See

discussions, stats, and author profiles for this publication at: https://www.researchgate.net/publication/273324393

Treatment of Menires Disease

Article in Current Treatment Options in Neurology April 2015

DOI: 10.1007/s11940-015-0341-x Source: PubMed

CITATIONS READS

7 1,766

4 authors, including:

Jeffrey D Sharon Michael C Schubert

Johns Hopkins Medicine Johns Hopkins Medicine
19 PUBLICATIONS 71 CITATIONS 92 PUBLICATIONS 1,926 CITATIONS

SEE PROFILE SEE PROFILE

Some of the authors of this publication are also working on these related projects:

SARA - Sensorimotor Assessment and Rehabilitation Apparatus View project

Magnetic Vestibular Stimulation View project

All content following this page was uploaded by Michael C Schubert on 12 March 2015.

The user has requested enhancement of the downloaded file.

Curr Treat Options Neurol (2015) 17:14
DOI 10.1007/s11940-015-0341-x

Neurologic Ophthalmology and Otology (RK Shin and DR Gold, Section Editors)

Treatment of Menires
Disease
Jeffrey D. Sharon, MD*
Carolina Trevino, MD
Michael C. Schubert, PhD
John P. Carey, MD
Address
*
Department of Otolaryngology-Head and Neck Surgery,
Johns Hopkins School of Medicine, Baltimore, MD, USA
Email: Jsharon2@jhmi.edu

* Springer Science+Business Media New York 2015

This article is part of the Topical Collection on Neurologic Ophthalmology and Otology

Keywords Menieres disease I Vertigo I Intratympanic steroids I Vestibular physical therapy I Intratympanic
gentamicin I Pharmacotherapy for Menieres disease I Unilateral hearing loss

Opinion statement
Diagnosis of Menires disease is made with a characteristic patient history, including
discrete episodes of vertigo lasting 20 min or longer, accompanied by sensorineural
hearing loss, which is typically low frequency at first, aural fullness, and tinnitus.
Workup includes audiometry, a contrast enhanced MRI of the internal auditory canals,
and exclusion of other diseases that can produce similar symptoms, like otosyphilis,
autoimmune inner ear disease, perilymphatic fistula, superior semicircular canal syn-
drome, Lyme disease, multiple sclerosis, vestibular paroxysmia, and temporal bone tu-
mors. A history of migraine should be sought as well because of a high rate of co-
occurrence (Rauch, Otolaryngol Clin North Am 43:10111017, 2010). Treatment begins
with conservative measures, including low salt diet, avoidance of stress and caffeine, and
sleep hygiene. Medical therapy with a diuretic is the usual next step. If that fails to control
symptoms, then the options of intratympanic (IT) steroids and betahistine are discussed.
Next tier treatments include the Meniett device and endolymphatic sac surgery, but the
efficacy of both is controversial. If the above measures fail to provide symptomatic control
of vertigo, then ablative therapies like intratympanic gentamicin are considered. Rarely,
vestibular nerve section or labyrinthectomy is considered for a patient with severe
symptoms who does not show a reduction in vestibular function with gentamicin.
Benzodiazepines and anti-emetics are used for symptomatic control during vertigo epi-
sodes. Rehabilitative options for unilateral vestibular weakness include physical therapy
and for unilateral hearing loss include conventional hearing aids, contralateral routing of
sound (CROS) and osseointegrated hearing aids.
14 Page 2 of 16
Introduction
Menires disease is named after the French neurologist
Prosper Menire, who in 1861 argued that symptoms of
episodic vertigo and hearing loss could be caused by an
inner ear disorder [1]. While much has been learned
since then, there are still many unanswered questions,
including the underlying pathophysiology, how to diag-
nose Menires disease with certainty, whether or not
current treatments are effective, and how to prevent end
organ damage. Current diagnosis is usually based around
1995 American Academy of Otolaryngology (AAO)
criteria [2]. In that schema, definite Menires disease is
diagnosed with two or more spontaneous episodes of
vertigo lasting at least 20 min, audiometrically docu-
mented hearing loss on at least one occasion, tinnitus
or aural fullness, and exclusion of other causes. Diagnosis
can be upgraded to certain Menires disease with histo-
pathologic confirmation of hydrops, which refers to dis-
tension of the endolymphatic space within the membra-
nous labyrinth. At the time of writing, the Barany Society,
an international collaboration of vestibular researchers,
was working on ratifying a new schema for diagnosis of
Menires disease [3]. Their criteria for diagnosis include
two or more spontaneous episodes of vertigo lasting
20 min to 12 h, audiometrically documented low to
medium frequency sensorineural hearing loss, fluctuat-
ing aural symptoms in the affected ear, and no better
diagnosis. The finding of hydrops is considered the path-
ological hallmark of the disease and is found consistently
in post-mortem temporal bones of subjects with clinical
Menires disease; however, it is also found in temporal
bones of subjects without that clinical history. Therefore,
it is unclear if endolymphatic hydrops is a byproduct
of the underlying pathology or directly causative [4].
Various forms of vestibular testing, including
electrocochleography (ECoG) and vestibular evoked
myogenic potentials (VEMPs), have been used with
the goal of diagnosing Menires disease, with variable
results. Cervical VEMPs, in which loud sounds pro-
duce brief inhibitory potentials of the ipsilateral
sternocleidomastoid muscle through a saccule mediat-
ed reflex arc, have higher frequency specific thresholds
and lower amplitudes, consistent with the histologi-
cally observed distention of the saccule [5]. However,
they have not been found to be helpful in
distinguishing Menires disease from migraine, which
is a common diagnostic dilemma [6]. ECoG, in which
an acoustic stimulus produces an electric response
from the cochlea and eight nerve, has been shown
Curr Treat Options Neurol (2015) 17:14
by some authors to reliably diagnose Menires disease,
while others have not found it to be helpful [7, 8]. With
newer high resolution T2 MRI, and perilymphatic up-
take of contrast agents, it is becoming possible to visu-
alize in vivo distention of the endolymphatic space, and
therefore diagnostic criteria may change [9, 10].
Furthermore, it is becoming possible to correlate endo-
lymphatic distention on MRI with symptomatology and
findings on vestibular testing and to assess changes in
hydrops in response to treatment [11, 12]. No cure
currently exists, nor a reliable method of arresting dis-
ease progression, which may result in permanent senso-
rineural hearing loss and vestibular hypofunction in the
affected ear. Long-term observation studies have shown
mean decreases in the word recognition score to 67 % in
medically treated Menires disease and 44 % in surgical
treated Menires disease, with quality of life indicators
showing moderate handicaps related to hearing impair-
ment, dizziness, and tinnitus [13]. Numerous treatment
options are currently used with the aims of reducing the
severity and incidence of dizziness attacks, preventing
disease progression, treating the effects of end organ
damage like hearing loss, tinnitus, and chronic imbal-
ance, and providing symptomatic relief (Fig. 1). These
include dietary modifications like salt restriction, oral
pharmacologic therapy like diuretics and betahistine,
intratympanic pharmacologic therapies like gentamicin
and steroids, ablative surgical therapies like vestibular
nerve section or labyrinthectomy, and other therapies
like the Meniett device, or endolymphatic sac surgery
[14]. Overall, the evidence for each of these therapeutic
modalities, except for ablation and intratympanic ste-
roids, is somewhat lacking (Table 1). Studies are ham-
pered by the variability and natural course of the disease,
in which vertiginous symptoms do tend to improve over
time as hearing worsens, consistent with the cumulative
effects of end organ damage. In addition, many patients
exhibit a waxing and waning pattern to their symptoms
and the degree of end organ impairment. Therefore, any
study that does not consider time to be therapeutic, and
therefore omits a placebo arm in which similar time
elapses but without the proposed therapeutic interven-
tion, should be considered flawed. Furthermore, since
patients generally seek treatment when most symptom-
atic, even without the natural history of the disease
causing improvement over time, regression to the mean
in terms of symptom frequency and severity will also
create the illusion of therapeutic efficacy.
Curr Treat Options Neurol (2015) 17:14 Page 3 of 16 14

