Enlarged vestibular aqueduct syndrome mimicking otosclerosis in adults

IntroductionAn enlarged vestibular aqueduct (EVA) results in hearing loss which is often progressive and heterogenous, the long-term natural history of which is not well understood. Patients born before the era of newborn hearing screening can present as adults with previously undiagnosed EVA.MethodsA retrospective chart review of patients seen at a tertiary adult academic otologic clinic from 2004 to 2012 were reviewed and cases of EVA were identified.ResultsAdult EVA was found to present with a mixed progressive hearing loss with absent stapedial reflexes.ConclusionEnlarged vestibular aqueduct abnormality can present in adulthood and mimic other more common pathology such as otosclerosis.