Meniere's disease

Abstract

Meniere's disease (MD) is a disorder of the inner ear that causes vertigo attacks, fluctuating hearing loss, tinnitus and aural fullness. The aetiology of MD is multifactorial. A characteristic sign of MD is endolymphatic hydrops (EH), a disorder in which excessive endolymph accumulates in the inner ear and causes damage to the ganglion cells. In most patients, the clinical symptoms of MD present after considerable accumulation of endolymph has occurred. However, some patients develop symptoms in the early stages of EH. The reason for the variability in the symptomatology is unknown and the relationship between EH and the clinical symptoms of MD requires further study. The diagnosis of MD is based on clinical symptoms but can be complemented with functional inner ear tests, including audiometry, vestibular-evoked myogenic potential testing, caloric testing, electrocochleography or head impulse tests. MRI has been optimized to directly visualize EH in the cochlea, vestibule and semicircular canals, and its use is shifting from the research setting to the clinic. The management of MD is mainly aimed at the relief of acute attacks of vertigo and the prevention of recurrent attacks. Therapeutic options are based on empirical evidence and include the management of risk factors and a conservative approach as the first line of treatment. When medical treatment is unable to suppress vertigo attacks, intratympanic gentamicin therapy or endolymphatic sac decompression surgery is usually considered. This Primer covers the pathophysiology, symptomatology, diagnosis, management, quality of life and prevention of MD.

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Figure 1: Clinical symptoms associated with Meniere's disease.
Figure 2: Diagram of the inner ear.
Figure 3: Cumulative age distribution of onset of symptoms in patients with Meniere's disease.
Figure 4: Endolymphatic hydrops.
Figure 5: Mouse model of endolymphatic hydrops.
Figure 6: Electrocochleography in a patient with left Meniere's disease.
Figure 7: Vestibular-evoked myogenic potential in a patient with right Meniere's disease.
Figure 8: Visualization of endolymphatic hydrops using MRI and intratympanic gadolinium-based contrast agent.
Figure 9: Visualization of endolymphatic hydrops using MRI and intravenous gadolinium-based contrast agent.
Figure 10: New proposed classification of Meniere's disease with the use of endolymphatic hydrops imaging.

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Introduction (T.N.); Epidemiology (M.L.C.); Mechanisms/pathophysiology (C.A.M., N.F.M. and I.P.); Diagnosis, screening and prevention (Y.-H.Y. and S.N.); Management (C.A.F.); Quality of life (M.A.A.); Outlook (T.N. and I.P.); Overview of Primer (T.N. and I.P.).

Correspondence to Tsutomu Nakashima.

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Nakashima, T., Pyykkö, I., Arroll, M. et al. Meniere's disease. Nat Rev Dis Primers 2, 16028 (2016). https://doi.org/10.1038/nrdp.2016.28

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