Large vestibular aqueduct syndrome

Case contributed by Dr Yi-Jin Kuok

Presentation

Mixed conductive/sensorineural hearing loss, since early childhood

Patient Data

Age: 19
Gender: Female
Modality: CT

Dilated vestibular aqueducts

Cochlear are slightly globular (left worse than right) raising the possibility of cochlear dysplasia (modiolus present though)

Horizontal semicircular canal is slightly dilated. the horizontal semicircular canal is the last canal form and often the first to go, in terms of development

Incidental likely calcified aneuyrsms - based on this finding, an MRI was performed

Modality: MRI

TOF MRA shows multiple aneurysms. right M1. right MCA bifurcation. right ICA paraclinoid. left PCOM. there is no known association between LVAS and berry aneurysms.

Tortuous clump of vessels affecting the right PCA mimics and aneurysm.

Vessels are tortuous. tortuous vessels are seen in connective tissue disease (erlers danhos), fibromuscular dysplasia. there is no known association between LVAS and a connective tissue vasculopathy.

Large vestibular aqueduct syndrome. Note how the endolymphatic sac lies outside the dura on the steady state sequences.

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Case Information

rID: 18876
Case created: 26th Jul 2012
Last edited: 6th Dec 2016
Inclusion in quiz mode: Included

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