If your child responds to the sounds of the world around him, yet fails to respond to verbal communication, you may assume that he is deliberately tuning you out or practicing what parents affectionately refer to as selective hearing. While this is possible, there is another cause to consider. Your child may be suffering from auditory neuropathy. The hallmark of the rare condition is the inability to distinguish human speech despite normal or slightly impaired hearing. 

What Is Auditory Neuropathy?

Auditory neuropathy is a hearing disorder characterized by sounds entering the ear normally, but becoming scrambled or impaired during the transmission to the brain. It most commonly affects speech recognition. This means you little one can hear you, he just can't make sense of what he is hearing. Auditory neuropathy can affect people of all ages but is most often noticed with young children. Someone with this condition may have hearing impairments, but it can also affect those with 'normal' hearing.

How Do You Find Out If Your Child Has Auditory Neuropathy?

The first step is to visit your child's pediatrician to rule out other causes, such as ear infections or conditions that affect your child's ability to hear or communicate with you when you speak to him. If the pediatrician suspects auditory neuropathy, he will typically work with an audiologist and an ear nose and throat specialist to test for the disorder. Two common tests are used to diagnose this condition.

• Auditory Brainstem Response (ABR): This test is performed using electrodes to measure the brain wave activity in response to sound. Electrodes placed in the ear and on the head record your child's response to sounds. This test is painless.

• Otoacoustic Emissions (OAE): This test uses a small microphone inserted in the ear canal to record sounds produced by the hairs in the outer ear in response to outside sounds. This test tells your child's medical professionals whether the ear is functioning properly.

A normal OAE reading combined with an abnormal ABR indicates that your child's ear is functioning properly but that the signal gets confused when it is transmitted to the brain. This reading typically indicates your child has auditory neuropathy.

What Causes Auditory Neuropathy?

The cause of this disorder is not known at this time. However,  it does tend to run in families, suggesting a genetic link. Other risk factors include medications taken by pregnant women, and conditions like jaundice, low birth weight and premature birth in infants. In some cases, auditory neuropathy may be accompanied by other neurological disorders. More research is needed to determine the cause of this disorder.

How Is It Treated?

There are currently several approaches to treating a child with auditory neuropathy. Because the condition may get better, worse or remain the same over time, treatment must be personalized to the individual. While hearing aids, cochlear implants and listening devices are effective for some children with this condition, some do not experience a benefit.

• Sign Language: Some doctors support teaching children to communicate with sign language. This may be most effective with those who have not already developed spoken language.
• Speech Reading: Older children who have already developed spoken language may be best served by learning speech reading, also known as lip reading.
• Combination Approach: The combination approach includes the use of hearing devices and teaching listening skills or lip reading. 

Will Your Child Ever Get Better?

Some children with auditory neuropathy do improve on their own, but there are no guarantees. Treatment typically produces an improved ability to communicate and may help your child compensate for his difficulties with understanding spoken language. As he learns listening techniques and lip reading skills, your child may appear to no longer have auditory neuropathy.

If you suspect that your child is having difficulty with understanding spoken language, even though he responds to environmental sounds, talk with his health care professionals right away and go to sites to learn more about this condition. Early treatment is vital to his cognitive and emotional development.