Vestibular schwannoma in the only hearing ear: cochlear implant or auditory brainstem implant?

OBJECTIVE: To explore the dilemma faced by neurotologists confronted with the patient who develops a vestibular schwannoma in the only hearing ear, the other having been deaf from birth, and to consider the choice between auditory rehabilitation using a cochlear implant (CI) on the congenitally deaf side and an auditory brainstem implant (ABI) on the tumor side.

STUDY DESIGN: A record review of two patients born deaf in one ear and who developed a vestibular schwannoma in the contra lateral ear, who then received a CI in the congenitally deaf ear.

SETTING: Tertiary referral center with special experience in vestibular schwannoma surgery, neurofibromatosis type 2 management, and cochlear implantation.

RESULTS: Neither patient was a good CI user. At 1 year postimplant, they both scored 0% on abbreviated words and 0% and 7%, respectively, on Bench Kowal Bamford sentences. They scored 54% and 57%, respectively, on City University of New York sentences with lip-reading, and both had fair access to environmental sound scoring at 45% each.

CONCLUSIONS: The results from cochlear implantation in the congenitally deaf ear in these patients were poor and suggest that stimulus deprivation in the early stages of the maturation of the auditory pathways is important even for a unilateral hearing loss. Unfortunately, the factors that predict a good ABI result are not known. In these circumstances, the authors advocate the insertion of an ABI at the time of tumor removal, retaining the option of CI in the congenitally deaf ear in the event of a poor outcome with the ABI.