ABSTRACT

Meniere's disease is an idiopathic inner ear disorder characterized by recurrent spontaneous vertigo accompanied by fluctuating or progressive sensorineural hearing loss, tinnitus and aural fullness in the affected ear. The histopathological correlate is highly linked to endolymphatic hydrops, a finding which is not pathognomonic to Meniere's disease, but more likely a marker for overall disordered inner-ear homeostasis. Meniere's disease affects the quality of life significantly and generally is treated with counselling on dietary restrictions, drug therapy and surgical interventions to prevent recurrence of vertigo. While Meniere's disease is sporadic in the majority of cases, an estimated 5–15% of cases are familial. Several candidate genes have been proposed for Meniere's disease, including AQP2, KCNE1, KCNE3, HCFC1, COCH, ADD, HSPA1A, PTPTN22 and IL1. The cause of Meniere's disease is unknown. Hallpike and Cairns suggested the relationship with endolymphatic hydrops in 1938. It has been observed that endolymphatic hydrops impairs blood flow autoregulation.