CT and MRI Evaluation of Bilateral Enlarged Vestibular Aqueduct Syndrome with Curved Reconstructions – A Case Report and Short Review of Literature

Chithra Ram,Richard Sherry,Erik Seroogy

doi:10.31579/2690-8808/079

Abstract

Enlarged Vestibular Aqueduct Syndrome (EVAS) is a known, but rare entity. It represents a common cause of congenital sensorineural hearing loss, diagnosed more often in children with a slight female predominance. Herein, we report a case of bilateral Enlarged Vestibular Aqueduct Syndrome [EVAS] in a 26-year-old male. As it is a subtle finding on imaging, the interpreting radiologist must be aware of this entity to make the diagnosis. The diagnostic CT and MRI images of this patient are given along with curved CT and MRI reconstructions along the plane of the Vestibular Aqueduct [VA] extending to the endolymphatic sac [ES]. The CT reconstructions demonstrate the bony anatomy in great detail and the bilateral dilated VA. These help in excluding diseases like otospongiosis and inner ear anomalies. The curved MRI reconstruction CISS images through the VA demonstrate the entire endolymph channel. The reconstructed MRI Images clearly demonstrate a patent endolymph channel without any focal abnormality such as stricture, or other associated congenital anomalies.

Highlights

Enlarged Vestibular Aqueduct Syndrome (EVAS) is a known but rare entity
The degree of aqueduct enlargement has not been correlated with the rate of attenuation of hearing in bilateral enlarged vestibular aqueduct syndrome (BEVAS)
BEVAS is associated with mutations of SLC26A4, and these mutations correlate with the rate of hearing attenuation

Summary

Introduction

Enlarged Vestibular Aqueduct Syndrome (EVAS) is a known but rare entity. It is an important cause of sensorineural hearing loss. The degree of aqueduct enlargement has not been correlated with the rate of attenuation of hearing in bilateral enlarged vestibular aqueduct syndrome (BEVAS). BEVAS is associated with mutations of SLC26A4, and these mutations correlate with the rate of hearing attenuation. Unilateral EVAS is not associated with SLC26A4, nor with any genetic syndromes. This condition can be congenital or acquired but is more commonly congenital. 26-year-old male was referred to the ENT clinic by his primary care physician for progressively worsening bilateral severe hearing loss. Patient was scheduled to undergo cochlear implantation

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Conclusion