SUPERFICIAL SIDEROSIS OF THE CENTRAL-NERVOUS-SYSTEM - AN UNDERDIAGNOSED CAUSE OF SENSORINEURAL HEARING-LOSS AND ATAXIA

Superfical siderosis of the central nervous system (CNS) is a rare but ultimately fatal disorder characterized by progressive hearing loss, ataxia, and dementia. Symptoms are caused by the deposition of hemosid erin in the CNS as a result of recurrent or persistent extravasation o f blood into the cerebrospinal fluid. Sites most commonly involved inc lude the cerebellum, brain stem, and eighth cranial nerve. In the past , diagnosis was most often made at autopsy, and this may account for t he lack of reported cases in the otolarynogologic literature and the p resumed rarity of the disorder. Now, diagnosis is confirmed by charact eristic magnetic resonance imaging findings and treatment is targeted toward the source of bleeding. The diagnosis of superficial CNS sidero sis should be entertained in all patients presenting with sensorineura l hearing loss and ataxia.