Monday, March 3

Cochlear Implants: Q & A

I have received several emails recently from parents of children newly diagnosed with hearing loss. I love the fact that this blog has allowed us to connect with other families all across the country (well, the world, really) that are going through similar experiences. Some of the questions I have received may be on the minds of others, so I thought I would post all of my answers here:

What genetic testing should I have run on my child now that he has been diagnosed with hearing loss? Do the genetic testing results have any relevance in regard to the treatment options? Did you see a geneticist or can an ENT order genetic testing?

While I am sure genetic testing varies from one hospital to the next, our genetic testing was ordered by Drew's ENT. He ordered the test after our initial meeting as a result of Drew's hearing loss. Drew's testing was run when he was about six weeks old. The genetic test is called a "Comprehensive Hearing Panel" and consists of testing for Connexin 26, Connexin 30 and a specific gene mutation. These are the most common, non-syndromic causes of hearing loss that can be tested and are genetic.

Many people never find a cause of their child's hearing loss. It is believed that there are many genetic causes of hearing loss that have not yet been discovered, so if these tests come back negative, that does not mean that your child's hearing loss was not a genetic cause, just not anything they can test for. I would however strongly recommend having this test run because if your child's hearing loss was caused by one of these genetic causes, you can rule out other causes, some of which are syndromic.

In addition to the initial genetic testing, your ENT will order a CT scan or an MRI to check the anatomy of the child's' ears. After the CT scan or MRI, the ENT may elect to run further genetic testing. If it is found that the child has enlarged vestibular aqueducts, malformation of the inner ear or other anatomical abnormalities, there are specific genetic tests that can be run for syndromes that have these abnormalities, such as Pendred Syndrome. This is just one of many syndrome's associated with hearing loss.

The genetic testing has no relevance in your actual CI candidacy. The only thing it can give you is an idea of how well your child may do with a CI. Children with hearing loss caused by Connexin 26 are known to do very well with cochlear implants because they don't have any other known problems, such as with thier balance, kidneys, eyes, just to name a few. So you might get an understanding of the possible outcomes with a cochlear implant. For instance, if a child has Mondini or EVS, all electrodes might not go in the cochlea, so results can vary. Ultimately, the success of the child is in the hands of the parents. The surgeon and audiologist can only do so much. Without parent support, intervention and therapy, the child will never perform to his maximum potential.

What made you so sure about having Drew simultaneously implanted? Furthermore, what made you feel that Drew needed two implants?

There were several reasons:

First, bilateral cochlear implants are proven to help with sound localization and with hearing in noisy situations, two areas we felt were vitally important for children. Drew can hear what direction a car horn is coming from if he is playing in the street. Also, for children in noisy situations - classrooms, play grounds, Chuck E Cheese - they can hear much better. For example, we were having dinner at Roosters the other night and I was talking to the waitress, who is an audiology major at Ohio State, about Drew's cochlear implants. I was listing all of the words he can say and he was repeating them! He was sitting on the other side of the table, I was turned talking to the waitress and he could hear everything I was saying. I don't think this would happen with just one cochlear implant.

Second, we did not see the need to put Drew through two surgeries when we already knew we wanted Drew to have bilateral implants.

Third, if you do sequential implantation, meaning one ear after the other 3-6 months, or more, apart, there is a "big" ear and a "baby" ear. The new ear has to catch up. You have to do specific therapy to catch up that second ear because it has gone longer without auditory stimulation to the brain. We didn't want to do that. It is important to know that many parents did not have this option when their children were first implanted, as research into bilateral implants has only been around for about two years. When Drew was diagnosed we received mixed reviews on the medical necessity of bilateral implants. The whole concept was very new. Drew is only the second child in Ohio to receive bilateral implants. I have a feeling that many families would have chosen simultaneous implants if it would have been a choice at the time.

We have also, over the last eight months, run into other reasons why there is a significant advantage to bilateral cochlear implants.

What made you decide to have Drew implanted at such a young age?

Drew got zero, and I mean zero, benefit from his hearing aids. He never responded to anything with his hearing aids! Seriously, we got one response in the testing booth in over six months time. We never saw him respond to any environmental sounds at home (phone ringing, dog barking, doorbell). Every day that went by was one more day that Drew was not getting any auditory stimulation and one more day further behind in his development. Had Drew shown even the slightest benefit with his hearing aids we probably would not have pushed as hard as we did. (Although, seeing the results, I can't imagine not getting him implanted as young as possible). Once a child is 6 months old the complications of surgery reduce considerably, as the risk of anesthetic declines.

