Enlarged vestibular aqueduct syndrome: A case of bilateral, sudden sensorineural hearing loss in a child☆

Summary 

Enlarged vestibular aqueduct syndrome (EVAS) is defined as progressive, fluctuating sensorineural hearing loss with radiologic evidence of large vestibular aqueducts. Since its first description multiple articles have illustrated the epidemiology, proposed mechanisms of altered inner ear development, and its association with sensorineural hearing loss. We describe for the first time in the English literature a child who experienced sudden onset of bilateral, sensorineural hearing loss after minor head trauma. There is no medical or surgical treatment for this condition, but amplification with hearing aids and cochlear implantation are viable options for patients.

Keywords: Large vestibular aqueduct syndrome, Enlarged vestibular aqueduct syndrome, Sudden hearing loss, Sensorineural hearing loss