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212 Disorders of the Auditory System
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and speech recognition scores often fluctuate in patients with active Ménière’s
disease.
Videonystagmography may further
be used to confirm clinical findings of
vestibulopathy; however, this test is normal in many patients with Ménière’s
disease, particularly in the early stages
of the disease. Electrocochleography is a
test that measures evoked potentials from
the cochlea and the cochlear nerve, which
has been used to assist in the diagnosis
of Ménière’s disease. Summating potentials (SP) and action potentials (AP) are
recorded and a SP/AP amplitude ratio
above 0.40 is associated with endolymphatic hydrops (Schwaber, Hall, & Zealear, 1991). However, one should carefully
interpret test results as abnormal electrocochleography results have been found
in patients with other auditory disorders.
If asymmetry in pure-tone audiometry,
tuning fork evaluation, or word recognition testing is identified, then radiologic
testing, such as MRI, or tests such as ABR
should be performed to rule out retrocochlear pathology.
Medical Examination
The diagnosis of Ménière’s disease is made
primarily by a thorough medical history
and not necessarily by an exam or diagnostic test. Specific questions should be
asked to rule out other potential diagnoses, such as perilymph fistula, ototoxicity,
autoimmune disease, infectious etiologies,
vestibular migraine, and retrocochlear
pathology. A thorough head and neck
examination augments the medical history and is typically unremarkable. In the
midst of a vestibular attack, patients will
exhibit spontaneous nystagmus. Neurotologic examination of vestibular function
may indicate the side of vestibulopathy
(see Clemmens & Ruckenstein, 2012).
Audiologic Management
Management of patients with Ménière’s
disease is often difficult from both a
medical and an audiologic standpoint.
This is because the fluctuations in the
disease process make it hard to predict
audiologic sensitivity and speech recognition ability from day to day. In addition,
although many patients’ hearing sensitivity warrants amplification, their speech
recognition abilities are often so poor that
they may receive little to no benefit from
amplification. Moreover, most patients
with Ménière’s disease are typically not
candidates for cochlear implants because
their noninvolved ear does not meet candidacy criteria. So, for many patients with
this inner ear disorder, the amplification
options are often limited and those that
are available may not provide optimal
benefits. This is typically a function of
poor word recognition ability. In cases in
which bilateral involvement is significant,
cochlear implantation may be considered.
As outlined by Valente and colleagues, it
is recommended that for those patients
with serviceable hearing, that hearing
aids with digital processing be employed
along with directional microphones and
assistive listening devices to aid in hearing in the presence of noise (Valente,
Mispagel, Valente, & Hullar, 2006). Most
patients with Ménière’s disease who are
to be fit with amplification will be managed most effectively with programmable
hearing aid devices that allow them the
flexibility to have multiple programs that
can accommodate their changing auditory
needs. And finally, counseling regarding
the use of such programs and the estab-
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lishment of reasonable expectations of
hearing aid benefit in various listening
conditions is also a critical component of
any hearing aid fitting, but it is especially
important when fitting a patient with
Ménière’s disease for reasons alluded to
previously (e.g., fluctuating hearing loss,
the potential of poor speech recognition
ability, changing auditory needs).
Medical Management
Acute episodes of vertigo are treated with
vestibular suppressants and antiemetics,
but long-term medical treatment of this
disease is directed primarily at preventing vertigo attacks. The uncertainty in
the etiology of this disease is reflected in
the types of management that are offered
to patients. No medical treatment has
been proven to be effective in improving
hearing or decreasing tinnitus and aural
fullness in all patients; however, some
patients may experience some relief in all
(or at least a subset) of these symptoms
with treatment. Although this disease is
not fatal and does not require treatment,
most patients suffering from recurrent
vertigo desire intervention. Hearing loss
related to Ménière’s disease is typically
treated with amplification. Prior to initiation of treatment, the degree of usable
hearing must be taken into account. Generally, patients with word recognition
testing scores of greater than 30% to 50%
are treated medically with hearing preservation options; although all patients
may be treated conservatively initially.