Fig. 1. Treatment algorithm for Menires disease. Blue arrows refer to decisions that should be made in all patients; red arrows refer
to disease progression. This algorithm was designed for treating unilateral disease.

Table 1. Level of evidence for treatment options in Menires disease

Type of treatment Specific treatment Level of Comment

evidence [66]
Dietary modification Salt restriction 5 Expert opinion [15]
Oral pharmacotherapy Diuretics 2b Low quality RTCs showing benefit [18]
Betahistine 2b Low quality RCTs showing benefit [21]
Intratympanic pharmacotherapy Steroids 1b RCT with clear benefit [26]
Gentamicin 1b RCT with clear benefit [33]
Surgical therapy Endolymphatic sac surgery 2b Two RCTs found no benefit, multiple
cohort studies with benefit [41]
Vestibular nerve section 2b Cohort studies with clear benefit [46]
Labyrinthectomy 2b Cohort studies with clear benefit [67]
Other Meniett device 2b 4 RCTs with no benefit, multiple cohort
studies with benefit [68]
RCT randomized controlled trial. Level of evidence is based on a grading scale from the Oxford Centre for evidence-based medicine [66]
14 Page 4 of 16 Curr Treat Options Neurol (2015) 17:14

Treatment

& Treatments in Menires disease are generally aimed at reducing

symptomology from the acute vertiginous episodes. To date, no treat-
ment has been convincingly shown to be effective in altering the
natural course of the disease, thereby preventing end organ damage
which results in hearing loss and vestibular impairment.

Diet and lifestyle

& Low salt diet is widely used as a first line treatment option [15, 16].
Daily sodium intake is recommended to be under 2000 mg, with some
recommending keeping intake below 1500 or 1000 mg. The low salt
intake is believed to be helpful in lowering endolymphatic pressure;
however, this is highly speculative. Currently, strong evidence that salt
restriction is beneficial does not exist. However, it is commonly used as
first line treatment because of anecdotal experience [17]. Stress reduc-
tion and avoidance of caffeine and alcohol are also commonly
recommended.

Pharmacologic treatment

& Pharmacologic therapy can be aimed at addressing the pathophysiol-

ogy of the disease, as with diuretics and betahistine, or at relieving
symptomatology from acute vertiginous episodes, as with
benzodiazepines.

Diuretics

& Diuretics are thought to alter endolymphatic electrolyte concentrations,

leading to decreased volume and pressure. Although they are commonly
used as first line treatments, a recent Cochrane review did not find any
quality evidence to argue for or against their usage [18]. However, other
smaller studies have found benefit in reduction of vertiginous episodes
[19]. Thiazide diuretics, with or without a potassium sparing diuretic, are
most commonly used for this purpose. However, many other diuretics
have been tried, such as acetazolamide, furosemide, and spironolactone,
the last of these being useful in patients with sulfa allergy.

Dyazide (hydrochlorothiazide/triamterene)

Standard dosage 25 mg hydrochlorothiazide with 37.5 mg triamterene taken orally once daily
Curr Treat Options Neurol (2015) 17:14 Page 5 of 16 14

Contraindications Renal impairment, hyperkalemia, concurrent potassium supplementation, hy-

persensitivity to thiazides, sulfonamides, or triamterene
Main drug interactions Caution with other drugs that increase serum potassium concentration to avoid
hyperkalemia, and also with antiarrhythmic drugs as Dyazide can potentiate
toxicity. Also interacts with methotrexate, lithium, cyclophosphamide,
pixantrone, and others.
Main side effects Common: weakness, dizziness, headache. Serious: cardiac arrhythmias,
hyperkalemia, renal failure, hypersensitivity.
Cost/cost-effectiveness Generic, inexpensive

Betahistine
& Betahistine is widely used in Europe as a first line treatment agent for
Menires disease. It is an H1 agonist and an H3 antagonist and is
thought to promote blood flow through the stria vascularis into the
cochlea in a dose-dependent fashion [20]. In addition, it may decrease
activity in the vestibular nuclei through neurotransmitter release. A
Cochrane review found some evidence for its efficacy but called for
larger trials [21]. The most effective dose is controversial, with recent
studies showing greater efficacy of 48 mg TID compared to 16 mg TID
and other studies showing further efficacy of doses up to 480 mg daily
[22, 23].

