Cochlear abnormalities associated with enlarged vestibular aqueduct anomaly

Abstract 

Objectives

The objective of this study is to examine the correlation between enlarged vestibular aqueduct (EVA) anomaly and other inner ear anomalies such as cochlear dysplasia, vestibulocochlear dysplasia and modiolar hypoplasia.

Methods

Retrospective chart review, with institutional review board approval, of patients with EVA who received treatment at Primary Children's Medical Center or University Hospital at the University of Utah, between 1997 and 2006. Review of radiographs was done to evaluate for the presence of EVA and other inner ear anomalies.

Results

Twenty patients (40 ears) were included in the study, 17 patients had bilateral EVA and three patients had unilateral EVA. There were 10 females and 10 males. The average age of all patients at the time of initial diagnosis was 1.7 years (0–6 years). Thirty-seven ears were shown to have EVA (92.5%). Of those ears with EVA, 29 (78.4%) had one or more inner ear anomalies. Twenty-three (62.2%) ears had cochlear dysplasia, six (16.2%) had vestibulocochlear dysplasia, and 18 (48.7%) had modiolar hypoplasia.

Conclusions

Using small field of view, thin section CT and/or MR imaging, cochleovestibular abnormalities are commonly identified in association with EVA.

Keywords: Enlarged vestibular aqueduct, Large vestibular aqueduct, Large endolymphatic sac and duct anomaly, Cochlear dysplasia, Vestibulocochlear dysplasia, Modiolar hypoplasia