Large vestibular aqueduct (LVA) is a genetically mediated inner ear developmental anomaly. It usually presents in childhood with hearing loss, typically starting on one side. Some patients also experience disequilibrium. The clinical course is quite variable, though the hearing loss tends to be progressive, and there are often abrupt, “stepwise” deteriorations in hearing. Workup may include hearing tests, vestibular tests, imaging studies, and genetic testing. The hearing loss is sometimes treated with cochlear implantation. Patients with LVA are usually managed by an otolaryngologist.