Hearing Moments: Life with Auditory Neuropathy

By Sue Thompson

My son Matthew, now 7 years old, was diagnosed with Auditory Neuropathy at the age of 2 ½.  We suspected something was up, because he stopped singing songs, he often looked "lost" so many times, and he was responding less.  However, many times he seemed to be hearing fine.  I took him to a preschool assessment and he flunked the hearing screening.  I then took him to the school audiologist.  I really was not sure what to expect.  Matthew did not respond to 80 decibels (dB) in the booth.  The audiologist ended up pounding on the window to get his attention.  After testing him, she explained to me that he had auditory neuropathy.  I thought, "Hey, I have a profound hearing loss, I know everything there is to know about deafness."

However, I had much to learn about Matthew and his unique hearing loss.  His experience has been different from mine growing up as a deaf child.  Matthew had an ABR done and according to that audiologist, he had a severe hearing loss.  The moment I suspected he had a hearing loss, I started total communication with him.  His face lit up once I started signing.  So I signed 24/7!  If he didn't understand something, I explained everything from the way the sky looked to what lightning was . . .  He started going to preschool in a hard of hearing program. 

We started with low gain hearing aids for awhile.  He liked wearing them for a short time.  In first grade, he complained that things sounded "funny."  So we switched to a FM system.  He seems to like that better.  My husband and I did not consider the implant because we felt with AN, there were more questions than answers.  Auditory Neuropathy is so different from your typical hearing loss.  It is defined as a hearing disorder in which sound enters the inner ear normally but the transmission of signals from the inner ear to the brain is impaired.  There is debate between professionals on the appropriate treatment for AN.  Some favor hearing aids, others recommend the implant.  Some professionals now prefer using a new term, auditory dysynchrony to describe this hearing loss.

As I watched Matthew the last couple of years, I realized that he is a deaf child with his hearing moments.  Yes, hearing moments.  I have real life examples. . . In the beginning, he could not hear 80 dB in the booth.  Approximately 2 years ago, he could identify words in the booth at 20 dB.  On another occasion, I took Matthew to the orthodontist and explained that he is deaf.  He heard all her questions and comments and answered appropriately! This orthodontist gave me a really strange look!  Another day, I took Matthew to the ophthalmologist.  Again, I explained, he is deaf.  Matthew did not understand him at all and I interpreted for him.  The ophthalmologist looked at me and said "Does he wear hearing aids?"  When I noticed Matthew's "hearing moments" in the beginning, I would say to him "Wait a minute, you are supposed to be deaf!"  Now I take it in stride, and I figure he is having an "on moment".  I use both sign and speech with him.  So that when he is having an "off day", he can watch me sign.  If he has an "on moment", he has the option of listening to me because I talk also.  Total communication really seems to work for him because his first language was English.  He already had speech before his hearing loss.  It is strange because he has retained his speech skills to this day despite his hearing loss.  He does have difficulty with new words.

Our 10 year old son, Alex, was also diagnosed with AN three years ago.  He has mild AN, he has less "off" moments than Matthew.  He functions more like a hard of hearing child.  He does not wear hearing aids.  He does not use a FM system.  He is slowly learning sign language.  His receptive skills are great!  His expressive skills are coming along slowly.  Alex is mainstreamed in his home school and receives services from a teacher of the deaf.  Alex also has a vision loss.

Matthew is now in second grade and is fluent in sign language and English.  He is an accomplished reader, now reading at the 3 rd grade level.  He also has been diagnosed with ADD, so the small classroom setting at the hard of hearing program is very beneficial for him.  He is a wonderful kid who sees himself as deaf.

Sue Thompson is a mother of three in Geneva, Illinois and a Hands & Voices member.

Recommended resources:

http://www.nidcd.nih.gov/health/hearing/neuropathy.asp

http://auditoryneuropathy. tripod.com/ANindex.html (a website set up by a parent of a child with AN.)

"Auditory Neuropathy:  A New Perspective on Hearing Disorders", editors Yvonne Sininger and Arnold Starr.