Time for an update.

UPDATE: I felt the need to come back and update this post as we have learned that Audrena's Usher Syndrome diagnosis was a mistake. Here is the post where we learned she does not have Usher Syndrome.

It has been awhile, so I thought it would be a good time to update! We have just been so busy! Audrena is really taking to being bilateral. She knows when the headpiece falls off, and she either puts it back on by herself, or she comes to us and pats her ear to let us know. We do have the alert beep as well, so we usually know before she reaches us. However, we are trying to teach her to be independent with her implants.

We did have some question as to whether she was getting much from the new implant yet since we're still on the first set of programs. Daddy took Audrena to her programming appointment yesterday, and she repeated "bird" to him after he had said it. That was with just the new implant turned on. So it's good to know things are at least starting to sound ok to her. Sometimes it does take awhile for the brain to adapt to the new implant.

They took her into the sound booth, and of course she was just not into it. Audrena rarely cooperates in the booth. She would rather be exploring. Even with that, she did cooperate just enough for them to determine that she is at least at 35-40 decibels with just the new implant on her first sort of generic MAPP, 30-40 with the first implant, and 30-40 with both. Dr. M. from USD has always said (and others have told us as well) that 30-40 decibels is sort of the best expectation for cochlear implant recipients. We do know it's possible to do better. We know plenty of implant patients who hear at 15-25 decibels, and that is our goal for Audrena once she gets old enough to give the Audiologist some verbal information about how she is hearing during programming. Also, we have gotten booth tests with her first implant that showed 20-30. That, combined with what we see at home, are enough for us to believe that she is hearing better than yesterday's test suggests. Both of our Audiologists (Boys Town and USD) have said she could very well be hearing those softer sounds, but she is just not paying enough attention to them to be motivated to turn away from her toy when she hears them.

Also, I forgot to include in some of my previous posts that Audrena heard birds (this was before the second implant)! She heard them before she saw them! We were at the police department's Bike Rodeo at the high school, and there were finches in the rafters of the lunch room. We were eating, and suddenly Audrena looked up (way up) at the birds, pointed, and said, "Tweet tweet!" She had to have heard them because she had not been looking up at all. She was eating. When we first found out that Audrena was deaf, I would take her for a walk, listen to the birds, and wallow in self-pity that my baby girl would never hear those beautiful outdoor sounds like birds chirping. Birds are generally about 15 decibels on the audiogram (although the chart below shows them even softer), so I never expected that she would hear them with implants either. That moment in the high school lunch room was a really defining moment for me, one of those WOW moments that just takes my breath away whenever I think back on it.

Here is an audiogram chart so you can see what I am talking about. The yellow area is the speech banana, or the range that speech sounds fall into.

From firstyears.org

Another little hiccup to report...we may be headed back to the operating room. Yesterday, Dr. M. couldn't find the tube in Audrena's left ear. That's the one Dr. L. inserted in March. Granted, her tools may not magnify quite as much as an ENT's tools, and those tubes are tiny. But she is good, so I trust that if it was there she would have found it. We have an ENT appointment tomorrow morning to check it out. If it's not there, then Dr. L. will need to insert another one. It's such a minor procedure, but it would be the 6th ear surgery and the 7th time under anesthesia for Audrena. And there is always the possibility that she will need a tube in her right ear in the future. So if you are the praying type, say a little prayer that the tube is in place tomorrow, although I don't have much hope that it is. Next week is also our appointment at University of Iowa with the Pediatric Ophthalmologist who will monitor her Usher Syndrome and the doctor with the genetics laboratory that conducted the genetic testing. So please keep Audrena in your prayers that everything turns out well there, too!

On a more positive note, Audrena is really on a role with her speech and language, and she is making progress with her balance! She is saying new words all the time and starting to put them together. She is beginning to understand that there are different words for the same thing, and starting to use them interchangeably. For example, "woof woof," "puppy," and "dog." She also says, "Look at that," or "Look at those." Here are a few of her favorite words/phrases lately.

Look at that/those!
What's that?
Where go? (Where Daddy go?)
Get down!
Good job!/Very good!
NO!
Stop!
Mine!
And when you call her name, she answers with, "What?"
She has also learned "Kelton, "Ana" (for Reyana), and "Zach" (my nephew).

Although we always question whether we could be doing more for her, or whether there is something we are missing, we are so pleased with Audrena's progress!