Betahistine

Standard dosage 8 to 48 mg orally three times daily

Contraindications Hypersensitivity, pheochromocytoma. Caution with asthma, gastric ulcers, and
liver disease.
Main drug interactions Anti-histamines may block effects.
Main side effects Nausea, headache, insomnia, GI disturbance, hypersensitivity reactions, rash,
pruritis, urinary frequency
Cost/cost-effectiveness Generic drug. Not FDA approved, so it can be difficult to obtain in the United
States, and requires a compounding pharmacy, which adds cost.

Benzodiazepines
& Benzodiazepines are used as vestibular suppressants for symptomatic
control during acute vertiginous attacks. Centrally acting H1 antago-
nists can also be used for this purpose.

Diazepam

Standard dosage 25 mg orally three times daily as needed for nausea

Contraindications Hypersensitivity, liver disease, glaucoma
Main drug interactions Caution with other drugs that cause CNS depression and drugs metabolized by
the liver
14 Page 6 of 16 Curr Treat Options Neurol (2015) 17:14

Main side effects Serious: respiratory depression, depression, suicidal thoughts. Common: som-
nolence, ataxia, dizziness
Special points Benzodiazepines should only be used on an as needed basis
Cost/cost-effectiveness Inexpensive

Intratympanic pharmacotherapy

& Intratympanic drug injections are aimed at delivering high doses of

drug to the inner ear via round window absorption while avoiding
systemic side effects. They carry a 1 % risk of creating a tympanic
membrane perforation and can induce middle ear inflammation
[24].

Intratympanic steroid injection

& Although originally assumed to have anti-inflammatory effects,

IT steroids like dexamethasone may have much more potent
mineralocorticoid effects in the inner ear than predicted from
renal studies. Dexamethasone, for example, increases the princi-
pal epithelial sodium transporter of semicircular canals by
threefold [25].
& In one placebo-controlled randomized clinical trial, intratympanic
dexamethasone was found to promote resolution of vertigo in 82 % of
subjects, as opposed to 57 % of controls [26, 27]. In another trial
using the concept of survival curves, 91 % of patients with Menires
disease had adequate symptom control with dexamethasone injections
and did not progress to require more ablative therapies [28]. One study
compared intratympanic gentamicin to dexamethasone in a random-
ized controlled trial, and found greater control of vertigo with genta-
micin, with minimal hearing damage [29]. IT steroids are well tolerat-
ed, and have proven efficacy in a majority of individuals, and are
therefore offered to patients who fail first line therapy. An ongoing
clinical trial is looking at a sustained-release formulation of
intratympanic dexamethasone to assess its efficacy in treating Menires
disease (clinicaltrials.gov, NCT01412177)
Standard procedure Risks and benefits are discussed, and consent is signed. The procedure is
performed in outpatient setting under an otologic operating microscope. The
tympanic membrane is topically anesthetized with either phenol or EMLA
(lidocaine 2.5 % and prilocaine 2.5 %) cream. A small ventilation hole is made
with a 25 gauge needle anteriorly, and then the drug is injected posteriorly/
inferiorly until the inferior middle ear space is full (~0.4 mL). The patient is
then instructed to lie with the injected ear facing up for 20 min to allow for
round window absorption.
Contraindications Active middle ear disease. Anatomy that would preclude round window uptake
of drug.
Curr Treat Options Neurol (2015) 17:14 Page 7 of 16 14

Complications Tympanic membrane perforation, middle ear inflammation.

Cost/cost-effectiveness Typically several hundred dollars per injection; however, usually, this provides
several months of symptomatic relief

Intratympanic gentamicin injection

& Gentamicin is an antibiotic that is both vestibulotoxic and

cochleotoxic. However, it has a high affinity for type 1 vestibular hair
cells and therefore produces relatively more vestibular impairment
than hearing loss. [30]. Streptomycin works in a similar fashion. It
should be noted that the aim of treatment, similar to other ablative
procedures, is reduction of acute vertiginous episodes, but the price
paid is unilateral vestibular hypofunction, which can cause symptoms
of imbalance with rapid ipsilateral head turns [31]. Rarely, postural
instability and oscillopsia are seen. Pharmacologic ablation with gen-
tamicin has been studied in a randomized controlled trial, which
showed a much greater reduction in the frequency of acute vertiginous
episodes as compared with saline injections, with no change in hearing
thresholds [32]. Therefore, it is thought that with proper dosing, there
is a therapeutic window available in which symptom reduction can be
achieved with only subclinical deleterious effects on hearing and
symptomatic balance function. In another randomized controlled trial
comparing intratympanic gentamicin to saline, significant reduction in
vertigo scores were seen in the gentamicin arm, along a small reduction
in hearing thresholds (mean 8 dB) [33]. Another paper showed a 17 %
risk of worsening hearing [34]. It is important to emphasize that
gentamicin is effective for vertigo control while only producing a partial
vestibular loss, while ablative surgical options create a total unilateral
vestibular loss [31]. Sometimes, multiple injections are required, and
these can be titrated to control vertigo symptoms, although some argue
for titration until there are signs of unilateral vestibular weakness, such
as a positive head thrust sign, post-headshake nystagmus, or sponta-
neous nystagmus beating towards the opposite ear. Titration of genta-
micin until there is complete loss of unilateral vestibular function, with
absent ice water caloric responses, is usually unnecessary and can result
in worse hearing and balance outcomes.
Standard procedure Same procedure as with intratympanic steroid injection, see above for
description.
Contraindications Only ear with hearing or balance function. Active middle ear infection.
Complications Hearing loss, unilateral vestibular hypofunction.
Cost/cost-effectiveness Typically several hundred dollars per injection; however, 54 % of patients only
require one injection, and 96 % are able to avoid ablative surgery through
injections [35].
14 Page 8 of 16 Curr Treat Options Neurol (2015) 17:14

