All October appointments are out the window...

I thought I had successfully secured October 19th for Lily's ASSR. I have been playing phone tag with the appointment setter at Shands for almost a month now. She and I had initially left very detailed phone messages with one another about October 19th and I had even written the date on our calendar here. When the appointment setter stopped calling me back and would never seem to answer her phone when I called (ever!), I started to get worried. I decided to keep calling her every other day and leave her the same message until I got a response. I did this for the past two weeks. In my last message though I said that I looked forward to seeing her on October 11th for Lily's pre-op appointment and could she please call me back to tell me what time we should arrive. That got a response from her and a month after our initial messages to one another, I finally got to speak to her personally. She tried to act like she had no idea about the October 19th date for Lily's ASSR and now says that the doctors are all booked for this day. She said she would call me back yesterday with a new date. Hoping to hear from her Monday. This clinic has not been easy to deal with so far. I would rather all this testing stuff not drag into November. We are scheduled to have our follow-up appointment with Dr. Antonelli on October 23rd.

Lily is binaural now (sorf of)...

Last Monday, Lily finally got her second hearing aid for her right ear. After somehow traveling to Brazil and back, the hearing aid arrived after a long wait. It is set to the maximum level and it has very loud feedback when not in Lily's ear. Her reaction has been hard to gauge (mostly because she has barely worn it but that is another story..). Lily's new audiologist says that because the neural pathways on the right side are not as developed as on her left side and that it possibly just sounds like a "bunch of junk" to her on her right side (can you tell we live in the South from the audiologist's quote?). The reason that we have had not much time with both aids in her ear yet is because as soon as we got in the car after her appointment, Lily ripped out both aids and threw them down to the floor of the car. We then picked up Charlotte from a friend's house. Charlotte was curious about Lily's new hearing aid so she picked it off of the floor to check it out (she had been the one to pick out pink with sparkles for Lily's new earmolds and she wanted to see how they tunred out). Lily freaked out when Charlotte picked up her hearing aids and ripped them out of Charlotte's hands. She then proceeded to separate every piece of the hearing aids from each other and throw them all aound the car. It happened in seconds. I found all the pieces except for one very essential part of her new aid. So Monday we will get the missing piece and I will make sure to grab the hearing aids as soon as she takes them out of her ear....

Here is a website created by a family with a little girl who was born profoundly deaf and now has a cochlear implant. Her family is currently pursuing a second implant for their daughter. Anyway, it is an interesting account of their journey:
http://www.laneyjane.com/

Head bumps and guilt....

The news that Lily's hearing loss is due to a genetic reason has really been a big surprise. Chris and I have had very different reactions. He has been relieved to find out what caused her loss, since 50% of cases of childhood deafness are never explained. I have had a strange (and admittedly illogical) sense of guilt about it that is hard to articulate- maybe just that some defective part of me caused her to be deaf. And equally illogical is my guilt that I was not able to prevent her from bumping her head as often as she did (and continues to do) in her everyday activities. The enlarged vestibular aqueducts (EVA) that cause hearing loss appear to be worsened in some cases by head trauma, sometimes even minor trauma. I cannot even tell you how many times Lily has clunked her head- I often threatened to get her a helmet when she was a new walker.

Another important characteristic is that, most people with LVAS experience sudden hearing loss following a minor head injury or other activity which causes increased intra-cranial pressure (increased cerebrospinal fluid pressure). In one study the figure was 85.7% while another study reported 61%. A mild bump on the head, tripping or falling down or even jumping can jar the head enough to result in more hearing loss. Also, sudden hearing loss can follow a minor illness such as a common cold, strenuous exercise or a sudden change in barometric pressure.

In one study of 12 children with LVAS, five had sudden hearing losses. Three of the episodes followed relatively minor head injuries. The fourth occurred while forcefully playing a trumpet, and the fifth occurred immediately after an airplane flight.

In another case, a six-year-old child ran into a goal post while playing soccer and hit his head on the left side. This resulted in sudden hearing loss in the left ear. Three years later, after mild head injury while on the school playground, the child again reported an immediate hearing loss, this time in the right ear. An audiogram done that same day revealed severe to profound hearing loss in the right ear and profound hearing loss in the left. A CT scan revealed bilateral large vestibular aqueducts with no other inner ear abnormalities. Over the next month, hearing fluctuated between moderate and severe.

