Focusing on the Commonalities of Auditory Neuropathy Spectrum Disorder (ANSD)

By: Jen Gow

In keeping with our trend of ‘specialized coffee nights’, we brought together parents of children with Auditory Neuropathy Spectrum Disorder (ANSD) last fall. The reason that ANSD is called a ‘spectrum’ disorder is because the effects of ANSD are variable and unique for each child.

Jenny Hatton, audiologist with the BC Early Hearing Program (BCEHP), was on-hand to answer the many technical questions that come with this specific type of hearing loss. In a new ANSD information handbook Jenny is creating for the BCEHP, ANSD is described as a type of hearing loss where sounds travel as they typically would through all parts of the ear (the outer, middle and inner ear) but there is a problem with sound transferring from the cochlea in the inner ear to the auditory (hearing) nerve. About 10% of children with sensorineural hearing loss have ANSD. What distinguishes ANSD from more common types of sensorineural hearing loss is that sound information reaching the brain isn’t organized in a way that the brain can readily understand. This causes speech sounds to be distorted. The degree of this distortion can vary greatly from child to child. Some children with ANSD will hear and understand speech in a way that is similar to those with the more common form of sensorineural hearing loss. Other children will experience greater distortion, with speech sounding like a radio that is out of tune. For a simulation of what it might sound like to have ANSD, click on the following link (simulation at the bottom of the page): http://hesp.ent.uci.edu/drupal/node/13

As unique as this type of hearing loss is for each child, the parents at our coffee night connected with one another more on the commonalities of their children rather than the differences in their stories. Parents shared a range of their children’s hearing levels from close to typical hearing to severe or profound levels. Some agreed that their children’s hearing seemed sporadic at times – where sometimes words were clear and other times they appeared to be all jumbled. Many found using sign language helpful, and some also connected with each other about additional medical needs their children have.

When your child has ANSD, it can feel like some of the resources you find about deaf/hard of hearing children don’t apply to your situation. This is one of the reasons a new ANSD information booklet is being created, with input from parents raising kids with ANSD, and other experts in the field. Until this booklet is ready…here are a few tips and some other resources:

Become an advocate for your child.
Have your child assessed and monitored regularly. Ask how to develop your own observation skills and how to document those observations. Show your child’s learning in creative ways such as videotaping.
Look at your “whole child” to see what other strengths and challenges they have.
Create a one-page handout about your child’s hearing that explains their ANSD in simplified terms, to give to their doctors or other professionals.
If possible find an Audiologist that is experienced with ANSD.
Connect with other parents and share your stories, rather than letting yourself feel isolated. This ANSD Yahoo Group can be a great resource for this: http://groups.yahoo.com/group/AuditoryNeuropathy/

Other Information Sources:

The BCEHP is creating a series of animations that describe the process of hearing and different types of hearing loss, including ANSD. Within these animations will be auditory simulations to give parents an idea of what speech may sound like with different types of hearing loss. Once available, these animations will be located here:

http://www.phsa.ca/our-services/programs-services/bc-early-hearing-program/understanding-hearing-loss/about-hearing-loss

Update: The ANSD booklet is now available at the following link: http://www.phsa.ca/bc-early-hearing/Documents/ANSD%20Information%20for%20BC%20Families.pdf