Audiometric findings in children with a large vestibular aqueduct

Objective: 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 cars. 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 L A had bilateral SNHI. The patients with bilateral LVAs generally had asymmetrical SNHI.