Auditory brainstem implants for hearing rehabilitation in NF2-schwannomatosis: A systematic review and single-arm meta-analysis

Abstract

BACKGROUND: NF2-schwannomatosis (NF2) is an autosomal dominant disorder prone to hearing loss. Auditory brainstem implants (ABIs) offer a promising solution for hearing rehabilitation in NF2. OBJECTIVE: To synthesize existing literature on ABI implantation in NF2, focusing on audiological outcomes and ABI-related complications. METHODS: The systematic review followed PRISMA guidelines and was registered in the PROSPERO database (CRD42022362155). Relevant studies were identified by searching PubMed, EMBASE, CENTRAL, CMB, and CNKI from inception to August 2023. Data on environmental sound discrimination, open-set discrimination, closed-set discrimination, and ABI-related complications were extracted and subjected to meta-analysis. Publication bias was evaluated using funnel plots and Egger’s test. RESULTS: Thirty-three studies were included. The pooled estimate was 58% (95% CI 49–66%) for environmental sound discrimination and 55% (95% CI 40–69%) for closed-set discrimination. Regarding open-set discrimination, the pooled estimates were 30% (95% CI 19–42%) for sound only, 46% (95% CI 37–54%) for lip-reading only, and 63% (95% CI 55–70%) for sound plus lip-reading. The pooled occurrence of ABI-related complications was 33% (95% CI 15–52%). CONCLUSION: This meta-analysis underscores the effectiveness and safety of ABIs in NF2, providing valuable insights for evidence-based decision-making and hearing rehabilitation strategies.