Large Vestibular Aqueduct Syndrome (LVAS) is the most common radiographic malformation in children with early onset of hearing loss. Usually its occurrence is non-familial, however intriguingly a portion of patients with LVAS is found to have evidence of genetic predisposition. We described cases of LVAS in two siblings of a Chinese family. The elder sister first presented with reduced hearing ...

The inner ear is composed of the membranous labyrinth and the osseous labyrinth (1). The membranous labyrinth has two major subdivisions, a sensory portion called the sensory labyrinth and a nonsensory portion designated the nonsensory labyrinth. The sensory labyrinth lies within the petrous portion of the temporal bone. It contains two intercommunicating portions: 1) the cochlear labyrinth tha...

We report a case of large vestibular aqueduct syndrome with a markedly dilated endolymphatic sac bilaterally. The density and signal intensity of the extraosseous portion of the sac were higher than those of cerebrospinal fluid on CT and MR studies. The findings may represent protein-rich and hyperosmolar fluid within the endolymphatic sac.

INTRODUCTION In the present case we report on the mismanagement of a patient misdiagnosed with otosclerosis, who was subsequently found to have enlarged vestibular aqueduct syndrome bilaterally. This highlights the need to not only be vigilant in pre-operative assessment of otosclerosis but also in post-operative investigations of stapedectomy failures. CASE PRESENTATION Our patient, a 56-yea...

UNLABELLED Children with LVAS can develop a severe sensorineural hearing loss early in childhood, but they can be rehabilitated with hearing aids to continue their regular studies and to have a normal life. The problem is that they can deteriorate their hearing capacity, and at this point a cochlear implant can be used to preserve their hearing skills and vocalization. AIM to evaluate the hea...

A controversy exists concerning whether or not roentgenographic narrowing or nonvisualization of the vestibular aqueduct is a specific sign for Meniere's disease. Of 190 ears that were evaluated, abnormal aqueducts were seen in 42.9% of ears with Meniere's disease, 45.4% of contralateral, noninvolved ears from patients with Meniere's disease, 41.3% of ears with diseases other than Meniere's dis...

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...

Enlarged vestibular aqueduct (EVA) syndrome is a common congenital inner ear malformation characterized by a vestibular aqueduct with a diameter larger than 1.5 mm, mixed or sensorineural hearing loss that ranges from mild to profound, and vestibular disorders that may be present with a range from mild imbalance to episodic objective vertigo. In our study, we present the case of a patient with ...