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Cochlear Implantation and Hearing Rehabilitation in CHARGE Syndrome: Expanding Clinical Knowledge and Outcomes
Soraya Abdul-Hadi1, Margaret Kettler2, Loga Iyer3
1Division of Pediatric Otolaryngology-Head and Neck Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Insights
Cochlear implantation (CI) is feasible for CHARGE syndrome (CS) patients, offering benefits despite anatomical challenges. An individualized approach is crucial for surgical planning and counseling.
Area of Science:
- Otolaryngology
- Pediatric Audiology
- Medical Genetics
Background:
- CHARGE syndrome (CS) presents complex challenges for auditory rehabilitation.
- Cochlear implantation (CI) is a potential intervention for hearing loss in CS.
- Understanding factors influencing CI outcomes in CS is critical.
Purpose of the Study:
- To characterize patients with CHARGE syndrome undergoing cochlear implantation.
- To evaluate factors influencing surgical approaches and outcomes in CS patients receiving CI.
- To provide guidance for CI candidacy and management in this population.
Main Methods:
- Retrospective study at a quaternary pediatric referral center.
- Analysis of demographic, audiologic, radiologic, operative, and postoperative data from 55 CS patients (≤21 years).
- Outcomes assessed included audiologic performance, device use, communication modality, and facial nerve stimulation (FNS).
Main Results:
- CI was performed in 67 ears of 55 CS patients; 91% had neurodevelopmental impairment.
- Complete electrode insertion achieved in 81% of ears; cochlear nerve anomalies and stenosis were common.
- Overall clinical benefit was identified in 64% of patients, with 49% active CI users; 15% experienced FNS.
Conclusions:
- Cochlear implantation is feasible and beneficial for CS patients, despite anatomical and neurodevelopmental complexities.
- An individualized approach to CI candidacy, surgical planning, and family counseling is supported.
- Findings offer practical guidance for managing hearing loss in CHARGE syndrome.
Objective:
Characterize patients with CHARGE syndrome (CS) undergoing cochlear implantation (CI) and evaluate factors influencing surgical approaches and outcomes.
Study Design:
Retrospective study.
Setting:
Quaternary pediatric referral center.
Patients:
Fifty-five patients with CS aged ≤21 years who underwent CI.
Intervention:
Demographic, audiologic, radiologic, operative, and postoperative data were reviewed. Outcomes included audiologic performance, device use, communication modality, and the presence of facial nerve stimulation (FNS).
Main Outcome Measures:
Surgical feasibility, anatomic characteristics, and auditory outcomes.
Results:
Fifty-five patients underwent CI, representing 67 ears. The cohort was 62% male, and 91% had neurodevelopmental impairment. Complete electrode insertion was achieved in 81% of ears (54/67), partial insertion in 5% (3/67), and insertion data were unavailable in 15% (10/67). Perimodiolar electrodes were used in 57% of ears. Imaging demonstrated cochlear nerve aplasia or hypoplasia in 54% of implanted ears and cochlear aperture stenosis in 25%. At analysis, 49% of patients were active CI users, while 27% had discontinued use. Audiologic threshold improvement was observed in 47% of patients, and overall clinical benefit was identified in 64% based on combined audiologic and functional measures. Ipsilateral facial nerve stimulation occurred in 15% of implanted ears; among these, 60% remained active users, while 40% discontinued CI use due to FNS unresponsive to reprogramming.
Conclusion:
CI is feasible and can provide meaningful benefit in patients with CS despite complex anatomic and neurodevelopmental challenges. These findings support an individualized approach to CI candidacy and offer practical guidance for surgical planning and family counseling regarding expected outcomes.