title: Cochlear implantation in superficial siderosis.
abstract: Superficial siderosis (SS) of the central nervous system has been thought to be a rare condition that generates progressive hearing loss, ataxia, pyramidal signs, and dementia. The main cause of hearing loss by SS is thought to be neuronal. Because there is no histopathologic report of the human temporal bone in SS, there is a debate about the possibility of cochlear involvement. We present a 25-year-old man who was investigated for bilateral progressive sensorineural hearing loss and vestibular failure after head trauma. On brain MRI, SS of the central nervous system was detected. Distortion product otoacoustic emission (DPOAE) and auditory brainstem response (ABR) showed no response on both sides. However, integrity of the eighth nerve was proved by the electrical ABR test on the right side and the patient benefited significantly from cochlear implantation. The sensorineural hearing loss in SS seems to be related to cochlear damage as well as neuronal damage. So, cochlear implantation would be a hearing rehabilitation modality for the sensorineural hearing loss caused by SS.
authors.affiliation: Department of Otorhinolaryngology-Head and Neck Surgery, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Chong-Sun
authors.initials: CS
authors.lastname: Kim
authors.forename: Jae-Jun
authors.initials: JJ
authors.lastname: Song
authors.forename: Min-Hyun
authors.initials: MH
authors.lastname: Park
authors.forename: Young Ho
authors.initials: YH
authors.lastname: Kim
authors.forename: Ja-Won
authors.initials: JW
authors.lastname: Koo
mesh_terms: D000328:Adult; D001927:Brain Diseases / Q000150:complications / Q000175:diagnosis / Q000534:rehabilitation; D019929:Cochlear Implantation; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000534:rehabilitation*; D006486:Hemosiderosis / Q000150:complications* / Q000175:diagnosis / Q000534:rehabilitation; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D011571:Psychoacoustics; D013063:Speech Discrimination Tests; D015837:Vestibular Diseases / Q000150:complications* / Q000175:diagnosis / Q000534:rehabilitation; D014724:Vestibular Function Tests