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.

Drew Is Helping Other Deaf Children!

At only one year old, Drew is already helping other deaf children to "turn on their ears".

A nine month old little girl in New York was scheduled for her cochlear implant surgery, but was later denied coverage by Anthem Blue Cross Blue Shield because she was under one year old. Their doctor submitted a letter explaining the need, and they tried to get help from the Let Them Hear Foundation, but nothing had worked. The family had their final appeal with the insurance company later that day and a family friend posted a plea for help on CI Circle:

I've heard there are studies supporting implantation under 12 months. Does anyone happen to know those studies offhand. I was hoping to put a few things together for them to take with them to their appeal this afternoon.

We saw her cry for help and emailed her, explaining that Drew had successful bilateral cochlear implant surgery at just under nine months while covered by Anthem. We sent to her a copy of Drew's approval of coverage letter and "Explanation of Benefits" from Anthem in the hopes that it would help the little girl in New York. We later got this response:

I'm happy to report that my husband's co-worker and his wife won their appeal with Anthem, so their daughter will be able to have her surgery as planned on Friday. Thanks for all your help and advice.

...I think Drew's EOB really made the difference. Thank you!

It feels great that Drew was able to help that girl, even though we don't know her or her family. If you or someone you know is denied insurance coverage for cochlear implants, don't give up! Please let us know if we (and Drew) can help you.

"Is That It? I'll Just Pay Cash."

We got Drew's "Explanation of Benefits" for his bilateral, simultaneous cochlear implant operation in the mail on Tuesday. We had a little bet going on who would come closest to the total cost, but both Drew's Mom and I were way too low.

The total "Provider Charges" were a whopping $155,232.71! However, because our insurance company Anthem Blue Cross and Blue Shield evidently "gets it" regarding how important bilateral cochlear implants can be for a child (or adult), the total out-of-pocket cost to us is only $1,170.15.
As I constantly "meet" people from Spain, Sweeden, California, South America, and all over who are dealing with hearing loss, hearing aids or cochlear implants, one thing is clear. That is the fact that whether one has insurance that will cover their chosen "plan of attack" directly impacts how successful that plan will be.

We are the fortunate ones. Our insurance company (Anthem of Virginia) gave phone approval in under five minutes for a procedure that some families never get. (I'd like to thank those who have decided to cover this procedure, as it truly makes a difference for Drew. I'll never meet you, but if you happen to see this please know we feel you have done a great service to your customers.)

Some insurance companies (now the minority) don't cover cochlear implants at all. Other insurance companies claim that a second cochlear implant is experimental or not medically necessary, despite growing evidence that they do benefit the users.

Because most of us can't quite find it in the budget to write a check for $155,000, not obtaining insurance approval means that they won't be able to take advantage of the cochlear implant technology. Families often fight for months or years to get insurance approval, while precious weeks roll away. These vanishing months quickly close the window of time during which a young child can grasp the foundations of language, with it often closing before the appeals, court arguments and battles have concluded.

Make sure this does not happen to your child. If you have decided that a cochlear implant is best for your son or daughter, I want you to do two things:

#1 - Start the insurance approval process immediately. Don't wait. Don't assume. Request a copy of what is covered by your policy. Politely demand that your chosen surgeon or audiology team contact your insurance company right away for approval.

#2 - If you are having difficulty, contact the Let Them Hear Foundation. They help families battle insurance companies that deny cochlear implants (or a second implant) to those who need them. Perhaps you should contact them even if you don't foresee trouble, just to take advantage of their educational resources.

We are going to send our approval letter and explanation of benefits to the Let Them Hear Foundation so they can demonstrate to the other insurance companies that bilateral, simultaneous cochlear implants are being covered. I hope that in this way we will be able to help others "Turn on their ears".

Bandages Off

Drew's Incision

Sitting Up

Drew's Sister Playing "Piggies"

I took Drew to have his bandages removed today. Dr. Kang removed them fairly quickly and said that Drew is healing nicely. It was a very fast - my kind of appointment.

As a side note, I have to say how thankful I am for Dr. Kang. He has given Drew and our family a gift that we will never be able to repay. Moreover, I think Dr. Kang genuinely cares about Drew's ability to communicate with the hearing world. I love the way that he casually talks with Drew. He will walk into the room and sweep Drew out of my hands and say, "How are you doing today, buddy? Are you ready to hear? We're expecting big things from you, young man!" It is so refreshing to have a doctor that genuinely cares for his patients. We knew from our research that we were working with a very skilled doctor but we got so much more! I am so thankful that he is in Columbus. We are very blessed to have him leading the cochlear implant program here.

