Pathogenesis and Etiology of Ménière Disease: A Scoping Review of a Century of Evidence.

IMPORTANCE: Ménière disease is a rare chronic benign disorder of the inner ear with a natural history of multiple clinical phenotypes of variable severity and a tendency to burnout with time. Although multiple treatment modalities have been shown to improve the disease process-some adversely affecting cochleovestibular function-it remains uncertain whether one, several separate, or a combination of pathophysiologic mechanisms affect the disease process. A scoping review of the evidence underlying proposed pathophysiologic mechanisms of Ménière disease is needed to determine which processes are most likely to be etiopathogenic factors.

OBSERVATIONS: Of the 4602 relevant articles found through Embase, Ovid, and PubMed, 444 met inclusion criteria. The most common reported causes of Ménière disease were autoimmune or immune-mediated, genetic, or structural dysfunction of the inner ear. During the study period from inception to March 2021, etiologic theories shifted from structural dysfunction to autoimmune and genetic causes of Ménière disease.

CONCLUSIONS AND RELEVANCE: This scoping review found that Ménière disease is a multifactorial disease with lifelong comorbidities and loss of quality-associated life-years whose most commonly reported causes were structural dysfunction, immunologic damage, and genetic susceptibility. Recent studies have examined how autoinflammatory processes and vestibular migraine may be associated with Ménière disease. Large heterogeneity among studies may be explained by historical differences in the clinical understanding of the disease, as well as evolving intervention methodologies and practitioner expertise. Ménière disease is a multifactorial disease with lifelong comorbidities and loss of quality-associated life-years; therefore, future studies of reliable biomarkers of endolymphatic hydrops and real-time imaging are warranted to improve understanding and treatment.