Cochlear implant patients with superficial siderosis.
BACKGROUND
Considered a rare disorder, superficial siderosis of the central nervous system (SSCN) has become more frequently diagnosed in recent years. As it is characterized by progressive sensorineural hearing loss, patients' needs may surpass the capability of hearing aid technology. Despite the retrocochlear nature of the disorder, patients have undergone cochlear implantation (CI) with varying success.


OBJECTIVE
To summarize the issues surrounding cochlear implant candidates with SSCN as well as highlight trends in performance postimplantation.


METHODS
Retrospective case reports of seven cochlear implant candidates detail the symptoms, typical audiologic presentation, and array of clinical issues for patients with this progressive and potentially fatal disease.


RESULTS
Despite the retrocochlear component of a hearing loss caused by SSCN, cochlear implantation may be a viable option.


CONCLUSIONS
It is essential that the CI audiologist not only be aware of the disorder but also be well versed in the resulting implications for the cochlear implant process. A more thorough case history, an expanded candidacy test battery, and knowledge of the typical presentation of SSCN are critical. The diagnosis of SSCN will impact expectations for success with the cochlear implant, and counseling should be adjusted accordingly.
Sydlowski
Sarah A
SA
Mayo Clinic Arizona, Department of Audiology, 13400 East Shea Boulevard, Scottsdale, AZ 85259, USA. sarah.sydlowski@gallaudet.edu
Cevette
Michael J
MJ
Shallop
Jon
J
Barrs
David M
DM
D000328:Adult; D000368:Aged; D001921:Brain / Q000473:pathology; D003054:Cochlear Implants; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000628:therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012806:Siderosis / Q000150:complications* / Q000473:pathology*; D013067:Speech Perception; D016896:Treatment Outcome