Modified transcanal cochlear implantation in CHARGE syndrome: A case report

Abstract

Rationale:Cochlear implantation (CI) in CHARGE syndrome is technically challenging because of the anatomical anomalies. This case aims to report a successful case of CI in CHARGE syndrome by using the modified transcanal approach with external auditory canal (EAC) obliteration.Patient concerns:The 3-year-old girl presented at the outpatient department with bilateral hearing loss and nasal obstruction since birth.Diagnosis:The patient had bilateral profound sensorineural hearing loss, patent ductus arteriosus, atresia of the choanae, middle and inner ear anomalies, and growth retardation, fulfilling the criteria for typical CHARGE syndrome. High resolution temporal bone computed tomography scan revealed a poorly developed mastoid cavity, cochlear dysplasia, hypoplastic semicircular canals, ossicular chain malformation, and sigmoid sinus engorgement. Magnetic resonance imaging revealed a narrow internal auditory canal and a hypoplastic cochlear nerve.Interventions:Modified transcanal approach with external auditory canal obliterationOutcomes:CI was successfully done and there are no intraoperative or postoperative complications occurred after 1 year of follow up.Lessons:The modified transcanal approach is a reasonable and safer option for CI in CHARGE syndrome.