Surgery

& Ablative surgery involves surgical interruption of unilateral peripheral

vestibular input. While this guarantees unilateral vestibular weakness,
central compensation for a static unilateral deficit occurs with time.
This compensation can be accelerated with vestibular physical therapy.
However, the potential for development of bilateral disease, which one
paper conservatively estimated at a 5 % risk, decreases the attractiveness
of totally ablative options [36]. The specific surgical procedure chosen
depends on the hearing status: for cases with residual hearing, vestib-
ular nerve section is preferred, and for cases without residual hearing,
labyrinthectomy is chosen. It should also be noted that since genta-
micin can achieve a partial but effective chemical labyrinthectomy in
most cases, these surgeries are usually now only performed for genta-
micin non-responders. Furthermore, there is clinical evidence that in
most gentamicin non-responders, anatomic factors like adhesions or
bone dust are physically obstructing the round window membrane,
precluding drug uptake. Therefore, middle ear exploration with
exposure of the round window membrane and direct application
of gentamicin pledgets is effective at controlling vertigo in 75 %
of gentamicin non-responders, thereby avoiding the need for
more invasive surgery [37].

Endolymphatic sac surgery

& The endolymphatic sac is an outpouching of the endolymphatic

membrane into the dura beneath the posterior fossa plate of bone. The
sac is connected to the membranous utricle through the endolymphatic
duct. It was classically thought to be involved in resorption of endo-
lymph. However, there is also evidence that the sac is involved in
immune function of the inner ear, possibly initiating immune re-
sponses after antigen processing and presentation [38]. Endolymphatic
sac surgery is aimed at shunting, draining, or decompressing the sac,
thereby preventing hydrops by facilitating outflow of endolymph. This
is considered a non-destructive procedure. It was originally described
by Portmann in 1927 and has been widely performed and hotly
contested since then [39]. A Cochrane review in 2013 examined the
evidence for surgery in the treatment of Menires disease [40, 41].
They included 2 RCTs, both of which involved endolymphatic sac
surgery compared to either pressure equalization tube insertion or
sham mastoid surgery. No differences were seen between treatment and
placebo groups. However, this remains a very controversial area [42].
One histologic study looked at temporal bones after sac surgery and
found that correct placement of the shunt into the sac had no relation
to postoperative relief of vertigo, which was commonly seen [43]. In
terms of surgical technique, similar results are seen with decompression
of the sac versus shunting [44].
Curr Treat Options Neurol (2015) 17:14 Page 9 of 16 14

Standard procedure The patient is placed under general anesthesia, and facial nerve monitoring
electrodes are placed. Antibiotics are administered, and a standard mastoidec-
tomy is performed, with decompression of the sigmoid sinus. Next, the endo-
lymphatic sac is identified posterior to the posterior semicircular canal, along
the posterior fossa plate, below Donaldsons line. In a decompression surgery,
the bone over the sac is widely removed. In a shunting procedure, a stent,
usually Silastic, is placed into the sac, which directs endolymph either into the
mastoid or CSF compartment.
Contraindications Active mastoid or middle ear disease is considered a contraindication.
Complications Complications of mastoid surgery include hearing loss, dizziness, CSF leak,
damage to the sigmoid sinus, facial paralysis, and general risks of surgery and
anesthesia. Furthermore, bone dust from surgery can be spread to the ossicles,
causing a conductive hearing loss, or to the round window niche, which may
impair future intratympanic drug administration [45].
Cost/cost-effectiveness Endolymphatic sac surgery is an outpatient surgical procedure that takes ap-
proximately 23 h under general anesthesia.

Vestibular nerve section

Standard procedure Introduced by Dandy in 1928, vestibular nerve section can be accom-
plished through a retrosigmoid or retrolabyrinthine approach. In the
former, a suboccipital craniotomy is performed with monitoring of the
facial nerve and auditory evoked potentials. The inferior and superior
vestibular nerves are identified and sectioned at the porus acusticus,
taking care not to injure the facial or cochlear nerve. Identification of the
vestibular nerves can be facilitated by decompression of the internal
auditory canal laterally so as to definitively locate landmarks such as the
horizontal and vertical crests (Bills bar) and the singular nerve to the
posterior semicircular canal ampulla. The wound is then closed in stan-
dard fashion after cranioplasty. In the retrolabyrinthine approach, a
mastoidectomy is performed, with decompression of the sigmoid sinus
and identification of the posterior canal and the vertical segment of the
facial nerve. The posterior fossa dura is then entered between the sigmoid
and the otic capsule, and the internal auditory canal is decompressed to
visualize the individual nerves. The vestibular, cochlear, and facial nerves
are identified, and the vestibular nerves are then carefully sectioned. A fat
graft is placed within the mastoid cavity to prevent CSF leak, and the
wound is closed.
Contraindications If there is no useful hearing, then labyrinthectomy is preferred because of lower
risk profile.
Complications Complications from posterior fossa surgery include CSF leak, meningitis, cra-
nial neuropathies, seizure, stroke, death, in addition, the usual risks of surgery
and anesthesia. The retrolabyrinthine route reduces the risks of posterior fossa
surgery but adds some risk from the mastoidectomy. In one large study, the risk
of greater than 10 dB sensorineural hearing loss for retrolabyrinthine vestibular
nerve sectioning surgery was estimated at 10 % [46].
Special points These procedures are now rarely performed because less risky and costly alter-
natives for vestibular ablation are available. Vestibular physical therapy is
helpful postoperatively to assist with central compensation and return to
functionality.
Cost/cost-effectiveness Inpatient surgery with 2 to 5-day hospital stay.
14 Page 10 of 16 Curr Treat Options Neurol (2015) 17:14