After episodes of sudden hearing loss, hearing may recover to the previous level, or much more commonly, it may recover partially to a new "normal." One girl with LVAS had characteristic attacks of sudden hearing loss following minor head trauma, common colds or exercise. Her high frequency hearing is now almost non-existent but her low-frequency hearing tends to recover shortly after each episode.

Hearing loss in LVAS generally follows a step-wise pattern. Each incident causes the hearing to drop another step. The hearing loss will follow one of two patterns. One is a gradual progressive stepwise hearing loss, but with fluctuations. The other is sudden bouts of hearing loss of at least 15 dB that may fluctuate to profound levels following minor head injuries or fluctuations in the pressure of cerebrospinal fluid (CSF). Usually these acute drops in hearing do not recover to previous levels. The end of this downward progression typically is profound hearing loss.

CT results are in- we have an answer....

Lily has enlarged vestibular aqueduct syndrome. I was convinced that we would never know the cause of her hearing loss. Will be adding more info as I learn more....

Here is a clearer explanation:
http://www.hearinglosshelp.com/articles/lvas.htm?zoom_highlight=lvas

What I was specifically told was that Lily has "enlarged vestibular aqueducts with mild cochlear dysplasia".

This is an autosomal recessive non-syndromic genetic disorder. I didn't take AP Biology in high school :P but this is my basic understanding: Chris and I are both recessive carriers of the gene that causes EVAS (and I am also finding info that she may have something called Mondini dysplasia). For each of our offspring, there is a 25% chance that we would have a child with hearing loss, a 50% chance of having a child with no hearing loss but is also a recessive carrier, and a 25% chance of having a child with no hearing loss and no recessive gene.

What is the vestibular aqueduct?
The vestibular aqueduct is the bony canal that links the inner ear with the fluid that surrounds the brain. In adults the vestibular aqueduct is narrow and shaped like the letter 'J'. However, when a baby is in the womb the vestibular aqueduct starts off being short, wide and straight before it develops, before birth, into a longer, narrower, J-shaped tube. The terms 'enlarged', 'wide' or 'dilated' refer to vestibular aqueducts that have not developed fully, and remain short and wide. In this document we will refer to an enlarged vestibular aqueduct.
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How does an enlarged vestibular aqueduct affect hearing?
An enlarged vestibular aqueduct can cause progressive or fluctuating deafness. (In other words, deafness that gets worse over time or changes from one level to another and back again.)

No one is quite sure why this happens. One explanation is that an enlarged vestibular aqueduct may allow the various pressure changes in the fluid around the brain to pass through to the inner ear (known as the cochlea), possibly affecting the hearing system.

The type of deafness usually associated with an enlarged vestibular aqueduct tends to be worse in the higher frequencies and usually affects both ears. Some people also experience a feeling of dizziness and imbalance (vertigo), and a general lack of co-ordination. It is difficult to know if your child's deafness will change over time or stay the same. However, the specialist may suggest that your child avoids certain sports, including contact sports and those that involve being exposed to changes in pressure (such as scuba diving). This is because it is thought that severe changes in pressure or a minor head injury can lead to an increase in deafness in children with an enlarged vestibular aqueduct.

CT scan a success

We went to Gainesville on Tuesday. Lily had her CT scan at 1:00. I rolled her into the room in her stroller and just plopped her up onto the table. Then the CT scan techs inflated a body pillow that was underneath Lily and it immobilized her arms, legs, and head. She just kept looking at me and blinking and never made a sound. I put on my lead vest and the techs went off into their control room. The bed moved into the scanning circle, the machine started whirring and then a red light started going around faster and faster in the circle. Lily looked around at the light and at me but never cried. Her chin quivered at one point, which was about at the same point I felt a strange vibrating sensation go through my body. It was over in seconds and we were done. Kind of weird and I didn't even explain it to her. One second you are happily sitting in your stroller and the next you are on a weird table in a strange room with bright lights and people you don't know fussing over you.....