Speech Banana Revisited

Here is a link to my original post about the audiogram and the speech banana. These are two very important things to Audrena's hearing journey. Today, I can say that Audrena cooperated beautifully in the sound booth at USD's Speech & Hearing Center, and we got a great audiogram!

Audrena tested at 20-30 decibels across the frequencies! We have been told that a cochlear implant patient will never achieve normal hearing, and typically they will be in the 30-40 decibel range. Now, I do know several who hear at 15-25 decibels with their implants, so it's entirely possible. However, we are thrilled with today's results! Thrilled! Audrena has achieved the speech banana levels! This means wonderful things for her speech and language development.

Through all the ups and downs of this journey, one thing has remained constant. The doctors, Audiologists, and other professionals at Boys Town National Research Hospital, and the doctors and clinical grad students at USD Scottish Rite Speech, Language, and Hearing Clinic have been top notch. Even if Audrena had not achieved such great results, we would still be able to say that with confidence. They have been nothing short of fabulous, and we would recommend them to anyone! But because they have changed our baby's life and opened up a world of opportunity for her, we will forever be grateful to them and to Advanced Bionics. If I sound like I'm delivering a sales pitch, well maybe I am. Just a little. Audrena is a success story in our eyes, and they have changed her life, so they deserve some public recognition.

Starting to think God works through Facebook, too.

We all know the Lord works in mysterious ways. But through Facebook? Why not! When we first posted this blog on our Facebook status updates, we had a flurry of good thoughts from friends and family. But a couple people actually put us in contact with cochlear implant families that they knew. Through Facebook messaging, this is what we have learned.

I have a relative whose granddaughter has a cochlear implant. She is 11, now in mainstream public school, on the honor roll, plays the violin in school, and won the school spelling bee. It has long been said that cochlear implant patients do not have the same ability to appreciate music as their normal hearing peers. Recent research is showing that music therapy is actually very good for people with cochlear implants. It seems that this little girl enjoys her music just fine!

Randy's friend put him in contact with her friend who has 4 daughters. The oldest is 13 and has bilateral cochlear implants. Her first implant was done at 4 years old, and her second was done at 8 years old. She went from being able to understand 12% of what was being said to her and an audiogram of 95 decibels to having 25 decibel hearing, which is in the normal range. Her mother says she is a straight A student, and people cannot tell she is deaf unless she shows them her implants. Her hair hides them completely. This couple's youngest daughter's hearing tests showed relatively the same hearing as the oldest child's. She is 16 months old and will be having bilateral cochlear implant surgery on May 7th. We are saying prayers for her, and we just know she will follow in her older sister's footsteps on the path to normal hearing with her implants.

For reference, here is some information about decibel levels from the California Ear Institute.

Whisper	30
Normal Conversation, Dishwasher	60
Vacuum Cleaner	70
Subway, Busy Street	80
Lawn Mower	90
Chain Saw, Snow Mobile	100
Rock Concert	120
Jackhammer	130
Gunfire, Jet Engine	140
Rock Music, Peak	150

Audrena's sedated ABR testing showed no response at 120 decibels. According to this chart, she could attend a rock concert, and she would not hear anything.

I got sidetracked a bit. The point of this post is that God does have a way of putting people in our path at the right moments. Apparently He is also keeping up with modern technology, working through Facebook as well! We love hearing about people who have done so well with their cochlear implants, and it's nice to talk to parents who have been through the process.

The Second Opinion

Last Thursday evening, we dropped Reyana and Kelton off at Grandma and Grandpa's house and traveled to Omaha with Audrena. We had booked a hotel room at the Sheraton since we would need to be at Boys Town by 5:30 am on Friday. The Sheraton offered a Boys Town patient discount, and it has been newly remodeled. Let's just say we highly recommend it. The room was clean and comfortable, with very nice furnishings, and it was affordable with the discount.

We arrived at Boys Town right at 5:30 and were checked in immediately. They took us to our room, where we put Audrena into a little pair of hospital pajamas and grippy socks. Then we went to the toy room to pass the time until they were ready for her.

At a little after 7:00, the anesthesiologist and nurse came to take Audrena. Another staff member brought us some breakfast. We waited for the ABR to be finished, not really expecting the results to be any different from the first ABR, even though Audrena would be under anesthesia for this one.