In order to decrease the endolymphatic
fluid pressure, conservative medical therapy involves the use of a low-sodium diet
and diuretics (Bojrab, Bhansali, & Battista,
1994). It has been found that some patients
who follow a low-sodium diet long term
note improvements in their symptoms
(Luxford, Berliner, Lee, & Luxford, 2013).
Such an approach has been shown to be
successful at vertigo control in 50% to 70%
of patients treated (Ruckenstein, Rutka, &
Hawke, 1991). The use of steroids, either
through a systemic or transtympanic
route, can also relieve recurrent vertigo
(Bojrab et al., 1994).
When the vertigo is not reasonably
controlled by these measures, further
intervention is indicated, which involves
ablating vestibular function on the affected side. It is counterintuitive to purposefully destroy vestibular function in
patients with vestibulopathy; however,
removal of residual vestibular function in
the diseased ear will allow the central vestibular centers to compensate for the unilateral involvement and prevent further
vertigo. Most of the ablative options have
efficacy measures that range from 70%
to 95%, but they carry a risk of hearing
loss. The transtympanic administration of
the vestibulotoxic antibiotic, gentamicin,
can be used to ablate vestibular function
if functional hearing is present, and typically this procedure can be performed in
an office setting. This type of nonsurgical
ablative procedure should be supported
with periodic audiologic and vestibular
monitoring.
A first-line surgical procedure in the
treatment of Ménière’s vertigo is a decompression of the endolymphatic sac and
duct and placement of a shunt into the
sac that allows for egress of excess endolymph into the mastoid, although the efficacy of this procedure has been debated.
This surgical procedure is performed
using a transmastoid surgical approach
and typically is completed as an outpatient procedure. The vestibular nerve
can also be sectioned surgically through
an intracranial retrosigmoid approach
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and carries a high success rate of vertigo
relief, but requires a craniotomy and a significant postoperative recovery period. In
cases where hearing is nonfunctional, a
transmastoid labyrinthectomy can be performed by drilling away the semicircular
canals and removing of all the neuroepithelium from the vestibule. This procedure removes the remainder of hearing,
but is more than 95% successful at vertigo
control (Schwaber, 2007). While several
destructive (i.e., vestibular nerve section)
and nondestructive (i.e., endolymphatic
sac surgery) surgical options are available, there have been no significant differences noted in patient-reported quality
of life outcome measures associated with
these procedures (Ballard et al., 2019).
Case 5–5: Bilateral
Ménière’s Disease
History
This 53-year-old male presented with
chronic and debilitating vertigo for a year
prior to his evaluation. He reportedly
experienced several episodes per week
lasting from a few minutes to hours. He
also presented with aural fullness and
“roaring” tinnitus bilaterally, and mentioned that these symptoms were more
severe in his left ear than in his right ear.
Hearing was reported to be significantly
decreased with daily fluctuations.
Audiology
An otoscopic check was unremarkable
bilaterally. The results of a comprehensive
audiologic evaluation indicated a moderately-severe rising to a mild sensorineural
hearing loss in the left ear and a severe rising to a moderately-severe sensorineural
hearing loss in the right ear with asymmetry noted between the ears (Figure
Speech recognition thresholds were in
good agreement with pure-tone averages
and word recognition was poor in the
right ear and good in the left ear.
5–5).
Medical Examination
The patient presented with an essentially
normal otolaryngologic examination.
Imaging results revealed no retrocochlear
involvement.
Impression
Probable bilateral Ménière’s disease.
Audiologic Recommendations
and Management
Binaural amplification was recommended
to assist the patient with his significant
hearing difficulties. The patient obtained
hearing aids, which were programmed
with multiple programs to help manage
the fluctuations in his hearing.
