Chapter 43: Disorders of Hearing

Hearing loss
 one of the most common sensory disorders in humans
 can present at any age
 Nearly 10% of the adult population
 one-third of individuals age >65 years have a hearing loss of sufficient magnitude
to require a hearing aid

PHYSIOLOGY OF HEARING

Function of the external and middle ear

- amplify sound
o to facilitate conversion of the mechanical energy of the sound wave into
an electrical signal by the inner ear hair cells (mechanotransduction)

Sound waves enter the external auditory canal

set the tympanic membrane (eardrum) in motion

moves the malleus, incus, and stapes of the middle ear

Movement of the footplate of the stapes causes pressure changes in the

fluid-filled inner ear

eliciting a traveling wave in the basilar membrane of the cochlea.

tympanic membrane and ossicular chain in the middle ear
- serve as an impedance-matching mechanism

Stereocilia of the hair cells of the organ of Corti,

- rests on the basilar membrane
- in contact with the tectorial membrane
- deformed by the traveling wave.

point of maximal displacement of the basilar membrane

- determined by the frequency of the stimulating tone
o High-frequency tones
 maximal displacement: near the base of the cochlea
o low-frequency sounds
 maximal displacement: toward the apex of the cochlea

inner and outer hair cells of the organ of Corti

- different innervation patterns
o afferent innervations: inner hair cells
o efferent innervations: outer hair cells
- both are mechanoreceptors

motility of the outer hair cells alters the micromechanics of the inner hair cells, creating
a cochlear amplifier, which explains the exquisite sensitivity and frequency selectivity of
the cochlea.

At low frequencies
 individual auditory nerve fibers can respond more or less synchronously with the
stimulating tone.

At higher frequencies,
 phase-locking occurs so that neurons alternate in response to particular phases
of the cycle of the sound wave.

Intensity is encoded by
 amount of neural activity in individual neurons
 numberof neurons that are active
 specific neurons that are activated
GENETIC CAUSES OF HEARING LOSS
hereditary hearing impairment (HHI)
- More than half of childhood hearing impairment
- can also manifest later in life. H
- may be classified as either:
o nonsyndromic
 hearing loss is the only clinical abnormality
 two-thirds of HHIs
 70 - 80% inherited in an autosomal recessive manner (DFNB)
 15–20% : autosomal dominant (DFNA)
 Less than 5%: X-linked (DFNX) or maternally inherited via the
mitochondria.

o syndromic
 hearing loss is associated with anomalies in other organ systems
 one third of HHIs

The hearing genes fall into the categories of:

 structural proteins (MYH9, MYO7A, MYO15, TECTA, DIAPH1)
 transcription factors (POU3F4, POU4F3)
 ion channels (KCNQ4, SLC26A4)
 gap junction proteins (GJB2, GJB3,GJB6).

GJB2, TECTA, and TMC1

 cause both autosomal dominant and recessive forms of nonsyndromic HHI.

hearing loss associated with dominant genes

- onset in adolescence or adulthood,
- varies in severity
- progresses with age,

hearing loss associated with recessive inheritance

- congenital and profound.

Connexin 26
- product of the GJB2 gene
- responsible for nearly 20% of all cases of childhood deafness
- half of genetic deafness in children is GJB2-related.
 Contribution of genetics to presbycusis is also becoming better understood.
 Sensitivity to aminoglycoside ototoxicity can be maternally transmitted through a
mitochondrial mutation.
 Susceptibility to noise-induced hearing loss may also be genetically determined.
PREVENTION

Conductive hearing losses may be prevented by

 prompt antibiotic therapy of adequate duration for AOM
 ventilation of the middle ear with tympanostomy tubes in middle ear effusions
lasting ≥12 weeks.

Loss of vestibular function and deafness due to aminoglycoside antibiotics can be

prevented by:
 careful monitoring of serum peak and trough levels.

Noise induced hearing loss can be prevented by:

 avoidance of exposure to loud noise
 regular use of ear plugs or fluid-filled ear muffs to attenuate intense sound.

Virtually all noise-induced hearing loss is preventable through education, which should
begin before the teenage years. Programs for conservation of hearing in the workplace
are required by the Occupational Safety and Health Administration (OSHA) whenever
the exposure over an 8-h period averages 85 dB. Exposure to loud sounds above 85 dB
in the work environment is restricted by OSHA, with halving of allowed exposure time
for each increment of 5 dB above this threshold.
‘DISORDERS OF THE SENSE OF HEARING
Hearing loss
- can result from disorders of the auricle, external auditor canal, middle ear, inner ear,
or central auditory pathways

conductive hearing loss

- lesions in the auricle, external auditory canal, or middle ear that impede the
transmission of sound from the external environment to the inner ear

sensorineural hearing loss

- mechanotransduction in the inner ear or transmission of the electrical signal
along the eighth nerve to the bin
Conductive Hearing Loss

External ear, external auditory canal, middle ear apparatus

- designed to collect and amplify sound and efficiently transfer the mechanical
energy of the sound wave to the fluid-filled cochlea.

