Since the report of Duke in which an allergic etiology was considered to be the cause of Meniere's disease, the hypothesis that a certain type of Meniere's disease is generated through immuno-pathological mechanisms has been advocated for 70 years. During this period, another entity of immune-mediated inner ear disorders, i. e., autoimmune inner ear disease was introduced. Fundamental immunological phenomena of the inner ear have been rapidly elucidated since 1980. The endolymphatic sac is the only site which contains immuno-competent cells within the inner ear. The inner ear is capable of mounting active immune responses when appropriately stimulated and the endolymphatic sac plays an integral function for inner ear immune response. Actually, many reports have been published that link immunity and Meniere's disease with a variety of proposed immune-related etiologies from autoimmunity to non-autoimmunity. It is suggested that immune injury to the endolymphatic sac plays an important role in the pathogenesis of Meniere's disease. These functional and morphological circumstances strongly suggest that an immunological etiology of Meniere's disease is not theoretically unfounded.