Cochlear Implantation in Patients With CHARGE Syndrome: A Systematic Review and Meta-Analysis

OBJECTIVE: Currently, there are limited large studies exploring cochlear implantation (CI) outcomes in patients with CHARGE syndrome. We aim to evaluate preoperative and postoperative speech and auditory outcomes in patients with CHARGE syndrome undergoing CI from a review of published single-center studies. DATABASES REVIEWED: PubMed, Scopus, and CINAHL. METHODS: A total of 15 studies were included for analysis, encompassing 85 CHARGE patients with a total of 99 implanted ears. Extracted data included demographic information, study characteristics, and speech perception and auditory outcomes. Pooled analyses of scaled auditory outcome measures were performed using standardized mean differences (SMDs). Risk of bias was assessed using the Joanna Briggs Critical Appraisal Checklist to evaluate the quality and validity of included studies. RESULTS: This meta-analysis demonstrates significant global postoperative improvement in auditory outcomes for patients with CHARGE syndrome who underwent CI. Meta-analyzed data showed a significant increase in Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) scores following CI, with an estimated overall pre/post mean difference of 11.9 (P = 0.036), and a significant increase in categories of auditory performance (CAP) scores following CI, with an estimated overall pre/post mean difference of 2.9 (P = 0.004). CONCLUSION: Despite the complexity of inner ear malformations in this population, these findings contribute to the growing body of evidence supporting the use of CIs in CHARGE patients.