A little about hearing and balance...

I'm trying to include research and educational materials in our blog as I find them. This video explains how we hear. At about 36 seconds, it talks about the hair cells. Those hair cells are what do not work in Audrena's ears, and it's called sensorineural hearing loss. Cochlear implants bypass those damaged hair cells by threading electrodes through that snail-shell shaped cochlea, delivering signals to the auditory nerve.

http://www.youtube.com/watch?v=vTiGskc1o48&feature=related

This video shows the ear structure, including the cochlea and the vestibular system. Audrena is missing the Posterior and the Lateral Semicircular Canals. She only has the Anterior Semicircular Canal, the one that sticks up on the top. This is the reason for her lack of balance and why she is late to walk.

http://www.youtube.com/watch?NR=1&feature=endscreen&v=WDfynsIk0PI&noredirect=1

Thank you to the posters of these Youtube videos!

Pink and Orange and Zebra Print, Oh My!

Audrena got her hearing aids on Tuesday! She had an appointment at USD with our much-loved Dr. M. We are excited to say that she does not touch the hearing aids at all, and we have been able to determine that she is getting some benefit from them! Now, keep in mind that with a profound hearing loss, she is not getting any useable sound from them. As Dr. M. explained, it's like giving her 3 or 4 pieces of a 100 piece puzzle. The bits and pieces she is getting are of no real benefit to her aside from providing stimulation to her auditory nerve. It's important to get sound to that nerve because it's kind of like a muscle, in that if it's not stimulated it will atrophy over time. So although Audrena still does not hear us talk, we have seen her dance to small bits and pieces of loud music (at Texas Roadhouse over dinner), and she does not like the feedback that the hearing aids create when she tries to lay her head on our shoulders. We have determined that it is in fact the noise, and not the feeling of the hearing aid on her ear, because if we turn the hearing aid off she will lay her head down and rest there.

Doesn't Audrena look adorable with them? (I apologize for the poor quality cell phone photos.)

She got to have lunch at McDonald's to celebrate. Audrena picked pink for one ear mold.

Daddy picked orange for the other ear mold because he knew Reyana would like it! Dr. M. picked out the zebra print hearing aids. This is Audrena waiting for her lunch at Perkins after a full morning of appointments, which I'll talk about in the next post.

Feeling Overwhelmed Today

I just talked with Dr. L. awhile ago. He was calling me back to answer some questions we had. Since he was at a conference and then trying to get caught up, it took awhile for him to get back to us.

First we discussed Audrena's risk factors for meningitis and the risk of facial nerve paralysis with the surgery. I'm relieved to hear that it is as we thought--those are not things that should keep us from going forward with the implants. The risk is still relatively small, especially because we do vaccinate on schedule. He has done at least 800 cochlear implant surgeries, and he has never had an issue with the facial nerve. Yes, these things are still risks, but even though they're low he still has to inform us.

We discussed Audrena's vestibular issues. He explained to me that a normal person has 3 semicircular canals, and she is missing 2 of them. The only one that formed is the superior semicircular canal. As a result, she'll be unsteady, and she'll have to use visual cues to be able to walk. He had explained that before, but now that I know exactly what the problem is, I'm a little more worried. I haven't had a whole lot of luck with finding information on how the absence of the other 2 canals will affect her. What I have found is that those canals control things like vertigo and posture, so I'm worried that her little world has been spinning and we don't know it. It's amazing what we take for granted. As part of the cochlear implant team's evaluations, she will undergo vestibular testing. I'm scared of what we'll find out, but I'm also looking forward to knowing more so that we know how to help her. I've said it before. Knowledge is power. In cases like this, when I don't know anything about what I'm facing, I feel weak and scared. I would just like to curl up in a ball and cry. So instead, I gather as much information as I can. I educate myself. If I know what I'm facing, if I have a game plan, then I deal much better with the situation. The problem right now is that I don't know, and I can't seem to find the information I need. So if anyone out there has experience with what happens when a person just doesn't have all 3 semicircular canals, please share it with me! Educate me.

As part of the discussion, I asked whether we would just treat any ear infections the same, using the Ciprodex drops. He explained that during the surgery, he would take Audrena's tubes out and patch the holes. That's when I essentially went, "Wait. I have big concerns with that." Audrena was very wobbly before her tube surgery. She had so much fluid built up that she was late to sit or crawl. She would steady herself with one hand while she sat. She army crawled to get around. After the tubes were put in, she stopped using her hands to steady herself, she crawled, and she began to cruise around the furniture. It was a HUGE improvement. Given that she has these vestibular abnormalities, I feel like she needs the tubes to keep the fluid at bay. He said what I already knew, that the tubes are an access point for bacteria. But he did say that he would consult with other surgeons around the country via e-mail to see what they would recommend in this situation.

He did reiterate that Audrena has other issues that will make surgery more challenging for him. She has small mastoids, which is where the implants are attached and where they drill to gain access for the electrode array. The auditory nerve canal opening is larger than normal, causing the potential for a spinal fluid leak which would have to be packed with tissue, and the auditory nerve canal narrows to a narrower-than-normal size. He did say that these are things he can deal with, but it does make things a challenge.

Now to the hearing aid trials. The cochlear implant team will meet today to review Audrena's case. They will discuss when to start the hearing aid trials and how long we will need them. That may determine whether we will be able to get them from USD, or whether we will need to get them from Boys Town.

I am just feeling a bit overwhelmed today. Some days are good. Some days I wonder how we will ever tackle all of this and get past it. Today is just an overwhelming day. But in the midst of this, I'm extremely thankful that we have a doctor (and a nurse) who is willing to take extra time to talk with us. He had patients waiting for him, yet he made sure my questions were answered. I'm SO grateful for that, and I do remember that when we are the waiting patients.

