Natural History of Hearing Loss in Children with Enlarged Vestibular Aqueduct Syndrome

Objective: To determine the natural history of hearing loss in children with enlarged vestibular aqueduct (EVA) syndrome. Design: (1) Retrospective cohort study and (2) systematic literature review. Setting: Tertiary pediatric centre. Methods: (1) Charts of children assessed by one physician between 1993 and 2000 were reviewed. (2) Source articles were identified by a search of Medline, Embase, and the Cochrane Library of the English-language literature through January 2006, with manual review of references. The search was limited to English, human, and age less than 18 years. Main Outcome Measures: Pure-tone average. Hearing was classified as stable, progressive and fluctuating. Results: (1) Twenty-one children (39 ears) with EVA were identified. Eighty-two percent of ears had stable hearing, and 18% of ears demonstrated progressive hearing loss. (2) Seven source articles were identified and combined with the present data for a total of 310 ears with a mean follow-up of 4 years. Bilateral EVA was found to be six times more common than unilateral EVA, and there was an equal male to female ratio. Stable hearing was found in 67% of ears and progressive hearing loss in 33% of ears. Subgroup analysis demonstrated hearing fluctuations in 50% of progressive hearing loss ears and 34% of stable ears. Conclusions: Stable hearing is observed in 67% of ears with EVA of which 34% will demonstrate fluctuations in hearing. Progression of hearing loss is seen in 33% of ears of which half will demonstrate fluctuations.