Meniere's Disease of Syphilitic Etiology

Congenital or acquired syphilis is the cause of Meniere's disease in six percent of all cases. The pathogenesis is endolymphatic hydrops and osteitis of the otic capsule which produces the characteristic fluctuating hearing loss, tinnitus and ear pressure associated with episodic spells of vertigo. The ear symptoms are, at first, typical of Meniere's disease of other etiologies. Failure to establish the specific etiologic diagnosis can result in unnecessary surgical treatment or inappropriate medical therapy. In some cases, if a steroid, the specific treatment, is not given promptly, hearing can be quickly and permanently lost. The etiology is confirmed by obtaining a reactive fluorescent treponema antibody absorption (FTA-abs) test. The clinical features and natural history of Meniere's disease of syphilitic etiology are unique. Ear symptoms often begin in the fifth decade of life, first in one ear and, after a few years, involving the second ear. Reduced or absent caloric responses are characteristic. When hearing loss is severe and sudden, this condition represents a true medical emergency and should be treated with prompt administration of steroids. An endolymphatic subarachnoid shunt operation is occasionally necessary as adjunctive management. An analysis of 34 cases shows that long-term use of steroids can maintain effective hearing for more than 20 years.