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.