I have to imagine that Drew feels a lot better with his bandage off and that his incisions will heal much faster now that they are exposed to some fresh air. The incisions are actually smaller than what I was expecting; I don't know why but I thought they would be larger, but I did. Also, I was amazed at how close the magnet is placed to the incision. And both look to be lined up quite nicely. (For all those that know my type A personality, you had to know I would want both coils lined up perfectly on his little head!)

I included two pictures of Drew sitting up because I am amazed at how much better his balance is since his surgery. Seriously. Amazed. He is actually sitting up! Tonight I had him sitting on the floor for 30 minutes by himself! He was looking around, watching television, chewing on his toys, thoroughly enjoying life. I am 100% convinced that the surgery made his balance better. Also, he is making attempts to crawl like never before, and when he is unsuccessful he gets really upset! My little boy will be crawling around here before too long.

We are so thankful for the way Drew has recovered from this surgery. We thank you for your prayers for Drew.

Now, is it June 28th yet?

Pictures

The perfect patient.

In recovery.

The next morning.

Surgery Complete - We're Home

We are now home. I just fed Drew and put him to bed - hopefully for the night, but we will see.

We arrived at the hospital at 8:30 AM this morning for the pre-op work. We then waited until 11:40 AM, Drew's surgery time. He was such a wonderful patient! Despite being hungry (and tired) he managed to remain a happy little boy. He was even flirting with the nurses and showing off his cute laughter!

The nurses took him to the operating room around 11:15 AM. It was a lot harder to let him go than what we thought it would be. We tried to each lunch shortly thereafter, but neither Drew's Dad nor I had much of an appetite.

The surgery lasted about 3 1/2 hours. Dr. Kang was very pleased with the insertion of each of the electrode arrays and there were no surgical complications. Drew had a bit of fluid in his right ear but nothing that complicated the surgery. In addition, Drew's audiologist tested both arrays and they are working beautifully! We are very happy with the results and look forward to activation day on June 28th.

It took Drew about one hour to wake up after surgery. He woke up fairly happy and VERY hungry! He ate four ounces of pedialyte in just a few short minutes. I could tell that he was not quite himself; he didn't want to play with his toys very much and he just laid in my arms, very different from my wiggle worm. Since he was able to keep his food down the nurse released him from the hospital a little before 6 PM.

We would like to thank each of your for your thoughts and prayers for Drew. They certainly worked!

We're Headed to Italy!

It's true! Our trip to Italy starts first thing tomorrow morning. There was a time about seven months ago when I thought I might never get to take Drew, but after months of planning we are finally ready to go!

We aren't going to arrive there tomorrow. There will likely be delays, flat tires, pit stops, and even lay overs. Some of these may be longer and more frustrating than what we think we can endure. We may even ask ourselves, "Are we there yet?" But I am looking forward to the journey just as much as I am looking forward to the destination. When you think about it, it is actually very special that we will get to visit so many places with Drew along the way. Not many people bound for Italy get to stop as many places as we will and relish in those moments. We are going to have so many that will shape the person Drew will become. I can not wait to watch the transformation, from beautiful Deaf baby to a hearing, speaking, communicating deaf child.

We are nervous for the beginning. Once we get through this I think we can handle the rest. We will let you know how our trip begins as soon as we can tomorrow.

Ciao!

Quick Update

I am feeling a little better tonight. I decided to take Drew to the pediatrician to see how he was doing with the fluid in his ears.

Drew's doctor found that the fluid in the right ear has completely cleared. He could not see the left ear drum, as Drew has a bit of an ear wax problem. Drew is not running a fever and is in overall good spirits so we know that he does not have an ear infection now, and with the fluid clear in at least one ear we are feeling better.

Final Meeting With the ENT

We had our final appointment with the surgeon today, just under two weeks before Drew's surgery. Dr. Kang wanted to have the meeting to answer any last minutes questions we may have and do one final evaluation on Drew.