I would have explained the whole procedure to Charlotte when she was 27 months old and I know she would have understood me. I didn't bother explaining to Lily because she cannot even begin to understand. It is becoming more and more apparent to me everyday how limited her language is compared to that of her peers. At the park today, she pointed to a little girl's Dora and Boots dolls and it just sounded like gobbledy-gook to everyone around us. I knew what she said and Charlotte probably understood her. The little girl was 10 days younger than Lily and said "Mommy, what did that girl say?". I have days when I think I fool myself that she is not that far behind but she really is. She is more like an angry little cavewoman than anything else these days. It goes way beyond what is typical 2 year old behavior. I think any barrier to communication, whether it is a speech delay, a hearing impairment, or autism just is so frustrating for the child. I hope we can get past this stage soon. I need buckets of patience these days.

-Kriste

New audiologist, new therapist, and new testing dates

This past Monday, Charlotte, Lily, and I spent two and half hours at FSU's Speech and Hearing Clinic. Lily had her first AV session since July 31. We have a new student intern that will work alongside Janet this semester. We all like her- she is fun and great with kids. I foresee that it will be hard getting attached to a new intern only to have her leave at the end of each semester. Lily has made some amazing progress since her 7/31 session and met many of the goals that were set then, like referring to herself by name (she calls herself "Neen"), responding to the question "where" with "in", "on", and "under", and following 2 step directions (like "pick up your shoes and put them by the chair". We are still working on that last one. I have noticed that Lily just smiles and nods "yes" when she doesn't understand what is being asked of her. Sounds like adults with hearing loss often do that too.

While at FSU, we also met with the audiologist there. She was wonderful and if we had seen her from the start, we wouldn't have had nearly the trouble that we've had trying to obtain Lily's aids. Lily will get her 2 pink hearing aids on Monday the 18th. It was no big deal for our new audiologist (Selena) to order them and get them within days. She actually already had them in possession at our appointment on the 11th, but she needed to get new molds made of Lily's outer ear. Selena was so helpful and took the time to explain what I need to do to take good care of Lily's aids and she took some of the mystery of it all away for me. I was kind of afraid of the thousand dollar equipment hanging from Lily's ears but not anymore. It is actually pretty ridiculous how little information our previous audiologist gave us about anything regarding Lily's hearing loss or her hearing aids.

I finally got a date for Lily's ASSR. It will be October 19th. I would have preferred for it to be sooner than that, but I will take what we can get. It means the September 27th appointment with Dr. Antonelli will need to be postponed because that was supposed to be a follow-up appointment after her testing was completed. I am a trouble-maker I guess though. If I just went along with what they scheduled for us, which was a non-sedated CT scan on September 19th and more audiological testing that same day, then we would still be meeting with Dr. Antonelli at the end of this month. But I did a lot of research and asked lots of questions on a Yahoo group for parents of children receiving cochlear implants and found out that for young kids, the ABR or ASSR is pretty much standard procedure. Most audiologists agree that soundbooth testing for kids under the age of 4 is unreliable. I saw that for myself when we were in Gainesville last time. They were leading Lily to respond to sounds when I knew she could not hear them. Waiting for the ASSR will be worth it if it means we will get accuracy regarding her level of hearing loss. I am trying to put on my thick skin and not care if I am labeled a trouble maker. My next task is trying to get them to coordinate the CT scan with the ASSR so that Lily only needs to be sedated once. That seems like it should not be a big deal but somehow I think I am asking something out of the ordinary. After looking at pictures of CT scan equipment, I am pretty sure that Lily would be too freaked to sit still long enough to get a good scan.

We go to a swim party on Sunday for local families that have followed the AV path for their children's hearing loss. We are looking forward to meeting other families that are in the same boat we are in.....


-Kriste

Bimodal, binaural hybrid hearing

The wondering "if" they will choose Lily as a cochlear implant candidate is hard sometimes and the end of this month when we meet with Dr. Antonelli again can't come quickly enough. I have been looking online lately for even more information that supports why I want to pursue the implant for Lily so that I will know what I am talking about when we see Dr. Antonelli and his team. It is not hard to find information supporting the importance of binaural hearing, either in the form of 2 hearing aids or 2 cochlear implants and I am hopeful that the team will be aware of the latest research supporting binaural hearing.

