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Ellis M. Arjmand, MD, PhD; Audra Webber, BS

Arch Otolaryngol Head Neck Surg. 2004;130:1169-1174.

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 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.

From the Department of Pediatric Otolaryngology, Children's Hospital of Pittsburgh, Pittsburgh, Pa. Dr Arjmand is now with the Department of Pediatric Otolaryngology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. The authors have no relevant financial interest in this article.

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