Enlarged vestibular aqueduct (EVA) is a congenital condition that can lead to various outcomes in pediatric patients including hearing loss and vestibular dysfunction. Our goal was to critically appraise the literature on the proportion of patients with EVA who report vestibular dysfunction, determine relevant risk factors for the development of these symptoms, and describe vestibular tests and interventions used to improve outcomes. A systematic review was performed in accordance with the PRISMA guidelines. We queried the EMBASE, Ovid Medline, and Cochrane Library databases for relevant literature. Studies were included if they had n > 10, reported vestibular symptoms or vestibular function testing in patients with EVA, and were published in English. Nonhuman studies, systematic reviews, and review articles were excluded. Of 808 identified studies, 20 met inclusion criteria. Subjective vestibular symptoms included dizziness, episodic vertigo, and imbalance. Seventeen studies reported subjective vestibular symptoms, ranging from 2% to 71% of patients between studies. Seventeen studies performed some form of vestibular function test, including physical exam maneuvers (Dix-Hallpike), caloric testing, electronystagmography, and vestibular evoked myogenic potentials. Of those who had vestibular function testing, 7% to 92% had an abnormal result. Two studies identified head trauma as a risk factor. One study successfully treated patients with BPPV using the Epley maneuver, but other vestibular symptoms were not targeted with treatment. The degree to which vestibular symptoms impact patients with EVA varies significantly. Performing vestibular function testing may help identify asymptomatic patients with vestibular dysfunction. Future studies should target improving treatment of vestibular symptoms in EVA patients. Laryngoscope, 132:873-880, 2022.