A case of sensorineural hearing loss due to superficial siderosis that improved with corticosteroids

Abstract

Superficial siderosis is a disease that causes neurological disorders due to iron (hemosiderin) deposition on the brain surface and in the brain parenchyma. Chronic or recurrent hemorrhage in the subarachnoid space is thought to be the cause of this disease. The clinical manifestations of the disease include sensorineural hearing loss, cerebellar ataxia, pyramidal tract signs, and cognitive dysfunction. The clinical manifestations are progressive and irreversible. Some reports suggest that chelating agents and corticosteroids may be effective as treatments; however, at this point, no effective treatment has been clearly identified. We report a case of sensorineural hearing loss due to superficial siderosis that was diagnosed by MRI, which improved after treatment with orally administered corticosteroids.