Labyrinthectomy

Standard procedure The goal of surgery is removal of neuroepithelium from the five vestibular end
organs: the three semicircular canals, the utricle, and the saccule. The patient is
placed under general anesthesia, and facial nerve monitoring electrodes are
placed. Antibiotics are administered, and a standard mastoidectomy is per-
formed. The tegmen, sigmoid sinus, horizontal canal, and facial nerve are all
identified. The horizontal semicircular canal is then entered, and followed
posteriorly to the posterior semicircular canal, which in turn is followed to the
common crus to identify the superior canal. Great care is taken at the anterior
aspects of the horizontal and posterior canals to avoid injury to the facial nerve.
The canals are then followed into the vestibule. The neuroepithelium is then
removed from the ampullated end of each semicircular canal, and the utricle is
removed from the elliptical recess superiorly in the vestibule, and the saccule
from the inferiorly located spherical recess. Once all neuroepithelium has been
removed, the wound is closed in layers, and a mastoid dressing is applied.
Contraindications The procedure produces hearing loss and unilateral vestibular weakness, and is
therefore contraindicated in an only hearing ear, and also with contralateral
vestibular impairment.
Complications Complications are related to mastoid surgery; they include dizziness, CSF leak, damage
to the sigmoid sinus, facial paralysis, and general risks of surgery and anesthesia.
Special points After surgery, depending on preoperative vestibular function, it is normal to
have a horizontal rotary nystagmus with the fast phase directed towards the
non-operated ear. This usually resolved in a few days. In addition, a skew
deviation can occasionally be observed due to acute disruption of unilateral
utricular input. Vestibular physical therapy is helpful postoperatively to assist
with central compensation and return to functionality.
Cost/cost-effectiveness Surgery usually takes 3 to 5 h, and an inpatient hospital stay of 1 to 3 days is expected.

Assistive devices

& Over time, many patients with Menires disease develop permanent
unilateral hearing loss. Treatment options include conventional hear-
ing aids if there is still useful hearing in the affected ear, and contra-
lateral routing of sound (CROS) hearing aids or osseointegrated hear-
ing aids if hearing is not useful in the affected ear. Useful hearing is
usually defined as a speech discrimination score above 50 %. In addi-
tion, cochlear implants are being studied for their utility in single-sided
deafness, and their use in bilateral end-stage Menires disease is well
supported [47].

Conventional hearing aid

Usage A hearing aid consists of, at minimum, a microphone that picks up sounds
from the environment, an amplifier that increases the sound intensity, and a
receiver that acts like a speaker delivering sound to the ear. Modern hearing aids
include on-board or remote processing circuitry to improve the quality of the
transmitted sound by fixed filters or context-specific filters that aim to increase
the signal to noise ratio.
Curr Treat Options Neurol (2015) 17:14 Page 11 of 16 14

Cost/cost-effectiveness Insurance coverage for hearing aids is limited and they may cost several thou-
sand dollars.
CROS (contralateral routing of sound) hearing aid

Usage This is a specialized hearing aid system in which a transmitter is placed on the
poorer hearing ear to collect sounds and wirelessly transmit them to a receiver
placed on the better hearing ear. This allows sound information to be collected
from the side of the head with hearing impairment, which helps with speech
understanding and sound localization.
Cost/cost-effectiveness Insurance coverage for hearing aids is limited and they may cost several thou-
sand dollars.
Osseointegrated mastoid implant + bone-conduction processor

Usage Osseointegrated mastoid implants coupled with bone-conduction processors,

commonly called bone-anchored hearing aids, depend on bone-mounted
hardware placed during a brief outpatient surgical procedure that can be
performed under general or local anesthesia. Once the implant is placed, there
is a waiting period up to several months during which osseointegration occurs.
After that, a sound processor is placed on the abutment, and sounds are then
transmitted via bone conduction through the skull base to the contralateral
cochlea, reducing the hearing impairment of single sided deafness.
Special points Wound care issues can occur, and local treatments are generally effective;
however, occasionally revision surgery is required.
Cost/cost-effectiveness Brief outpatient surgical procedure. The cost of the processor, which is several
thousand dollars, is usually covered by insurance as part of the procedure cost.

Physical/speech therapy and exercise

& In addition to the acute vertiginous episodes, patients with Menires

disease will usually experience a gradual decline in vestibular function
on the affected side. This may result in baseline imbalance if the brain
does not properly adapt to asymmetric peripheral vestibular input.
Therefore, vestibular physical therapy (VPT) is often helpful to assist
with central compensation for the progressive unilateral loss.
Numerous trials have shown benefit for vestibular therapy in improv-
ing function with unilateral vestibular loss [48].

Vestibular physical therapy

Usage VPT is a specialized rehabilitation that instructs patients in home exercise

routines that improve gaze and postural stability while reducing fall risk. The
gaze stability exercises are designed to challenge the brain to keep the eyes still
in space during head rotation, when the vestibulo-ocular reflex (VOR) is
deficient in doing so. Evidence suggests that this occurs from two means: an
increase in the vestibular generated slow phase eye velocity (VOR gain) and an
increase in the frequency of compensatory saccades [49, 50, 51]. Postural
exercises challenge balance by altering the visual and proprioceptive afferents.
There is now strong evidence summarized via Cochrane reviews that VPT is an
efficient means to improve balance, gait, and reduce fall risk [48, 52, 53].
14 Page 12 of 16 Curr Treat Options Neurol (2015) 17:14

Recently, VPT using virtual reality methods was shown to reduce perception of
handicap due to dizziness and improve postural stability in patients with
Menires disease [54].
Cost/cost-effectiveness VPT is generally covered by insurance. The duration of physical therapy will
depend on the extent of vestibular hypofunction. Most patients tolerate a
home-based exercise program with weekly or biweekly outpatient visits for
exercise progression over 4 to 8 weeks [55, 56]. Estimates suggest VPT costs from
$5001500 per month depending on need.

Other treatments

& The Meniett Device (Medtronic Xomed, Jacksonville, Florida, USA) is a

handheld air pressure generator which delivers brief pulses of pressure
to the inner ear. Treatments are given for 5 min, three times daily.

