Abstract
Objective:CHARGE syndrome（CS） is an autosomal dominant congenital malformation involving multiple organs and sensory systems. The incidence of hearing impairment and developmental retardation in CS patients is 87% and 84%, respectively, which is often accompanied by malformations such as abnormal development of temporal bone, venous sinus, and facial nerve that directly affect cochlear implant surgery. Other systemic malformations increase perioperative risks, such as heart defects, micrognathia, laryngeal and trachea malformations. Cochlear implants are significant in promoting hearing and language rehabilitation and improving the psychomotor development of children with CS. Still, the process and outcome assessment of cochlear implantation are challenging. The Chinese Multi-center Clinical Research Collaboration Group on Genetic Screening and Diagnosis of Deafness organized domestic otologists, experienced audiologists, and speech rehabilitation experts to form an expert consensus on the perioperative management, key points of surgical operation and postoperative rehabilitation of cochlear implantation to standardize the clinical diagnosis, treatment, and rehabilitation for CS.