During the evaluation Dr. Kang found fluid in Drew's ears. He has had a runny nose for the past few days which we attributed to teething (Drew has cut two teeth in the last week). But Dr. Kang thinks that Drew has a touch of a cold and that is causing fluid to fill in his ears. If the fluid does not clear before surgery it does not really add any risk to the surgery itself, but should the fluid become infected before his surgery it will be postponed.

At this point we are getting really nervous. Nervous that Drew won't be able to have surgery on June 6th because of an ear infection. We are also getting really nervous about possible complications with the surgery. Drew's Dad is really worried about complications that would cause facial paralysis and severing the taste nerve. All are extremely rare, but none the less, worrisome.

We have full confidence in Dr. Kang - he is a very experienced and talented surgeon and is the chief of the department at Children's. We trust him fully, but we are Drew's parents so we worry about every detail. We are trying to make the best decisions for Drew and ultimately surgery is a necessary evil to allow Drew to hear. We'll be a lot better once June 7th is here.

Under Two Weeks Until CI Surgery

Well, the countdown clock that Drew's Mom put to the right of the posts tells me that we are now only 13 days away from Drew's cochlear implant surgery.

I'm happy that it is almost here, but I've been getting progressively more nervous. The surgery itself is not very risky, but I suppose that even if it was a simple procedure of putting tubes in his ears I would be nervous as well. I just want to fast-forward two weeks so that I can know that he is comfortable and that everything is working correctly.

At that point we will have a lot of work ahead of us, but at least we can get started on that as opposed to waiting and waiting. He is now very active, very interested in watching us (and The Wiggles), and I can't wait to pair his obvious communication techniques with sound!

I believe that Drew is going to do great with his implants, and we're going to do whatever we can to help him. I just want to get through the next few weeks so we can begin together.

Auditory Verbal Therapy

With Drew's surgery now scheduled and a much easier than anticipated insurance approval process behind us, I have now begun to seek out therapists that will help our hearing Drew learn to identify and listen to sound and eventually speak!

Right after his surgery was scheduled I began to get really nervous about the surgery itself. I think that focusing on the therapy has really helped me to keep the butterflies about the surgery itself buried, at least for now. In my efforts to get to the point of implantation I knew surgery was inevitable, but that doesn't make it any easier. I want my son to hear; to do so requires surgery. Do I wish we didn't have to go through this? Certainly, but I know on the other side of June 6th there is a world full of opportunity that will open for Drew with his cochlear implants.

We are taking a strictly oral approach for Drew's therapy and intervention. This leaves us with two choices of therapy: Auditory Oral and Auditory Verbal Therapy. We have decided to utilize the principles of the Auditory Verbal Therapy (AVT). The main reasons we have decided on this therapy are:

• AVT focuses on having children grow up in typical listening and learning environments, enabling them to become independent, participating, and contributing members of the mainstream.
• AVT is a family entered approach where parents are active participants in the therapy.
• AVT relies on building auditory skills without the ability to lipread, stressing the normal patterns of language development. These children gain spoken language skills equal to their hearing peers, have normal voice inflection and tone and learn the rules that guide spoken language.

While we will certainly incorporate pieces of the Auditory Oral approach, primarily the natural gesturing and lipreading all hearing people do in everyday setting. However, during Drew's therapy session there will be a focus on gaining information auditorally, instead of through auditory access and visual cues.

In addition to two hours of therapy a week, there is a toddler program at a new Oral Deaf Education School in Dublin. We will enroll Drew in the Parent-Toddler program one half day a week in January at the Auditory Oral Children's Center. This school utilizes the principles of AVT in their therapy sessions, but allows natural gestures in the classroom setting. This is a new program, showing the commitment within the cochlear implant team in Columbus to develop better resources for children with hearing loss.

Special Delivery

I was surprised to find in the mail today the letter from the insurance company approving Drew's cochlear implant surgery! I have tucked it away in a special place for safe keeping - just in case. I want to celebrate the fact that everything is scheduled, but I am so nervous that something is going to happen to change the approval status for Drew' surgery.

I don't think I am going to answer my phone until the little countdown clock reaches zero.

Too Good to be True?

I have experienced some of the greatest challenges of my life over the last seven months. There were days after Drew was diagnosed that I did not want to get out of bed and face his deafness each morning. Luckily my family and friends were there to support me, and finally I realized that staying in bed, crying all day would not change Drew's diagnosis. So I made a decision; I would get of bed each day. And I would get myself dressed. And I would learn as much as I could about deafness, cochlear implants and oral education. And that's what I have done. Today I feel like I have reaped my first reward of hard work and dedication.

