38 Clinician Reviews • OCTOBER 2013

CE/CME

CE/CME INFORMATIONEarn credit by reading this article and successfully completing the posttest at www.clinicianreviews.com/cecme/cecme.html. Successful completion is defined as a cumulative score of at least 70% correct. This program has been reviewed and is approved for a maximum of 1.0 hour of American Academy of Physician Assistants (AAPA) Category I CME credit by the Physician Assistant Review Panel. [NPs: Both ANCC and the AANP Certification Program recognize AAPA as an approved provider of Category 1 credit.] Approval is valid for one year from the issue date of October 2013.

LEARNING OBJECTIVES • Describe the pathophysiology of

Ménière’s disease, as it is currently understood.

• Discuss the triad of symptoms that should prompt suspicion for Ménière’s disease in a primary care patient.

• List the diagnostic criteria for “definite” Ménière’s disease, as defined by the American Academy of Otolaryngology–Head and Neck Surgery.

• Review pharmacologic management, intratympanic injection and other nonoperative therapies, and surgical treatment for Ménière’s disease. Tamera Pearson is an Associate Professor in the School of Nursing at West-

ern Carolina University.

Ménière’s disease can significantly affect a person’s quality of life and is a challenge to diagnose and treat effectively. The French physician Prosper Mé-

nière first described this disorder approximately 150 years ago. Yet, researchers are still uncertain of its exact etiology and un-derlying pathophysiology.1,2

Ménière’s disease is defined as a chronic condition with recurrent episodes of vertigo that are associated with sen-sorineural hearing loss, tinnitus, and/or a sensation of aural fullness.3,4 Thanks to researchers’ evolving knowledge of Mé-nière’s disease, a new definition has been proposed: a degen-erating inner ear leading to impaired homoeostasis, hearing loss, and vertigo.5

In the United States, the prevalence of Ménière’s 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 Ménière’s 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 Ménière’s disease is based on recognition of the clinical symptoms that characterize the disorder, and man-agement is centered on heuristic treatment options. Thus, a person may experience mild to severe symptoms of Ménière’s disease for months to years before receiving either the diagno-sis or first-line treatment. This article reviews the current un-derstanding of the underlying physiologic mechanisms that cause Ménière’s disease and discusses the criteria for diagno-sis and various treatment options.

Ménière’s DiseaseA Lifelong Merry-Go-Round

Tamera Pearson, PhD, ACNP, FNP

Ménière’s 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 person’s quality of life can be significant. Here are the symptoms, criteria for

diagnosis, and appropriate treatment or referrals for Ménière’s disease.

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ETIOLOGY AND PATHOPHYSIOLOGYThe cause of Ménière’s disease and the subsequent mechanical, physiologic, and biochemical changes that occur are poorly understood, but several theo-ries have been proposed. According to the current central theory, a buildup of fluid (endolymph) with-in the cochlear and saccular ducts in the inner ear causes distention of these structures into the endo-lymphatic space, resulting in the development of en-dolymphatic hydrops.4 Pressure from endolymphat-ic 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 Ménière’s dis-ease completely to hydrops and focused on identify-ing 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 Meniere’s disease symptoms.9

Recently recognized factors that contribute to the development of Meniere’s disease include autoim-mune reactions, genetic irregularities, vascular ab-normalities, and viral influences. Approximately one-third of Ménière’s disease cases can be attributed to an autoimmune origin.1,6 Researchers hypothesize that several immunologic processes may contribute to Ménière’s 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 person’s immune sys-

tem and increase the probability of Ménière’s disease.1

The probability of a genetic influence is supported by the fact that one in 20 people with Ménière’s disease reports a positive family history of the disorder.4

Many patients with Ménière’s disease experience migraine headaches, and thus vascular abnormali-ties are another area of consideration among the eti-ologies of this disease.10 Researchers are also studying a potential viral cause in the development of Ménière’s disease.1,6

Regardless of the specific cause or physiologic changes that occur, the one common finding in pa-tients with Ménière’s disease is a dysfunction of fluid homeostasis within the inner ear.

