Inner ear diseases

Brhanu (M.D ENT R4)

 Tinnitus

• Tinnitus is a symptom, not a disease

• It is the sensation of sound not brought about
by simultaneously externally applied mechanoacoustic or
electrical signals—the perception of
sound generated involuntarily within the head
of an individual
• Suggests problem between cochlea and auditory
cortex
• Central auditory system plays vital and critical
role in experience of tinnitus
• Classification
• • Somatosounds:
◦ Cochlear—spontaneous otoacoustic emissions
◦ Extracochlear
- Muscular [eustachian tube (ET) function, stapedial, tensor
tympani or palatal myoclonus]
- Vascular (internal carotid artery or
ICA)—pulsatile
- Crepitus (temporomandibular joint)
- Respiratory (patulous eustachian tube)
• • Subjective tinnitus:
◦ May arise from a source/trigger =
tinnitus-related neural activity (TRNA)
in cochlea, brainstem or higher centres
(neurophysiological)
◦ Usually unable to determine the source
clinically
◦ May be facilitated and sustained by hearing
loss (HL)—reduced perception of external
environmental sounds leading to increased
central gain
• Non-organic tinnitus: little known, and no
definitive test
 Incidence and Epidemiology
• 94% of normal-hearing subjects placed in a
soundproof room for up to 5 min have tinnitus like
experiences
• 10% of adults have experienced tinnitus for
>5 min
• 4% have tinnitus causing sleep disturbance
• 0.5% severely disabled by tinnitus
 • Risk factors:
◦ Noise exposure
◦ Hearing loss
◦ Increasing age
◦ Pre-existing anxiety/depression
◦ Association with ear pathology (e.g., otosclerosis,
Ménière, tympanic membrane perforation,
vestibular schwannoma)
◦ Ototoxic medication (e.g., salicylates, aminoglycosides,
loop diuretics)
• Impact
• Emotional distress
• Insomnia
• Concentration
• depression
• potential self-harm
 Management Algorithm
• Pulsatile—if yes, unilateral, and no conductive
HL or negative middle ear pressure, consider
glomus tumour (Ix by magnetic resonance imaging or
MRI, then CT scan if nothing shown on
MRI): when have conductive HL, body sounds
are heard more easily (as environmental sounds
attenuated) so often hearing a normal pulsation.
 • Non-pulsatile with normal hearing: refer;
consider hearing therapy
• Non-pulsatile with deafness: if symmetrical
loss, for hearing therapy; if unilateral consider
MRI for acoustic neuroma
• Clicking noises may imply muscle twitches of
the palate, stapedius, and tensor tympani (rare)
 Treatment
• Most habituate naturally
• Tinnitus retraining therapy: objective is to
effect permanent changes in auditory filters and
pattern recognition involved in the detection
and enhancement of TRNA
• Instrumentation:
◦ Masking devices (white noise generators);
change attentional focus
◦ hearing aids (HAs)—10% will experience total
suppression of tinnitus, many experience
partial inhibition
◦ White noise generator and HA combined
sometimes useful
• Directive counseling, e.g., information and
explanation
• • Relaxation therapy
• Cognitive-behavioural therapy: explanation,
relaxation, change of beliefs about tinnitus
• Sound therapy for hyperacusis
• Transcranial magnetic stimulation under
investigation
 Hyperacusis

•
• Decreased sound tolerance or emotional dislike
of noise
• 40% of tinnitus patients
• Can be demonstrated by loudness discomfort
testing (but unpleasant for patient and of little
value)
• Mechanism usually increased central gain
• Treat with information, relaxation, wide band
sound generators
 vertigo

• 1. Vertigo: False sense of motion, such as rotation

(spinning
or whirling)
2. Disequilibrium: Unsteadiness, or loss of balance
3. Lightheadedness: Floating feelings, head fullness,
or out of
body sensations
4. Presyncope: Near faint
• Hx
• • Description of the dizziness
– Lightheadedness
– Disequilibrium and unsteadiness
– Vertigo and oscillopsia
– Presyncope
• Episodic or continuous
• Duration of the individual attack: Seconds, minutes,
hours, days or months
• Effect of head movements: Worse, better or no effect
• Specific positions that induce vertigo
• Preceding history of
– Trauma: Physical, barotraumas, surgical
– Medicines: For infections, hypertension,
hyperglycemia
and cardiac arrhythmias and CNS disorders
• Medical conditions: Hypothyroidism, diabetes
mellitus,
anemia, autoimmune diseases and hypoperfusion of
brain
from postural hypotension or cardiac arrhythmia
• Psychogenic disorders: Anxiety and panic disorders
and agoraphobia
• Triggering events: Stimuli (sound, pressure, or
movements),
rolling onto the side in bed
•
• Associated/concomitant symptoms
– Ear: Discharge, pain, hearing loss, tinnitus, ear
fullness
– Eye: Diplopia, vision loss
– Headache
– CNS: Paralysis and paresthesias, dysarthria,
dysphagia
– Sweating, dyspnea and palpitation
 Sensorineural Hearing Loss

