Establish outcomes following cochlear implantation (CI) in patients with Pendred syndrome. Systematic review and narrative synthesis. Databases searched: Medline, Pubmed, Embase, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on language or year of publication. Review conducted in accordance with the PRISMA statement. Searches identified 251 abstracts and 242 full texts. Of these, 22 studies met inclusion criteria reporting outcomes in 231 patients with at least 234 implants. Hearing outcomes were generally good with patients experiencing useful functional improvement. A total of 46 minor complications were reported in 78 cases. The methodological quality of included studies was modest, predominantly consisting of case reports and non-controlled case series with small numbers of patients. All studies were OCEBM grade III-IV. Hearing outcomes following CI in Pendred syndrome are generally good with useful functional improvement. However, outcomes reported in published studies lack long term follow up.