A case of bilateral enlarged vestibular aqueducts and unilateral hearing loss at birth

Screening of newborn hearing is nowadays widespread, and as many as 3.4% of all screened infants are found to have unilateral hearing loss. However, we, as clinicians and parents, usually dismiss the severity of unilateral hearing loss in pediatric patients. Recently, we experienced the case of unilateral hearing loss since birth in a 2-year-old girl who was finally found to have bilateral enlarged vestibular aqueducts when the disorder progressed to bilateral hearing loss. Proper evaluation of the cause of hearing loss, including imaging studies of the inner ear, and consecutive hearing evaluations should be mandatory in children with unilateral sensorineural hearing loss, due to the possibility of progression of hearing loss bilaterally.