Meniett device

Standard procedure Usage of the Meniett device requires placement of a pressure equalization tube
within the tympanic membrane. The device is then placed within the external
auditory canal, and pulses of air pressure are generated that are transmitted
from the external ear to the middle ear, and are thought to vibrate the round
window. This is thought to aid in endolymphatic redistribution and turnover.
Three treatments of 5-min duration are administered daily.
Contraindications The Meniett device is safe and well tolerated. It cannot be used if a pressure
equalization tube cannot be placed. Relative contraindications include disor-
ders of the external ear like chronic otitis externa that would complicate
frequent placement of a device within the ear canal and any middle ear disease
that blocks access to the round window.
Complications Complications are rare with the Meniett device. Most reported complications
are related to the PE tube, which can cause otitis media or a tympanic mem-
brane perforation.
Special points Several recent meta-analyses looking at the effectiveness of the Meniett device
have found conflicting results. One study looked at 4 randomized clinical trials
and found no evidence for effectiveness of the device over placebo [57].
Another meta-analysis included 12 studies, including 2 RCTs, 3 prospective
studies, 4 retrospective studies, and 3 cross-sectional/unknown studies, and
found that the Meniett device was effective at restoring some hearing (3.5 dB
pure tone average gain) and in reducing vertigo frequency. However, that study
mostly pooled single armed studies without control groups, and should there-
fore be interpreted with caution [58].
Cost/cost-effectiveness The Meniett device costs approximately $2500, and this is usually not covered
by insurance. In addition, a PE tube must be placed.

Emerging therapies

& OTO-104 is a novel formulation for sustained release of dexametha-

sone. It has been evaluated in a recent single-dose and placebo-
controlled clinical trial. The steroid is contained in a solution with a
thermoreversible polymer excipient which gels once exposed to body
Curr Treat Options Neurol (2015) 17:14 Page 13 of 16 14

temperature after it injected into the middle ear. This delivery method
allows prolonged steroid exposure to the inner ear. The results dem-
onstrated OTO-104 to be safe and suggested a decrease in vertigo
frequency at 3 months as well as tinnitus handicap [59]. At the time
of writing, this drug was being evaluated in a multicenter clinical trial
for Menires disease [60].

Pediatric considerations

& Perhaps because of its rare occurrence, only a few case series have
investigated Menires disease in pediatric populations. They reported
low incidences ranging from 0.4 % by Stahle et al. [61], approximately
3 % by Meyerhoff et al. [62], and 7 % by Filipo et al. [63].
& There are no large-scale studies that evaluate the effectiveness of
Menires disease therapies in children. In published case series, treat-
ment was offered in a stepwise manner starting with dietary restriction
of sodium. Medications can be offered if there is insufficient control
over symptomatology; diuretics are suggested as a first line therapy.
Vestibular depressants and anti-histamines can be administered as well.
Placement of transtympanic ventilation tubes and intratympanic ad-
ministration of gentamicin have also been reported to provide symp-
tomatic relief [64, 65]. Endolymphatic sac drainage surgery can be
pursued if more conservative treatments fails [62, 63].

Acknowledgments
A grant from Otonomy to the Johns Hopkins School of Medicine funds this work. The contract is approved and
monitored by the institutions Office of Research Administration, the Conflict of Interest Committee and the
Institutional Review Board.

Compliance With Ethics Guidelines

Conflict of Interest
Jeffrey D. Sharon, Carolina Trevino, and Michael C. Schubert declare no conflicts of interest. John P. Carey
declares that he is a site investigator for a clinical trial of OTO-104 for control of vertigo in MD.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References and Recommended Reading

