In this systematic review, the authors describe the complications and outcomes of cochlear implantation in patients with enlarged vestibular aqueduct (EVA). The authors reviewed five major databases and included 4035 patients from 34 studies. Of these, 853 patients (21.14%) had EVA. Perilymph gusher was documented in 112 patients, CSF oozing in 18 patients and partial electrode insertion in six patients. In 56.7% patients, the cochleostomy was closed using temporalis fascia, muscle, connective tissue or fibrin glue. Facial nerve injury was observed in 4.2% patients. Immediate postop complications included transient imbalance, protracted vomiting, intermittent pulsing sensation, and dizziness. In one patient, postoperative infection progressed to cholesteatoma. Although audiological outcomes improved after CI in all studies, the outcomes could not be compared as different audiological evaluation tests were used in the different studies. The authors conclude that CI in patients with EVA is associated with improved audiometric and speech outcomes. Standardised tests to measure cochlear implant outcomes may in future help us to understand the true outcomes in this patient population.