Related Experiment Video
Updated: Jul 19, 2025
Robotic Cochlear Implantation for Direct Cochlear Access
Published on: June 16, 2022
Hearing Rehabilitation in Patients With Neurofibromatosis Type 2: The Quebec's Experience With Auditory Implants
Edwina Khneisser1, Richard Bussieres1, Mathieu Côté1
1Department of Otolaryngology-Head and Neck Surgery, CHU de Québec-Université Laval, Hotel Dieu de Quebec.
Objective:
To review a single-center experience with hearing rehabilitation in patients with neurofibromatosis type 2 (NF2) and to describe the auditory outcomes of cochlear implants (CIs) and auditory brainstem implants (ABI) in this population.
Study Design:
Retrospective case series.
Setting:
Tertiary referral center.
Patients:
Twelve adult patients with NF2 who received a CI (n = 10) and/or an ABI (n = 5) between 2000 and 2021.
Interventions:
Insertion of a CI and/or an ABI in NF2 patients with bilateral vestibular schwannomas (VSs).
Main Outcome Measures:
Patients' demographic data, treatment history, hearing evolution, hearing rehabilitation methods, implant details, and auditory outcomes after implantation.
Results:
Among those who received a CI, five patients had a stable untreated VS, one patient underwent a cochlear nerve preserving surgery, and four patients received radiotherapy treatments. Six patients became regular users of their device. The median open-set sentence recognition scores were as follows: 0.5% preoperatively, 60% at 1 year postoperatively, and 80% on the most recent audiological evaluation. All patients with an ABI were implanted concomitantly with VS surgical excision, and three of them also received radiotherapy treatments. The median open-set sentence recognition scores were as follows: 4% preoperatively, 26% at 1 year postoperatively, and 0% on the most recent evaluation. Three patients became regular ABI users.
Conclusion:
Despite major technological advances, auditory outcomes with ABIs remain deceiving. Considering the overall improvement in postoperative auditory performances provided by CIs compared with ABIs, cochlear implantation standouts as a primary mean of hearing rehabilitation in NF2 patients.
