Bilateral Auditory Brainstem Implants in Patients With Neurofibromatosis 2

Otolaryngol Head Neck Surg. 2021 Aug;165(2):339-343. doi: 10.1177/0194599820977420. Epub 2020 Dec 15.

Abstract

Objective: To discuss indications for bilateral auditory brainstem implants (ABIs), compare audiometric outcomes of unilateral vs bilateral ABIs, and determine if patients have improved outcomes with addition of a second-side implant.

Study design: Retrospective review of 24 patients with neurofibromatosis 2 (NF2) who underwent sequential placement of ABIs from 1989 to 2019.

Setting: Tertiary referral center.

Methods: Charts were reviewed for indication for second-side surgery, use of implants, and audiometric outcomes. Implants placed in the past 30 years were included in the study. Northwestern University Children's Perception of Speech (NU-CHIPS) and/or City University of New York (CUNY) sentence scores were compared in unilateral and bilateral conditions.

Results: Indications for a second-side implant included first-side implants with severe nonauditory symptoms (11), marginal audiometric results (9), outdated technology (2), or deterioration of first side (2). Seven patients are bilateral users and 1 patient discontinued bilateral use after a year due to no significant improvement over unilateral use. One patient with initial bilateral use was lost to follow-up. Thirteen patients are unilateral users due to nonaudiometric side effects or poor audiometric outcomes with the first side. Two patients are complete nonusers. Seventy-five percent had improved audiometric outcomes after the second-side implant, and 20% had stable findings.

Conclusions: Second-side ABIs should be consider in patients with poor performance from a first-side implant. Most patients demonstrate subjective improvement with the second ABI. More research is needed for better objective assessments of improvements.

Keywords: auditory brainstem implant; bilateral; cochlear implant; neurofibromatosis type 2; treatment.

MeSH terms

  • Adolescent
  • Audiometry
  • Auditory Brain Stem Implantation*
  • Auditory Brain Stem Implants*
  • Child
  • Child, Preschool
  • Female
  • Hearing Disorders / diagnosis
  • Hearing Disorders / etiology
  • Hearing Disorders / therapy*
  • Humans
  • Male
  • Neurofibromatosis 2 / complications*
  • Neurofibromatosis 2 / diagnosis
  • Neurofibromatosis 2 / therapy
  • Patient Selection
  • Retrospective Studies
  • Treatment Outcome