Large vestibular aqueduct syndrome

Case contributed by Fakhry Mahmoud Ebouda
Diagnosis certain

Presentation

Sensory neural hearing loss since birth.

Patient Data

Age: 4 years
Gender: Male
This study is a stack
Axial
non-contrast
This study is a stack
Axial
non-contrast
This study is a stack
Axial
non-contrast
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Info

Dilated vestibular ducts and sacs bilaterally. Clear middle ear clefts with normal ossicles shape and location as well normal morphology of the oval and round windows.

This study is a stack
Axial 3
D FIESTA
This study is a stack
Axial
T1
This study is a stack
Coronal
T2
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Info

Dilated vestibular ducts and sacs bilaterally however the vestibule and SCCs as well as cochlear turns with the modiolus show normal morphology and development. 

Case Discussion

Dilated vestibular ducts and sacs bilaterally causing congenital sensory neural hearing loss that resulting in large vestibular aqueduct syndrome.

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