of sickle cell disease Progressive or sudden Associated with sickle cell crises 28-Dec-2006

Leukemias & Lymphomas SNHL  Leukemic infiltrates  Inner ear hemorrhage  Vascular occlusion  Labyrinthine ischemia 28-Dec-2006

Immune disorders Cogan’s syndrome
Attacks of acute non-syphilitic interstitial keratitis Auditory and vestibular dysfunction Unilateral or bilateral SNHL Severe vertigo, nausea, vomiting, and tinnitus Progresses to a profound loss over months Ophthalmologic findings If treated  SNHL is responsive Aggressive treatment with steroids 28-Dec-2006

Immune disorders Polyarteritis nodosa
Necrotizing vasculitis of small- and medium-sized arteries Myriad of findings, including weight loss, fatigue, fever, anorexia, arthritis, neuropathy, hypertension, renal failure, abdominal pain, and SNHL Biopsy  Necrotizing vasculitis Unilateral or bilateral Facial paralysis also may be seen Management - Aggressive doses of steroids - Immunosuppressive drugs 28-Dec-2006

Relapsing polychondritis
Immune disorders Relapsing polychondritis An inflammatory reaction in multiple cartilages The auricles  1st affected Arthritis and eye findings HL  Conductive  Sensorineural  Mixed HL SNHL  Sudden or progressive May be associated with vestibular disturbances Rx  Steroids  Immunosuppresive  Dapsone 28-Dec-2006

Wegener’s granulomatosis
Immune disorders Wegener’s granulomatosis Necrotizing granulomatous vasculitis involving principally the lungs, airway, and kidneys Usually  Conductive HL CHL  Involvement of the eustachian tube or middle ear SNHL  If extends into the inner ear 28-Dec-2006

Primary autoimmune inner ear disease
Immune disorders Primary autoimmune inner ear disease McCabe  Bilateral SNHL responsive to immunosuppressive drugs Sudden or progressive HL Involves both ears Associated with vestibular symptoms Strongly mimic Ménière’s disease Humoral autoimmunity  Abnormal Responsiveness of the HL to steroids or cytotoxic drugs  The hallmark Used Methotrexate  Reduce the need for continued high-dose steroids 28-Dec-2006

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

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

Vestibular schwannoma
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

Vestibular schwannoma
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

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

Presbycusis Neural presbycusis Sensory presbycusis
- Progressively hair cells loss - Steeply sloping HF-SNHL 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 28-Dec-2006

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 28-Dec-2006

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 28-Dec-2006

28-Dec-2006

SENSORINEURAL HEARING LOSS IN ADULTS

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