DIAGNOSIS Establishing the diagnosis of Ménière’s disease can be difficult and time-consuming because the symptoms of the disorder are nonspecific and variable. Ménière’s 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

According to current theory, Ménière’s disease, characterized by chronic vertigo, tinnitus, and hearing loss, may be caused by damage to the structures of the inner ear.

Pinna

Earcanal

Malleus Incus

Stapes

INNER EAR

Cochlea

Eustachiantube

Middle ear

Tympanic membrane

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TABLE 1Ménière’s Disease: Diagnostic Criteria from AAO-HNS

Symptom Criteria

Certain Ménière’s Disease

Probable Ménière’s Disease

Possible Ménière’s Disease

Spontaneous vertigo > 2 vertigo episodes > 20 minutes’ duration

1 vertigo episode > 20 minutes’ duration

Episodic vertigo without 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 Otolaryngology–Head and Neck SurgerySource: 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 Ménière’s episode may include nausea, vomiting, gait imbal-ance, and tinnitus. Most vertigo attacks from Ménière’s 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 Ménière’s disease can be made. Also, it is important to note that specialists may differ-entiate Ménière’s disease, an idiopathic condition, from Ménière’s 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 Ménière’s disease, defined in 1995 by the American Academy of Otolar-yngology–Head and Neck Surgery (AAO-HNS), remain the gold standard for diagnosis.3 A “definite” diagnosis of Ménière’s 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 cat-

egories of “probable” and “possible” Ménière’s dis-ease based on the frequency of vertigo episodes or the presence of a combination of associated symptoms (see Table 1).3,7

Patients with Ménière’s disease may experience different patterns of symptoms. “Auditory dominant” Ménière’s disease produces more hearing loss changes than vertigo, while “vestibular dominant” causes fre-quent episodes of severe vertigo and less severe hear-ing changes. A “mixed” pattern of Ménière’s disease manifests with both hearing fluctuations and vertigo.5

Unilateral symptoms are most common; however, bilateral disorder occurs in approximately 25% of pa-tients, either at onset or with changing symptomatol-ogy over time.5,8,9

HISTORY AND EXAMINATIONObtaining a detailed history from the patient and com-pleting thorough neurologic and otologic examinations are essential components of the diagnostic process.* Audiometry should be completed to evaluate neuro-sensory hearing loss, as audiometrically documented hearing loss is part of the AAO-HSN diagnostic criteria for Ménière’s disease.6

Based on findings from the patient’s history, physi-cal exam, and audiometric testing, a tentative diagno-sis 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 Ménière’s disease, electrical vestibular stimulation and videonystagmography are useful tests to assess abnormalities in vestibular function and monitor dis-ease progression, which may help determine inter-vention options.6 Additional diagnostic tests may be suggested due to the essential need to exclude other potential causes of vertigo prior to determining the fi-nal diagnosis of Ménière’s disease.

* For more information, read “Ototoxicity and Vestibulotoxicity,” Clinician Reviews. 2013;23(4):32-40.

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Triggers of VertigoSelected triggers of vertigo that must be considered are benign paroxysmal positional vertigo (BPPV), labyrin-thitis, acoustic neuroma, migraine with vertigo, and ce-rebral vascular events.6 Diagnostic tests are indicated to rule out certain problems, such as MRI to exclude a tu-mor or an acoustic neuroma. Distinct differences noted during a complete assessment may help eliminate cer-tain disorders. BPPV is triggered by a change in physi-cal position and usually lasts less than one minute; the diagnosis can be confirmed by the Dix-Hallpike ma-neuver.4 Labyrinthitis is characterized by acute vertigo associated with continuing imbalance, while instabil-ity with walking resolves completely between vertigo episodes in Ménière’s disease.4 If abnormal neurologic manifestations are noted during the exam or reported in a patient’s account of a vertigo episode, then a tran-sient 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 Ménière’s disease, and the evidence sup-porting the efficacy of currently used therapies is in-consistent. However, several medicines and treatments are useful in relieving symptoms and improving a pa-tient’s quality of life.

