Auditory Neuropathy Spectrum Disorder (ANSD)

Many children with hearing loss have a problem with the cochlea (the snail-shaped, fluid-filled chamber of the inner ear), but we’ve found that many children have another condition called auditory neuropathy disorder spectrum (ANSD).

ANSD is a broad term that describes any of a variety of problems involving “misfirings” of the auditory nerve, or problems affecting the nerve’s connection with the cochlea. The result of ANSD is the disordered transmission of sound from the inner ear that makes sound disorganized when it reaches the brain.

Children with ANSD benefit from specialized testing that is often different than what is offered in a newborn hearing screening. In fact, it is because many newborns with the disorder were going undiagnosed that we developed a unique program to test for and manage ANSD.

Nemours’ ANSD program consists of many different types of testing, including an Auditory Brain Response (ABR) test. This test, performed when your child is either asleep or sedated, measures whether the auditory nerve transmits sound from the inner ear to the brain and how loud sounds have to be for the brain to detect them.

Knowing that ANSD is the cause of your child’s hearing loss allows us to approach the problem from the correct perspective. As with other types of hearing loss, a team approach is always best.

The ANSD team consists of a group of professionals including a neuroscientist (a scientist who studies brain and nerve function) 

• pediatric audiologists
• an educator of the deaf
• research staff
• pediatric otologist (an ENT doctor who specializes in abnormalities of the ear)
• speech and language specialists
• consultants from various other departments including neurology, ophthalmology, and genetics 

This team not only manages your child’s treatment plan, but is also actively involved in educating the community regarding the diagnosis and management of children with ANSD. Our goal is to use our specialized knowledge about ANSD to create a protocol for treatment that we can share with other facilities throughout the country.