Medical Recommendations
and Management
The decision was made to treat the patient’s
left ear due to the fact that the symptoms
(hearing loss, aural fullness, and tinnitus)
were more severe on this side per patient
report and audiologic test results, and the
patient underwent a course of prednisone
and gentamicin treatments and demonstrated some improvements with this
approach. The prednisone and gentamicin treatments were employed in an effort
to control the vertigo by pharmacologic
intervention. A transtympanic gentamicin
titration was employed to depress labyrinthine function, resulting in relief from
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Figure 5–5. Pure-tone thresholds, speech audiometry, and tympanometry results for a 53-year-
old male with bilateral Ménière’s disease (Case 5–5).
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the vestibular symptoms. A low-sodium
diet was also recommended to help control symptoms. In addition, vestibular
rehabilitation to assist with the patient’s
imbalance was recommended.
Additional Information
The patient continues to struggle with
bilateral Ménière’s disease; however, he
has demonstrated significant improvement
in his vestibular symptoms since their
onset. As one would predict, no significant
changes in audiologic test results for either
pure-tone thresholds or word recognition
were observed following treatment.
Case 5–6: Unilateral
Ménière’s Disease
History
Audiology
An otoscopic check was unremarkable
bilaterally. Results of a comprehensive
audiologic evaluation indicated a relatively flat, moderate sensorineural hearing
loss in the right ear and normal peripheral
hearing sensitivity for the left ear (Figure
5–6A). Speech recognition thresholds
were in good agreement with pure-tone
averages, and word recognition scores
were excellent for both ears. Both VNG
(including ocular motor, positional, and
caloric evaluation) and vestibular evoked
myogenic potentials (VEMP) examinations were found to be within normal
limits. Due to presence of the congenital
hearing loss and normal VNG findings,
determining which ear was the involved
ear was difficult. Electrocochleography
testing was completed using a 90 dB nHL
click stimulus. Results from the ECochG
demonstrated a SP/AP ratio of about 60%
to 70% on the right side (Figure 5–6B).
A 27-year-old graduate student was initially seen for medical evaluation of a
long-standing hearing loss. At the time of
her evaluation, she reported that she has
had a hearing loss since birth and that her
hearing loss was originally diagnosed as a
mild sensorineural hearing loss. However,
she additionally noted that her hearing loss
had become progressively worse in recent
years and that, over the last several months
prior to her evaluation, she had developed some episodic vertigo. She denied
any tinnitus or aural fullness. She initially
thought that the change in her hearing
loss and the origin of her episodic vertigo
were caused by a mild carbon dioxide
exposure, but these symptoms continued
to persist following the exposure, with the
severity of the symptoms becoming progressively worse, resulting in both nausea
and vomiting. She also reported an unsuccessful attempt to use amplification.
Medical Examination
The patient presented with an essentially
normal otolaryngologic examination.
Imaging results revealed no retrocochlear
involvement.
Impression
Probable unilateral Ménière’s disease.
Audiologic Recommendations
and Management
Unilateral amplification was recommended
to assist the patient with her significant
hearing difficulties. The patient obtained
a hearing aid following medical clearance,
which was programmed with multiple
programs to help manage the fluctuations
in her hearing sensitivity. Hearing was
monitored following treatment with no
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A
Figure 5–6. Pure-tone thresholds, speech audiometry, and tympanometry results for a 27-year-
old female with unilateral Ménière’s disease (Case 5–6) (A) and electrocochleographic recordings
from a second individual with Meniere’s disease (B). The latter figure is included to demonstrate the
application of EcochG in Ménière’s disease. continues
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9.02 µV
(transtympanic)
3.31 µV
(canal)
.69 µV
05msec
B
(tympanic)
Figure 5–6. continued Transtympanic, tympanic, and ear canal record-
ings are shown from top to bottom (inverting electrode was the ECochG
electrode and the noninverting electrode was on the forehead). Note the differences in amplitude for the three recording sites and the presence of the ABR
waves in the canal electrode recording. The amplitude measures reflect measurements from the base of the SP to the peak of the AP wave I as shown by
dotted arrows. The SP/AP ratio remains about the same for all three recordings and exceeds 50% in each case.
significant changes in audiologic test
results for either pure-tone thresholds or
word recognition documented.
