Ménière’s Disease: A Lifelong Merry-Go-Round

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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.

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 Ménière first described this disorder approximately 150 years ago. Yet, researchers are still uncertain of its exact etiology and underlying pathophysiology.1,2

Ménière’s 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 Ménière’s 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 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 management 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 diagnosis or first-line treatment. This article reviews the current understanding of the underlying physiologic mechanisms that cause Ménière’s disease and discusses the criteria for diagnosis and various treatment options.

The cause of Ménière’s 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 Ménière’s 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 Meniere’s disease symptoms.9

Recently recognized factors that contribute to the development of Meniere’s disease include autoimmune reactions, genetic irregularities, vascular abnormalities, 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 system 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 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 Ménière’s disease.1,6

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

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