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A Review of 12 Cases and the Description of a New Clinical Entity

Mark J. Levenson, MD; Simon C. Parisier, MD; Morton Jacobs, MD; David R. Edelstein, MD

Arch Otolaryngol Head Neck Surg. 1989;115(1):54-58.

Abstract
• The large vestibular aqueduct as an isolated anomaly of the temporal bone has been previously identified radiologically, and its association with sensory neural hearing loss has been recognized. It has not, however, been defined as a distinct clinical entity in children. We studied 12 children, ages 3 to 9 years, with downward-fluctuating progressive high-frequency neurosensory hearing losses whose symptoms were thought to be related to the isolated enlargement of the vestibular aqueduct identified by highresolution computed tomographic scanning. Previously it had been assumed that a large vestibular aqueduct is a temporal bone dysplasia that is a variant of the Mondini type of deformity and that the associated hearing loss is congenital in nature. Our clinical observations, however, indicate that the hearing loss in children with an isolated enlargement of the vestibular aqueduct is acquired during childhood. The natural history of this progressive deafness is reviewed, and a pathophysiologic hypothesis is presented.

(Arch Otolaryngol Head Neck Surg 1989;115:54-58)

Author Affiliations
From the Departments of Otolaryngology Head and Neck Surgery (Drs Levenson, Parisier, and Edelstein) and Radiology (Dr Jacobs), Manhattan Eye, Ear & Throat Hospital, New York.

Footnotes
Accepted for publication Sept 21, 1988.

Read before the annual meeting of the Triologic Society, Denver, April 28, 1987.

Reprint requests to Department of Otolaryngology Head and Neck Surgery, Manhattan Eye, Ear & Throat Hospital, 210 E 64th St, New York, NY 10021 (Dr Levenson).

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