Auditory Neuropathy

Auditory Neuropathy Spectrum Disorder (ANSD) is a hearing problem in which the ear detects sound normally, but has a problem sending it to the brain. It can affect people of all ages.

Someone with ANSD may have trouble telling one sound from another. They may also have trouble understanding speech clearly. Sounds may seem to fade in and out or are ‘out of sync’. Children with ANSD may have hearing that varies or gets worse over time. They may also appear to hear one day and not hear the next. This can be very frustrating for parents and make a definite diagnosis difficult.

The amount of hearing loss associated with ANSD can vary greatly.

Common Causes

ANSD likely has more than one cause.  Children can inherit it from their parents, or it may be a result of trauma or disease.  Some of the common causes of ANSD include:

• Lack of oxygen at birth
• Severe jaundice during the newborn period 
• Infectious diseases such as mumps
• Immune disorders
• Neurological disorders such as Charcot-Marie-Tooth syndrome or Friedreich’s ataxia

Diagnosis

Audiologists use several tests to diagnose ANSD. These tests include auditory brainstem response (ABR), acoustic reflex testing and otoacoustic emissions (OAE). These tests are painless and are used in both newborns and older children.

Depending upon what test is used for newborn hearing screening, some children with ANSD may pass the first hearing screening in the hospital. This may delay the identification of ANSD. Parents and doctors may not suspect hearing loss right away if an infant’s response to sound changes from day to day.

As children get older, more information about their hearing becomes available. Ongoing testing may show a poor understanding of speech and/or problems with hearing in noise.

Some newborn babies who have been diagnosed with ANSD improve over time. They may start to hear and speak within a year or two. Other infants stay the same or even get worse. At this time, no tests can tell us if a child’s hearing will change.

How Will You Know?

You may notice your child having any of these issues:

• Hearing that appears to change daily or even hourly
• Inconsistent responses to speech
• Trouble understanding speech, especially in noisy places
• Acting as though they have a hearing loss

Symptoms can develop at any age. However, most children with ANSD are diagnosed in the first months of life.

Treatment

Currently, there is no medical treatment for ANSD. Some children benefit from hearing aids. However, some children get limited or no hearing aid benefit. It is important to see a pediatric audiologist when looking at treatment options. It is especially important if hearing aids are recommended.

Some professionals recommend the use of remote microphone systems. Remote microphone systems can improve listening in noisy backgrounds.

Some children benefit more from a cochlear implant than from hearing aids. However, there are no current tests that can determine if a child with ANSD might benefit more from a hearing aid or cochlear implant.

What Can You Do to Help Your Child?

There is no single approach that fits all children with ANSD. It is helpful to work closely with a team of professionals. These may include a pediatric audiologist, neurologist, speech pathologist, otologist, physical therapist, occupational therapist, early intervention specialists, and teachers. A team can help parents observe their child and determine what techniques bring about the best responses.

Some children benefit from teaching that focuses only on learning to listen and speak.

Some children have great difficulty understanding what is heard. That child will benefit more from visual communication such as sign language or cued speech.

It is difficult to predict how any one child will do. It is important to be flexible. Teachers and other professionals can help families decide if therapy is working. Communication and understanding should improve over time if therapy is working.