Objective:To evaluate the clinical efficacy of surgical intervention for laryngeal airway lesions with concurrent cochlear implantation in CHARGE syndrome concomitant laryngeal airway lesions, and provide clinical data for cochlear implantation in children with CHARGE syndrome concomitant laryngeal airway lesions. Methods:The medical records of five cases diagnosed with CHARGE syndrome were retrospectively reviewed, two of them treated with surgical intervention for laryngeal airway lesions and concurrent cochlear implantation. One child treated with balloon dilatation of laryngeal stenosis and Cochlear implant, and another case received with modified supraglottoplasty for laryngeal malacia and Cochlear implant. Results:Two cases of CHARGE syndrome concomitant laryngeal airway disease, who underwent Cochlear implant and concurrent surgical intervention, recovered well after treatment. The remining three cases treated with Cochlear implant, who previously received deformity-correction surgery. All of the five cases presented with CHD7 mutation. Conclusion:Cochlear implant concurrent with surgical intervention of laryngeal airway lesions for the treatment of CHARGE syndrome concomitant laryngeal airway disease was safe and efficient, which could be a treatment option for children in this situation.