목적: This study aimed to develop a reliable way for preoperative determination of optimal surgical approaches for electrode insertion in cases with aberrant facial nerve (FN) course in CHARGE syndrome. 방법:A total of 14 cases with CHARGE syndrome who got cochlear implantation (CI) were included. The displacement of FN, intrusion of FN into round window (RW) or oval window (OW), various anomalies, applied surgical approach, audiologic and speech performance were analyzed. For the preoperative determination of optimal surgical approach, the spatial relation among basal turn of cochlea, posterior canal wall (PW) and FN was analyzed. 결과:Average age of implantation was 43 months old (range 14-84, MF=37, RLB=154). FN displacement was observed in 11 cases mostly anterior (5) or anteroinferior displacement (6). RW was violated in all cases and OW in 8 cases. All cases showed aplasia of semicircular canals with hypoplasia of vestibule and six cases had cochlear hypoplasia. Nine cases which had spaces between the parallel extension of posterior wall (PW) and FN (average 2.89 0.99 mm, range 1.78 - 4.50 mm) and successfully got CI through facial recess approach (RW approach 1, cochleostomy approach 8). In contrast, five cases without any space got alternative surgical approach (transcanal approach 3 cases, osteoplastic approach of PW 2 cases). The choice between two alternative approaches was determined mainly by the existence of otitis media (OM) considering the protective role of transcanal approach against OM. Preoperative evaluation resulted 0~1 on CAP, 0~5 onIT-MAIS and they were changed into 4 1.7 (range 1-7) on CAP and 30.5 11.6 (range 12-40) on IT-MAIS 20 months after CI. No difference was found according to applied surgical approach, status of RW and OW. 결론:Preoperative evaluation for FN course and their spatial relation with PW, status of RW, OW could provide useful information in the choice of surgical approach for CI in CHARGE syndrome with successful hearing rehabilitation.