Conductive hearing loss

- result from factors that obstruct the transmission of sound or serve to dampen
the acoustical energy
- occur from:
o obstruction of the external auditory canal by cerumen, debris, and foreign
bodies
o swelling of the lining of the canal
o atresia or neoplasms of the canal
o perforations of the tympanic membrane
o disruption of the ossicular chain
 as occurs with necrosis of the long process of the incus in trauma
or infection
o otosclerosis
o fluid, scarring, or neoplasms in the middle ear
o inner ear malformations or pathologies,

Eustachian tube dysfunction

 extremely common in adults
 may predispose to acute otitis media (AOM) or serous otitis media (SOM)

usual factors responsible for tympanic membrane perforation:

 Trauma
 AOM
 chronic otitis media

small perforations
- often heal spontaneously

larger defects
- usually require surgical intervention.
o Tympanoplasty - highly effective (>90%) in the repair of tympanic
membrane perforations.

Otoscopy
- usually sufficient to diagnose AOM, SOM, chronic otitis media, cerumen
impaction, tympanic membrane perforation, and eustachian tube dysfunction

tympanometry
 - useful to confirm the clinical suspicion of these conditions
Cholesteatoma
- benign tumor
- composed of stratified squamous epithelium in the middle ear or mastoid
- occurs frequently in adults
- slowly growing lesion
- destroys bone and normal ear tissue
- Theories of pathogenesis:
 traumatic immigration and invasion of squamous epithelium through a
retraction pocket
 implantation of squamous epithelia in the middle ear through a
perforation or surgery,
 metaplasia following chronic infection and irritation
- On examination:
o perforation of the tympanic membrane filled with cheesy white squamous
debris
o presence of an aural polyp obscuring the tympanic membrane  highly
suggestive of an underlying cholesteatoma.
o chronically draining ear that fails to respond to appropriate antibiotic
therapy  suspicion of a cholesteatoma.

- Conductive hearing loss secondary to ossicular erosion is common.

- Surgery is required to remove this destructive process.

Conductive hearing loss with a normal ear canal and intact tympanic membrane
 suggests either ossicular pathology
 or the presence of “third window” in the inner ear

Fixation of the stapes from otosclerosis

o common cause of low-frequency conductive hearing loss.
o occurs equally in men and women
o inherited as an autosomal dominant trait with incomplete penetrance
o may be a manifestation of osteogenesis imperfect
o Hearing impairment usually presents between the late teens and the forties
o In women, otosclerotic process is accelerated during pregnancy
o hearing aid or a simple outpatient surgical procedure (stapedectomy) can
provide adequate auditory rehabilitation.

cochlear otosclerosis
o Extension of otosclerosis beyond the stapes footplate to involve the cochlea
o can lead to mixed or sensorineural hearing loss
o Fluoride therapy to prevent hearing loss from cochlear otosclerosis is of
uncertain value.

Disorders that lead to the formation of a pathologic “third window” in the inner ear
 - associated with conductive hearing loss
- normally two major openings, or windows:
o oval window - connect the inner ear with the middle ear
o round window - serve as conduits for transmission of sound
- A third window
o formed where the normally hard otic bone surrounding the inner ear is
eroded

Dissipation of the acoustic energy at the third window is responsible for the “inner ear
conductive hearing loss.”

Vertigo
- evoked by:
 loud sounds (Tullio phenomenon)
 Valsalva maneuvers that change middle ear pressure
 applying positive pressure on the tragus

Sensorineural Hearing Loss

Sensorineural hearing loss

- results from either:
o damage to the mechanotransduction apparatus of the cochlea
o disruption of the electrical conduction pathway from the inner ear to the
brain
- injury to hair cells, supporting cells, auditory neurons, or the central auditory
pathway can cause sensorineural hearing loss

Damage to the hair cells of the organ of Corti may be caused by:
o intense noise
o viral infections
o ototoxic drugs
 (e.g., salicylates, quinine and its synthetic analogues, aminoglycoside
antibiotics, loop diuretics such as furosemide and ethacrynic acid, and
cancer chemotherapeutic agents such as cisplatin)
o fractures of the temporal bone
o meningitis
o cochlear otosclerosis
o Meniere’s disease
o Aging

