Beginning the Process

On Tuesday we met with the coordinator at the CDRC Pediatric Hearing Amplification Service at Doernbecher Children’s Hospital. This is our first step in beginning the process to determine if Ben is a candidate for a Cochlear Implant. Just because he is deaf does not automatically make an implant possible. He will need to undergo several tests to determine the cause of his deafness and to be certain an implant will be effective.

The first test Ben will need is a CAT scan (x-ray) and MRI of the inner ear. These will show the condition of the cochlea and the auditory nerve. The doctor will be looking to see if the cochlea has any ossification (bony growth) or is malformed. Neither of these conditions would prevent an implant, but may determine how well the implant will work or be a deciding factor of which ear to implant. Though the implant is placed within the cochlea, sound must be transmitted to the brain via the auditory nerve. If this nerve is not intact or is not present, the implant will not be possible in that ear. Ben will have his MRI and CAT scan on April 3rd.

We also discussed possible causes for Ben’s deafness. Unfortunately, congenital deafness can be one of the symptoms of a syndrome, with other symptoms showing up later in life. The doctor suggested our first step be genetic counseling. A very common cause of congenital deafness is a genetic condition called Connexin 26. Connexin kids tend to do very well with cochlear implants. Their hearing anatomy is all present and intact. The only problem is that the hair cells along the basilar membrane, which translate mechanical sound energy into nerve impulses, are defective, due to a lack of Connexin protein. Finding out Ben has Connexin 26 would be positive.

When Ben is older, around six or seven months, he will undergo some cognitive testing. These tests will be used to help determine the possible results of the implant. If Ben has developmental delays, speech and language may be harder for him to grasp, even with an implant.

Doernbecher Hospital performed about 55 implants last year. All of those parents went though this process. All of those parents survived. All of those kids are now hearing. We are riding on the optimism they provide.

Happy Bear

Ben's Aunt Joy went on a mission to find a toy a deaf baby would enjoy. She hit the jackpot with the "Happy Bear". The flashing lights on it's tummy keep Ben starring for minutes (hours in baby time!).

Well, come to find out, Ben can enjoy the songs of the "Happy Bear" also. Last night we put the speaker of the bear next to Ben's left ear (the better ear). I think he hears it! What do you think?

What is a Cochlear Implant?

A Cochlear Implant opens up a whole new batch of questions. What exactly does a Cochlear implant do? Does it fix his deafness? What does "activated" mean? Will he wear something noticeable?

A little of what we learned....

Ben will always be deaf. The implant is like a more powerful hearing aid, although it is very different from a hearing aid. Instead of simply amplifying sound, it creates signals that the brain interprets as sound, making it ideal for people who have little or no hearing. Cochlear implants use a device called an external speech processor, worn outside the ear, to capture sound and convert the sound into digital signals. Those signals are then sent to a surgically implanted electronic receiver inside the head, which tells the implant to stimulate the electrodes inside the cochlea (inner ear). The brain then recognizes these signals as sound. Those that are qualified for an implant must have a certain degree of hearing loss (meaning "bad") and not get any use out of a hearing aid. I used to see a person with hearing aids and ask myself, "Why don't they just get that surgery done to 'fix' their hearing." I now know that most that wear the hearing aids, get enough sound from them, so that there is no need for an implant. Ben's left ear may be like that.

Normal hearing is about 10-20 decibels. Ben is considered profoundly deaf in the right ear, hearing between 90 and 110 dB (depending on the frequency, meaning high pitch sounds vs. low pitch sounds). He is severe in the left, hearing between 70 and 90 dB.



The Cochlear Implant surgery can only be done after Ben is 12 months old and has worn hearing aids for six months. He has to show no benefit from the aids. We hope he will have good hearing with the aid in the left side, meaning Ben will most likely only qualify for an implant in the right ear. The implant is not simple-- it has an inner device that has to be surgically implanted. He wouldn't hear with just that, he would have to attach the outer device-also called the processor (it is a magnet that attaches to the magnet on the inner device) in order to receive sound. So at night or in water, when we would take the processor off, he wouldn't hear anything.

"Activated" basically means "turned on" or "hooked up". Ben would be activated several weeks after the implant surgery. At that appointment he would receive the processor and will begin hearing in "surround sound!" :)

Here are some pictures for you to see what the implant looks like.



This is what is implanted inside...


This is the speech processor that is seen on the outside...



Isn't technology amazing!

Special Education

As a teacher, I am familiar with Special Education. I have often said, "Why would a parent be resistant of an education plan specifically tailored to their child's needs?" But, now I am on the other side of the table. My child needs Special Education. As we checked the boxes and signed our names, a small twinge of panic went through me. Emily Perl Kingsley explained my feelings well.


It's like going to Holland...

"When you're going to have a baby, it's like planning a fabulous vacation trip to Italy. You buy a bunch of guidebooks and make your wonderful vacation plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very, very exciting!

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The flight attendant comes in and says, "Welcome to Holland."

"Holland??" you say. "What do you mean, Holland? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They landed in Holland and there you must stay.

The important thing is that they haven't taken you to a horrible place, full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guidebooks. And you must learn a whole new language. And you will meet a while new group of people that you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you have been there for a while and you catch your breath, you look around, and you begin to notice that Holland has windmills. Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy, and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say, "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever go away, because the loss of that dream is a very significant loss.

But if you spend your life mourning the fact that you didn't go to Italy, you may never be free to enjoy the very special, the very lovely things about Holland."



We will go to Italy. A change of flight plans will not stop our family.

Questions

It's been amazing to see people come out of the wood work with support. Just days after Ben's diagnosis, he had a team of people with ideas and previous experience with babies like him eager to help. We have been filled with confidence that he will hear soon.

Today we met with a good friend of Grandpa Paul. She is a Pediactric Audiologist at the ESD, where Paul works. We want to understand Ben's hearing loss and learn about the options for his hearing.

We have researched Cochlear Implants and hearing aids. Our hope, boosted greatly by our meeting, is that Ben will have normal hearing in his left ear with the use of a hearing aid.

We made ear molds and orderd hearing aids. April first cannot come fast enough!