Primary care clinicians can initiate treatment for Ménière’s 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 Ménière’s disease.5,9

Lifestyle ChangesDietary changes. Patients diagnosed with Ménière’s 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 de-creasing fluid retention and reducing the endolym-phatic hydrops.2,7,11 Decreasing alcohol and caffeine consumption is also routinely recommended as part of the treatment of Ménière’s disease.2,5

Researchers have recently suggested a different ap-proach to dietary changes for Ménière’s disease that reflects the underlying loss of ability to regulate fluid in the inner ear. This alternate method of dietary regula-tion 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 deli-cate fluid balance in the inner ear.9 When caring for patients with Ménière’s 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 oc-currence of Ménière’s 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 Ménière’s disease on a pa-tient’s life. Stress reduction techniques that can be rec-ommended include progressive relaxation, meditation with deep breathing, yoga, and exercise.

Although studies of the effect of stress reduction methods on Ménière’s disease are not available in the current literature, the association of stress with Mé-nière’s disease is well documented.5 By avoiding stress, it is hoped, patients may experience a reduction in the frequency and severity of Ménière’s disease– associated episodes of vertigo. Researchers also suggest that stress reduction and patient education may help alleviate patients’ feelings of frustration resulting from misinfor-mation about their condition.2,11

Oral Medications for Acute ReliefAcute attacks of vertigo associated with Ménière’s dis-ease can be treated with benzodiazepines, antiemet-ics, 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 Ménière’s disease but has not undergone audiometry, the plausible diag-nosis may lead to tentative treatment for acute episodes if other causes of vertigo have been ruled out.

Antihistamines, such as meclizine or dimenhydri-nate, may help reduce vertigo symptoms and associ-ated 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 cer-tain activities while taking this medication. Antihista-mines should not be given to patients with glaucoma or prostate disease due to the potentially strong anticho-linergic effects of these drugs.4,6

Scopolamine is a belladonna alkaloid that can be ap-plied 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-

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ety associated with an acute episode of vertigo.6,11 Ben-zodiazepines 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 Meniere’s dis-ease.11

Other antiemetic medications, such as prometha-zine 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 MedicationMedication for long-term management of Ménière’s disease can promote improvement in symptoms and reduce the frequency of vertigo episodes. A mild di-uretic, such as hydrochlorothiazide with or without triamterene, taken on a regular basis reduces extracel-lular fluids and may decrease pressure from endolym-phatic hydrops.2,7 While strong evidence regarding the efficacy of diuretics is lacking, the majority of patients with Ménière’s disease who are treated with diuretics do experience improvement in vertigo.2,5

Betahistine hydrochloride, a vasodilator and his-tamine receptor antagonist, is another medication to consider for management of Ménière’s disease.1 This agent is not approved by the FDA; however, the FDA classifies betahistine as an inert chemical, so it is avail-able in compounding pharmacies in the United States. The efficacy of betahistine has not been clearly or con-sistently established in research studies, but it has been and continues to be widely used to treat Ménière’s dis-ease 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 Ménière’s disease.2,8,11

Intratympanic MedicationPatients who do not respond well to the previously de-scribed management should be referred to a specialist for additional treatment options. An otolaryngology specialist may administer intratympanic medications to patients with Ménière’s disease who have not re-sponded to primary medical therapy.

Patients in the US who have not responded posi-tively to lifestyle or diuretic medication are commonly offered treatment with intratympanic dexamethasone. The primary goal of this therapy is to improve vertigo

without affecting a patient’s hearing; an added effect may be a potential positive impact on the immune system.11 Studies show that intratympanic steroid in-jection results in control of vertigo in patients with Mé-nière’s 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 Ménière’s disease is a type of chemical ablation of the labyrinth induced by injecting gentami-cin 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 Mé-nière’s disease, but this treatment is only recommend-ed 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 pa-tients to follow up with the specialist if additional treat-ments are indicated.

Portable Pressure Device Use of the Meniett device is a minimally invasive treat-ment for Ménière’s disease based on the principle of using alternating pressure to stimulate the flow of en-dolymph.11 This handheld device delivers low-pressure pulses within the inner ear through a standard ventila-tion 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 pa-tients who use the Meniett device routinely for several weeks.2,12 As noted, the use of this device, which is ob-tained by prescription from an otolaryngologist, re-quires placement of ventilation tubes.