After awhile, Dr. L. came in to tell us that the ABR showed no response. That means they maxed out their equipment at 120 decibels, and Audrena couldn't hear any of it. She has profound hearing loss. In simple terms, she is deaf. I handled that news fine. I had prepared myself for it. Then Dr. L. explained that they would be taking her for an MRI next to check for the presence of an auditory nerve. Immediately we felt like he had yanked the rug out from under us. What?!? How could it be possible not to have an auditory nerve? I had never read that in any of my research. I vaguely remember asking what option we might have if she did not have an auditory nerve. He explained that there are limited options. They are just starting to do things like brainstem implants, and the only doctor doing them is in Italy. My world started to spin at that point. I wanted to throw up. I'm a planner. I have to know what is going to happen next. In this case, I was not prepared.

Dr. L. left the room, and I cried. Randy and I hugged each other and tried to convince ourselves it would be ok. After all, my cousin's daughter is deaf, and she is graduating high school, having lettered in sports, etc. She does wonderful. We know other people who cannot hear, and they do great in life. But they are not our baby girl.

Shortly after I finally got myself composed, the Audiologist came in to speak with us. She brought a hearing aid with her so we could see what Audrena might have. They typically do 3 months of hearing aid trials before cochlear implants for a few reasons. It gets the child used to something being on her head, and it stimulates the auditory nerve to some extent while the pre-op and insurance approval process takes place. I remember asking her what the chances were that Audrena would not have an auditory nerve. She said she didn't have any statistics, but she knew it was very rare. I cried again. Then she offered to forward the records to the Birth to 3 program and to Dr. M. at USD. She knows her from college days and had good things to say about her, suggesting that it really would save us a lot of time to work with Dr. M. on things that USD was equipped for. I signed some release forms, and off she went.

At some point the Audiologist came back with a cochlear implant packet. It had brochures and paperwork for us to fill out. We hesitatingly took that to mean that the MRI had shown an auditory nerve.

The nurses brought Audrena back to us, took her IV out, and told us we could take her to the toy room while we waited for Dr. L. I snuggled my girl and hoped for the best.

Dr. L. came in and said the neurologist said the auditory nerve was normal. He was not convinced it looked quite as it should, but it could have been a difference in computer screen contrast. He would have to call the neurologist and consult with him some more about it. They had also done the CT scan, which explained why things took a little longer.

Then he explained that Audrena has a larger-than-normal cochlear opening, which will cause a "gusher" in surgery. As he drills through the mastoid, spinal fluid will gush. He will need to pack it with tissue to seal it up. This is something he is used to handling, and he can take care of it. However, it will raise Audrena's risk for catching bacterial meningitis throughout her lifetime. As Randy got completely hung up on the word "gusher," I had it together enough to ask how vaccination affects that risk. Dr. L. said it does bring the risk down some, and he explained that as she is now, she's already at a higher risk due to that malformation. We were both trying to take it all in and neglected to ask for statistics or percentages so that we could assess what we're facing.

Dr. L. said Audrena also has smaller-than-normal mastoids, but again, he said he is used to dealing with that. All of this combined, though, means that he will not do a bilateral implant surgery. Audrena will have to have two separate surgeries, about 6 or so weeks apart. He wants to do the first one and assess how well she does with the implant before proceeding with the second surgery.

He did say that we could expedite hearing aid trials since we know they will not help. Our biggest delay, he said, will be getting insurance approval. He would get us scheduled to meet with the cochlear implant team in the meantime.

He told us that Audrena has a "very abnormal vestibular system," which means that her balance is bad. He said her vision will compensate for that, allowing her to walk, but she will need a night light or a flash light in the dark where she cannot see. Again, I had the rug pulled out from under me, and the only thing I could think to ask was, "Will she be able to do normal kid things like riding a bike?" He didn't know if she would be able to do those balance-dependent activities like bike riding. Randy had the presence of mind to ask if physical therapy would help, and Dr. L. did recommend it.

Dr. L. also referred us for genetic testing since we now know that Audrena's ear conditions are congenital, meaning she was born with them.

We left Boys Town feeling completely defeated. What if the neurologist was wrong, and her auditory nerve was not normal? How much benefit would she receive from cochlear implants? How huge of a risk would she be for meningitis compared to now? We were not prepared for two surgeries. And what about her vestibular system? How bad will it be for her? I was just sick, and the entire way home, I just wanted to throw up. Why couldn't have the MRI just been normal?