Cochlear implantation in patients with superficial leptomeningeal hemosiderosis

Superficial leptomeningeal hemosiderosis is a rare disease of the central n ervous system. Chronic bleeding into the subarachnoid space causes depositi on of hemosiderin in glial cells and subsequent damage to adjacent brain ti ssue. There is a characteristic predilection for the cerebellum and eighth cranial nerve. Accordingly,among a variety of symptoms, cerebellar ataxia a nd sensorineural hearing loss progressing to total deafness commonly occur. To date,the hearing loss has been believed to be purely neural. We present a case of superficial hemosiderosis in a patient with total deafness who w as successfully provided with a cochlear implant. Audiometry demonstrated t otal bilateral cochlear hearing losses but with preserved function of the e ighth cranial nerve. These findings make us conclude that in contrast to cu rrent opinion, superficial hemosiderosis can initially damage the cochlea a lone. Thus, patients with total deafness due to this disorder may benefit f rom cochlear implantation. All patients require careful audiometric assessm ent, including promontory stimulation.