Auditory Neuropathy Spectrum Disorder in Children

Auditory neuropathy is a condition which causes hearing loss in people of all ages, but is most commonly diagnosed in children. The hearing impairment is of neurological origin, which simply means the nerve pathway form the ear to the brain is faulty.

In practical terms, this means the sufferer hears noises, but can’t make total sense of them: a bit like listening to a poorly tuned radio set. The child may hear speech but it may not make sense, or there is a time lag – like a miming singer with poor lip sync. 

In some cases, auditory neuropathy may have a genetic component, as the problem does seem to run in families. In other cases the child may have experienced health problems at or around birth, which had a negative impact on their hearing health. This might have been oxygen deprivation at birth, being of a low birth weight, having jaundice requiring blood transfusions, or being very premature.

If you are the parent of a child affected by this form of hearing loss, you are likely wondering if the condition gets better. This is a difficult question to answer as some babies diagnosed with auditory neuropathy at birth do show improvements by one or two years of age and learn to hear and speak.  Unfortunately, the opposite is also possible and some cases deteriorate.

The audiologist makes a diagnosis with a combination of two tests. The OAE (otoacoustic emissions) test checks out the mechanical parts of the ear, and the result is usually normal. The ABR (auditory brainstem response) looks at the brain’s response to sound, and in this case is highly abnormal. Neither test is painful or invasive, and it can be safely carried out on newborn babies.

At this point in time there is no definitive or effective treatment for auditory neuropathy. Some individuals may benefit from the use of a hearing aid or a cochlear implant (an electronic device which compensates for damage to the inner ear). Research is ongoing into the latter, especially for children. However, to our knowledge neither solution fully addresses the underlying issue, which is a nerve problem.

What seems to be key is working as a team – parents, child, and audiologist – to teach an affected child communication skills. This might be sign language, or different ways of learning listening techniques such as lip reading. One thing is for sure: everyone should talk and interact with the child, and make sure to face the child and smile regularly.

Of course there are many other causes of hearing loss in children. If you suspect your child doesn’t hear as well as they should, schedule an appointment with a pediatric audiologist who is skilled in testing and treating children. There are sophisticated, non-invasive tests which can pinpoint the exact nature of the problem and get your child back to their hearing best.