Sunday, May 3, 2009

Auditory Neuropathy

Blair had her second ABR (auditory brain response) test this past week. I was sure that her hearing had improved like the doctor had hoped it would. She turns her head when her name is called, and she reacts to noise a lot of the time. However, she failed the ABR again. It showed a 65 dB loss in the left ear and 80 dB in the right. At the same time, she has a good pass on the OAE, the test that measures the function of her ear. That tells us that she has Auditory Neuropathy (AN). Many children who have have an issue with the hair cells in the cochlea. Blair's ear works properly, but there is a problem with the communication between the ear and the brain.

AN is very complicated. In fact, I'm trying to learn more about it before we go back to the ENT. From what I can tell, hearing with AN can go in and out and vary greatly from hour to hour and day to day, thus the complication of it. It can also fluctuate over the first several years of a child's life. Severity can range from hearing normally to profound deafness. AN is a rare form of hearing loss, and there aren't many doctors who deal with it on a regular basis.
It can result of many things including prematurity, jaundice, respiratory distress at birth, prenatal infections, and genetics. Blair falls into many of the acquired risk factors, so we do not think that it would be genetics in her case, but testing can be done to make sure.

Treatment for AN is varied. Some use hearing aids, but the success rate in testing done by experts is not very high. Cochlear implants work in most cases, but it is recommended to wait longer than normal (1 year) with these kids (2 to 3 years) to see how speech develops first. Being unaided works but not usually. I guess you get the picture. We are going to have to do some research to see what the best option is for Blair. There is a professor at USF in Tampa who specializes in AN, and I hope to be in contact with him as a resource. There are many different communication recommendations for infants with AN, one of them being cued speech, and we will have to work with Blair to see what works best.

The next step is to do booth testing in two weeks to see what kind of speech tones she hears and if she might be able to benefit from hearing aids. I have been told that the booth testing with six month olds is very frustrating, because they don't always understand what to do. Please pray that we do get some answers about what Blair does hear and can find a good option for her.

6 comments:

Jamie said...

We are praying that the next tests show that everything is just as it should be. You are a miracle baby, Blair...this little bump in the road won't stop you!!!
Love
Jamie

Semi-Slacker Mom said...

I had no idea ya'll had this to deal with too! Who's her ENT? Bo has major ear issues w/ some hearing loss in his left ear, but nothing this major.

I know I'm praying for ya'll! Love ya!

The Morris Family said...

Your kitchen looks very nice! As I scrolled down and you shared about your daughter, we have family in our home church and their little boy just had the cochlear implant a while back, I thought you might be blessed in reading about their journey. http://causeme2hear.blogspot.com/
Cindy

Jen O'Daniel said...

Oh, please keep us updated as to how the booth testing goes. Blair is in my prayers (as well as her parents!) and I'm hoping for clear answers!

XOXO
Orr

Julia said...

I hate to hear this about Baby Blair. Hopefully her case will be less severe and can be treated easily. My little sister has worn hearing aids her entire life because of a malformation in the ear that fused her hammer and anvil together. We will be thinking of you guys and praying for little Blair's hearing to improve!

Anna said...

Ashlee, I read your post on the AN web site and traveled over to read your blog. Nice kitchen. I also love to cook and knit...and quilt.

I have a son who is now 9 and is bilaterally implanted with Cochlear Freedom. He hears very well, plays piano and loves music. After our research of AN when he was 2 1/2, we knew we wanted him to speak and hear. So we had him implanted and we haven't looked back. The initial decision was hard. We later learned the surgery is hardest on the parents then the child. Lee was running around 6 hours post op. We asked to be discharged and away we went. I'd be happy to visit with you by phone is you'd like. 320-252-0751 and my name is Anna~ Blessings~
ps. Blair is Beautiful!