Cochlear implantation in patients with CHARGE syndrome: a 10-year institutional experience and literature review

OBJECTIVE: This study aimed to report a 10-year single-centre experience of cochlear implantation in children with CHARGE syndrome (manifests as coloboma, heart defects, atresia of the choanae, growth retardation and genital and ear anomalies) and evaluate surgical strategies and outcomes. METHODS: Children with CHARGE syndrome who underwent cochlear implantation between 2014 and 2024 were retrospectively reviewed. Patient characteristics and post-operative auditory outcomes were analysed. RESULTS: Six patients (nine ears) were included. Most had airway anomalies, one-third required post-operative intensive care. Temporal bone malformations were present in 78 per cent of ears. Standard posterior tympanotomy was feasible in one-third; the remainder required transcanal or combined approaches. Full-electrode insertion was achieved in all ears. Median follow-up was 3.4 years; aided thresholds ranged 25 to 50 dB hearing level. Three patients developed reliable open-set speech perception, two achieved closed-set recognition and one demonstrated environmental sound awareness. CONCLUSION: Cochlear implantation provides meaningful auditory benefit in selected children with CHARGE syndrome. Pre-operative cochlear nerve imaging, flexible surgical planning and coordinated peri-operative airway management are essential.