sensorineural hearing loss in adults นพ. อัครรัตน์ ศาสตร์สูง...

SENSORINEURAL HEARING LOSS IN ADULTS

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28-Dec-2006 2

Sensorineural hearing loss

Introduction

Clinical evaluation• History• Physical examination• Auditory testing• Vestibular testing• Laboratory testing• Radiographic testing

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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**

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Clinical evaluation

History• Unilateral / Bilateral• Chronicity• Tinnitus / Vertigo• Otalgia / Otorrhea• Headache• Eye symptoms

• Underlying disease• Ototoxic drugs• Hx of trauma• Noise exposure• Family Hx

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Clinical evaluation

Physical examination• Weber / Rinne test• Otoscopy• Cranial nerve• Stigmata of associated disease• Generally no abnormality**

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Clinical evaluation

Auditory testing• Conventional audiometry• Tympanometry• Acoustic reflex threshold• Auditory brainstem response• Electrocochleography• Otoacoustic emission

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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?

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Etiology

• Development & Hereditary disorder• Infectious disorder• Pharmacologic disorder• Trauma• Neurologic disorder• Vascular & Hematologic disorder• Immune disorder• Bone disorder• Neoplasms• Disorder of unknown etiology

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Development & Hereditary disorder

Nonsymdromic

hereditary HL• Not associated with

hereditory abnormalities

• Genetic factor

• ~ 90% Recessive fashion

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

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Waardenburg syndrome• Autosomal-dominant1) Dystopia canthrum (Lateral

displacement of the media canthi)

2) Broad nasal root3) Confluence of the

medialportions of the eyebrows

4) Partial or total heterochromia iridis

5) A white forelock6) Sensorinearal hearing loss• Vary hearing loss• Unilateral / Bilateral

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Alport syndrome• Autosomal-dominant• More common in

woman• Much severe in men

(55-75% ESRD)• Interstitial nephritis• SNHL• Ocular manifestations• Progressive hearing

loss

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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.

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Etiology


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Infectious disorder

LabyrinthitisSerous 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

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Infectious disorder

Herpes zoster oticus• Varicella-zoster infection• Most commonly associated

with facial paralysis • HL and vertigo can occur

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Infectious disorder

Measles• Not uncommon cause

of deafness in children• Bilateral HL• Moderate-to-profound

HL• Vestibular function can

be similarly affected

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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

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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

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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

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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)

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Infectious disorder

Lyme disease • Tick-borne infection • Borrelia burgdorferi• Facial paralysis• Possible etiology of

SNHL in endemic areas

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Etiology


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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

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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

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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

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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

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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

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

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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 dose-related SNHL

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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

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Etiology


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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

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Trauma

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

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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

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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

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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

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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

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Etiology


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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 T2-weighted images

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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

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Etiology


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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

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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

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Sickle cell anemia• Incidence of SNHL• ~ 22% of sickle cell

disease• Progressive or sudden• Associated with sickle

cell crises

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Leukemias & Lymphomas• SNHL

Leukemic infiltrates

Inner ear hemorrhage

Vascular occlusion

Labyrinthine ischemia

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Etiology


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Immune disorders

Cogan’s syndromeo Attacks of acute non-

syphilitic interstitial keratitis o Auditory and vestibular

dysfunctiono Unilateral or bilateral SNHLo Severe vertigo, nausea,

vomiting, and tinnituso Progresses to a profound

loss over monthso Ophthalmologic findings o If treated SNHL is

responsiveo Aggressive treatment with

steroids

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Immune disorders

Polyarteritis nodosao Necrotizing vasculitis of small- and

medium-sized arterieso Myriad of findings, including weight

loss, fatigue, fever, anorexia, arthritis, neuropathy, hypertension, renal failure, abdominal pain, and SNHL

o Biopsy Necrotizing vasculitiso Unilateral or bilateral o Facial paralysis also may be seeno Management

- Aggressive doses of steroids - Immunosuppressive drugs

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Immune disorders

Relapsing polychondritiso An inflammatory reaction in

multiple cartilageso The auricles 1st affectedo 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

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Immune disorders

Wegener’s granulomatosis

o Necrotizing granulomatous vasculitis involving principally the lungs, airway, and kidneys

o Usually Conductive HLo CHL Involvement of the

eustachian tube or middle ear

o SNHL If extends into the inner ear

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Immune disorders

Primary autoimmune inner ear disease

o McCabe Bilateral SNHL responsive to immunosuppressive drugs

o Sudden or progressive HLo Involves both earso 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

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Etiology


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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

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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

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Etiology


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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

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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

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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

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Presbycusis

Neural presbycusis - Loss of auditory nerve fibers

- Reduced speech discrimination out of proportion to their pure tone thresholds

Cochlear presbycusis - Mechanical CHL

Sensory presbycusis - Progressively hair cells loss - Steeply sloping HF-SNHL

Strial presbycusis - Atrophy of the stria vascularis - Flat audiograms

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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

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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

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sensorineural hearing loss in adults นพ. อัครรัตน์ ศาสตร์สูง...

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