Meniere’s Disease: Genetics and the Immune System

Abstract

The purpose of this review was to describe the ongoing research that supports that Meniere’s disease (MD) is a heterogeneous set of clinical disorders. Different clinical subgroups of patients have been identified, which may have different etiological factors. Genes associated to the initiation or progression of MD can be classified in four main categories: immune-associated, proliferation and cell survival, cell surface channels, and extracellular matrix proteins. Two major mechanisms have been involved in MD, the innate immune response involving proinflammatory cytokines and NF-κB-mediated inflammation, but the molecular events that initiate the disorder are not known. The challenge of the immune system by antigens or allergic reactions may involve several targets in the cochlea, such as the endolymphatic sac or the spiral ligament, leading to inflammation and finally endolymphatic hydrops. Several clinical variants have been described, and genetic and immunological factors seem to play a central role in MD.