ANSD results from the abnormal transmission of sound from the hair cells of the inner ear to the hearing center of the brain. Children who are born prematurely or with hypoxia (low oxygen) or elevated bilirubin at birth are at greater risk of this condition. A family history of the disorder can also play a role.
ANSD occurs in approximately 10% of people with profound hearing loss, and is being diagnosed more frequently in children due to newborn neonatal screening. A consequence of this disorder is poor word understanding relative to the level of hearing sound.
There is no cure for ANSD. Early diagnosis and treatment, however, can result in children developing strong language and communication skills with the help of assistive listening devices such as hearing aids and cochlear implants.
