Meniere syndrome

Douglas J Lanska MD FAAN MS MSPH (Dr. Lanska of the Great Lakes VA Healthcare System and the University of Wisconsin School of Medicine and Public Health has no relevant financial relationships to disclose.)
Originally released December 7, 2004; last updated May 22, 2016; expires May 22, 2019

This article includes discussion of Meniere syndrome and endolymphatic hydrops. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

The author discusses the clinical presentation, pathophysiology, diagnostic workup, and management of Meniere syndrome. Meniere disease can be confused with migraine-associated vertigo, and current diagnostic criteria may not differentiate the two adequately in all patients. Although some analytic epidemiologic studies have suggested an association between migraine and Meniere disease, more recent studies have not found an elevated incidence of migraine headache in patients with Meniere syndrome compared to the general population. A low-sodium diet and diuretics are considered the mainstays of treatment for Meniere disease. Ablative therapy is a second-line treatment approach for failures of medical therapy. Among the various forms of ablative therapy, the most common are intratympanic aminoglycoside administration and vestibular neurectomy.

Key points

 

• The key clinical features of Meniere syndrome are recurrent episodes of spontaneous vertigo, fluctuating hearing loss, aural fullness, and subjective tinnitus, with a progressive loss of audiovestibular function. The vertiginous episodes typically last hours (with a range generally of 30 minutes to 12 hours), with residual nonvertiginous disequilibrium lasting possibly several days.

 

• With otolithic crises (also called “otolithic catastrophe”), patients may report the sensation of being suddenly thrown to the ground without warning.

 

• Audiometry is essential for diagnosis, staging, and monitoring.

 

• Caloric tests and either electronystagmography or video nystagmography are recommended, particularly before and after surgical treatment or aminoglycoside therapy.

 

• In at least two thirds of patients, vertigo is successfully controlled with medical management and the natural history of the disease, and many individuals reach a steady-state phase of the disease.

 

• Most studies report a frequency of bilateral disease of between 10% and 33%.

 

• A family history of Meniere disease is convincing (confirmed or probable) in nearly a quarter of patients, and genetic studies suggest that Meniere disease may be transmitted as an autosomal dominant trait with reduced penetrance and anticipation.

 

• Staging is based on the average of pure-tone thresholds at 0.5 kHz, 1 kHz, 2 kHz, and 3 kHz of the worst audiogram in the 6-month period prior to treatment.

 

• The aims of management of Meniere disease are to (1) reduce the number and severity of acute vertiginous attacks and their associated symptoms (eg, hearing loss and tinnitus), (2) alleviate chronic symptoms (eg, hearing loss and tinnitus), and (3) prevent progression of the disease and its associated symptoms (eg, hearing loss and tinnitus).

 

• A low-sodium diet and diuretics are considered the mainstays of treatment for Meniere disease.

 

• Ablative therapy is a second-line treatment approach for failures of medical therapy. Among the various forms of ablative therapy, the most common are intratympanic aminoglycoside administration and vestibular neurectomy.

Historical note and terminology

In 1861 French otologist Prosper Meniere (1799-1862) described a group of 11 patients, mostly young men in their 30s and 40s, with recurrent episodes of vertigo, deafness, and tinnitus with associated nausea, vomiting, gait ataxia, and falls (Charcot 1881; M'Kenzie 1924; Williams 1949; Atkinson 1960; Gros 1967; Shapiro 1970; Pappas and Galanos 1982; Arenberg 1989; Beasley and Jones 1996).

Image: Prosper Meniere (1799-1862)
The intermittent attacks of vertigo were accompanied by diminution of hearing and increased tinnitus. Only one case had mention of aural pressure and fullness, but Meniere did not include this as part of the clinical description. Over time the hearing loss progressively worsened. He localized the responsible lesion to the peripheral vestibular apparatus, and specifically the semicircular canals.

Meniere's localization of responsible pathology to the semicircular canals was apparently based in part on a controversial autopsy (M'Kenzie 1924; Atkinson 1945; Williams 1949; Shapiro 1970; Pappas 1982). Meniere seemed to suggest hemorrhage into the semicircular canals as the responsible pathology, but subsequent historians have carefully reviewed his 1861 report (and an earlier published comment) and have concluded that Meniere was using a separate case of hemorrhage into the semicircular canals to support his contention that the disease was localized to the peripheral cochleo-vestibular system rather than the central nervous system (Pappas and Galanos 1982).

In 1874 French neurologist Jean Martin Charcot (1825-1893) labeled the disease “Maladie de Meniere” and noted that episodic symptoms ceased when the deafness became complete (Charcot 1881).

Image: Jean Martin Charcot (1825-1893)

Surgical treatments for Meniere disease began in the 1900s. In 1904 Richard Lake at the Royal Ear Hospital in London performed a labyrinthectomy and radical mastoidectomy on a 21-year-old woman with a 5-year history of episodic vertigo, nausea, vomiting, deafness, and tinnitus. In 1928 American neurosurgeon Walter Dandy (1886-1946) described 9 cases of intracranial auditory nerve section for Meniere disease: all of the cases survived and all had symptomatic relief (Dandy 1928).

In 1938 endolymphatic hydrops was found to be the pathological correlate of Meniere syndrome (Hallpike and Cairns 1938; Paparella 1992), a finding subsequently confirmed by other investigators (Hallpike and Wright 1939; Hallpike and Wright 1940; Paparella 1992). The initial discovery was made independently by English otologist Charles Skinner Hallpike (1900-1979) and Australian-born neurosurgeon Hugh Cairns (1896-1952) in England, and Japanese otolaryngologist Kyoshiro Yamakawa (c1891-1980) in Japan (Paparella 1992).

Meniere disease is generally defined as the idiopathic syndrome of endolymphatic hydrops, whereas the term Meniere syndrome is generally used for patients with the same clinical features but who have an identified cause. Other authors, however, use the term Meniere disease for both idiopathic and secondary cases (Paparella 1985; Paparella 1991).

So-called delayed endolymphatic hydrops develops in an ear that was previously damaged, usually from a bacterial or viral infection. A number of variants of Meniere disease have also been proposed including vestibular and cochlear Meniere disease (characterized, respectively, by isolated episodes of vertigo or hearing loss, without the complete Meniere syndrome); however, there are no clinical-pathologic studies correlating isolated vestibular and auditory disorders with selective endolymphatic hydrops of the vestibular and auditory labyrinth.

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