title: Cochlear implantation in a patient with superficial siderosis.
abstract: Superficial siderosis is a rare disease characterized by accumulation of hemosiderin in the leptomeninges, subpial tissue, spinal cord, and cranial nerves. The first clinicopathological description was reported in 1908. Most patients (95%) experience progressive bilateral hearing loss that begins early in the course of disease. The initial management of a patient with hearing loss secondary to superficial siderosis involves sound amplification using hearing aids. However, cochlear implants can be considered in cases where the patient can no longer gain significant benefit from hearing aids. Only 10 studies have described the use of cochlear implants in superficial siderosis and results have been variable. We report the case of a 65-year-old woman with bilateral progressive sensorineural hearing loss caused by superficial siderosis who benefited significantly from cochlear implantation.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Kanazawa University Graduate School of Medical Science, Kanazawa, Ishikawa, Japan.
authors.forename: Hisashi
authors.initials: H
authors.lastname: Sugimoto
authors.forename: Makoto
authors.initials: M
authors.lastname: Ito
authors.forename: Miyako
authors.initials: M
authors.lastname: Hatano
authors.forename: Tomokazu
authors.initials: T
authors.lastname: Yoshizaki
mesh_terms: D000368:Aged; D019929:Cochlear Implantation; D003389:Cranial Nerve Diseases / Q000209:etiology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology / Q000601:surgery*; D006485:Hemosiderin / Q000633:toxicity; D006486:Hemosiderosis / Q000150:complications*; D006801:Humans
chemical_list: D006485:Hemosiderin