Role of Hearing Loss Genes in the Sensory Epithelia Associated With Meniere Disease

ABSTRACT Meniere disease (MD) is an inner ear disorder characterized by episodic vertigo, tinnitus, fluctuating sensorineural hearing loss (SNHL), and aural fullness. Its hallmark pathological feature is endolymphatic hydrops. MD shows significant familial clustering in European and East Asian populations, supporting a strong genetic component in disease susceptibility. Dysfunctional sensory epithelia, particularly in the cochlea and vestibular organs, are increasingly recognized as central to the development of SNHL in MD patients. Notably, numerous non‐syndromic hearing loss genes such as OTOG, MYO7A, TECTA, or TRIOBP are expressed in sensory epithelial cells across the inner ear, including hair cells and supporting cells. Rare variants in these MD‐associated genes may contribute to MD pathogenesis through disruption of hair cell stereocilia, interfering mechanoelectrical transduction, disturbing ionic homeostasis, or affecting inner ear development. This review aims to summarize the current evidence on SNHL genes expressed in the inner ear sensory epithelium and their potential role in the molecular pathophysiology of MD.