The child received CT scans and has a preliminary diagnosis of enlarged vestibular aqueduct syndrome (also called large vestibular aqueduct syndrome), with hearing loss progression expected.

Here are the audiograms at age 3 years 10 months and 4 years 1 month.

• Comment on the air-bone gap.
• Is there a genetic component to EVAS?  Fully discuss.

Audiogram 3 years 10 months

Audiogram 4 years 1 month