• Noise-Induced HL
• Excessive noise can damage cochlear by both
metabolic and mechanical mechanisms:
◦ Metabolic: oxidative stress, synaptic
hyperactivity, altered blood flow implicated;
outer hair cell intracellular calcium levels rise
causing cell injury
 ◦ Mechanical: large-amplitude motion may
disrupt cochlear structures and even cause cell
membrane rupture
• After exposure, hearing threshold may return to
normal—temporary threshold shift
• Greater cellular damage leads to a permanent
threshold shift; ~125 dB is the critical level at
which temporary becomes permanent
• Loss typically seen as notch at 3, 4, and 6 kHz
• Hazards: occupational, military, social (music),
fireworks
 Sudden Sensor neural Hearing Loss

• Loss of >30 dB in three contiguous frequencies

over 3 days
• Usually unilateral and idiopathic (~60%)
• Identifiable causes include:
◦ Head injury, barotrauma, blast injuries
◦ Ototoxic drugs
◦ Bacterial infections—suppurative labyrinthitis,
meningitis, syphilis
◦ Viral infections—mumps, measles, rubella,
varicella zoster virus
◦ Vestibular schwannoma
◦ Iatrogenic, after ear surgery
◦ Cardiovascular accident (CVA)
◦ Multiple sclerosis (MS)
• Ix: pure tone audiogram, magnetic resonance
imaging; consider FBC, erythrocyte sedimentation rate
(ESR), autoimmunity screen, syphilis
serology, Lyme disease
• Poor prognostic factors: raised ESR, severe
vertigo, age >60 years, degree of sensorineural hearing
loss (SNHL) (greater loss = worse
prognosis)
• • Rx:
◦ 60% of idiopathic group recover spontaneously
◦ Steroids: oral prednisolone 40 mg once daily
for 1/52 (or up to 1 mg/kg per day); earlier
started, better the prognosis
◦ Intratympanic steroids may be of benefit
◦ Acyclovir of dubious benefit
◦ Peripheral vasodilator, e.g., carbogen (5% CO2,
95% O2) dubious benefit
◦ Possible role for hyperbaric oxygen (problem is
availability)
◦ Psychological help and rehabilitation, hearing
aid
• Some evidence that sudden SNHL is an early
warning sign of impending stroke
 Presbyacusis

• The lessening of the acuteness of hearing that

characterizes old age
• 40 to 50% >75-year-olds have HL that can impair
communication; ~80% of 80-year-olds
• Cause probably multifactorial; an accumulation
of metabolic and physiological changes associated
with increased susceptibility to disease
• Accumulative oxidative damage and mitochondrial
DNA mutations leading to cellular dysfunction and a
decrease in auditory sensitivity
 Ototoxicity

• Can cause SNHL, tinnitus, and vertigo.

◦ Aminoglycosides:
- May be predominantly cochleotoxic (e.g., neomycin)
or vestibulotoxic (e.g., gentamicin)
- Cause mitochondrial dysfunction in
susceptible patients that leads to toxic
accumulation of intracellular ions and
ultimate cell death
- Apparent cumulative effect because 6-month
half-life in outer hair cells
- Damage enhanced by synchronous use of
loop diuretic
- Antioxidants may have protective effect, as
may aspirin
- Renal failure and advanced age increase
chance ototoxicity
- C/o: tinnitus, HL (high-frequency SNHL),
vertigo
• Aminoglycosides cause damage to the
dark cells of the stria vascularis, therefore
reducing endolymph production and thus
helping in the control of Ménière disease.
• ◦ Loop diuretics:
- May produce reversible high-tone SNHL
◦ Cisplatin:
- Cochleotoxic; can cause permanent
high-tone SNHL
- Effect similar to aminoglycosides
◦ Salicylates:
- High-frequency tinnitus preceding SNHL
- Usually reversible on withdrawal of aspirin
◦ Quinine:
- Effects similar to aspirin
- Potentially huge implications in developing
world as inexpensive treatment for malaria
 Ménière Disease

• Pathology: uncertain and all theories have flaws:

