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CE/CME

Mnires Disease

A Lifelong Merry-Go-Round
Tamera Pearson, PhD, ACNP, FNP

Mnires disease is a complex disorder of intermittent vertigo, tinnitus, and hearing loss whose
symptoms usually manifest between ages 20 and 60. Although this disorder is uncommon,
its impact on a persons quality of life can be significant. Here are the symptoms, criteria for
diagnosis, and appropriate treatment or referrals for Mnires disease.

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LEARNING OBJECTIVES
D
 escribe the pathophysiology of
Mnires disease, as it is currently
understood.
D
 iscuss the triad of symptoms
that should prompt suspicion for
Mnires disease in a primary care
patient.
L ist the diagnostic criteria for
definite Mnires disease, as
defined by the American Academy
of OtolaryngologyHead and Neck
Surgery.
R
 eview pharmacologic management,
intratympanic injection and other
nonoperative therapies, and surgical
treatment for Mnires disease.

38 Clinician Reviews OCTOBER 2013

nires disease can significantly affect a persons


quality of life and is a challenge to diagnose and
treat effectively. The French physician Prosper Mnire first described this disorder approximately 150 years ago.
Yet, researchers are still uncertain of its exact etiology and underlying pathophysiology.1,2
Mnires disease is defined as a chronic condition with
recurrent episodes of vertigo that are associated with sensorineural hearing loss, tinnitus, and/or a sensation of aural
fullness.3,4 Thanks to researchers evolving knowledge of Mnires disease, a new definition has been proposed: a degenerating inner ear leading to impaired homoeostasis, hearing
loss, and vertigo.5
In the United States, the prevalence of Mnires disease is
estimated at 15 to 150 cases per 100,000 persons. (This wide
variation in prevalence reflects a lack of standard diagnostic
criteria, as well as differences based on geographic area.6,7)
Many affected individuals experience symptoms significant
enough to lead to disability.6,7 Patients with Mnires disease
usually present between ages 20 and 60, with a peak incidence
occurring between ages 40 and 50.1,4,6,8 This disease affects
both genders, but is slightly more common in women.1,4,6,8
Diagnosis of Mnires disease is based on recognition of
the clinical symptoms that characterize the disorder, and management is centered on heuristic treatment options. Thus, a
person may experience mild to severe symptoms of Mnires
disease for months to years before receiving either the diagnosis or first-line treatment. This article reviews the current understanding of the underlying physiologic mechanisms that
cause Mnires disease and discusses the criteria for diagnosis and various treatment options.
Tamera Pearson is an Associate Professor in the School of Nursing at Western Carolina University.

Pinna

Malleus

Incus
Stapes
INNER
EAR

Ear
canal

Cochlea
Tympanic
membrane
Middle ear

Eustachian
tube

According to current theory, Mnires


disease, characterized by chronic vertigo,
tinnitus, and hearing loss, may be caused by
damage to the structures of the inner ear.

ETIOLOGY AND PATHOPHYSIOLOGY


The cause of Mnires disease and the subsequent
mechanical, physiologic, and biochemical changes
that occur are poorly understood, but several theories have been proposed. According to the current
central theory, a buildup of fluid (endolymph) within the cochlear and saccular ducts in the inner ear
causes distention of these structures into the endolymphatic space, resulting in the development of endolymphatic hydrops.4 Pressure from endolymphatic hydrops may cause damage to hair follicles and
to the vestibular epithelium, resulting in symptoms
of vertigo, tinnitus, and hearing loss.1,4 Researchers
previously attributed the symptoms of Mnires disease completely to hydrops and focused on identifying anatomic abnormalities.7,9 However, studies now
suggest that a range of pathophysiologic processes
resulting from intrinsic and/or extrinsic factors may
be responsible.7,9 While hydrops may develop, it is
not always the definitive or only cause of Menieres
disease symptoms.9
Recently recognized factors that contribute to the
development of Menieres disease include autoimmune reactions, genetic irregularities, vascular abnormalities, and viral influences. Approximately onethird of Mnires disease cases can be attributed to
an autoimmune origin.1,6 Researchers hypothesize
that several immunologic processes may contribute
to Mnires disease:

Antibodies may cause inner ear damage,


Injury to the inner ear may result in the release of
cytokines which provoke immune reactions, and
Certain genes may affect a persons immune system and increase the probability of Mnires
disease.1
The probability of a genetic influence is supported
by the fact that one in 20 people with Mnires disease
reports a positive family history of the disorder.4
Many patients with Mnires disease experience
migraine headaches, and thus vascular abnormalities are another area of consideration among the etiologies of this disease.10 Researchers are also studying
a potential viral cause in the development of Mnires
disease.1,6
Regardless of the specific cause or physiologic
changes that occur, the one common finding in patients with Mnires disease is a dysfunction of fluid
homeostasis within the inner ear.

