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

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.

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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