One Small Sigh of Relief

Dr. L.'s nurse called me this morning. She did confirm that the auditory nerve was normal. And apparently either Dr. L. misspoke or we misunderstood. It was a radiologist that he needed to consult with about it, not a neurologist. In any case, that part is in fact normal, so we can breathe a small sigh of relief. In the meantime, we'll wait anxiously for the rest of our answers as Dr. L. is on a plane bound for the annual cochlear implant conference. His nurse was not sure if he would check in again today, so it may be a day or two before we have answers to the rest of our questions.

She did not know what exactly Dr. L. has planned for the hearing aid trials. She would check with him on that since we are able to get loaner hearing aids through USD. While we may be able to expedite the time frame, it's still important to get some stimulation to the auditory nerve until surgery, even if the actual sound benefit is not much.

I did ask what kind of professionals make up the cochlear implant team that we will meet with. She gave me a long list, and I'm not sure I even wrote all of them down. But here is the general makeup:

Audiologists
Speech Language Therapists/Pathologists
Psychologists
Researchers
The Team Manager
The Two Surgeons
The Surgeons' Nurses

She told me to rest assured that we would know every aspect of the cochlear implant process before Audrena is cleared for surgery. They will make sure we are completely ready, mentally, physically, or otherwise. I can't speak for the process at other CI centers, but I definitely feel like Boys Town is a great fit for us.

Beginning to Digest Everything

It has been a few days since our second opinion. We have cried, talked, cried some more, talked to family, and cried again.

Saturday morning at our local deli/bakery, Randy ran into a man with a cochlear implant. He approached the man, P., and asked if he could talk to him about his cochlear implant. They had a nice conversation, discussed the experience, the risks, and his results. This man had normal hearing and lost it suddenly overnight. The cochlear implant restored 90-95% of his hearing. He and his friends basically said, "How could you not give her a chance to hear?" Then they asked to put Audrena on their prayer list. ABSOLUTELY! Keep 'em coming!

We keep coming back to that question, "How could we not give her a chance to hear?" We have come to the conclusion that even some hearing would be a benefit. If Dr. L. was still comfortable doing the surgery, then things can't be that bad. Then the meningitis risk can't be that high. We vaccinate on time. Ok, so we do still need to know, and I did call his nurse this morning to ask about it, as well as to make sure Audrena's auditory nerve was really normal as the neurologist said. She was out today, so I will probably have an answer tomorrow.

Over the past few months, we have considered Total Communication (sign language as well as hearing, speaking, and lip reading), and we've decided that we will prefer to use Auditory Verbal or Auditory Oral Communication. The parent of an adult deaf man said to me, "Face it. It's a hearing world." She would have gotten an implant for her son, had they been available at the time. I know that there is a wonderful Deaf culture out there. Deaf people are very successful in life and have great careers. But I also know that I want to give my daughter the chance to hear. I want her to marvel at those chirping birds. Randy wants her to hear him say, "I love you." We want to hear her little voice. The studies I have read find that a small child (the younger the better) who receives cochlear implants and Auditory Verbal or Auditory Oral rehabilitation will do so much better with the implants. With the proper therapies, it's possible to have the child mainstreamed in Kindergarten, and really maybe even before. 30,000 words per day. I think I can. I think I can. I think I can.

I called our Birth to 3 coordinator today to tell her about Dr. L.'s recommendation for physical therapy. Audrena had not previously qualified because she showed fine in that area of the evaluation, although she's not yet walking. So Audrena will now receive physical therapy and speech therapy. The coordinator had also arranged for an outreach worker with the South Dakota School for the Deaf to be involved in Audrena's care. Wonderful! I had planned to contact them, but now I don't need to.

I received a call today from the genetics team. They meet once a month, and they only take 3 patients each time. It could take us 6 months or longer to see them. That should give me plenty of time to get insurance on board with the testing. I'm more worried about getting insurance to approve both implant surgeries. They've been good so far, so let's just keep praying. Time is of the essence for these surgeries.

As for Audrena's abnormal vestibular system, I really wonder if I have the same defect. I have kind of bad balance, especially at night. If I can't see, I have to steady myself with a wall, or else I fall all over the place. My mom's balance was not good, either. So maybe it is genetic. And maybe it's not going to be as bad as Dr. L. warned us it could be. I rode a bike. I walked a balance beam in gym class. Audrena seems pretty steady after her ear tube surgery. Maybe she's already compensating well. I think with some physical therapy, she'll do great. I bet she will ride a bike.

I'm a little afraid that the counselors on the cochlear implant team will think I'm really too unstable to take this on. They will want to discuss reasonable expectations for the results of the implantation. Reasonable expectations. I'm well aware that my expectations are probably completely unreasonable. I want as much therapy as possible for Audrena. WE are dedicated to getting her caught up to her peers in speech/vocabulary before Kindergarten. We want her to be mainstreamed. I envision her playing sports and participating in extracurricular activities. I see her becoming something hugely important, like the first deaf woman president. Small but mighty.

Will I need to adjust my expectations? Maybe. Will I be completely devastated if she doesn't have a fantastic outcome with the cochlear implants? Probably. But then I will pick myself up and move on. If she has some learning disabilities in the process and is not mainstreamed as we hope, we'll do our best. If cochlear implants do not work well for her and she needs sign language, then we'll change our focus. Because she is our child, we will make sure she has everything she needs to pave her path to success, whatever that success entails. But as of right now, I see no reason to adjust my expectations. Randy and I are dedicated parents. Besides, Dr. C. has proven over the years that she is rarely wrong. Small but mighty. I see a bright future ahead.

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?