Outcomes of cochlear implantation in patients with neurofibromatosis type 2

Abstract

In neurofibromatosis type 2 (NF2) bilateral vestibular schwannomas (VS) or their treatment usually results in bilateral hearing loss. Cochlear implantation (CI) was traditionally not used in these patients due to concern that retrocochlear disease would render the implant ineffective. This paper describes the auditory outcomes of CI in 13 patients with NF2 and includes patients with untreated VS and patients undergoing VS removal with cochlear nerve preservation. The non-user rate was 7.7%. Of the active users, median CUNY score was 98%, median BKB score in quiet was 90% and median BKB score in noise was 68%. CI is a viable option in selected patients with NF2.

Keywords:

• Cochlear implantation
• Neurofibromatosis type 2
• Vestibular schwannoma base of skull
• Auditory brainstem response

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Contributors None.

Funding None.

Conflicts of interest None declared.

Ethics approval None.