Cochlear implantation in superficial siderosis

Chong Sun Kim, Jae Jun Song, Min Hyun Park, Young Ho Kim, Ja Won Koo

Research output: Contribution to journalArticlepeer-review

24 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)892-896
Number of pages5
JournalActa Oto-Laryngologica
Issue number8
Publication statusPublished - 2006 Aug 1
Externally publishedYes

Bibliographical note

Copyright 2011 Elsevier B.V., All rights reserved.


  • Cochlear implantation
  • Hearing loss
  • Siderosis

ASJC Scopus subject areas

  • Otorhinolaryngology


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