ObjectiveThis study aimed to establish hearing outcomes after cochlear implantation in patients with otosclerosis.Materials and MethodsWe conducted a systematic review and narrative synthesis. Databases searched were as follows: MEDLINE, PubMed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov. No limits were placed on language or year of publication. The review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement.ResultsSearches identified 474 abstracts and 180 full texts, with 68 studies meeting the inclusion criteria and reporting outcomes in a minimum of 481 patients with at least 516 implants. Patient-reported outcome measures (PROMs) were reported in five studies involving 51 patients. Intraoperative adverse events/surgical approach details and preoperative radiological assessment were reported in 46 and 38 studies, respectively. The methodological quality of included studies was modest, predominantly consisting of case reports and noncontrolled case series with small numbers of patients. Most studies were Oxford Centre for Evidence Based Medicine grade IV.DiscussionAccess to good rehabilitation support is essential to achieving the good hearing outcomes and PROMs that can be expected by 12 months after implantation in most cases. There was a significant association between the radiological severity of otosclerosis and an increase in surgical and postoperative complications. Postoperative facial nerve stimulation can occur and may require deactivation of electrodes and subsequent hearing detriment.ConclusionsHearing outcomes are typically good, but patients should be counseled on associated surgical complications that may compromise hearing. Modern diagnostic techniques may help to identify potentially difficult cases to aid operative planning and patient counseling. Further work is needed to characterize PROMs in this population.