Case report: cochlear implantation in a patient with superficial siderosis.

OBJECTIVE

This is a retrospective case review study. Current understanding of cochlear implantation in patients with superficial siderosis is limited, with roughly 15 reported cases worldwide. The purpose of this study is to report an additional case of cochlear implantation in a patient with superficial siderosis and to contribute to the current literature available on outcomes of implantation in these individuals.

PATIENTS: One cochlear implant user with superficial siderosis in a tertiary referral center.

INTERVENTION(S): Therapeutic and rehabilitative.

MAIN OUTCOME AND

RESULTS

Open set speech perception word scores increased from 0% preimplantation with bilateral hearing aids to 8% postimplantation in the bimodal condition (cochlear implant in the left ear and hearing aid in the right ear). Phoneme scores increased from 16% preimplantation to 36% postimplantation. The patient also demonstrated improvement from 38% preimplantation to 83% postimplantation on a closed set word test. Performance on open set recorded sentences did not improve; however, open set sentences administered live voice revealed an increase in performance from 4% preimplantation to 25% postimplantation in the auditory only condition and from 59% to 86% in the auditory-visual condition.

CONCLUSION

For this patient with superficial siderosis, only marginal improvements in test scores at 11 months postimplantation were seen. Minimally, the use of a cochlear implant did prevent further deterioration of auditory performance. Therefore, patients with superficial siderosis who choose to proceed with cochlear implantation may still report perceived benefit from the maintenance of speech perception abilities even in the presence of minimal improvement. Rapid deterioration in hearing thresholds and speech discrimination before implantation may be an indicator of an actively progressing case of superficial siderosis, which may suggest possible limited benefit from cochlear implantation.