Although the textbook view of Pendred syndrome is that of an autosomal recessive condition characterized by deafness and goitre, it is increasingly clear that not all such patients present this classical clinical picture. Malformations of the inner ear, specifically enlargement of the vestibular aqueduct, are common in Pendred syndrome and mutations in the PDS (Pendred Syndrome) gene have been ...

OBJECTIVE To evaluate the vestibular aqueduct in patients with sudden sensorineural hearing loss. METHODS We evaluated 19 patients (12 men and seven women; age range, 22-79 years) with unilateral sudden sensorineural hearing loss, using computed tomography and magnetic resonance imaging. All these patients had unilateral sudden sensorineural hearing loss. We also evaluated 47 control subjects...

OBJECTIVE To further define the spectrum of clinical presentation and explore the histologic sequelae of jugular bulb abnormalities (JBAs). DESIGN Retrospective review. SETTING Academic medical center. PATIENTS Thirty patients with radiologic evidence of inner ear dehiscence by JBA. MAIN OUTCOME MEASURE Thirty patients with radiologic inner ear dehiscence by JBA and 1579 temporal bone s...

The large vestibular aqueduct (LVA) syndrome is a congenital malformation that predisposes the patient ultimately to a loss of hearing and possible continuing vestibular disorder. If the LVA patient is diagnosed, it typically is not until later life, when he/she exhibits profound sensorineural hearing loss. To better understand this disorder and to bring it to the attention of audiologists, we ...

A high-resolution three-dimensional Fourier transform technique and prototype bilateral dual phased-array surface coil technique was used to make inner ear structures visible on MR. Multiplanar reformatted images, parallel to the plane of the vestibular aqueduct, allowed viewing of the entire endolymphatic sac/vestibular aqueduct on one section, producing a "Christmas tree" shape. The reformati...

Introduction Large vestibular aqueduct syndrome (LVAS) is characterized by the enlargement of the vestibular aqueduct associated with sensorineural hearing loss. It is the most common radiographically detectable inner ear anomaly in congenital hearing loss. LVAS may occur as an isolated anomaly or in association with other inner ear malformations. Objective To report three cases of isolated LVA...

Enlarged vestibular aqueduct (EVA) is one of the commonly identified congenital temporal bone abnormalities associated with sensorineural hearing loss. Hearing loss may be unilateral or bilateral, and typically presents at birth or in early childhood. Vestibular symptoms have been reported in up to 50% of affected individuals, and may be delayed in onset until adulthood. The details of nystagmu...

The Enlarged Vestibular Aqueduct Syndrome is characterized by a widening of the vestibular aqueduct, associated with sensorineural hearing loss, or sometimes with mixed hearing loss, which may be congenital or acquired during childhood. The sensorineural hearing loss may be classified into mild, moderate and severe, associated with sudden periods of improvement or aggravation. The enlargement o...