AcupunctureA traditional Chinese medical approach, acupunc-ture is one complementary and alternative medicine therapy that has been studied as a treatment option for Ménière’s disease. Studies on the use of acupunc-ture 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. Acu-puncture has a positive effect in both acute episodes of vertigo in those without Ménière’s disease and in patients who have had Ménière’s disease for many years.13

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Vestibular Rehabilitation An additional adjunctive treatment option to con-sider 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 Ménière’s disease who have undergone medi-cal or surgical intervention used to treat vertigo. Pa-tients who have significant balance problems occur-ring between acute vertigo episodes may also benefit from vestibular rehabilitation.6

Surgical Treatment Surgical intervention should be the last resort to treat Ménière’s disease due to the higher risk involved with any surgical procedure and the potential adverse effect on hearing. Endolymphatic sac decompression sur-gery 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 possi-bly 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 with-out the recommendation of an otolaryngology special-ist. The decision should be made based on the sever-ity of the disease and its effect on the patient, weighed against the risks involved in such an invasive treatment option.

CONCLUSIONMénière’s disease is a complex disorder that can sig-nificantly alter a person’s quality of life. While nei-ther the exact cause nor pathophysiology underlying Ménière’s 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 diag-nosis based on a detailed history and comprehensive assessment of recurrent vertigo with tinnitus, hearing loss, and possibly a sensation of aural fullness. Estab-lishing the diagnosis of Ménière’s disease requires rul-ing 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 recommenda-tions for patients with Ménière’s disease. Oral medi-cations from a range of drug categories may be used to improve acute and chronic symptoms, including antiemetics, anticholinergics, antihistamines, benzo-diazepines, and mild diuretics. Additionally, a com-pounded substance with vasodilator and histamine- receptor–antagonist properties (betahistine) can be used for treatment of Meniere’s.

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 Mé-nière’s disease by recommending lifestyle changes, prescribing oral medications, providing patient edu-cation, and recognizing indications for referral. CR

REFERENCES 1. Greco A, Gallo A, Fusconi M, et al. Ménière’s disease might be an autoim-

mune condition? Autoimmun Rev. 2012;11:731-738. 2. Greenberg SL, Nedzelski JM. Medical and noninvasive therapy for Ménière’s

disease. Otolaryngol Clin North Am. 2010;43:1081-1090. 3. American Academy of Otolaryngology–Head and Neck Foundation, Inc.

Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Ménière’s disease. Otolaryngol Head Neck Surg. 1995;113:181-185.

4. Syed I, Aldren C. Ménière’s disease: an evidence based approach to assess-ment and management. Int J Clin Pract. 2012;66:166-170.

5. Rauch SD. Clinical hints and precipitating factors in patients suffering from Ménière’s disease. Otolaryngol Clin North Am. 2010;43:1011-1017.

6. Dinces EA, Rauch SS. Ménière’s disease. In: UpToDate. Deschler DG, Lin FH, eds. 2012:November 27,2012.

7. Semaan MT, Megerian CA. Ménière’s disease: a challenging and relentless disorder. Otolaryngol Clin North Am. 2011;44:383-403.

8. Martin González C, González FM, Trinidad A, et al. Medical management of Ménière’s disease: a 10-year case series and review of literature. Eur Arch Otorhinolaryngol. 2010;267:1371-1376.

9. Berlinger NT. Ménière’s disease: new concepts, new treatments. Minn Med. 2011;94:33-36.

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 Ménière’s disease. Laryn-goscope. 2008;118:1099-1108.

12. Gates GA, Green JD Jr, Tucci DL, Telian SA. The effects of transtympanic micropressure treatment in people with unilateral Ménière’s disease. Arch Otolaryngol Head Neck Surg. 2004;130:718-725.

13. Long A, Xing M, Morgan K, Brettle A. Exploring the evidence base for acu-puncture in the treatment of Ménière’s syndrome-a systematic review. Evid Based Complement Alternat Med. 2011;2011:1-13.

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 Ménière’s disease. Acta Otolaryngol. 2012;132:491-497.

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