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.  
Lopez-Escamez 2015

History

In the 18th 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 19th 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.

  • Un appareil auditif, jusque-là parfaitement sain, peut devenir tout a coup le siège de troubles fonctionnels consistent en bruits de nature variables, continus ou intermittents, et ces bruits s’accompagnant bientôt d’une diminution plus ou moins grande l’audition;
  • Ces troubles fonctionnels avant leur siège dans l’appareil auditif interne peuvent donner lieu à des accidents réputes cérébraux, lels que vertiges, étourdissements, marche incertaine, tournoiement et chute, et de plus ils sont accompagnés de nausées, de vomissements et d’un état syncopal;
  • Ces accidents, qui ont la forme intermittente, ne tardent pas à être suivis de ‘surdité de plus grave, et  est souvent l’ouïe est subitement et complètement abolie;
  • Tout porte à croire la lésion matérielle qui est case de ces troubles fonctionnels réside dans le canaux demi-circulaires.

Menière 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

  • The auditory apparatus may be suddenly affected causing tinnitus, and diminution of hearing;
  • The inner ear is the site which can suddenly be affected causing attacks of vertigo, dizziness, uncertain gait, staggering and falling, and may be accompanied by nausea, vomiting and syncope;
  • Attacks are intermittent followed by hearing loss of increasing severity;
  • The lesion is likely to be in the semicircular canals.

Menière 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

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

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

References

eponymictionary CTA

eponymictionary

the names behind the name

Studied at Univerisity of Cambridge - BA MB BChir. British doctor working in emergency medicine in Perth, Australia. Special interests include primary care and emergency medicine.

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