Klockars Tuomas, Kentala Erna
Department of Otorhinolaryngology, Helsinki University Central Hospital, PL220, 00029 HUS, Helsinki, Finland.
Auris Nasus Larynx. 2007 Mar;34(1):101-4. doi: 10.1016/j.anl.2006.09.020. Epub 2006 Nov 21.
The etiologies of Meniere's disease and otosclerosis are largely unknown. An association between these two diseases has been proposed on both a clinical and histopathologic basis but the causal relationship is controversial. In this paper we report two families in which both otosclerosis and Meniere's disease are inherited as independent phenotypes suggesting that the two diseases represent different outcomes of the same mutation. Thus the Meniere's disease occasionally seen in otosclerotic patients might not be caused by otosclerosis, but rather by a molecular defect leading to endolymphatic hydrops and/or clinical otosclerosis.