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There have been many hypotheses regarding the underlying etiology of Meniere's disease since its first description in 1861. There appears to be a genetic component or familial predisposition that in the presence of certain "triggers" or external events, (see below) may cause Meniere's disease.

Evidence of a genetic component for Meniere's disease includes:

• Family history - Approximately 5-20% of patients diagnosed with Meniere's disease report that a family member has/had Meniere's disease or similar symptoms.
• Racial/ethnic factors - Meniere's disease appears to be more common in northern parts of Europe and the U.S.
• Physical anomalies or malformations are present in some people with Meniere's disease
• Ongoing chromosomal studies appear to be closer to identifying certain genetic markers in patients with Meniere's disease
• Some people with Meniere's disease have autoimmune conditions
• Otosclerosis - formation of spongy bone around the stapes (a rare condition). Many patients with otosclerosis have symptoms of Meniere's disease. It is thought that otosclerotic formations may cause malformation in the endolymphatic sac or may change the chemical properties of the perilymphatic and endolymphatic fluid.