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Superficial siderosis is a disease in which iron from hemoglobin is deposited in the central nervous system, resulting in conditions such as progressive sensorineural hearing loss, cerebellar ataxia, dementia, and pyramidal signs. A 30-year-old man with superficial siderosis received a cochlear implant in the left ear, which had shown complete hearing loss. Good auditory responses were obtained at 14 days after implantation. The postoperative average hearing level with the cochlear implant was 56.7 dB at 3 months and 55.0 dB at 6 months. However, the patient showed gradual hearing loss, and the dynamic range changed each time the electrode parameters were adjusted. To assess residual hearing ability, single-photon emission computed tomography was performed together with an assessment of electrical auditory brainstem response, which showed a good response and increased blood flow in both the temporal lobes. Based on this result, we asked the patient to continue using the cochlear implant to see whether a perception of speech response would be obtained. However, the patient discontinued using the cochlear implant because he could not hear satisfactorily. Hearing outcomes after cochlear implant surgery for patients with superficial siderosis are not necessarily good. Therefore, the possibility of unsatisfactory results should be fully explained before recommending this surgery to patients.

A Case of Cochlear Implantation in a Patient with Superficial Siderosis