Menière’s disease

Description

Menière’s disease is a condition characterized by the triad of episodic vertigo, tinnitus, and hearing loss, caused by endolymphatic hydrops of the labyrinthine system of the inner ear.

Epidemiology

• Age of onset 20-50years
• Higher incidence of migraine in cases of Menière’s disease compared to the general population

Pathogenesis

• The exact disease mechanism of Menière’s disease is unknown
• Endolymphatic hydrops of the labyrinthine system of the inner ear is characteristic. This is usually a histopathological finding at post-mortem and therefore  not very useful clinically, although MRI with gadolinium can now be used to visualise endolymphatic hydrops.

Clinical features

Menière’s disease is characterized by a triad of three symptoms:

• Vertigo
  • usually spontaneous in onset
  • episodic – lasting 20minutes to 24hours
  • sometimes occurring in clusters, sometimes more sporadically

• Tinnitus
  • variable for individuals
  • may be associated with aural fullness
  • can be constant or fluctuating
  • can occur both concurrently or independently from other symptoms
  •  may become persistent as permanent hearing loss develops.

• Hearing loss
  •  fluctuating, unilateral, sensorineural hearing loss
  • may be associated with aural fullness or pressure
  •  initially hearing loss affects low frequencies and in the short-term repeat audiogram may show recovery of low-frequency hearing; over several years hearing loss will be progressive and affect all frequencies of hearing.

Diagnostic criteria ()

in 2015, Lopez-Escamez defined four criteria which must be met for a diagnosis of definite Menière’s disease. This may take months to years given the episodic and progressive nature of the condition. If criteria A, C and D are met only a diagnosis of “probable” Menière’s disease can be made.

Differential diagnoses include transient ischaemic attack, vestibular migraine, vestibular paroxysmia, recurrent unilateral vestibulopathy and other vestibular disorders

A. Two or more spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours.

B. Audiometrically documented low- to medium-frequency sensorineural hearing loss in one ear, defining the affected ear on at least one occasion before, during or after one of the episodes of vertigo

C. Fluctuating aural symptoms (hearing, tinnitus, or fullness) in the affected ear.

D. Not better accounted for by another vestibular diagnosis.

History

In the 18^th century a large umbrella term of “apoplectiform cerebral congestion” was used to describe a collection of conditions that generally caused people to collapse. This included vertiginous conditions that today we recognise as labyrinthitis and Menières disease, as well as those such as epilepsy and subarachnoid haemorrhage which we now know are vastly different disease processes.

In the early 19^th century Prosper Menière (1799 – 1862) and others began to recognise some cases of “apoplectiform cerebral congestion” followed a more benign course than others, and went on to describe cases of vertigo associated with hearing loss and tinnitus. In 1861 he was the first to conclude that these symptoms were cause by a defect of the semicircular canals.

In 1874 Charcot was the first to use the term “Maladie de Menière” to describe the condition of vertigo, tinnitus and deafness [Vertiges ab aure laesa (maladie de Menière)].

Over the next 50 years or so there became increasing confusion and controversy over what constituted Menière’s disease and what others were calling Menière’s syndrome, Menière’s attacks, Menière’s symptom complex and pseudo-Menière’s. This was recognized by the Committee on Hearing and Equilibrium in 1972 who went on to set out a criterion to define Menière’s disease is which has since been redefined twice (see below).

Associated Persons

• Prosper Menière (1799 – 1862)

Alternative names

• Maladie de Meniere (1874)
• Menière’s disease

References

• Ménière P. Mémoire sur des lésions de l’oreille interne donnant lieu à des symptômes de congestion cérébrale apoplectiforme. Gazette médicale de Paris, 1861; 16: 597-601
• Charcot JM. Conferences cliniques de Salpetriere: Vertiges ab aure laesa (maladie de Menière). Gazette des Hospiteaux Civils et Militaires 1874; 47: 73-74
• Williams HL. A review of Ménière’s original papers in the light of our present knowledge of Ménière’s disease. Ann Otol Rhinol Laryngol. 1949;58(3):761-770.
• Arenberg IK, Countryman LL, Bernstein LH, Shambaugh GE Jr. Vincent’s violent vertigo. An analysis of the original diagnosis of epilepsy vs. the current diagnosis of Meniére’s disease. Acta Otolaryngol Suppl. 1991;485:84-103
• Beasley NJ, Jones NS. Menière’s disease: evolution of a definition. J Laryngol Otol. 1996; 110(12): 1107-1113.
• Lopez-Escamez JA, Carey J, Chung WH, et al. Diagnostic criteria for Menière’s disease. J Vestib Res. 2015;25(1):1-7.