Sedated ABR

This week Ben had his first sedated ABR (Auditory Brainstem Response). The ABR evaluates how well sounds travel along the hearing nerve pathways from the ear to the brainstem (i.e how well he hears).

The ABR monitors changes in brain activity as repetitive clicking sounds are sent through the ear. The audiologist places four small stickers on Ben's head, chest, and ear lobes, connected to leads going into a computer. Sounds are be sent through an earphone to each ear separately while a computer analyzes the changes in the brain wave pattern in response to sounds.

In order for the results to be accurate, the brain needs to be relaxed. When Ben was younger, he was able to sleep peacefully for the ABR. Each time he would twitch or suck on his pacifier, we would see a change in his brain waves on the computer screen. Now, to have Ben's brain relaxed enough and for long enough, he needs to be sedated.

Ben doesn't mind the process at all- except that we had to starve him for 6 hours before the sedation. Nurses put numbing cream on his skin in several places so the I.V wont hurt as much.

A Sedation team administers the medication and Ben falls peacefully asleep. The hospital wont allow parents to stay with their children once they are fully sedated. Mom and dad have a few hours to wonder, eat and worry.

According to the ABR, Ben's hearing has not changed since his last ABR three months ago. We had a small bit of hope (or wishful dreaming) that his hearing would have improved somehow. But, we are also glad to have the left ear working just as strong. We know that with EVA
hearing loss is progressive. We would hate to have the hearing he currently has decline before he is implanted. In this case, December seems so far away.

Water Fun!

Ben loves his bath. He splashes, gurgles, and tries to eat the wash cloth. But, he can't hear any of it because he has to have is hearing aids off.

So....we had some water fun outside.

Some splashing, no gurgling.

Ben spent most of his time looking at his feet.

I guess toes in the water look different with hearing aids on.

Don't worry Nadine. The aids are in the dryer tonight :)

One Step Closer

This week we headed up to OHSU to meet with their Genetic Counselors. Our goal is to find the cause of Ben's hearing loss.

After giving our family history (no congenital hearing loss on either side), Ben was given a physical exam. He is right on track with his growth and is meeting all the age appropriate developmental markers. We told the counselors about Ben's ABRs and how his MRI and CT scans were normal.

That's where we were stopped short. The counselors had reports from the scans we had not yet seen. Apparently, Ben's MRI and CT scans were not normal. The doctor noted that Ben has enlarged vestibular aqueducts.

So, what does that mean? It's hard to explain, and we are still learning, but this is what we know so far:

• Vestibular aqueducts are narrow, bony canals that travel from the inner ear to deep inside the skull. The aqueducts begin inside the temporal bone, the part of the skull just above the ear.


• A vestibular aqueduct is considered enlarged if it is greater than 1.5 millimeters in size, roughly the diameter of the head of a pin.


• Scientists also are finding that five to 15 percent of children with sensorineural hearing loss, or hearing loss caused by damage to sensory cells inside the cochlea, have EVA.


• The presence of EVA can be a symptom of a genetic disorder called Pendred syndrome, a cause of childhood hearing loss. Approximately one-third of individuals with EVA and hearing loss have Pendred syndrome. With Pendred syndrome, the hearing loss is progressive, which means that a Ben could have less hearing over time.


• EVA may also be linked with balance problems.


• No treatment has proven effective in reducing the hearing loss associated with EVA or in slowing its progression.

Through my many hours Googleing and reading posts on my favorite cochlear implant connection, cicircle yahoo group, I have found that quite a few kids have EVA and receive cochlear implants. Whew! Although there can be some extra complications, implantation is possible.

After letting our new discoveries sink in for a bit, we are actually relieved. Often, children with congenital hearing loss have other physical or developmental challenges that surface as the child ages. With the findings of the EVA we can let many of our fears go.

Our next step is to have Genetics counselors at OHSU test Ben for Pendred Syndrome. We'll worry about that when the time comes.

Let the games begin!

Look what Ben found!

These things are so cool!

And tasty?




Mom has her own plan!

Salem Heights

This week I had the opportunity to visit Salem Heights Elementary School. This is where deaf and hard of hearing children in three local counties are able to come together for mainstream education. The school provides sign language services and speech and language therapy specific to the kid's needs. My mom actually taught kindergarten there several years ago.

Quite a few of the kids at Salem Heights have hearing aids or Cochlear implants. I met with several kids who were excited to tell me all about their implants. They took off their processors and coils, proudly showing me how they hear. To these boys, Cochlear Implants and hearing aids are just a part of their lives. As natural as the shoes we put on each day.

I had conversations and played games with children who were deaf, just like Ben. It was enlightening to have a vision into Ben's future. I could picture him giggling with a group of boys or arguing over the rules of a game.

I brought a book about Ben's first day with his hearing aids to show the kids. One fifth grade boy, with an implant and a hearing aid, carefully read through each page of the book. He finished reading and said:

"What is your baby's name?"

