[citation needed] Although large vestibular aqueducts are a congenital condition, hearing loss may not be present from birth.

Age of diagnosis ranges from infancy to adulthood, and symptoms include fluctuating and sometimes progressive sensorineural hearing loss and disequilibrium.

It is possible that a small portion of those with large vestibular aqueducts will not exhibit auditory symptoms such as hearing loss.

Enlarged vestibular aqueducts are caused by mutations in gene SLC26A4, which is also associated with Pendred syndrome and non-syndromic recessive deafness.

Enlarged vestibular aqueducts often occur with other inner ear development problems, such as cochlear deformities.

During an audiologic assessment, an air-bone gap in the low frequencies can occur due to the enlarged vestibular aqueduct acting as a third mobile window.

Hearing loss in the contralateral ear to the enlarged vestibular aqueduct can also occur due to the chemical composition on both sides.

[6] Identification of the enlarged vestibular aqueduct in a child is usually by MRI scan which identifies the fluid within the endolymphatic duct and sac.