DIAGNOSIS
Establishing the diagnosis of Mnires disease can be
difficult and time-consuming because the symptoms
of the disorder are nonspecific and variable. Mnires
disease is a clinical diagnosis, and thus the clinician
must conduct a thorough physical exam and elicit a
very specific history, including a detailed description of
vertigo incidents and associated symptoms. Often, the
greatest challenge is encouraging patients to articulate

OCTOBER 2013 Clinician Reviews 39

CE/CME

TABLE 1

Mnires Disease: Diagnostic Criteria from AAO-HNS

Symptom
Criteria

Certain
Mnires Disease

Probable
Mnires Disease

Possible
Mnires Disease

Spontaneous vertigo

> 2 vertigo episodes


1 vertigo episode
Episodic vertigo without
> 20 minutes duration > 20 minutes duration documented hearing loss

Hearing loss

Audiometrically
documented on
at least 1 occasion

Audiometrically
documented on
at least 1 occasion

Fluctuating or fixed sensorineural


hearing loss with disequilibrium,
but without definitive episodes

Tinnitus or aural fullness

Present

Present

Not present

Other causes

Excluded

Excluded

Excluded

Abbreviation: AAO-HNS, American Academy of OtolaryngologyHead and Neck Surgery


Source: Otolaryngol Head Neck Surg. 1995.3

the details of their episodes. Patients may not keep a


record of the variations of episodes, nor do they always
know what information is needed. Thus, the provider
needs to elicit specific information by asking questions
regarding frequency and duration of episodes, as well
as fluctuation of hearing loss, nausea, and tinnitus.
Symptoms associated with vertigo during a Mnires
episode may include nausea, vomiting, gait imbalance, and tinnitus. Most vertigo attacks from Mnires
disease occur in clusters, but they may also occur
sporadically.6
An additional challenge for clinicians is that other
potential diagnoses related to vertigo must be excluded
before the diagnosis of Mnires disease can be made.
Also, it is important to note that specialists may differentiate Mnires disease, an idiopathic condition, from
Mnires syndrome, which results from known causes
of damage to the inner ear. In the literature, however,
this distinction in terminology is not always clear.7
Specific diagnostic criteria for Mnires disease,
defined in 1995 by the American Academy of OtolaryngologyHead and Neck Surgery (AAO-HNS), remain
the gold standard for diagnosis.3 A definite diagnosis
of Mnires disease is based on:
A history of two or more episodes of spontaneous
vertigo lasting 20 minutes or longer,
Hearing loss documented by audiometry at least
once,
Presence of tinnitus, and/or
A sensation of aural fullness.2,6,7
The AAO-HNS diagnostic criteria also define categories of probable and possible Mnires disease based on the frequency of vertigo episodes or the
presence of a combination of associated symptoms
(see Table 1).3,7

40 Clinician Reviews OCTOBER 2013

Patients with Mnires disease may experience


different patterns of symptoms. Auditory dominant
Mnires disease produces more hearing loss changes
than vertigo, while vestibular dominant causes frequent episodes of severe vertigo and less severe hearing changes. A mixed pattern of Mnires disease
manifests with both hearing fluctuations and vertigo.5
Unilateral symptoms are most common; however,
bilateral disorder occurs in approximately 25% of patients, either at onset or with changing symptomatology over time.5,8,9

HISTORY AND EXAMINATION


Obtaining a detailed history from the patient and completing thorough neurologic and otologic examinations
are essential components of the diagnostic process.*
Audiometry should be completed to evaluate neurosensory hearing loss, as audiometrically documented
hearing loss is part of the AAO-HSN diagnostic criteria
for Mnires disease.6
Based on findings from the patients history, physical exam, and audiometric testing, a tentative diagnosis can be made. The role and inclusion of adjunctive
tests in the diagnostic process varies considerably by
region in the US. While not required for the diagnosis
of Mnires disease, electrical vestibular stimulation
and videonystagmography are useful tests to assess
abnormalities in vestibular function and monitor disease progression, which may help determine intervention options.6 Additional diagnostic tests may be
suggested due to the essential need to exclude other
potential causes of vertigo prior to determining the final diagnosis of Mnires disease.
* For more information, read Ototoxicity and Vestibulotoxicity,
Clinician Reviews. 2013;23(4):32-40.