Medical Recommendations
and Management
The patient underwent a course of prednisone and gentamicin treatments and
demonstrated some improvements in
vestibular symptoms with this approach.
Specifically, a reduction in frequency
and severity of vertiginous spells were
observed. A low-sodium diet was recommended to help control her symptoms.
In addition, vestibular rehabilitation to
assist with the imbalance was recommended.
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autoimmune inneR
eaR disease
Introduction
Dysfunction of the immune system can
involve the inner ear resulting in fluctuating hearing loss with potential tinnitus
and vestibular dysfunction. This disease
may be caused by antibodies or activated
immunologic cells that are directed at the
inner ear. An autoimmune inner ear disease (AIED) classification system has been
developed and consists of (1) primary
disease, which originates within the inner
ear, and (2) secondary disease, which is
characterized by systemic disease that
affects the inner ear (Hughes, Barna, Calabrese, & Koo, 1993). Multiple systemic
autoimmune diseases have been known
to be related to AIED, several of which are
Cogan’s syndrome, rheumatoid arthritis,
lupus, Hashimoto’s thyroiditis, ulcerative
colitis, and Wegener’s granulomatosis
(Hughes et al., 1993). Cogan’s syndrome
is of particular interest because it mimics
Ménière’s disease in its presentation, but
it frequently is accompanied by ocular
symptoms. A diagnosis of AIED is typically made by exclusion of other diseases
that cause similar symptoms.
Symptoms
Autoimmune inner ear disease is characterized by bilateral fluctuating sensorineural hearing loss. The hearing
loss typically occurs over a few months,
unlike noise-induced or age-related hearing losses, which tend to have a much
longer time course. In addition, tinnitus
typically accompanies the hearing loss,
whereas vertigo or imbalance may or
may not accompany the cycles of hearing loss. Although the audiometric configuration in AIED may often mimic that
of Ménière’s disease, in many patients
with AIED, hearing loss is not the primary symptom. In cases with secondary
autoimmune disease, such as lupus or
Wegener’s granulomatosis, the external
and middle ears may also be affected.
Incidence and Prevalence
The incidence of AIED is debated, but
likely accounts for less than 1% of all cases
of hearing loss. About 50% of patients
with AIED have symptoms related to
balance (dizziness or unsteadiness) and
AIED is more common in patients with
other forms of systemic autoimmune disease (Ruckenstein, 2004).
Etiology and Pathology
The dysfunction of the immune system
that leads to inner ear disease is complex
and has yet to be fully elucidated. T-cell
lymphocytes have found to be reactive
against inner ear structures (McCabe &
McCormick, 1984). Antibodies that are
directed at the human inner ear also have
been identified (Arnold, Pfaltz, & Altermatt, 1985). The mechanism by which this
autoimmune process occurs, however,
is unknown.
Site of Lesion
Relatively little information is available
regarding the site at which AIED-induced
compromise occurs due to a paucity of
temporal bone specimens from patients
with active disease processes. It has been
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assumed to be a cochlear disorder. In onethird of patients, the 68 kilodalton protein
has been identified in inner ear extracts,
which would implicate the cochlea as the
site of action for autoantibodies (Harris &
Sharp, 1990).
Audiology
Audiologic evaluation of AIED typically
involves a comprehensive audiologic
evaluation along with immittance audiometry. As these patients often present
with fluctuations in hearing sensitivity,
serial audiograms are often necessary.
Medical Examination
The diagnosis of AIED is primarily made
through careful review of a patient’s history and the exclusion of other common
causes of hearing loss. Laboratory testing may be normal, especially in times
of normal hearing. Specific questions
should be asked to rule out other potential diagnoses, such as perilymph fistula,
ototoxicity, Ménière’s disease, infectious
etiologies, and retrocochlear pathology.