◦ Disease of the membranous inner ear, with
excess endolymph (endolymphatic hydrops)
production (stria vascularis), or reduced
absorption (endolymphatic sac)
o syndrome associated
with other diagnoses (e.g., syphilis, otosclerosis)
 • Epidemiology:
◦ Middle-aged F>M, 10% familial, usually
unilateral; at 2 years 15% bilateral, at
20 years 40%
◦ Incidence ~1:1000
 • Symptoms:
◦ Episodic triads of intermittent disabling
vertigo, fluctuating HL, tinnitus and associated
pressure sensation; attacks tend to occur in
clusters over several weeks
- Vertigo: recurrent episodes lasting 20 min
to 24 h, associated nystagmus, nausea and
vomiting, no neurological symptoms
- Deafness: low-frequency fluctuating SNHL,
progressive.
- Tinnitus: variable, louder during attacks,
unilateral on affected side
- Drop attacks possible: acute attack of vertigo
causing patient to drop to the floor as if
struck by a sledgehammer (with nausea and
vomiting).
 • Diagnostic categories:
◦ Possible: episodic vertigo without HL, or SNHL
with dysequilibrium but not definite episodes
◦ Probable: one definite episode vertigo,
audiometrically documented HL at least once,
tinnitus or aural fullness.
◦ Definite: two or more definite episodes vertigo
≥20 min, audiometrically documented HL at
least once, tinnitus or aural fullness
◦ Certain: definite with histopathological
confirmation
• Ix:
◦ Diagnosis made mainly on history:
- Exclude vestibular schwannoma when
unilateral inner ear symptoms (MRI)
- PTA
- Calorics: develop canal paresis on side of
lesion, directional preponderance towards
normal ear
- ECoG: broadening of summating potential/
action potential
 • Nonsurgical Rx:
◦ Spontaneous resolution ~70%
◦ Vestibular sedatives during attacks (may
be counterproductive if used regularly for
prevention)
◦ Betahistine up to 16 mg three times a day;
little evidence of benefit (but little harm)
◦ Salt-restricted diet (and possibly alcohol,
caffeine)
◦ Thiazide diuretics have little proven benefit
◦ Intratympanic gentamicin: control of attacks
in up to 90%; hearing preserved or improved
in ~75% (gentamicin is more vestibulotoxic
than cochleotoxic); aminoglycosides
cause damage to the dark cells of the stria
vascularis, therefore reducing endolymph
production
 ◦ Intratympanic steroid injection has been
shown to be better than placebo.
 Other Causes of
Sensorineural Hearing Loss

• Other causes include:

◦ General disease: diabetes, syphilis, Paget
disease, human immunodeficiency virus
◦ Cardiovascular disease: myocardial infarct,
CVA
◦ Genetic: connexin 26 mutation responsible for
most cases of nonsyndromic SNHL (30–40%
of all childhood genetic deafness in white
Western Europeans)
◦ Retrocochlear lesions (e.g., vestibular
schwannoma, meningioma, MS)
 Benign Paroxysmal Positional Vertigo

• Pathology:
◦ Usually in posterior Scc
◦ Canalolithiasis or free-floating debris
( otoconia) in endolymph causes continuing
stimulation after movement of head has
ceased
◦ Cupulolithiasis: debris from otolith organ
becomes attached to cupula
 • Aetiology:
◦ Usually idiopathic; ~20% associated with minor
head trauma
• Epidemiology:
◦ Any age (peak 6th–7th decades); most
common cause of vertigo
◦ Associated with preceding inner ear disease
(e.g., Ménière, vestibular neuritis)
 • Symptoms:
◦ Vertigo of seconds to minutes duration
associated with rapid changes in head position
(e.g., rolling over in bed, turning to reach
objects on high shelves)
◦ Attacks often in clusters, may recur after
period of remission
• Ix:
◦ Dix–Hallpike manoeuvre: provokes vertigo and
nystagmus (affected ear downwards)
- Nystagmus rotatory with latency of a few
seconds, fatigues after 30 to 40 s
- Torsional, beats towards floor (geotropic),
direction reverses on sitting up
• • Rx:
◦ Spontaneous remission rate high
◦ Epley manoeuvre (Fig. 17.1) to disperse canal
debris into utricle (where it is inactive);
effective in ~85% cases
◦ Avoid vestibular sedatives (e.g.,
prochlorperazine)
◦ Posterior Scc occlusion in severe recurrent
cases; singular neurectomy possible
 Acute Vestibular Failure

• Definitions:
◦ Labyrinthitis suggests coexistent HL ± tinnitus
◦ Neuronitis implies just vestibular symptoms
• Aetiology:
◦ Possible viral role (HSV)
◦ Vascular, trauma, iatrogenic (e.g., post
gentamicin), bacterial infection, local disease
(cholesteatoma, vestibular schwannoma),
autoimmune (e.g., Cogan syndrome)
• Symptoms and natural history:
◦ Vertigo lasting > 24 h; often bed-bound for a
few days
◦ Recovery usually spontaneous over ~1 week as
central compensation occurs
 ◦ Decompensation episodes with disequilibrium
common over next two years, but generally
gradually reduce in intensity, duration, and
frequency
• Rx:
◦ Compensation promoted by vestibular
rehabilitation (often physiotherapy led)
◦ Those with visual vertigo (overreliance on
visual input) require physiotherapy exercises
in visually stimulating environments
◦ Vestibular sedative helpful in acute period but
counterproductive in long term
• Sequelae:
◦ Increased incidence of BPPV
 Deafness in Children