What is hard to find is information about someone in Lily's position with relatively no hearing in one ear and a small, but fairly significant amount in the other. There isn't much out there about it and I wasn't sure what to call it when I first began my search but now have found that there are people out there with a cochlear implant in one ear and a hearing aid in the other and that this is called bimodal hearing or hybrid hearing.

The reason that Lily is a borderline candidate for the cochlear implant is because she does not have a severe-profound loss in both ears. That appears to be the minumum criteria for most clinics that I have found. There are clinics in various parts of the country who are implanting adults 18 and up with just moderate hearing loss in both ears. On a side note, people who are completely deaf in one ear and have normal hearing in the other ear are not candidates at all for cochlear implants, even though it can be severely debilitating to have hearing in only one ear.

The thing about Lily's hearing loss that is in her favor for receiving a cochlear implant is that her loss in her left ear (her "good" ear) is "sloping". This means that if you look at her audiogram, her loss is in the high frequency range and it slopes down to the bottom of the chart. No matter how much her hearing is amplified with a hearing aid, there are sounds in the speech banana that she will never hear. Hearing aids only amplify sounds, they can't do anything to change what frequencies she hears. And that is why a cochlear implant is so important for her speech and language development. She will never hear "s" sounds or any of the higher frequency sounds without an implant. I have already decided that if she is not a candidate at this time for an implant that we will start introducing more and more signs, even though that is not what we are supposed to do with AV therapy. I just want to get words into her and stop the misunderstandings and frustrations we deal with everyday due to her limited way of expressing herself.

Speaking of binaural hearing, Lily is scheduled to get 2 new hearing aids on Monday. One will be one that we are purchasing. The other will be a loaner that CHS is buying on our behalf that they will be able to loan out to other families when and if Lily gets her implant. We will no longer be dealing with the audiologist at Tallahassee Ear Nose and Throat and will be going to the audiologist at the FSU Speech and Hearing Clinic, which is a good thing in my opinion. I am curious about what Lily's reaction will be to the 2nd aid. She has been trying to put her hearing aid into her right ear lately even though it is custom fit for her left ear only.

-Kriste

What a cochlear implant sounds like....

Chris found this link last night and it you can hear what it sounds like to hear with a cochlear implant. Pretty interesting and gives some insight into what Lily might face if she receives an implant. Since she is hanging onto some hearing in her other ear, there is the possibility that she would not like the mix of the 2 different types of sound.

Tuning In: What a Cochlear Implant Sounds Like

Heather Whitestone McCallum, mentioned a few posts ago, spent several years with one cochlear implant and a hearing aid in her other ear, a situation very much like the one Lily may find herself in. Her is her account of what that was like for her:

While I have a cochlear implant in my right ear, I want you to know that I continue to depend on the hearing aid that I wear on my left ear. Interestingly, there are some sounds that I do not hear well with my cochlear implant that I hear well with my hearing aid. The same thing is also true of my implant. There are sounds that I hear very well with the implant that I do not hear well with the hearing aid. One thing is certain, with the combination of both devices working together I hear much better than I did before as is evidenced by my speech recognition testing.

Still have some more research to do. I want to find accounts from others who have had a hearing aid in one ear and an implant in the other. I also want to find the latest information on the importance of binaural hearing (hearing in both ears) on speech development.

-Kriste

Thoughts after the big appointment

It will be a week tomorrow that we spent an entire day in Gainesville. We left at 7:00 am that day and didn't get home until 9:30pm (and that was with Chris driving 80mph both ways). Charlotte had some dental work done at 10:00am at Shands by her beloved "Dr. Jason" and then we had 5.5 hours to kill until Lily's appointment with Dr. Antonelli. Thankfully, a trip to the Butterfly Rainforest at the Florida Museum of Natural History, lunch at Macaroni Grill, and an afternoon showing of "How to Eat Fried Worms" helped to pass the time away. The only problem was the girls were beyond worn out by the time Lily's appointment happened.