Triggers of Vertigo
Selected triggers of vertigo that must be considered are
benign paroxysmal positional vertigo (BPPV), labyrinthitis, acoustic neuroma, migraine with vertigo, and cerebral vascular events.6 Diagnostic tests are indicated to
rule out certain problems, such as MRI to exclude a tumor or an acoustic neuroma. Distinct differences noted
during a complete assessment may help eliminate certain disorders. BPPV is triggered by a change in physical position and usually lasts less than one minute; the
diagnosis can be confirmed by the Dix-Hallpike maneuver.4 Labyrinthitis is characterized by acute vertigo
associated with continuing imbalance, while instability with walking resolves completely between vertigo
episodes in Mnires disease.4 If abnormal neurologic
manifestations are noted during the exam or reported
in a patients account of a vertigo episode, then a transient ischemic attack or stroke must be ruled out by
more detailed diagnostic testing.

TREATMENT OPTIONS
Presently, no evidence-based guidelines exist for the
treatment of Mnires disease, and the evidence supporting the efficacy of currently used therapies is inconsistent. However, several medicines and treatments
are useful in relieving symptoms and improving a patients quality of life.
Primary care clinicians can initiate treatment for
Mnires disease through lifestyle recommendations
and prescription of specific medications. Everyday
adjustments that incorporate dietary changes, stress
reduction, adequate sleep, and regular exercise have
been shown to improve vertigo symptoms in 60% of
patients with Mnires disease.5,9

Lifestyle Changes
Dietary changes. Patients diagnosed with Mnires
disease may benefit from following a low-sodium diet,
limiting their daily sodium intake to between 1,000 and
2,000 mg.2,7,11 A low-sodium diet is believed to have a
positive impact on inner ear fluid homeostasis by decreasing fluid retention and reducing the endolymphatic hydrops.2,7,11 Decreasing alcohol and caffeine
consumption is also routinely recommended as part of
the treatment of Mnires disease.2,5
Researchers have recently suggested a different approach to dietary changes for Mnires disease that
reflects the underlying loss of ability to regulate fluid in
the inner ear. This alternate method of dietary regulation aims to maintain fluid homeostasis by avoiding
variations in the daily intake of sodium, caffeine, or al-

cohol, rather than limiting daily consumption.5


The goal of any proposed dietary changes is to limit
fluid and electrolyte shifts that could disrupt the delicate fluid balance in the inner ear.9 When caring for
patients with Mnires disease, clinicians need to keep
in mind that dietary changes may be difficult and will
probably require ongoing encouragement.
Stress reduction. Stress is associated with the occurrence of Mnires disease and often is the trigger
for an acute episode of symptom exacerbation.5 Thus,
clinicians should encourage stress management as a
way to reduce the impact of Mnires disease on a patients life. Stress reduction techniques that can be recommended include progressive relaxation, meditation
with deep breathing, yoga, and exercise.
Although studies of the effect of stress reduction
methods on Mnires disease are not available in the
current literature, the association of stress with Mnires disease is well documented.5 By avoiding stress,
it is hoped, patients may experience a reduction in the
frequency and severity of Mnires diseaseassociated
episodes of vertigo. Researchers also suggest that stress
reduction and patient education may help alleviate
patients feelings of frustration resulting from misinformation about their condition.2,11

Oral Medications for Acute Relief


Acute attacks of vertigo associated with Mnires disease can be treated with benzodiazepines, antiemetics, or anticholinergic medications.4,6 Alleviation of
symptoms is achieved through different physiologic
pathways, based on the drug category prescribed. If a
patient reports typical symptoms of Mnires disease
but has not undergone audiometry, the plausible diagnosis may lead to tentative treatment for acute episodes
if other causes of vertigo have been ruled out.
Antihistamines, such as meclizine or dimenhydrinate, may help reduce vertigo symptoms and associated nausea by blocking the effects of histamine.4,6 One
of the most common side effects of antihistamines is
drowsiness, so patients must be cautioned to avoid certain activities while taking this medication. Antihistamines should not be given to patients with glaucoma or
prostate disease due to the potentially strong anticholinergic effects of these drugs.4,6
Scopolamine is a belladonna alkaloid that can be applied topically on the tissue just behind the ear to help
reduce nausea and vomiting related to vertigo.11
Another option for treatment of acute vertigo is a
benzodiazepine, such as alprazolam, to suppress active
cerebellar responses; this agent may also reduce anxi-

OCTOBER 2013 Clinician Reviews 41

CE/CME

ety associated with an acute episode of vertigo.6,11 Benzodiazepines should be started at the lowest dose and
increased as needed to the maximum recommended
for individual medications based on symptom relief
and side effects.6 Although caution needs to be used
when prescribing benzodiazepines, studies show that
they can be effective for persons with Menieres disease.11
Other antiemetic medications, such as promethazine or ondansetron, may be needed to treat severe
nausea, but these agents should be used cautiously
with other medications due a potential side effect of
sedation.

