Cochlear implant patients with superficial siderosis

Sarah A. Sydlowski, Michael J. Cevette, Jon Shallop, David M. Barrs

Research output: Contribution to journalArticlepeer-review

17 Scopus citations


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 o of hearing aid technology. Despite the retrocochlear nature of the disorder, patients have undergone cochlear implantation (CI) with varying success. Purpose: To summarize the issues surrounding cochlear implant candidates with SSC SSCN as well as highlight trends in performance postimplantation. Research Design: Retrospective case reports of seven cochlear implant candidates detail the symptoms, typical audiologic presentation, and array of clinical issues for patien patients with this progressive and potentially fatal disease. Results: Despite the retrocochlear component of a hearing loss caused by SSCN, coch 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 presentati presentation of SSCN are critical. The diagnosis of SSCN will impact expectations for success with the cochlear i implant, and counseling should be adjusted accordingly.

Original languageEnglish (US)
Pages (from-to)348-352
Number of pages5
JournalJournal of the American Academy of Audiology
Issue number6
StatePublished - 2009


  • Cochlear implant
  • Hemosiderin
  • Retrocochlear
  • Superficial siderosis

ASJC Scopus subject areas

  • Speech and Hearing


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