Presbycusis
o age-associated hearing loss
o most common cause of sensorineural hearing loss in adults
o early stages: symmetric, gentle to sharply sloping high-frequency hearing loss
o With progression, the hearing loss involves all frequencies
 o hearing impairment is associated with significant loss in clarity.
o loss of discrimination for phonemes, recruitment (abnormal growth of loudness),
and particular difficulty in understanding speech in noisy environments
o Hearing aids
 helpful in enhancing the signal-to-noise ratio by amplifying sounds that are
close to the listener
 they cannot restore the clarity of hearing
 may provide only limited rehabilitation once the word recognition score
deteriorates below 50%.
o Cochlear implants
 treatment of choice when hearing aids prove inadequate

Meniere’s disease
- characterized by episodic vertigo, fluctuating sensorineural hearing loss, tinnitus,
and aural fullness.
- annual incidence is 0.5– 7.5 per 1000
- onset is most frequently in the fifth decade of life
- Histologically: distention of the endolymphatic system (endolymphatic hydrops)
leading to degeneration of vestibular and cochlear hair cells.
- may result from:
o endolymphatic sac dysfunction secondary to infection, trauma,
autoimmune disease, inflammatory causes, or tumor;
o an idiopathic etiology constitutes the largest category and is most
accurately referred to as Meniere’s disease.
- Typically, low-frequency, unilateral sensorineural hearing impairment is present.
- Magnetic resonance imaging (MRI)
o obtained to exclude retrocochlear pathology
- Therapy is directed toward the control of vertigo.
o 2-g/d low-salt diet
 mainstay of treatment for control of rotatory vertigo.
o Diuretics, a short course of glucocorticoids, and intratympanic gentamicin
 useful adjuncts in recalcitrant cases
o Surgical therapy
 reserved for unresponsive cases
 includes:
 endolymphatic sac decompression
 labyrinthectomy
 vestibular nerve section
- no effective therapy for hearing loss, tinnitus, or aural fullness from Meniere’s
disease.

Sensorineural hearing loss may also result from any neoplastic, vascular,
demyelinating, infectious, or degenerative disease or trauma affecting the central
auditory pathways.
 HIV leads to both peripheral and central auditory system pathology and is associated
with sensorineural hearing impairment.

Primary diseases of the central nervous system can also present with hearing
impairment.
- a reduction in clarity of hearing and speech comprehension is much greater than
the loss of the ability to hear pure tone.
- Auditory testing is consistent with an auditory neuropathy; normal otoacoustic
emissions (OAE) and an abnormal auditory brainstem response (ABR) is typical

mixed hearing loss

- finding of conductive and sensory hearing loss in combination
- due to pathology of both the middle and inner ear

Trauma resulting in temporal bone fractures may be associated with conductive,

sensorineural, or mixed hearing loss. These abnormalities can be surgically corrected.
Profound hearing loss and severe vertigo are associated with temporal bone fractures
involving the inner ear.

A perilymphatic fistula associated with leakage of inner ear fluid into the middle ear
can occur and may require surgical repair. An associated facial nerve injury is not
uncommon.

Computed tomography (CT)

- best suited to assess fracture of the traumatized temporal bone, evaluate the ear
canal, and determine the integrity of the ossicular chain and the involvement of
the inner ear.

Cerebrospinal fluid leaks that accompany temporal bone fractures are usually self
limited; the value of prophylactic antibiotics is uncertain.

Tinnitus
- perception of a sound when there is no sound in the environment
- may have a buzzing, roaring, or ringing quality and may be pulsatile
(synchronous with the heartbeat)
- often associated with either a conductive or sensorineural hearing loss
- pathophysiology is not well understood

APPROACH TO THE PATIENT: Disorders of the Sense of Hearing

Goal is to determine the:

1. nature of the hearing impairment (conductive vs sensorineural vs mixed)
2. severity of the impairment (mild, moderate, severe, or profound)
3. anatomy of the impairment (external ear, middle ear, inner ear, or central
auditory pathway)
4. etiology
The history should elicit:
 characteristics of the hearing loss, including the duration of deafness, unilateral
versus bilateral involvement
 nature of onset (sudden vs insidious)
 rate of progression (rapid vs slow).
 Symptoms of tinnitus, vertigo, imbalance, aural fullness, otorrhea, headache,
facial nerve dysfunction, and head and neck paresthesias should be noted
 Information regarding head trauma, exposure to ototoxins, occupational or
recreational noise exposure, and family history of hearing impairment

sudden onset of unilateral hearing loss, with or without tinnitus

- may represent a viral infection of the inner ear, vestibular schwannoma, or a
stroke.
- usually complain of reduced hearing, poor sound localization, and difficulty
hearing clearly with background noise.

