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!

IFSP Meeting

We had our IFSP meeting yesterday.

Audrena showed off a little for her team. She walked between me and Daddy, holding our fingers, many times. The physical therapist was happy to see that! We decided that Audrena should have physical therapy once a week for 30 minutes. Her 6 month goal is to play while standing and to walk 10 steps independently. I know the point of the goal is to be realistic, but it seemed a tad silly to me. I feel like she'll reach them much sooner than 6 months. But I do know that her balance will cause challenges. In the meantime, we can adjust the schedule as needed, so if we feel like she needs more sessions we can work that in. I had not met our physical therapist at the initial evaluation, so I didn't know what to expect. I'm happy to report that I think he'll be good to work with.

The early childhood education teacher recommended evaluating Audrena once a month using the HELP method and set some goals for her as well. We feel confident with those things as well.

The speech therapist from the hospital was able to be there as well. We felt like speech therapy once a week until she has her first implant activated was a good approach to take. Then from there we can increase to twice a week. I'm thinking more like 3 times a week would be good, but we'll cross that bridge after we speak with the professionals at Boys Town next week. Our team was open to us consulting with Boys Town and switching things up as necessary. We were glad to hear that, although we don't have a certified Auditory Verbal therapist in our area, our speech therapist is educated in it. That was one thing I was not sure about since I admittedly don't know a whole lot about speech therapists' educational training.

We did tell the team that, although it may be a bit out of the norm, we want AV therapy and possibly down the road when Audrena's language is caught up to her peers we would work in sign language. I'm not sure that really fits any therapy model we've seen yet. I think that usually a family would pick just one method, which is essentially what we're doing with AV therapy, but I'm not sure that other families work in sign language at a completely different point in time. The fact is, we don't know if we will even need sign language. With bilateral implants, the chances of both implants failing at the same time are probably pretty slim, and there are waterproof implants now, so she can wear them while swimming. We do know absolutely, however, that our main focus over the next few years will be developing Audrena's ability to speak normally and to really learn to listen with her implants.

Yesterday was also the first time we met our SD School for the Deaf outreach person. I had talked to her on the phone a few weeks ago, and I had a good impression then. She stayed after the meeting for quite awhile, just talking with us about the implants and different things. She doesn't get many clients who use Boys Town. A lot of them go to the Mayo Clinic (which was one of our options, too) or just use one of the two surgeons in Sioux Falls. We shared our experiences with her, and we can truly say that we do not have one bad thing to say about Boys Town up to this point. Every single person there has been nothing short of amazing. When she finally did leave our house, we had a great feeling about her. We think she will be really good to work with!

So overall, we feel like we're on the right track. While we're at Boys Town, we hope to be able to go over things with them a bit to determine if there is anything we need to change. Next week is truly the beginning of an intensive journey for us. We began this journey several months ago, but it's about to get more crazy with many, many therapy appointments, pre-op appointments, the surgeries themselves, activations, mapping appointments, etc. The next 3-4 years will be hard, but this is one time shot. We cannot afford to screw up. These coming years will be a sacrifice in some ways for us, but they will affect Audrena's entire life. Yes, this is the beginning of building the foundation for her whole life. We are dedicated to giving her the best foundation that we possibly can.

Just a Little Research

I have a few interesting research items to share. I hope I'm within copyright laws by sharing these, but I have linked directly to the websites where they came from, so I think I'm good. Both deal with research coming from the University of Wisconsin, Madison, WI, an important cochlear implant research location.

This is just a snippet of the full article, but it does highlight the reasons we have chosen bilateral cochlear implants and why it is important for Audrena to get her implants ASAP. Earlier is better, especially before age 2.

Deaf children: Study shows significant language progress after two cochlear implants

“It’s a huge success to see these children making such strides in language acquisition,” says Christi Hess, a Ph.D. student in communicative disorders. “Many, after as little as one or two years with the implant, have language scores within the normal range, especially those who got the implant before age two.”

Thousands of children get cochlear implants each year, and the surgery is done at an ever-younger age, says Ruth Litovsky, professor of communicative disorders and surgery/otolaryngology.

It’s known that implants made at a younger age deliver results more quickly, and that a second implant helps children both locate the source of a sound and understand speech in a noisy room. But until now, it was not clear if the second implant would improve understanding of spoken language.

“The most exciting finding is that having two implants does correlate with an improvement in receptive language,” says Hess.

“Many of these children go through an ‘Aha!’ moment, a revelation, when the inputs they are trying to process suddenly start to make sense,” says Hess. “They have not had a framework for organizing these stimuli, but at some point, their brains start to make new connections and they begin to understand the auditory world.”

There are several reasons why two implants could be better, says Litovsky, who is director of the binaural hearing and speech lab at the Waisman Center and has studied cochlear implants for 12 years.

“As good as cochlear implants are, they provide input that is degraded, and the input to each ear is imperfect. The hypothesis is that with two implants, the children receive ‘two looks’ at a signal. The success of surgery in the two ears can vary, so the electrical impulses reaching the brain from each ear are not identical, and getting a signal to both ears gives another opportunity to sample and understand the auditory world," Litovsky says.

The children, who ranged from 4 to 9 years of age at the time of testing, had had at least one year of experience with the first implant and got the second implant by age 6.

There are several reasons to study language acquisition in children with “electric hearing,” Litovsky says. “This helps us understand the incredibly complex process of converting pressure waves in the air into sound and then into meaningful information, but this kind of research also helps parents make momentous decision about implants for their children who are deaf.”

The surgery is often covered by health insurance, but parents must decide whether and when to implant and whether to do both ears. Understanding the possible benefit of a second implant becomes a critical component of the decision process.

Again, this article highlights the benefit of bilateral implants. Studies continue to show that children with two implants, and who have enough therapy and language exposure, do very nearly as well in life as their normal hearing peers.

Helping To Hear: Cochlear Implants x 2

Little Isabella Rosa is a bright, happy 6 -year-old girl and she's also almost completely deaf. Her dad couldn't believe it when tests showed his little girl would never hear.

"We were in denial, we thought that there was something with the test, how can they tell? She’s just a little baby,” Vasile Rosa, Isabella's father, told Ivanhoe.

But Isabella can hear now with the help of not one, but two cochlear implants. A new study from hearing scientists, found that implanting one implant in each ear increases the benefit.

“We see that they’re better able to understand speech in quiet and also in the presence of noise, and we see a general ease of listening,” Ruth Litovsky, Ph.D, a Hearing Scientist at the University of Wisconsin-Madison explained.

Researchers found that two implants can help restore a child’s ability to identify which direction a sound is coming from, a skill that helps deaf children acquire language and improve their quality of life.

“Being able to simply listen without having to constantly look at what you’re listening for, improves your ease of listening in a way that is very important for everyday environmental functioning,” Dr. Litovsky said.

Scientists played a moving human voice through speakers placed at different points around a child. Then, they asked the child which direction the voice was coming from. Most children could tell where the voice was coming from until the voice was directly in front of them.

“You’re giving somebody two opportunities to be able to hear well,” Dr. Litovsky said.

Testing showed that most children performed almost as well as children with normal hearing. Isabella can look forward to a life with sounds all around.

“Those cochlear implants will help her and she shouldn’t be any different than any other child,” Rosa said.

What a difference two implants can make.