Your child's hearing
Auditory Neuropathy Spectrum Disorder
What is auditory neuropathy spectrum disorder (ANSD)Auditory neuropathy spectrum disorder (sometimes called auditory neuropathy or auditory dys-synchrony) are terms used to describe a particular type of hearing problem when sounds may be transmitted normally through the middle ear and the cochlea but then do not transmit normally from the cochlea, or along the auditory nerve. This means that the child may be hearing something, but this is probably very distorted.
What does it mean if the tests show the cochlea’s hair cells are working normally but the auditory brainstem response is not normal?
This means that although the ear is picking up sound correctly, the hair cells within the cochlea or the hearing nerve itself may not be passing the sound on to the brain correctly.
Usually the auditory brainstem response will closely match the levels at which a child responds to sound. However, when a child has auditory neuropathy spectrum disorder, the auditory brainstem response will not match the level at which the child responds to sounds. Very often, there will be no auditory brainstem response even though the child does respond to some sounds.
The auditory brainstem response test measures the function of the auditory nerve. In some babies, particularly those born prematurely, the auditory nerve may not be fully developed and the results of the auditory brainstem response test will be poor because of this delay in development. For this reason, very young babies will have their tests repeated when their hearing system has had time to develop.
back to top
The hearing loss measured (by hearing responses to sound) with this type of hearing problem varies greatly from child to child, from normal to profound hearing loss. For some children with this condition, their hearing problem will seem to change from time to time or their hearing will be better on some days than others. Sometimes the hearing loss can be progressive (get worse with time), and in some children their hearing has improved with time.
These unusual test results are creating new challenges for families and professionals working with these children. A diagnosis of auditory neuropathy spectrum disorder warns professionals that a child may not act or respond as a typical deaf child. The child will need to be monitored closely as they grow up.
back to top
back to top
Background noise is a common problem for children with any hearing problem, including auditory neuropathy spectrum disorder. It is important to try to improve your child’s listening environment in order to help them make the most of the speech that they hear. You can do this by using soft furnishings, as well as wall and floor coverings. (Sound will bounce off hard surfaces, creating an echo effect, and soft surfaces reduce this.) Try to reduce background noise as much as possible (for example, turning off the television or radio) when talking together. Toddlers and children at school often benefit from using a personal FM system. The teacher or parent wears a microphone and the child picks up the voice directly through the hearing aids.
Soundfield systems, where the teacher’s voice is fed through a loudspeaker, may also be helpful in the classroom. These systems help to raise the speaker’s voice to a comfortable listening level above the background noise.
It is possible that some children with auditory neuropathy, even those wearing good-quality hearing aids, cannot make enough use of speech sounds to develop speech. Children with this condition are likely to benefit from learning a manual system of communication (for example, a sign language) especially in the early years. This helps to ease the frustration of not being able to communicate when they are very young.
My child has had hearing aids for some time and I think he may have this condition
The tests described above are now routinely used for testing newborn babies. The results of the otoacoustic emissions and the auditory brainstem response tests, and other tests, need to be interpreted together in order to get an accurate diagnosis. In the past babies were not necessarily tested in this way and may only have had one or the other, or a hearing test where the health visitor records your child’s responses to sounds behind them. In children with this condition, their response to the otocoustic emissions test may naturally reduce or disappear altogether with time. For this reason, the way in which auditory neuropathy spectrum disorder is diagnosed in an older child may differ, but do talk to your consultant or audiology department for further advice.
back to top
You can also get further information from the following websites
Information provided by National Deaf Children's Society
Reproduced with permission.
Date reviewed: 19.5.2011
Disclaimer: This website is for general information only and is not intended as a substitute for independent professional advice.
Note: We use the term ‘deaf’ to mean all types of deafness, including temporary deafness such as glue ear.