I was very persistent and direct with our ENTs on my expectation that Drew be implanted at eight months of age. I finally called each of the ENTs we were considering and asked them for a surgery date the first week of June for bilateral implants. One agreed, one did not. We would have gone to the end of the earth to get him implanted when we did. Travel distance to the implant center did not matter to us. We would have searched out skilled doctor after skilled doctor to have Drew implanted at that age.

What is the difference between Auditory Verbal and Auditory Oral philosophies? I know there is an Auditory Oral school near us - is that successful as well from what you have heard?

The line between AV and AO is blurring.

Traditionally, AV believes in not allowing the child to do any lipreading, mainstreaming from the beginning (meaning there are no AV schools because they believe in putting the child in a preschool with typical, hearing peers) and the process of catching up a child's hearing age to their actual age happens without any special needs schooling.

Traditionally, AO allows you to see the lips of the person talking. In fact, AO used to encourage the use of lipreading as a means of communication, although I think that idea is fading as amplification options become better and better. In addition, with AO, the idea is to give the child intensive therapy from the beginning, in a special needs setting, so that the child can mainstream in school by kindergarten, or when the child is ready.

We are doing a combination of both therapy methods with Drew. In his therapy sessions we use a lot of AV, where we don't allow Drew to see our lips, making him perform all tasks with auditory input only. But in everyday life, Drew can see our lips. We also strongly believe in sending Drew to an oral preschool that will focus on his specific needs and give him a head start to mainstreaming in kindergarten. I would not feel comfortable sending him to a mainstream preschool. To me, that would be like giving up precious years that we can use to educate him with intense auditory and speech therapy.

How old was Drew when he received hearing aids? Were you able to use loaner hearing aids? I'm thinking my child will be 3-4 months of age before he receives his hearing aids through our states loaner program.

Drew received his hearing aids when he was 10 weeks old. We did get loaner aids through our Regional Infant Hearing Program, because we knew that the hearing aids were likely not going to give Drew enough amplification to develop normal speech and hearing.

There is a mandatory hearing aid trial in order to develop candidacy for a cochlear implant, so the sooner your child receives hearing aids the sooner you can head down the CI path (if the aids aren't working). We had to wait 3 months from when we got the aids to begin down the path toward the cochlear implant, and this time frame may vary by program. We actually started the official cochlear implant process in March. We then had to do the candidacy process, which included meeting with a Social Worker, SLP, ENT and our audiologist. I had scheduled many of these appointments in advance to speed things up. The best advice I can give it to advocate for your child in order to move the process along. I went with the old saying, "The squeaky wheel gets the oil." I called the doctors office everyday to see if there were cancellations so that I could get Drew in for his evaluations sooner. I think that the receptionist got so sick of me calling everyday that she finally just gave me an earlier appointment.

What forums would you recommend I join?

There is a great Yahoo! Group: CI Circle. It is a group for parents with kids who have cochlear implants and for parents of kids in the cochlear implant process, although there are many professionals involved with the group as well. This group has been an excellent source of information for me throughout this entire process. This group was where I initially discovered that kids were being implanted under 12 months of age. They were also great with providing information on insurance companies that were providing coverage for bilaterals and for under 12 months of age.

If you have any recommendations or would like to expand on these questions, please leave a comment.


MB said...

I am so jealous you had such a short wait for genetics testing. In DC the wait was 6 months for an appointment! Had we known from the start it was a genetics issue it would have saved us a lot of time and money with all the other tests. I should have been a much bigger pain about calling to get an earlier time. Lesson learned!

Tales from the CI Gal said...

Thank you for so valuable information. I have a family at my school who is going through the CI process, I am going to pass this blog along to them. They are feeling so isolated and without support. It is fantastic that you take the time to put this information out for parents, children, and adults.

As an adult who went through bilateral cochlear implant sugery, I am so happy I did it together. My view - one surgery - one time.

Laurie said...

This is a great post. My whole family underwent genetic testing when I was 16 years old to determine what the cause of my and my brother's hearing loss. A cause was never determined and we were told that both parents probably had the recessive gene for this type of hearing loss. We were also told that as long as my brother and I married persons with normal hearing, the chances of having a child with a hearing loss was VERY slim (1%).

I did marry a hearing person and all four of our children have normal hearing. I always said that if I DID end up with a child with a hearing loss, that we could deal with it because I knew what it was like.

Glad to hear Drew is doing so well!