Papers of particular interest, published recently, have been
highlighted as:
Of importance
Of major importance
14 Page 14 of 16
1. Baloh RW. Prosper Meniere and his disease. Arch
Neurol. 2001;58(7):11516.
2. Monsell EM et al. Committee on hearing and equilib-
rium guidelines for the diagnosis and evaluation of
therapy in Menieres disease. Otolaryngol Head Neck
Surg. 1995;113D3]:1815.
This article established commonly used guidelines for diag-
nosing Menires disease and evaluating treatment efficacy.
3. Society B. Diagnostic criteria for Menires disease.
Available from: http://www.jvr-web.org/images/
55807-Lopez-Escamez.pdf
4. Rauch SD, Merchant SN, Thedinger BA. Menieres syn-
drome and endolymphatic hydrops. Double-blind
temporal bone study. Ann Otol Rhinol Laryngol.
1989;98D11]:87383.
A temporal bone study that helped clarify the relationship of
histological endolymphatic hydrops and clinical Menires
disease.
5. Rauch SD et al. Vestibular evoked myogenic potentials
show altered tuning in patients with Menieres disease.
Otol Neurotol. 2004;25(3):3338.
6. Zuniga MG et al. Can vestibular-evoked myogenic po-
tentials help differentiate Meniere disease from vestib-
ular migraine? Otolaryngol Head Neck Surg.
2012;146(5):78896.
7. Hornibrook J et al. Transtympanic
electrocochleography for the diagnosis of Menieres
disease. Int J Otolaryngol. 2012;2012:852714.
8. Claes GM et al. The Menieres disease index: an objec-
tive correlate of Menieres disease, based on audio-
metric and electrocochleographic data. Otol Neurotol.
2011;32(5):88792.
9. Nakashima T et al. Visualization of endolymphatic
hydrops in patients with Menieres disease. Laryngo-
scope. 2007;117D3]:41520.
This important paper showed that it was possible to visualize
endolymphatic hydrops in Menires disease using high reso-
lution MRI and intratympanic contrast.
10. Pyykko I et al. Magnetic resonance imaging of the inner
ear in Menieres disease. Otolaryngol Clin North Am.
2010;43(5):105980.
11. Gurkov R et al. Herniation of the membranous labyrinth
into the horizontal semicircular canal is correlated with
impaired caloric response in Menieres disease. Otol
Neurotol. 2012;33D8]:13759.
This study is important because it showed that novel MRI
techniques used to visualize endolymphatic hydrops in vivo
do correlate with objective measures like vestibular testing.
12. Gurkov R et al. In vivo visualization of endolymphatic
hydrops in patients with Menieres disease: correlation
with audiovestibular function. Eur Arch
Otorhinolaryngol. 2011;268(12):17438.
13. Kinney SE, Sandridge SA, Newman CW. Long-term
effects of Menieres disease on hearing and quality of
life. Am J Otol. 1997;18(1):6773.
14. Sajjadi H, Paparella MM. Menieres disease. Lancet.
2008;372(9636):40614.
Curr Treat Options Neurol (2015) 17:14
15. Minor LB, Schessel DA, Carey JP. Menieres disease.
Curr Opin Neurol. 2004;17(1):916.
16. Smith WK, Sankar V, Pfleiderer AG. A national survey
amongst UK otolaryngologists regarding the treatment
of Menieres disease. J Laryngol Otol.
2005;119(2):1025.
17. Greenberg SL, Nedzelski JM. Medical and noninvasive
therapy for Menieres disease. Otolaryngol Clin North
Am. 2010;43(5):108190.
18. Thirlwall AS, Kundu S. Diuretics for Menieres disease
or syndrome. Cochrane Database Syst Rev.
2006;3:CD003599.
19. van Deelen GW, Huizing EH. Use of a diuretic
(Dyazide) in the treatment of Menieres disease. A
double-blind cross-over placebo-controlled study. ORL
J Otorhinolaryngol Relat Spec. 1986;48(5):28792.
20. Ihler F et al. Betahistine exerts a dose-dependent effect
on cochlear stria vascularis blood flow in guinea pigs
in vivo. PLoS One. 2012;7(6):e39086.
21. James AL and Burton MJ. Betahistine for Menieres
disease or syndrome. Cochrane Database Syst Rev.
2001(1):CD001873.
This systematic review examined the evidence for betahistine,
which is widely used around the world as a treatment for
Menires disease.
22. Strupp M et al. Long-term prophylactic treatment of
attacks of vertigo in Menieres diseasecomparison of
a high with a low dosage of betahistine in an open trial.
Acta Otolaryngol. 2008;128(5):5204.
23. Lezius F et al. High-dosage betahistine dihydrochloride
between 288 and 480 mg/day in patients with severe
Menieres disease: a case series. Eur Arch
Otorhinolaryngol. 2011;268(8):123740.
24. Rauch SD et al. Oral vs intratympanic corticosteroid
therapy for idiopathic sudden sensorineural hearing
loss: a randomized trial. JAMA. 2011;305(20):20719.
25. Pondugula SR et al. Glucocorticoids stimulate cation
absorption by semicircular canal duct epithelium via
epithelial sodium channel. Am J Physiol Renal Physiol.
2004;286(6):F112735.
26. Garduno-Anaya MA et al. Dexamethasone inner ear
perfusion by intratympanic injection in unilateral
Menieres disease: a two-year prospective, placebo-
controlled, double-blind, randomized trial.
Otolaryngol Head Neck Surg. 2005;133D2]:28594.
A well-conducted trial that helped establish the utility of
intratympanic steroids as a treatment for Menires disease.
27. Phillips JS and Westerberg B. Intratympanic steroids for
Menieres disease or syndrome. Cochrane Database
Syst Rev. 2011(7):CD008514.
Systematic review that looked at evidence for intratympanic
steroids in treating Menires disease.
28. Boleas-Aguirre MS et al. Longitudinal results with
intratympanic dexamethasone in the treatment of
Menieres disease. Otol Neurotol. 2008;29(1):338.
29. Casani AP et al. Intratympanic treatment of intractable
unilateral Meniere disease: gentamicin or dexametha-
sone? A randomized controlled trial. Otolaryngol Head
Neck Surg. 2012;146(3):4307.
Curr Treat Options Neurol (2015) 17:14
30. Lyford-Pike S et al. Gentamicin is primarily localized in
vestibular type I hair cells after intratympanic admin-
istration. J Assoc Res Otolaryngol. 2007;8D4]:497508.
This animal study showed the differential uptake of gentamicin
by type 1 vestibular hair cells.
31. Carey JP et al. Changes in the three-dimensional an-
gular vestibulo-ocular reflex following intratympanic
gentamicin for Menieres disease. J Assoc Res
Otolaryngol. 2002;3D4]:43043.
This study examined the physiologic changes that occurred to
the vestibulo-ocular reflex after gentamicin administration.
32. Stokroos R, Kingma H. Selective vestibular ablation by
intratympanic gentamicin in patients with unilateral
active Menieres disease: a prospective, double-blind,
placebo-controlled, randomized clinical trial. Acta
Otolaryngol. 2004;124D2]:1725.
A placebo controlled study that added to the body of literature