"Ben"

"Is Ben going to get an implant too?"

"Yes. I think so."

"He will say: Thank you Mommy for letting me hear."

"Really?"

"He will say it was the best day of his life."



I smiled the whole way home. My baby has such a bright future.

HB 2589

I just heard that HB 2589, the children's hearing aid bill, will be headed to the House floor with bipartisan support from the Health Care Committee. Only one member of the committee voted no, Ron Maurer, and it was noted that he very rarely supports mandates. This is great news! Within the next week or two, we should find out when the bill is likely to be up for a vote of the full House.

Anyone who is interested in contacting their state representative and urging his or her support of the bill is welcome to do so.

To find your representative:
http://www.leg.state.or.us/findlegsltr/home.htm

Scroll down to State Representative and the phone and email for your representative will be listed.

Thank you very much!

Growing Pains

Our little boy is growing up! He weighs almost 12 pounds and has grown three inches since birth! (If you know our family, you will understand how remarkable this height growth is!) He has outgrown his newborn clothes, rolls from tummy to back and back to tummy, holds his head up, and has even had his first cold- A true sign of childhood.

Ben's ears are growing at a rapid pace as well. He is already on his third set of ear molds, the soft plastic portion of his hearing aid that sits inside his ear. Ben's audiologist makes impressions of his ears and sends them to a factory. In two weeks ear molds return. Ear molds that are already too small for Ben's growing ears!

The microphone, on the top of the hearing aid, picks up sounds and sends them to an amplifier that makes them louder. After sounds are made louder, they go through the earhook to an ear mold. The ear mold's job is to hold the sound inside Ben's ear.



Since Ben's ear molds don't fit tightly, the sound leaks out- feedback. If you have ever been near a person whose hearing aids squeal, they have poor fitting ear molds. Ben's sound like bees buzzing around is head. Sometimes I can hear my voice coming from his ear...kinda creepy.

Here he is rolling like a champ! As he rolls, he loosens the ear mold. Ah, feedback!

Ben Practices Politics

We first learned Ben had a hearing loss through the Newborn Infant Hearing Screening. Since 1999, when the Hearing Screening began, hearing loss has been found to effect 1 in 22 infants. Oregon mandated the Hearing Screening as a way to insure early identification of hearing loss. Early identification and intervention can prevent psychosocial, educational, and linguistic repercussions. Infants who are not identified before 6 months of age have delays in speech and language development. Intervention at or before 6 months of age allows a child with impaired hearing to develop normal speech and language. However, when it comes to interventions, often times hearing aids, health insurance companies lack support.

At more than $3,000 apiece for hearing aids and no help from insurance, many families are forced to make financial sacrifices or have their child go without hearing aids. Unfortunately, the identification of a child's hearing loss becomes a financial burden. Families become aware their child needs hearing aids, but are left without means for attaining them.

The issue of hearing aid insurance is heating up, and several states are responding with bills to require coverage of hearing aids for some people. This Friday House Bill 2589 was proposed to the House Committee on Health Care at the Oregon State Capital. The passing of this bill would require insurance companies to pay a portion of pediatric hearing aids.

Ben and I attended the committee meeting, along with several experts in the field of pediatric hearing loss. Ben did a fantastic job modeling his hearing aids for the committee while I told our story. Hopefully we tugged at some heart strings. This is what I said:

Finding out your baby is deaf is like being hit with a pile of bricks. Invisible bricks. He was warm and soft and snuggled in my arms. He looked perfect. How could something be wrong? But, at twelve hours old, I was told he failed his hearing test. Impossible. The nurses must have botched the test. But still, through the denial, I felt pain coming from somewhere.

At two weeks old, we took our baby to get another hearing test. He failed. The dull, throbbing pain of those invisible bricks returned. I could have sworn he flinched when we banged the pots and pans over his head desperately tying to perform our own hearing tests.

After a visit to an ENT, a different audiologist, and two more hearing tests we had our answer. Our son is deaf. All the “I love you” whispers and lullaby tunes had truly gone unheard. He’ll really never know my voice? The bricks pushed so hard I couldn’t breathe. That long, deep exhale of relaxed contentment, of a world full of bright futures and sunny skies that exists in the world of our son were crushed by the dark news of which I had just heard.

I’m his mother. My job is to fix what goes wrong in his world. I hung on the glimmer of hope provided by the audiologist: “He may be able to hear some sounds with a hearing aid.” Hearing aids would be the answer. I knew it.

I researched and found that with my son’s severe loss, he would need high powered hearing aids. Expensive hearing aids. Our insurance doesn’t cover hearing aids. We couldn’t deny our child a chance to hear. Sacrifices could be made. We have a car we no longer drive. Someone might buy it. My husband could work longer hours. I could cut my maternity leave short. Those bricks grew heavier.

We were lucky. Blessed. The Willamette ESD loaned us hearing aids to see if our son responded to sound. On April 1st, at 9 weeks old, my son tried on his first pair of hearing aids. As we snapped the battery door shut, we held our breath. I didn’t know what to say.