Small vestibular schwannomas

- typically present with asymmetric hearing impairment, tinnitus, and imbalance
(rarely vertigo

Hearing loss with otorrhea

- most likely due to chronic otitis media or cholesteatoma.

In examining the eardrum, the topography of the tympanic membrane is more

important than the presence or absence of the light reflex.

Insufflation of the ear canal

- necessary to assess tympanic membrane mobility and compliance.

Unilateral serous effusion

- should prompt a fiberoptic examination of the nasopharynx to exclude
neoplasms.

Rinne and Weber tuning fork tests

- 512-Hz tuning fork
- used to screen for hearing loss
- differentiate conductive from sensorineural hearing losses
- confirm the findings of audiologic evaluation

Rinne test
- compares the ability to hear by air conduction with the ability to hear by bone
conduction.
- tines of a vibrating tuning fork are held near the opening of the external auditory
canal, and then the stem is placed on the mastoid process;
 - patient is asked to indicate whether the tone is louder by air conduction or bone
conduction.
- Normally, and in the presence of sensorineural hearing loss
o tone is heard louder by air conduction than by bone conduction
- conductive hearing loss of ≥30 dB
o bone-conduction stimulus is perceived as louder than the air-conduction
stimulus
Weber test
- stem of a vibrating tuning fork is placed on the head in the midline and the patient
is asked whether the tone is heard in both ears or better in one ear than in the
other.
- With a unilateral conductive hearing loss
o tone is perceived in the affected ear
- With a unilateral sensorineural hearing loss
o Tone is perceived in the unaffected ear.
- 5-dB difference in hearing between the two ears is required for lateralization.

LABORATORY ASSESSMENT OF HEARING

Audiologic Assessment
minimum audiologic assessment for hearing loss should include:
 measurement of pure tone air-conduction and bone-conduction thresholds,
 speech reception threshold,
 word recognition score,
 tympanometry,
 acoustic reflexes
 Acoustic reflex decay

Pure tone audiometry

- assesses hearing acuity for pure tones
- administered by an audiologist
- performed in a sound attenuated chamber.
- Audiometer
o an electronic device that allows the presentation of specific frequencies
(generally between 250 and 8000 Hz) at specific intensities.
o Delivers pure tone stimulus
- Air- and bone-conduction thresholds are established for each ear.
- audiogram
o plot of intensity in decibels of hearing threshold versus frequency
- decibel (dB)
o equal to 20 times the logarithm of the ratio of the sound pressure required
to achieve threshold in the patient to the sound pressure required to
achieve threshold in a normal-hearing person.
- establishes the presence and severity of hearing impairment, unilateral versus
bilateral involvement, and the type of hearing loss
Speech audiometry
- tests the clarity with which one hears

speech reception threshold (SRT)

- intensity at which speech is recognized as a meaningful symbol
- obtained by presenting two-syllable words with an equal accent on each syllable.
- The intensity at which the patient can repeat 50% of the words correctly is the
SRT.
- Once the SRT is determined, discrimination or word recognition ability is tested
by presenting one-syllable words at 25–40 dB above the SRT.
- normal hearing or conductive hearing loss can repeat 88–100% of the
phonetically balanced words correctly.
- Patients with a sensorineural hearing loss have variable loss of discrimination.

Tympanometry
- measures the impedance of the middle ear to sound
- useful in diagnosis of middle ear effusions
- Tympanogram
o graphic representation of change in impedance or compliance as the
pressure in the ear canal is changed
o Patterns:
 Type A
 compliance of middle ear at atmospheric pressure
decreases as the pressure is increased or decreased
 Seen with normal hearing or in the presence of
sensorineural hearing loss.
 Type B
 Compliance that does not change with change in pressure
 suggests middle ear effusion (type B).
 Type C
 With a negative pressure in the middle ear, as with
eustachian tube obstruction, the point of maximal
compliance occurs with negative pressure in the ear canal
 Type Ad
 no point of maximal compliance can be obtained
 seen with discontinuity of the ossicular chain
 Type As
 reduction in the maximal compliance peak
 seen in otosclerosis
acoustic reflex
- intense tone elicits contraction of the stapedius muscle.
- change in compliance of the middle ear with contraction of the stapedius muscle
can be detected
- important in determining the etiology of hearing loss as well as in the anatomic
localization of facial nerve paralysis.
 - help differentiate between conductive hearing loss due to otosclerosis and that
caused by an inner ear “third window”:
- absent in otosclerosis and present in inner ear conductive hearing loss
- Normal or elevated acoustic reflex thresholds in an individual with sensorineural
hearing impairment suggest a cochlear hearing loss.
- absent acoustic reflex in the setting of sensorineural hearing loss is not helpful in
localizing the site of lesion.

acoustic reflex decay

- helps differentiate sensory from neural hearing losses.
- In neural hearing loss: reflex adapts or decays with time.
- Presence of OAEs :
o Indicates that outer hair cells of the organ of Corti are intact
o used to assess auditory thresholds
o distinguish sensory from neural hearing losses.