Long-Term Oral Medication


Medication for long-term management of Mnires
disease can promote improvement in symptoms and
reduce the frequency of vertigo episodes. A mild diuretic, such as hydrochlorothiazide with or without
triamterene, taken on a regular basis reduces extracellular fluids and may decrease pressure from endolymphatic hydrops.2,7 While strong evidence regarding the
efficacy of diuretics is lacking, the majority of patients
with Mnires disease who are treated with diuretics
do experience improvement in vertigo.2,5
Betahistine hydrochloride, a vasodilator and histamine receptor antagonist, is another medication to
consider for management of Mnires disease.1 This
agent is not approved by the FDA; however, the FDA
classifies betahistine as an inert chemical, so it is available in compounding pharmacies in the United States.
The efficacy of betahistine has not been clearly or consistently established in research studies, but it has been
and continues to be widely used to treat Mnires disease in Europe, with good results. Betahistine affects
the microcirculation in the inner ear and inhibits the
vestibular nuclei, which may reduce the frequency of
vertigo episodes and improve tinnitus associated with
Mnires disease.2,8,11

Intratympanic Medication
Patients who do not respond well to the previously described management should be referred to a specialist
for additional treatment options. An otolaryngology
specialist may administer intratympanic medications
to patients with Mnires disease who have not responded to primary medical therapy.
Patients in the US who have not responded positively to lifestyle or diuretic medication are commonly
offered treatment with intratympanic dexamethasone.
The primary goal of this therapy is to improve vertigo

42 Clinician Reviews OCTOBER 2013

without affecting a patients hearing; an added effect


may be a potential positive impact on the immune
system.11 Studies show that intratympanic steroid injection results in control of vertigo in patients with Mnires disease, but up to four injection treatments may
be required for optimal effectiveness.2,7 Improvement
of vertigo is achieved in more than 80% of patients who
undergo intratympanic steroid injections.9
An option reserved for patients with severe, frequent
vertigo related to Mnires disease is a type of chemical
ablation of the labyrinth induced by injecting gentamicin into the middle ear.2 Gentamicin has a toxic effect
on the vestibular hair cells in the inner ear, resulting
in elimination of vestibular function.2 Intratympanic
gentamicin is reported to reduce symptoms from Mnires disease, but this treatment is only recommended for patients with unilateral disease because it may
induce permanent hearing loss.11
The primary care clinician needs to be aware of
these intratympanic procedures and encourage patients to follow up with the specialist if additional treatments are indicated.

Portable Pressure Device


Use of the Meniett device is a minimally invasive treatment for Mnires disease based on the principle of
using alternating pressure to stimulate the flow of endolymph.11 This handheld device delivers low-pressure
pulses within the inner ear through a standard ventilation tube in order to increase exchange of fluids and
improve homeostasis.8 The Meniett device should be
used for five-minute intervals three times per day.12
Several studies have shown excellent results in patients who use the Meniett device routinely for several
weeks.2,12 As noted, the use of this device, which is obtained by prescription from an otolaryngologist, requires placement of ventilation tubes.

Acupuncture
A traditional Chinese medical approach, acupuncture is one complementary and alternative medicine
therapy that has been studied as a treatment option
for Mnires disease. Studies on the use of acupuncture to treat vertigo demonstrate a beneficial effect for
persons with this disease. While the optimal number
and frequency of treatments has not been determined,
all types of acupuncture studied showed benefit. Acupuncture has a positive effect in both acute episodes
of vertigo in those without Mnires disease and in
patients who have had Mnires disease for many
years.13

Vestibular Rehabilitation
An additional adjunctive treatment option to consider for patients with residual disequilibrium is
vestibular rehabilitation. Vestibular rehabilitation is
designed to desensitize or retrain the balance system
response through a series of exercises and activities
supervised by a physical or occupational therapist.
This rehabilitation may improve balance in patients
with Mnires disease who have undergone medical or surgical intervention used to treat vertigo. Patients who have significant balance problems occurring between acute vertigo episodes may also benefit
from vestibular rehabilitation.6

