Objective:To review outcomes of cochlear implantation (CI) in children diagnosed with autism spectrum disorder (ASD).Study Design:Retrospective case review and parent survey.Setting:Tertiary care children's hospital.Patients:Thirty children with ASD who underwent CI between 1991 and 2018. Mean age at CI = 3.5 years (0.8–11.8), mean age at diagnosis of ASD = 5.1 years (2.0–15.0) (22/30 diagnosed after CI), mean follow-up = 10.5 years (1.4–21.6). Parents of 7 children returned a survey.Intervention:Unilateral or bilateral cochlear implantation.Main Outcome Measures:Speech perception; expressive communication mode; educational placement; social engagement; consistency of CI use; parent survey of child behavior change.Results:Thirty-three percent of all and 45% of the 22 consistent device users developed measurable open-set speech perception by an average of 4.5 years of device use. Educational placement at last follow-up included 13% mainstreamed without interpreter, 50% Special Education programs, 10% therapeutic residential or day programs, 23% total communication programs, and one home schooled. Spoken language alone was used by 31% and spoken plus sign by 14%, with the remainder using sign alone, augmentative communication devices or no mode of communication. By parent report, 86% showed improvement in social engagement compared to pre-CI. Survey results showed the behaviors most frequently ranked as most affected by CI were communication and attention, while awareness of environment had the lowest (most affected) mean ranking.Conclusions:Findings support a growing body of literature that cochlear implantation has the potential to improve auditory skills, language, and enhance social engagement in some deaf children with autism spectrum disorder.