Search

Introduction: CHARGE syndrome is a congenital collection of anomalies of various organs including various functional and structural anomalies of the ears. Surgical management of hearing loss in CHARGE syndrome is challenging due to various anatomical malformations of the middle and inner ear. Cochlear implantation surgery for profound sensorineural hearing loss is technically difficult due to these malformations whilst cochlea nerve hypoplasia or aplasia may compromise outcomes. Aberrant facial nerve position may increase perioperative morbidity whilst developmental delay often makes global assessment of patients with CHARGE difficult. We aim to provide surgical guidance on managment of patients with CHARGE Methods: We performed a systematic review of all trials involving cochlear implantation in CHARGE syndrome. We searched Cochrane Central Register of Controlled Trials, PubMed, EMBASE through Ovid SP and Web of Science. Primary outcome measures included Health-related quality of life, audiological outcomes as well as adverse events and complications. We also assessed the most commonly recorded ear anomalies including the findings of aberrant facial nerve positon. A retrospective series of patients with CHARGE syndrome assessed for cochlear implantation at Great Ormond Street Hospital was also performed. Results: 15 patients with CHARGE were assessed at our centre between 1993 and 2017 with ten cochlear implantations performed. Semicircular canal aplasia and vestibulocochlear dysplasia were seen in all patients. All patients had abnormal facial nerve anatomy. These findings were in keeping with the findings of our systematic review which consisted of case series/retrospective reviews. No randomised controlled trials were identified. Data from the systematic review is presented in an easy-to-interpret table. Conclusions: Cochlear implantation in CHARGE patients is surgically challenging with variable outcomes. We describe our experience and compare this to the literature through a systematic review of published outcome data. [ABSTRACT FROM AUTHOR]