Audiometric findings in children with unilateral enlarged vestibular aqueduct

To evaluate the prevalence of bilateral hearing loss in children with unilateral enlarged vestibular aqueduct (EVA) at a single institution.

A retrospective case review was performed at a tertiary care pediatric referral center involving children with radiologic findings of unilateral EVA and normal labyrinthine anatomy of the contralateral ear diagnosed via CT and/or MRI. The main outcome measure of interest is the number of patients with unilateral EVA who were diagnosed with bilateral hearing loss.

Sixty-one pediatric patients were identified. The mean audiometric follow-up was 48.2 months (0-150). Three (4.9%) patients with unilateral EVA were noted to have bilateral hearing loss, and this rate was not significantly different (p = 1.0) from the rate reported in a comparison group of patients with contralateral hearing loss (6.0%) without an EVA. The pure-tone average (defined as the average dB HL at 500, 1000, 2000, and 4000 Hz) in the group with bilateral hearing loss was 31.3 dB HL in the better hearing ear and 79.6 dB HL in the worse hearing ear, with the difference being statistically significant (p = 0.02). In the unilateral EVA patients without contralateral hearing loss (n = 56, 91.8%), the PTA was 9.4 dB HL in the better hearing ear and 51.9 dB HL in the worse hearing ear, with the difference being statistically significant (p < 0.001). Two patients (3.3%) with unilateral EVA were found to have hearing within normal limits bilaterally. The EVA was ipsilateral to the worse hearing ear in all cases.

The prevalence of bilateral hearing loss in children with unilateral EVA appears to be low. Specifically, it may be no different than the rate of contralateral hearing loss in children with unilateral hearing loss without an EVA. The present report is somewhat different than the previously described prevalence in the literature. This difference could be related to the imaging type and diagnostic criteria used, the patients included, the source of the identified patents, and the overall population of patients studied.