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Updated: May 7, 2026
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Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
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Cochlear Implantation in Patients With CHARGE Syndrome: A Systematic Review and Meta-Analysis
Kalena Liu1, Jacob Beiriger1, Eric Fei1
1Department of Otolaryngology, Thomas Jefferson University, Philadelphia, PA.
Summary
Cochlear implantation (CI) significantly improves auditory outcomes in children with CHARGE syndrome. This review of 15 studies shows enhanced speech and hearing abilities post-implantation, supporting CI use in this complex population.
Area of Science:
- Otolaryngology
- Pediatric audiology
- Genetics
Background:
- CHARGE syndrome presents complex inner ear malformations impacting hearing.
- Limited large-scale data exists on cochlear implantation (CI) outcomes for these patients.
Purpose of the Study:
- To evaluate preoperative and postoperative speech and auditory outcomes in CHARGE syndrome patients undergoing CI.
- To synthesize evidence from published single-center studies via meta-analysis.
Main Methods:
- Systematic review and meta-analysis of 15 studies involving 85 CHARGE patients (99 ears).
- Data extraction on demographics, study characteristics, and auditory/speech outcomes.
- Pooled analysis of auditory outcome measures using standardized mean differences (SMDs).
Main Results:
- Significant global postoperative improvement in auditory outcomes observed post-CI.
- Meta-analysis revealed significant increases in Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) scores (mean difference 11.9, P=0.036).
- Significant increases in Categories of Auditory Performance (CAP) scores were also noted (mean difference 2.9, P=0.004).
Conclusions:
- Cochlear implantation demonstrates efficacy in improving auditory function in CHARGE syndrome patients.
- Findings support the use of CIs despite the challenges posed by inner ear malformations in this population.
- This meta-analysis adds valuable evidence to the growing body of literature on CI benefits for CHARGE syndrome.
