Monday, May 6, 2013

ENT, School. ABR Results

We got the official results of Jack's ABR. They said he has profound hearing loss in his left ear and something called auditory neuropathy/dysynchrony in his right ear. This means his right ear has normal cochlear function, but for some reason sound is not being properly sent to his auditory nerve. In other words, he can't process sound in his right ear.

This is a rare diagnosis, and only about 2% of people with this diagnosis have it on only one side. This can happen in babies that had jaundice that was not properly treated. Jack did have jaundice, but he was under the photo therapy lights. In babies that have severe jaundice they should have an exchange blood transfusion. I do not know what Jack's bilirubin levels were and I am not sure how long they were elevated.

I am not certain that this diagnosis is accurate. It may be, but from what I am reading it is so rare that he would have it on only one side that I am wondering if they either missed it on his left side or that he doesn't actually have it on the right. In some cases babies with this condition have a spontaneous recovery. If they will have a spontaneous recovery it is usually in the first year of life and it generally would happen in babies that acquired this condition due to jaundice. The other thing is that there are a lot of people who say this diagnosis really can't be accurate if he still has fluid in his ears, and he does. So we will have to see if he shows this in further testing.

I took Jack to another ENT. He said he can do Jack's tubes. They prefer not to do it before 6 months of age. I am sure he will probably be over 6 months old by the time it is actually all set up. He also said he can do another ABR after the tubes are in and while Jack is still under general anesthesia. That would be great because I think it would be much more accurate. He also said he thinks Jack will be a good candidate for cochlear implants. Cochlear implants can help profound deafness as well as auditory neuropathy/dysynchrony. He wanted me to speak to the cochlear implant surgeon there at LIJ. I decided not to because I have already researched this and I have found that NYU has the premier program for infant cochlear implants. Obviously I didn't tell him that. I just said I wanted to wait to see what happens after the tubes are in.

I went to see the deaf infant program today. They have kids from birth until they would start kindergarten. We saw a little boy who is 5 years old and has bilateral cochlear implants. He became deaf as an infant after contacting meningitis. He received his implants at just over a year old.  His speech was beautiful and it seemed like his hearing was great. I was so happy to see him and I almost starting crying when I saw how normal and happy he was. He is starting kindergarten in the fall in a regular classroom. They had a lot of other kids there but the rest of them had regular hearing aids. It is likely Jack will get regular hearing aids but they do not think they will help him hear sound. They will just be to keep his sensory pathways open until he can get cochlear implants, providing he doesn't have any issues that will keep him from being a candidate for them.

They teach the kids to use spoken language. They no longer teach sign language. I told them I taught Julianna some signs when she was a baby and I was told that actually accelerates language development. They said that is true with a hearing baby and it would also be fine for Jack before he gets cochlear implants (if he does). However, they said they do not use sign for a baby with implants or   hearing aids. They said that it is a lot of work for them to learn to hear with these assistive devices and the signs are easier. They don't want them to take the easy way out. They want them to learn to hear and discriminate between different sounds.

I really liked all the people at the school. They said Jack would have about a half an hour a week at first. They said that is really them teaching me how to interact with him. As he gets older he will spend more time there. They said that most of their kids leave the program to go to regular preschool and almost all of them are mainstreamed in kindergarten. They are generally at grade level then and a lot are ahead due to all the early intervention. This sounded very promising to me.

I told them I was still hoping for a miracle that he will be able to hear when he gets his tubes. I realize that it is unlikely and I told them I would like to call them up and apologize for wasting their time. They said they wouldn't be upset about wasted time and they would be thrilled if that happened.

So right now we are waiting on scheduling the tubes. We couldn't do it right now anyway because Jack has a cold that is causing him to have some difficulty breathing. They would not let him have general anesthesia with it. He seems to be doing better today but he was having a really bad time on Friday and Saturday.

He loves to smile and laugh and everyone says he is the cutest boy ever. I agree!!


  1. He is the cutest little boy and I do pray for that miracle for Jack.
    Can't wait to see him again. You are such a great mom!!! Oh, and Happy Mother's Day!!!

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