Abstract
Background Many patients with neurofibromatosis type 2 (NF2) suffer from sensorineural hearing
loss, and associated cochlear nerve compromise in NF2 patients makes auditory brainstem
implant (ABI) an attractive treatment option. The long-term outcomes and benefits
of the device are still being explored.
Methods A retrospective review was conducted for 11 ABI recipients at a single-institution
tertiary center between November 2017 and August 2022. Patients diagnosed with NF2
undergoing resection for concurrent vestibular schwannoma (VS) were included. Pre-
and postaudiometric assessments were reviewed. Evaluation included pure-tone audiometry
and speech testing.
Results Our cohort included 11 patients with a median age of 34 years. All patients underwent
a translabyrinthine approach for implant placement with concurrent VS resection. Average
tumor size of VS was 2.87 cm. Preoperatively, 8 patients had pure-tone averages with
no response at 110 dB, 2 were within mild–moderate hearing loss (25–45 dB), and one
patient had a PTA of profound loss (92 dB). Postoperatively, 9 (81%) patients had
improvement in PTA. In total, seven patients reported mild side effects upon ABI activation
which included dizziness (n = 2), tinnitus (n = 1), and abdominal and lower extremity tingling sensation (n = 3). Of the seven patients with early speech perception (ESP) scores, five had a
score >75%, indicating the auditory ability to detect pattern perception upon auditory
stimulation through the ABI alone.
Conclusion Nine of 11 patients derived benefits from ABI placement. These findings demonstrate
that ABI placement during concurrent VS resection can provide a significant hearing
benefit for NF2 patients.
Keywords
auditory brainstem implant - vestibular schwannoma - NF2 - neurofibramatosis 2 - audiometric
outcomes
