Endoscopic-assisted cochlear implant procedure in CHARGE syndrome: Preliminary report

The endoscopic approach demonstrated to be useful for cochlear implantations of children with CHARGE syndrome. It allows the surgeon to perform a direct 'safe' cochleostomy in difficult anatomical conditions. Three children with CHARGE syndrome underwent endoscopic-assisted cochlear implant surgery at the Tertiary University Referral Center of Modena and Verona between January 2014 and September 2015. A review of clinical data and videos from the operations was made. All procedures were re-analyzed and codified. Three children, all females (mean age 4.6 years; range: 3-7 years) underwent surgery. Two primary surgical procedures and one revision surgery, for secondary cholesteatoma, were performed. CT scans demonstrated complex malformations of middle and inner ear with anomalous course of the facial nerve. In all subjects, a transcanal endoscopic cochleostomy was performed; no immediate or late postoperative complications were observed. Discharge from hospital was the day post-surgery. The current mean follow-up is 12.5 months (range: 8-19 months).