“Ben it’s mommy.” He opened his eyes. He looked at my face. He heard my voice.

My son wears his hearing aids whenever he is awake. He smiles more. He enjoys Patty Cake, and Twinkle Twinkle. We listen to Mozart. I can soothe him with my voice when he cries and he hears my goodnight whispers.

If we were not able to borrow hearing aids, my child would be missing these sounds. Soon we will need to buy Ben his own pair of hearing aids. Unfortunately, the high cost of the hearing aids creates a barrier for families like ours. Our insurance does not cover any of the expenses associated with hearing aids. Sacrifices will need to be made. It is not fair that Ben should have to get less expensive, less effective hearing aids, or our family have financial constraints.

We pay for health insurance to cover our family’s medical needs. My child’s eyesight is covered. Why not his hearing? Should he be denied the opportunity to attend regular education classes, develop normal speech, and listen to Mozart?

MRI and CT

Today we spent a long day at OHSU. We arrived at 7 am to have Ben sedated for a MRI and a CT. The nurses began by putting a cream on Ben's hand and feet to numb the skin over his entire body. After forty minutes, the cream worked and Ben was ready for his IV. He was dressed in a hospital gown, given a light sedation medication, hooked up to a blood pressure monitor, and given oxygen. Once he was fully sedated, the nurses wheeled him away for the scans. In an hour and a half a groggy little boy returned. He took a long time waking up and orienting himself.



We also met with Dr. McMenomey, OHSU's cochlear implant surgeon. He has preformed over 1,000 implant surgeries and averages around sixty each year on children. Dr.McMenomy feels very strongly that a child should be around a year old before getting an implant. After learning more about the surgery itself, we agreed.

The actual surgery takes 2 to 4 hours, a long time for babies to be sedated. It involves securing the receiver/stimulator under the skin and inside the skull, and then threading the wires containing the electrodes into the spirals of the cochlea. The internal portion of the implant includes a strong magnent for ataching the external portion.

To secure the receiver/stimulator, the surgeon first makes a small depression in the temporal bone (the skull bone that contains part of the ear canal, the middle ear, and the inner ear). Next, he opens up the mastoid bone behind the ear to allow access to the middle ear. Then, a small hole is drilled in the cochlea and the wires containing the electrodes are inserted. The implant is then secured and the incision is closed. Swelling is minimized by applying a pressure bandage. Babies usually need to stay over night, but adults can go home the same day.


Two to four weeks after surgery, the sound and speech processor is matched with the implant and is programmed and fine-tuned to meet the child's individual hearing needs (This is the portion of the implant you see on the outside of the head.).

After reviewing Ben's scans, Dr. McMenomy feels Ben is a perfect candidate for a cochlear implant! This means Ben could be implanted before Christmas and hearing before his first birthday. What great gifts!

Ben can hear!

Today Ben got his hearing aids!

Ben was asleep while his dad put on the first hearing aid in his left ear. When he closed the battery door and turned the aid on, Ben's eyes opened. He looked around the room with wide eyes. He kept looking to left to find where the sound was coming from. It was wonderful! Our little boy heard our voices for the first time!

Look at those smiles!


He loves to hear!

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!

Diagnosis

How did we find out?
Ben was born on January 13, 2009. It was a terrific day -- everything went smoothly, and I was so thrilled to hold him in my arms.

That night, he was given a newborn hearing screening. He failed. Strictly speaking, he was "referred" -- meaning that the machine showed no response and he would need follow-up testing. (They don't like to call it "failure".) The nurses assured us that there was nothing to worry about. He might still have some gunk in his ears from birth. He probably squirmed and disconnected the electrodes. We had plenty of other things on our minds and we were more than willing to believe their assurances, so we took their advice and didn't worry.

The next night, they tested him again, and again he was "referred". Again they told us not to worry, but we asked my doctor, every nurse, and the on-call pediatrition for advice. I made an appointment with an audiologist for more testing. In the meantime, we performed our own hearing 'tests' by clapping and banging pots and pans over his head. We tried to convince ourselves he was responding.

At the audiologist's, Ben took a test called an ABR (auditory brainstem response test). He had to sleep for three hours while the test was preformed. Once again, no response. But, we were told Ben's ear canals were collapsed and it was probably nothing to worry about. They might 'pop' back in a few weeks. We should come back then.

I didn't want to wait. I took Ben to an ENT the next week. He insisted his canals were not collapsed. This was not the problem. By now, we were no longer in denial. We knew something was wrong.

At this point it seemed like Ben had some sort of hearing loss. We went to OHSU for a more comprehensive ABR. After two visits, six hours of sleeping, and two long tests we had our answer.

It wasn't good news.

We found that Ben is profoundly deaf in his right ear, and severely-to-profoundly deaf in his left.

We knew he was deaf, but to have it confirmed and to have it be so bad....

We drove home in silence, both thinking the same thing: some how Ben will hear.