establishing the effectiveness of gentamicin in treating
Menires disease.
33. Postema RJ et al. Intratympanic gentamicin therapy for
control of vertigo in unilateral Menieres disease: a
prospective, double-blind, randomized, placebo-con-
trolled trial. Acta Otolaryngol. 2008;128D8]:87680.
Another well conducted study that showed the effectiveness of
gentamicin for treating Menires disease.
34. Wu IC, Minor LB. Long-term hearing outcome in pa-
tients receiving intratympanic gentamicin for Menieres
disease. Laryngoscope. 2003;113(5):81520.
35. Nguyen KD et al. Time course of repeated
intratympanic gentamicin for Menieres disease. La-
ryngoscope. 2009;119(4):7928.
36. Perez R, Chen JM, Nedzelski JM. The status of the
contralateral ear in established unilateral Menieres
disease. Laryngoscope. 2004;114(8):13736.
37. Crane BT et al. Middle ear exploration in patients with
Menieres disease who have failed outpatient
intratympanic gentamicin therapy. Otol Neurotol.
2009;30(5):61924.
38. Satoh H et al. Proinflammatory cytokine expression in
the endolymphatic sac during inner ear inflammation.
J Assoc Res Otolaryngol. 2003;4(2):13947.
39. Portmann G. The saccus endolymphaticus and an op-
eration for draining for the relief of vertigo. Proc R Soc
Med. 1927;20(12):18627.
40. Sood AJ et al. Endolymphatic sac surgery for Menieres
disease: a systematic review and meta-analysis. Otol
Neurotol. 2014;35D6]:103345.
A systematic review that examined the evidence for endolym-
phatic sac surgery in Menire's diease.
41. Pullens B, Verschuur HP, van Benthem PP. Surgery for
Menieres disease. Cochrane Database Syst Rev.
2013;2:CD005395.
42. Welling DB, Nagaraja HN. Endolymphatic mastoid
shunt: a reevaluation of efficacy. Otolaryngol Head
Neck Surg. 2000;122(3):3405.
43. Chung JW et al. Histopathology after endolymphatic
sac surgery for Menieres syndrome. Otol Neurotol.
2011;32(4):6604.
Page 15 of 16 14
44. Brinson GM, Chen DA, Arriaga MA. Endolymphatic
mastoid shunt versus endolymphatic sac decompres-
sion for Menieres disease. Otolaryngol Head Neck
Surg. 2007;136(3):41521.
45. Minor LB. Intratympanic gentamicin for control of
vertigo in Menieres disease: vestibular signs that
specify completion of therapy. Am J Otol.
1999;20(2):20919.
46. Goksu N et al. Combined retrosigmoid
retrolabyrinthine vestibular nerve section: results of our
experience over 10 years. Otol Neurotol.
2005;26(3):4813.
47. Lustig LR et al. Cochlear implantation in patients with
bilateral Menieres syndrome. Otol Neurotol.
2003;24(3):397403.
48. Hillier SL and McDonnell M. Vestibular rehabilitation
for unilateral peripheral vestibular dysfunction.
Cochrane Database Syst Rev. 2011(2):CD005397.
A systematic review that provided strong evidence that vestib-
ular physical therapy is helpful in Menires disease.
49. Schubert MC et al. Mechanism of dynamic visual acuity
recovery with vestibular rehabilitation. Arch Phys Med
Rehabil. 2008;89D3]:5007.
An important study that helped elucidate the physiologic
changes that occur with vestibular physical therapy.
50. Schubert MC et al. Oculomotor strategies and their
effect on reducing gaze position error. Otol Neurotol.
2010;31(2):22831.
51. Scherer M, Migliaccio AA, Schubert MC. Effect of ves-
tibular rehabilitation on passive dynamic visual acuity.
J Vestib Res. 2008;18(23):14757.
52. Hall CD, Schubert MC, Herdman SJ. Prediction of fall
risk reduction as measured by dynamic gait index in
individuals with unilateral vestibular hypofunction.
Otol Neurotol. 2004;25(5):74651.
53. McDonnell MN, Hillier SL. Vestibular rehabilitation for
unilateral peripheral vestibular dysfunction. Cochrane
Database Syst Rev. 2015;1:CD005397.
54. Garcia AP et al. Vestibular rehabilitation with virtual
reality in Menieres disease. Braz J Otorhinolaryngol.
2013;79D3]:36674.
This study is important because it highlighted the use of virtual
reality to deliver vestibular rehabilitation therapy to Menires
patient, and showed it to be highly effective.
55. Herdman SJ et al. Recovery of dynamic visual acuity in
bilateral vestibular hypofunction. Arch Otolaryngol
Head Neck Surg. 2007;133(4):3839.
56. Herdman SJ et al. Recovery of dynamic visual acuity in
unilateral vestibular hypofunction. Arch Otolaryngol
Head Neck Surg. 2003;129(8):81924.
57. Syed MI et al. Positive pressure therapy for Menieres
syndrome/ disease with a Meniett device: A systematic
review of randomised controlled trials. Clin
Otolaryngol. 2014. A systematic review that examined
the evidence for the Meniett device.
58. Ahsan SF, Standring R and Wang Y. Systematic
review and meta-analysis of Meniett therapy for
Menieres disease. Laryngoscope. 2014. Another
systematic review that looked at evidence for the
Meniett device
 14 Page 16 of 16
59. Lambert PR et al. A randomized, double-blind, place-
bo-controlled clinical study to assess safety and clinical
activity of OTO-104 given as a single intratympanic
injection in patients with unilateral Meniere's disease.
Otol Neurotol. 2012;33D7]:125765.
This study is important because it examined a novel delivery
vehicle for intratympanic dexamethesone, which has been
proven to be an effective, non-ablative treatment.
60. ClinicalTrials.gov. OTO-104 for the Treatment of
Menieres Disease. Available from: https://clinicaltrials.
gov/ct2/show/study/NCT01412177?term=meniere%
27s&rank=4
61. Stahle J, Stahle C, Arenberg IK. Incidence of Menieres
disease. Arch Otolaryngol. 1978;104(2):99102.
62. Meyerhoff WL, Paparella MM, Shea D. Menieres
disease in children. Laryngoscope. 1978;88(9 Pt 1):
150411.
63. Filipo R, Barbara M. Juvenile Menieres disease. J
Laryngol Otol. 1985;99(2):1936.
View publication stats
Curr Treat Options Neurol (2015) 17:14
64. Hausler R et al. Menieres disease in children. Am J
Otolaryngol. 1987;8(4):18793.
65. Akagi H et al. Menieres disease in childhood. Int J
Pediatr Otorhinolaryngol. 2001;61(3):25964.
66. Medicine, O.C.f.E.B. Levels of Evidence. 2009 [cited
2015 Jan 16]; Available from: http://www.cebm.net/
oxford-centre-evidence-based-medicine-levels-
evidence-march-2009/
67. Diaz RC et al. Quality-of-life assessment of Menieres
disease patients after surgical labyrinthectomy. Otol
Neurotol. 2007;28(1):7486.
68. Syed I, Aldren C. Menieres disease: an evidence based
approach to assessment and management. Int J Clin
Pract. 2012;66(2):16670.
69. Rauch SD. Clinical hints and precipitating factors in
patients suffering from Menieres disease. Otolaryngol
Clin North Am. 2010;43(5):10117.