A thorough head and neck examination
augments the medical history and typically is unremarkable. Other systemic
autoimmune disease may cause other
otologic manifestations, such as a middle
ear effusion in Wegener’s granulomatosis.
Care should be taken to document other
head and neck manifestations of disease,
such as skin rashes or mucosal lesions.
Neurotologic examination of vestibular
function typically is normal. A panel of
serologic blood tests are ordered to examine for markers of systemic inflammation
and autoimmune antibodies. If asymmetry in pure-tone audiometry, tuning fork
evaluation, or word recognition testing
is identified, then further testing such as
ABR or MRI should be performed to rule
out retrocochlear pathology.
Audiologic Management
Patients with AIED often present in a manner similar to patients with Ménière’s disease. That is, they often have unpredictable
fluctuations in their hearing sensitivity.
Hearing aids may be utilized for hearing loss; however, significant fluctuations
make appropriate programming more
challenging. In patients who progress to
severe or profound hearing loss, cochlear
implants can be used successfully.
Medical Management
The mainstay of treatment for this disorder is systemic steroids, which may have
to be titrated to manage acute changes in
auditory function. Long-term steroid use
is not without complication and typically
is not recommended; thus, chemotherapeutic agents, such as cyclophosphamide
or methotrexate, are utilized for long-term
management. More recently, intratympanic steroid injections have been utilized
in an attempt to achieve the same clinical
effect without the systemic side effects.
Case 5–7: Autoimmune
Inner Ear Disease
History
This is a 60-year-old female who has been
followed for fluctuating hearing loss for
10 years. She was first evaluated for a sudden sensorineural hearing loss. Through-
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out her treatment, she has presented with
issues of imbalance and intermittent vertigo. Since the onset of her sudden hearing loss, she has had significant fluctuations in her hearing sensitivity.
Audiology
This patient has undergone many serial
audiograms over the years. Audiometric
test results both prior to and following
treatment are presented in Figures 5–7A
and 5–7B. Results obtained upon initial
presentation demonstrated a severe sensorineural hearing loss and poor word
recognition scores bilaterally (see Figure
5–7A). Posttreatment results are discussed
in the following sections.
Medical Examination
This patient has undergone extensive
medical evaluation by both rheumatology
and otolaryngology. Imaging test results
ruled out any retrocochlear involvement.
tion, following medical evaluation, treatment, and clearance. Following medical
intervention and clearance, the patient
returned for a hearing aid fitting and was
fit with binaural hearing aids that were
programmed with multiple programs to
assist with her hearing ability when fluctuations in hearing occurred. The patient’s
hearing continues to be closely monitored
due to fluctuations in hearing sensitivity.
Medical Recommendations
and Management
The patient was prescribed oral prednisone and underwent transtympanic dexamethasone injections. The patient continues to be treated with oral prednisone and
dexamethasone as needed to manage the
fluctuating hearing loss.
age-Related heaRing
loss (PResBycusis)
Impression
Probable autoimmune inner ear disease.
Audiologic Recommendations
and Management
The patient responded well to treatment
(see the medical recommendations and
management section) and posttreatment
audiologic results revealed significant
improvements in both hearing sensitivity
and word recognition ability with only a
mild sensorineural hearing loss and fair
to good word recognition scores being
observed for both ears (see Figure 5–7B).
Given the significant fluctuations noted in
hearing sensitivity, it was recommended
that the patient obtain binaural amplifica-
Introduction
Age-related hearing loss (ARHL), which
also has been referred to as presbycusis,
is the decrease of auditory function that
occurs with aging. Although the severity
ranges greatly, this type of auditory condition involves a decrease in acuity, understanding, and overall communication
and cognitive function. Although nearly
all adults experience a decrease in hearing sensitivity with time, some individuals are affected earlier in life and more
severely. Unfortunately, the peripheral
auditory system has very little reparative
capacity, so insults to the auditory system are additive over time. In addition to
changes in the peripheral system, changes
in the central auditory system are also
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