Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology · 2023
Hearing Rehabilitation in Patients With Neurofibromatosis Type 2: The Quebec's Experience With Auditory Implants.
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Khneisser Edwina, Bussieres Richard, Côté Mathieu, Philippon Daniel, Fradet Gaétan, Côté Martin, Lessard Nathalie, Champagne Pierre-Olivier, Trudel Mathieu
Аннотация
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.
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.