Cochlear implantation in superficial siderosis.

Superficial siderosis is a rare central nervous system disorder characterized by deafness, ataxia, and pyramidal signs. The hearing loss is believed to be predominantly neural and is usually progressive and bilateral. Careful assessment is therefore necessary to determine the best approach to hearing rehabilitation. A case is presented of superficial siderosis in a young woman who has benefitted significantly from cochlear implantation using the Nucleus device.