Surgical Treatment
Surgical intervention should be the last resort to treat
Mnires disease due to the higher risk involved with
any surgical procedure and the potential adverse effect
on hearing. Endolymphatic sac decompression surgery involves removing a portion of the mastoid bone,
resulting in decompression of the sac adjacent to the
sigmoid sinus. This procedure has been used for more
than 40 years to control vertigo and has the advantage
of preserving hearing.7,9 However, the benefit of this
procedure is now somewhat controversial and possibly related to a placebo effect.6 Researchers also report
positive results with the use of tenotomy surgery, which
involves severing tendons to the stapedius and tensor
tympani muscles in the middle ear.14
No surgical procedure should be considered without the recommendation of an otolaryngology specialist. The decision should be made based on the severity of the disease and its effect on the patient, weighed
against the risks involved in such an invasive treatment
option.

CONCLUSION
Mnires disease is a complex disorder that can significantly alter a persons quality of life. While neither the exact cause nor pathophysiology underlying
Mnires disease is well understood, several solid
theories are being investigated and contribute to the
current understanding of treatment options. Primary
care clinicians can help determine this clinical diagnosis based on a detailed history and comprehensive
assessment of recurrent vertigo with tinnitus, hearing
loss, and possibly a sensation of aural fullness. Establishing the diagnosis of Mnires disease requires ruling out other possible causes of vertigo.
Lifestyle changes that improve the consistency
of dietary intake of sodium, caffeine, and alcohol as

well as reduction of stress are ongoing recommendations for patients with Mnires disease. Oral medications from a range of drug categories may be used
to improve acute and chronic symptoms, including
antiemetics, anticholinergics, antihistamines, benzodiazepines, and mild diuretics. Additionally, a compounded substance with vasodilator and histaminereceptorantagonist properties (betahistine) can be
used for treatment of Menieres.
Patients who do not respond well to conservative
therapy should be referred to an otolaryngologist for
possible intratympanic medications, ventilation tube
placement with a prescription for pulse pressure
therapy (ie, Meniett device), or surgical intervention.
Primary care clinicians can initiate treatment for Mnires disease by recommending lifestyle changes,
prescribing oral medications, providing patient education, and recognizing indications for referral. CR

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REFERENCES

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 reco A, Gallo A, Fusconi M, et al. Mnires disease might be an autoimmune condition? Autoimmun Rev. 2012;11:731-738.
2. Greenberg SL, Nedzelski JM. Medical and noninvasive therapy for Mnires
disease. Otolaryngol Clin North Am. 2010;43:1081-1090.
3. American Academy of OtolaryngologyHead and Neck Foundation, Inc.
Committee on Hearing and Equilibrium guidelines for the diagnosis and
evaluation of therapy in Mnires disease. Otolaryngol Head Neck Surg.
1995;113:181-185.
4. Syed I, Aldren C. Mnires disease: an evidence based approach to assessment and management. Int J Clin Pract. 2012;66:166-170.
5. Rauch SD. Clinical hints and precipitating factors in patients suffering from
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disorder. Otolaryngol Clin North Am. 2011;44:383-403.
8. Martin Gonzlez C, Gonzlez FM, Trinidad A, et al. Medical management of
Mnires disease: a 10-year case series and review of literature. Eur Arch
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9. Berlinger NT. Mnires disease: new concepts, new treatments. Minn Med.
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10. von Brevern M, Neuhauser H. Epidemiological evidence for a link between
vertigo and migraine. J Vestib Res. 2011;21:299-304.
11. Coelho DH, Lalwani AK. Medical management of Mnires disease. Laryngoscope. 2008;118:1099-1108.
12. Gates GA, Green JD Jr, Tucci DL, Telian SA. The effects of transtympanic
micropressure treatment in people with unilateral Mnires disease. Arch
Otolaryngol Head Neck Surg. 2004;130:718-725.
13. Long A, Xing M, Morgan K, Brettle A. Exploring the evidence base for acupuncture in the treatment of Mnires syndrome-a systematic review. Evid
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14. Loader B, Beicht D, Hamzavi JS, Franz P. Tenotomy of the middle ear
muscles causes a dramatic reduction in vertigo attacks and improves
audiological function in definite Mnires disease. Acta Otolaryngol.
2012;132:491-497.

OCTOBER 2013 Clinician Reviews 43

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