Large vestibular aqueduct syndrome

Case contributed by Dr Sherif Shebl Kotkata

Presentation

Bilateral sensorineural hearing loss.

Patient Data

Age: Child
Gender: Female

The vestibular aqueducts are bilaterally dilated (more than 3.5 mm in its portion), giving a conical shape appearance. 

MRI axial 3D T2 for the petrous bone revealed dilated vestibular aqueduct and sac bilaterally.

Case Discussion

Large vestibular aqueduct syndrome is the presence of congenital sensorineural hearing loss (SNHL) in combination with an enlarged vestibular aqueduct. It is thought to be one of the most common congenital causes of SNHL. 

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