Auditory neuropathy spectrum disorder (1955)

Key points below

Diagram of a normal ear

What is ANSD?

ANSD is a hearing problem between the cochlea (organ of hearing) and the hearing nerve or with the hearing nerve itself

With ANSD, sound signals are not always sent to the brain. Sometimes the signals are sent but are not clear.

The amount of hearing problems for each person with ANSD is different. It can change over time.

Some people have little or no problem hearing.
Some people say they can hear but not understand what is said. This happens more if there is background noise.
Some people have changing hearing abilities. They say they have good-hearing days and bad-hearing days.
Some people with ANSD cannot hear at all.

About 1 out of 10 people with permanent hearing loss have ANSD.

How is ANSD diagnosed?

The audiologist tests different parts of the ear. They will:

Look in your child’s ears to make sure they are clear of wax.
Test your child’s eardrum to make sure the middle ear is healthy.
Test your child’s cochlea to see if it is working normally. This test is called otoacoustic emissions (OAE).
Test how well the ear picks up sounds and how well the auditory nerve sends sounds to the brain. This test is called an auditory brainstem response (ABR) evaluation. In children with ANSD, the ABR will be absent or not normal.

What can help a child with ANSD?

In children with ANSD, the ABR evaluation cannot tell us how the child hears. We will need to test your child’s hearing in the sound booth. This tells us the softest level of sound your child hears. This is called their hearing threshold. The first test can be done when a child is six months old. It may take several appointments to get this information. For some children, it takes a long time to get helpful test results.

Once we know how your child hears, we can suggest ways to help them.

For children who hear in the mild to moderately-severe hearing loss range, we recommend hearing aids.
For children who hear in the severe to profound hearing loss range, also recommend a trial with hearing aids. Because hearing aids sometimes do not help these children, we also make a referral to the cochlear implant team to see if that is a better option.
A child who wears a hearing aid may be able to get a cochlear implant, at some point. This happens if their speech is not getting better and the hearing aids aren’t helping. Ask your provider for more information about cochlear implants.

For children with ANSD in one ear, there may be other suggestions. First, hearing thresholds are checked. Then, the best options for your child will be discussed.

What is best way to communicate with child with ANSD?

Audiologists and speech-language pathologists will work together to see how your child is learning to hear and talk.

If your child responds to speech, we suggest you use speech.
Sometimes, sign language or speech and sign language together may help your child.

For more health and wellness information check out this resource:https://kidshealth.org/ChildrensWi/en/parents

ALERT

Call your child’s doctor, nurse, or clinic if you have any questions or concerns or if your child has special health care needs that were not covered by this information. This sheet was created to help you care for your child or family member. It does not take the place of medical care. Talk with your healthcare provider for diagnosis, treatment and follow-up.