Menière's symptoms resulting from bilateral otosclerotic occlusion of the endolymphatic duct: an analysis of a causal relationship between otosclerosis and Menière's disease.

An association between otosclerosis and Menière's disease has been proposed on both a clinical and temporal bone histopathologic basis for well over three-quarters of a century. Controversy persists over a causal relationship between these two entities, however, and the underlying pathophysiologic mechanisms relating capsular otosclerosis with Menière's disease remain speculative. The first case of total, bilateral endolymphatic duct occlusion resulting from extensive capsular otosclerosis is presented in a woman manifesting Menière's symptoms. The severe endolymphatic hydrops resulting from otosclerotic endolymphatic duct occlusion is given as histopathologic proof of a causal relationship between these two entities. Analysis of 18 of our own temporal bone cases of extensive capsular otosclerosis without endolymphatic hydrops and review of the literature indicates the uniqueness of such a firm causal relationship in the present case.