Most of us have a moment: a dog barked, a pot dropped, or maybe a balloon popped. And then nada. No jump, twitch, or even glance from your baby in the direction of such a loud noise. Nothing. That was us. Except my husband and I were also sneaking behind our son, calling his name. That’s when I knew there was something wrong. But then, how could that be when at the same time, I had to whisper at night so we didn’t wake him?
This was my reality for the first year of our son’s life.
Our son, C.J. is almost 20 months at the time of this writing and is growing into such a boy. Anything that has wheels (and I mean anything – even his sister’s Barbie stroller) he loves. He has a great laugh, an even greater “shy face”, and loves to snuggle. C.J. also has bilateral auditory neuropathy spectrum disorder (ANSD). ANSD is a condition where the auditory nerve has trouble sending the correct signals to the brain. He hears sounds, but not always the way they are supposed to sound, and sometimes not at all. Some children hear normally with ANSD, thus the inclusion of “spectrum disorder” in the terminology. Someone with ANSD can test profoundly deaf while another person places in the normal to slight range of hearing loss…or they can be the same person at a different moment of the same day. (Are you still with me?) A child diagnosed with ANSD can have their hearing fluctuate depending on health, weather, allergies, lack of sleep, and even time of day. (If you are now thoroughly confused, welcome to the club.) Needless to say, doctors and professionals are still trying to understand the intricacies of ANSD. So don’t expect a black and white answer. We are still waiting for ours.
For the first six months, we don’t think C.J. heard much at all. Many children who are premature or extremely jaundiced are predisposed to ANSD, neither of which C.J. was. This group also has a tendency to grow out of the condition. They just need extra time for the auditory nerve to mature. This usually happens after a year. Well, maybe this was what C.J. needed, extra time. So we waited with baited breath for his one-year ABR test. Unfortunately, the ANSD was still present in both ears. Booth testing brought us more confusion, as some visits he would test very well, and others, not so much. When he turned 14 months, we began Visually Reinforced Infant Speech Discrimination, or VRISD testing. Similar to traditional booth tests for children, VRISD testing relies on conditioning your child to turn at a change in stimulus. The difference though is that instead of just hearing a sound and turning, the change is a different consonant/vowel combination (i.e. ba, pa, de, me). Some individuals with auditory neuropathy have trouble hearing certain sound combinations and being able to pinpoint which ones have no response can help narrow (or widen) the area of therapy. The audiologists narrowed down to at least two consonant/vowel combinations that C.J. didn’t respond to after two separate appointments. How wonderful for us to get some concrete information, finally!
At times I almost wished he was a more typical deaf child. I don’t know how many times I have heard, “let’s just wait a few more months,” or “he seems to be hearing fine today,” and my personal favorite “maybe he’ll grow out of it.” A parent of a child with a more typical hearing loss can get that identification right after a soundbooth or an OAE test, “Sir/ma’am, your child has a loss of xx Db in three frequencies. He or she might respond well to hearing aids.” Thanks and have a nice day. For someone diagnosed with ANSD, they might expect to endure, say, six booth tests in three months. Why so many? Because C.J. was responding as his hearing was fluctuating. And also to track improvement. So just keep testing, just keep testing (said like Dory on Finding Nemo, “Just keep swimming, just keep swimming”). But right about the time where I was starting to lose hope and research other methods of treatment (i.e. cochlear implants, FM system), something awesome and amazing happened. C.J. started to respond to sounds. A large variety of sounds. Frequently.
Because he wasn’t a “typical” deaf child, I hesitated joining the D/HH community. I mean, what did I have in common with anyone? We were slowly learning ASL just in case he proved to benefit from it. My signing ability is still quite embarrassing compared to other parents. And what about cochlear implants? Many parents of children with ANSD choose to implant early. And some are very successful. But we decided to wait, which was the best choice for our family. What I realized though is that it is not about the level of assistance your child may need. It’s about support. Supporting a family through the unknown. And I know that feeling quite well.
Over the past nine months, C.J. has shown so much growth. There are times when we don’t know for sure; he could not be hearing…or he could just be a toddler ignoring his mother. Here shortly, we will be entering the world of FM systems; a trial to see if it may help with his language development. Either way, we know we made the right decision for our family. With our audiologist, his Colorado Home Intervention Program (CHIP) facilitator, and our Integrated Reading Program sign language instructor, C.J. is getting the best individualized care to meet his ever-changing needs.
Need additional information on ANSD? Here are a few links that helped me through the early days:
www.wikipedia.org/wiki/Auditory_neuropathy
www.childrenscolorado.org/conditions/speech/danielscenter/ANSD-Guidelines.aspx
And here’s a great audio of what it sounds like to have mild, moderate, severe, and profound ANSD: www.youtube.com/watch?v=IY5YIiu_4t4
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