Audiometric Findings in Children With a Large Vestibular Aqueduct

Abstract

To characterize audiometric findings in children with a large vestibular aqueduct (LVA).Design: Retrospective review.Methods: Audiometric records of children with an isolated LVA, diagnosed by computed tomography of the temporal bone, from 1995 through 1998 were reviewed.Results: Nineteen children had an isolated LVA in one or both ears. In all, 26 ears with an isolated LVA were identified. An LVA was seen in association with another inner ear anomaly in an additional 7 ears. The hearing impairment was sensorineural in 22 ears (85%) with an isolated LVA and mixed in 3 (12%). The hearing was normal in 1 ear. The sensorineural hearing impairment (SNHI) was moderate-severe in 12 ears (46%) and severe-profound in 10 ears (38%). Thirteen (50%) of 26 ears had a downsloping or high-frequency SNHI, and 8 (31%) of 26 ears had a midfrequency-peaked audiogram. Bilateral LVAs were seen in 7 children, 6 of whom had bilateral and asymmetrical SNHI. Of 12 patients with a unilateral LVA, 5 had bilateral SNHI. Conclusions:In this series, the children with LVAs typically had moderate-severe or worse SNHI. An unusual midfrequency-peaked audiogram was present in approximately one third of the study patients. The majority of the patients had a unilateral LVA; however, nearly 50% of the patients with a unilateral LVA had bilateral SNHI. The patients with bilateral LVAs generally had asymmetrical SNHI.

Enlarged Vestibular Aqueduct in Congenital Non-Syndromic Sensorineural Hearing Loss in Egypt

“…There was no significant difference between VA operculum or midpoint and the PTA configuration in the three groups. Similar findings were reported in the literature [1,8,12,19,22,29].…”

“…This is a relatively high incidence when compared with that reported in the literature [3][4][5][6][7][8]12] and might be accepted in a pilot study with a limited number of patients. Developmental growth of the VA throughout infancy and early childhood has been described, indicating that EVA may result from abnormal postnatal and pre-adolescent development and reaches the adult size at 3-4 years [13,14].…”

“…The EVA syndrome has been reported to be bilateral in 55 to 94% of cases [20,[23][24][25] and is generally reported to be asymmetrical [8,19]. Some authors claimed slight female preponderance [23,24] whereas others reported the inverse [25].…”

“…These anomalies are presented either isolated or in combination. Previous studies reported an incidence of isolated EVA ranging from 3.7 to 11.4% [3][4][5][6][7][8].…”

Enlarged Vestibular Aqueduct in Pediatric SNHL

“…[12][13][14] The significant correlations between measurements taken on the right and left sides of the same patient suggest that EVA may not be as morphologically asymmetric as previously thought. While there is a significant correlation between the midpoint and operculum measurements, it is not perfect.…”