A 7 yearold boy brought by his parents on complaint of hearing loss after a minor head impact.

1. What is the diagnosis?

2. What is the classic history?

3. How often is this finding bilateral?

4. This is associated with absence of the “? ” 90% of the time

Correct answer:

1. What is the diagnosis?

Wide Vestibular Aqueduct

2. What is the classic history?

Sensorineural hearing loss

3. How often is this finding bilateral?

 Usually

4. This is associated with absence of the “? ” 90% of the time

 Bony Modiolus

Large Vestibular Aqueduct Syndrom e (LVAS)

Possibly resulting from reflux of fluid from the endolymph sac into the inner ear, this is a very common cause of sensorineural hearing loss.


KEY FEATURES

- You aren't born deaf. It’s progressive.

- As a helpful rule, if the Vestibular Aqueduct is larger than the posterior semicircular canal (or facial nerve canal) then they have LVAS

- It is usually bilateral (50-90%)

- LVAS is often associated with other malformations - the most common of which is absence of the bony modiolus (seen in 90%).

Extra Credit: Mondini Abnormality

- Abnormal Cochlea (missing a turn)

- Enlarged Vestibule, but normal SCC

- Enlarged Vestibular Aquaduct

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