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.