Enlarged vestibular aqueduct concomitant with endolymphatic hydrops.

BACKGROUND

Enlarged vestibular aqueduct (EVA) is one of the most common inner ear anomalies associated with sensorineural hearing loss (SNHL) in childhood.

OBJECTIVE

This study aimed to investigate the prevalence of EH in patients with large vestibular aqueduct syndrome (LVAS).

METHODS

A total of 20 patients with congenital SNHL underwent an inner ear test battery aligned with Hydrops MRI. Among them, 7 patients (unilateral involvement in 2 and bilateral involvement in 5, totaling 12 ears) with LVAS were enrolled.

RESULTS

Abnormality rates across the inner ear test battery showed a significantly decreasing order, moving from audiometry (100%), cervical vestibular-evoked myogenic potential (VEMP) test (92%), caloric test (67%), to the ocular VEMP test (58%), which did not parallel with the declining trend of abnormality rate in Meniere's disease. Hydrops MRI demonstrated EH in 4 (57%) of 7 LVAS patients, comprising 5 ears in the cochlea and 4 ears in the vestibule. All patients received supportive treatment except one patient who underwent sac and shunt surgery, which resulted in profound deafness and recurrent postoperative vertigo.

CONCLUSION

Hydrops MRI enables the simultaneous identification of EVA and EH. In this study, 57% of LVAS patients were found to have concomitant EH.