Dr. Kang called today with a date for Drew's surgery. Drew will have simultaneous cochlear implant surgery on June 6. Although not confirmed, he should be activated sometime during the week of June 25. I can not believe that we have traveled to this point in our journey. During these months I have felt like we would never get to the point of having him implanted. Now there is a date on my calendar! We are getting closer to the day that Drew will hear.

Even more amazing (and I'm still pinching myself to make sure I am not dreaming) is that Dr. Kang's office received verbal approval for the surgery from our insurance company today. We should receive a written approval letter within the next week. We are going to celebrate when we receive the letter, but until Drew's ears have two electrode arrays in them nothing is certain. I have read horror stories from other parents receiving calls from their insurance company the week of the surgery with a change in the approval status.

The Cochlear Implant team will finalize Drew's candidacy at their May 8th meeting, but we do not anticipate any issues. At this point, we are just waiting for June 6th to come and praying that nothing changes in the approval status of Drew's implants. If all goes as planned, Drew will be hearing by the end of June. Someone pinch me!

CT Scan Results

Dr. Kang called yesterday with the results of Drew's CT scan. Drew's inner ear formation is in tact and there are no structural issues with his inner ear. This confirms his candidacy for his cochlear implants! While this is the news we were expecting, we are very happy that our son has two perfectly shaped cochlea's!

As with the course of this journey, things do not goes as we plan, or as we are told. Dr. Kang did not schedule a date for Drew's surgery yesterday. He confirmed that he will implant Drew in June, but would like to schedule the surgery after our CI Consult with the mapping audiologist, which is Wednesday and after our meeting with a speech pathologist, which is April 25th. So stay tuned, it will probably be another month before we have a confirmed surgery date.

CT Complete...Barely

Drew had his CT scan today, a critical step in moving forward as a cochlear implant candidate. This will allow Dr. Kang to see the anatomy of Drew's inner ear, and to ensure that Drew has a cochlea for the electrode array of the implant to be inserted in.

When the CT scan was scheduled we were told that Drew would need to be sedated because he would need to remain still for the scan. To say that the sedation did not go as planned is an understatement. Drew managed to fight off all of the medicine he was given, never was drowsy, let alone sedated, and remained awake for the entire procedure. To say that Drew's Dad and I were concerned that they would have to reschedule the scan would be an understatement.

Thanks to the wonderful nurse and radiologist at Columbus Children's, Drew was still enough for the scan to be completed today. The nurse and radiologist managed to take a pacifier (which Drew has never had before), dip it in cherry syrup, place it in his mouth, securing it temporarily by using medical tape wrapped around his head. Then they strapped him to the table to reduce as much movement as possible, and ran the scan. Drew was amazing, allowing all of this to happen to him while starving, since he had not been allowed to eat after 2 AM!

We are expecting the results in the next two to four days. Dr. Kang told me that he would call with the results and assuming the scan comes back as we anticipate, we should have a surgery date within the next few days!

Moving Forward

We are very excited with the pace at which we are now moving toward having Drew bilaterally implanted. I am so happy that we re-evaluated the decisions we had previously made and that we decided to change course.

In the beginning, we had only looked outside of Columbus because the reputation of the cochlear implant program through Children's Hospital was poor. Lately we felt we were not being aggressive enough with Drew's intervention, and decided to re-evaluate our previous decisions.

Since then, we have learned that the tide is changing with an increase in the strength of the Columbus implant program. Similarly, we have discovered that many of the negative comments we heard regarding Columbus audiology program were not entirely applicable to our situation. Some of these initial negative impressions other parents had have changed since the arrival of Dr. Kang and the perspective he brings to the audiology program, and other opinions we heard on the perceived weakness of the audiology program were due to certain children struggling with their hearing progress due to other barriers that those particular children were facing.

With this new, aggressive path we are taking, Drew will be having a CT Scan on Wednesday. Dr. Kang told us that as soon as he receives the results from the CT Scan, he will schedule Drew's simultaneous surgery. Our goal is to have the surgery when he is nine months old. Dr. Kang's office will work with our insurance company to ensure coverage for his surgery.

Please keep Drew in your prayers on Wednesday, as he will be under an anesthetic for the procedure. We will let you know as soon as we have the results from the scan and Drew's surgery date!