Evoked Responses
Electrocochleography
- measures the earliest evoked potentials generated in the cochlea and the
auditory nerve.
- useful in the diagnosis of Meniere’s disease, where an elevation of the ratio of
summating potential to action potential is seen

Brainstem auditory evoked responses (BAERs)

 aka as auditory brainstem responses (ABRs)
 useful in differentiating the site of sensorineural hearing loss
 valuable in situations in which patients cannot or will not give reliable voluntary
thresholds
 used to assess the integrity of the auditory nerve and brainstem in various clinical
situations, including intraoperative monitoring, and in determination of brain
death

vestibular-evoked myogenic potential (VEMP) test

 elicits a vestibulocolic reflex whose afferent limb arises from acoustically
sensitive cells in the saccule, with signals conducted via the inferior vestibular
nerve
 a biphasic, short-latency response recorded from the tonically contracted
sternocleidomastoid muscle in response to loud auditory clicks or tones.
 diminished or absent in patients with:
o early and late Meniere’s disease
o vestibular neuritis
o benign paroxysmal positional vertigo
o vestibular schwannoma.
 threshold may be lower in cases of
o superior canal dehiscence
 o other inner ear dehiscence
o perilymphatic fistula

Imaging Studies
Largely determined by whether the goal is to:
 evaluate the bony anatomy of the external, middle, and inner ear
 image the auditory nerve and brain

Axial and coronal CT of the temporal bone

 with fine 0.3- to 0.6-mm cuts
 ideal for determining the:
- caliber of the external auditory canal
- integrity of the ossicular chain
- presence of middle ear or mastoid disease
- detect inner ear malformations
 ideal for the detection of bone erosion with chronic otitis media and
cholesteatoma.

Poschl reformatting in the plane of the superior semicircular canal

- required for the identification of dehiscence or absence of bone over the superior
semicircular canal

MRI
- superior to CT for imaging of retrocochlear pathology

Both CT and MRI

- equally capable of identifying inner ear malformations and assessing cochlear
patency for preoperative evaluation of patients for cochlear implantation.

Treatment: Disorders of the Sense of Hearing

conductive hearing losses

- amenable to surgical correction

sensorineural hearing losses

- usually managed medically.

Atresia of the ear canal

- surgically repaired

Tympanic membrane perforations

- repaired with an outpatient tympanoplasty

conductive hearing loss associated with otosclerosis

- treated by stapedectomy,
middle ear effusions
- Tympanostomy tubes allow prompt return of normal hearing

conductive hearing losses

- Hearing aids

sensorineural hearing losses

- regularly rehabilitated with hearing aids

Unilateral deafness may benefit from:

 CROS (contralateral routing of signal) hearing aid
o microphone is placed on the hearing-impaired side and the sound is
transmitted to the receiver placed on the contralateral ear
 bone-anchored hearing aid (BAHA)
o hearing aid clamps to a screw integrated into the skull on the hearing
impaired side
o transfers the acoustic signal to the contralateral hearing ear, but it does so
by vibrating the skull
o
profound deafness on one side and some hearing loss in the better ear
 Candidates for a BICROS hearing aid
o patient wears a hearing aid, and not simply a receiver, in the better ear.

In the event that the hearing aid provides inadequate rehabilitation

 cochlear implants may be appropriate

Criteria for implantation include:

 severe to profound hearing loss with openset sentence cognition of ≤40% under
best aided conditions.
Cochlear implants
 neural prostheses that convert sound energy to electrical energy
 used to stimulate the auditory division of the eighth nerve directly
 consist of electrodes that are inserted into the cochlea through the round
window, speech processors that extract acoustical elements of speech for
conversion to electrical currents, and a means of transmitting the electrical
energy through the skin.

Tinnitus
- Therapy is usually directed toward minimizing the appreciation of tinnitus.
- obtained by masking it with background music
- Hearing aids are also helpful in tinnitus suppression,
- Antidepressants have been shown to be beneficial in helping patients cope with
tinnitus.

Hard-of-hearing individuals
- benefit from a reduction in unnecessary noise in the environment to enhance the
signal-to-noise ratio.
- Speech comprehension is aided by lip reading