Classification
• Only 5 to 10% deaf children have deaf parents
• Congenital: sensorineural, conductive, or mixed;
isolated or part of syndrome; 1:1000 children
born with SNHL (50% of these profound)
◦ Genetic: usually SNHL, usually from single
gene disorders
- Single gene disorders
- Syndromic
◦ Perinatal/intrauterine causes
◦ Congenital disorders causing deafness to
develop in childhood
• Acquired: hearing normal at birth
◦ Meningitis (bacterial): commonest cause
of acquired SNHL seen in ~10% children
infected;
◦ Mumps, measles
◦ Trauma
◦ OME
• Genetic Nonsyndromic Sensorineural Hearing Loss
• • Autosomal or X-linked; 1:8 people carry a
recessive gene for deafness; of hereditary
deafness (1:4000 live births) 80% autosomal
recessive, 15% auto dominant, 4% X-linked,
1% mitochondrial
• Connexin 26 most common type (50%
non-syndromic autosomal recessive HL)
◦ Mutation in GJB2 gene alter function of
connexin-26 gap junction protein causing
potassium intoxication of organ of Corti
◦ Homozygous mutations usually cause
non-progressive HL, heterozygous ones cause
progressive HL
◦ Genetic testing available
• Syndromal
• ◦ Autosomal recessive:
- Pendred syndrome: SNHL and thyroid goitre
±vestibular disturbance; usually euthyroid
• Usher syndrome: SNHL and retinitis
pigmentosa; some types have absent
vestibular responses
• Branchio-otorenal syndrome; branchial
arch anomalies (abnormal external ear,
preauricular pits), renal dysplasia, and mixed
HL; gene mapped to long arm chromosome 8
• ◦ Autosomal dominant:
- Waardenburg syndrome: SNHL and hypertelorism, pigment disorder
(e.g., white
forelock, heterochromia iridis); various genes
and types identified
- Treacher–Collins syndrome: hypoplasia of
zygoma, maxilla, mandible ± microtia, downslanting palpebral
fissures, external and
inner ear deformities; deafness—CHL, mixed,
or SNHL
- Pierre Robin syndrome: hypoplastic mandible, cleft palate,
glossoptosis ± external,
middle, and inner ear deformities, club foo
• ◦ X-linked:
- Alport syndrome: X-linked recessive, defect in
collagen gene; chronic renal failure, progressive
SNHL, ophthalmic signs, milder in F; some
pedigrees exist that are autosomal dominant
◦ Down syndrome:
- Trisomy 21, 1:600 live births (1:100 for
mothers >40 years old)
- Prone to OME, recurrent URTIs, narrow EACs,
increased rate of ossicular abnormalities
(hence CHL) and cochlear abnormalities
(hence SNHL)
• Perinatal/Intrauterine
• • Potentially preventable
• Maternal infection: TORCH
◦ Rubella: a common identifiable cause of
congenital SNHL (cookie bite pattern on PTA)
that is progressive in ¼; deafness affects ~1/3
rubella children; infection can cause deafness at
any stage of pregnancy; maternal infection often
subclinical—mothers often not immunized
• ◦ CMV: infection can be congenital, perinatal, or
acquired; most infections subclinical at birth;
get destruction of cochlear and labyrinthine
structures, usually severe bilateral SNHL;
commonest intrauterine infection causing
deafness (0.5% births, 4% of which will be deaf,
rising to 8% by 5 years, i.e., can be progressive);
treatment can arrest progressive HL
• ◦ Toxoplasmosis: often subclinical at birth;
progressive blindness, SNHL (in ~15% infected
children)
◦ Congenital syphilis: onset deafness usually
early adulthood; middle ear problems + SNHL
◦ Herpes simplex: vertical transmission
possible; incidence rising (Rx: acyclovir)
• Ototoxic medication: effect on fetal cochlea,
e.g., loop diuretics, aminoglycoside antibiotics
(risk from latter less in children than adults),
cisplatin chemotherapy, quinine
• Metabolic: e.g., maternal diabetes, fetal alcohol
syndrome
• Perinatal: prematurity associated with
hypoxia, hyperbilirubinemia, low birthweight,
susceptibility to infection—may act
synergistically to affect hearing
• Investigations
◦ Imaging: CT petrous temporal bones
(dysplasias, widened vestibular aqueduct); MRI can also be helpful
◦ Infection screen (i.e., TORCH)
◦ Ophthalmology: 40% of children with SNHL
have ophthalmic conditions
◦ Serological testing, immunologic screening,
renal tests—only if otherwise directed