We first met with a first-year resident and shared Lily's background with her. She was young, most likely with no kids and no clue what it is like to be the parent of a child facing a big uphill battle in life. She was a bit condescending and I was glad that our interaction was brief. Despite bringing in an entire notebook of copies of all of Lily's audiological testing, OAE results, language testing, etc, she wanted us to do a "quick" round of audiological testing in the soundbooth before we met with Dr. Antonelli. This "quick" session with the student audiologists turned into about an hour and a half of unnsuccessful testing. Part of the reason it took so long was because Lily was tired, the other part was probably because of their inexperience.

After talking with the older, more experienced audiologist on staff, we made plans to return on September 19th to do more testing with her first thing in the morning when Lily is at her best.

We finally met with Dr. Antonelli, who is a really nice and very smart man, around 6:45pm. Dr. Antonelli was first briefed by the first-year resident and then we had a chance to ask questions and find out his plan of action for us. Because Lily's hearing loss is not complete, she is not a clear cut case for a cochlear implant. There is more testing to do. So on September 19th, we return for a CT scan (hopefully non-sedated), the before mentioned audiological testing, and more dental work for Charlotte. I am hoping to schedule an Auditory Steady State Response (ASSR) for the following day. This would need to be a sedated test for Lily but I think it is really important for her to have this one before Dr. Antonelli and his team meet to discuss Lily's case. The ASSR is a newer test and it is more precise than the ABR. It may give us the clearest picture yet of Lily's hearing loss. Dr. Antonelli mentioned that this is a test he would like Lily to have so I am going to take the initiative and try to have it scheduled while we are there for our next visit. We also found out that the brand of implant that they use there is Cochlear. (There are 2 other brands out there, Advanced Bionics and Med-El. Some clinics offer all three brands and let you choose, others only have one or two to choose from. This all depends upon what brand the surgeon has been trained to implant.)

It was difficult to talk and listen to Dr. Antonelli because Charlotte and Lily were so tired. They were wild and took turns jumping off the footrest of the examination chair while we talked. I had a list of questions that I had intended to ask but it was too hard to ask questions. Right as we were about to leave, Dr. Antonelli asked if there was anything else we'd like to discuss. I almost let the moment slip away and then attempted to ask the question that had been burning in my mind for so long: "How would implanting Lily with one cochlear implant be different from a child who is bilaterally implanted? Do you bilaterally implant children at your clinic?". I am not sure how it actually came out because I burst into tears as I spoke. It had been a long day and I felt like we were about to leave with no answers and no idea where he stood on bilateral implants and whether or not Lily even had a chance to receive an implant there. He answered kindly and said that is what they do, they do bilaterals all the time. There was more to his response but I was preoccupied with getting my tears under control. His response did give me hope though.

I barely slept that night after we got home as my mind swirled around the day's events. I think that appointment made me really think about *why* I want a cochlear implant for Lily. The chance for as normal a life as possible is certainly part of it. The chance to learn as naturally as possible from absorbing language around her rather than having it drilled into her head during intense therapy is also pretty appealing.

I wrote Dr. Antonelli an email the next day when my thoughts were clearer than at our appointment. Here is part of my email to him:

”. I have really been researching a lot about cochlear implants and specifically about the benefits of binaural hearing. It seems to me that Lily has nothing but good things to gain from hearing from both ears. From what I have read, binaural hearing helps young children acquire language more naturally and allows for better speech perception, more natural and balanced sound, and better sound localization abilities. For those reasons, I have chosen to seek out your advice and expertise. I just want my daughter to live as normal a life possible and it seems to me that a cochlear implant will help us realize that dream for her.

We have chosen at this time to pursue Auditory-Verbal Therapy with Lily and we are very lucky to have Janet Kahn as our therapist. We will officially begin therapy on September 11. Part of my desire in choosing AV is to have Lily a part of the hearing world as much as possible. I have my doubts that this will be possible with Lily only having limited hearing in one ear. I don’t want her entire childhood revolving around rigid therapy that keeps her from learning in the most natural way possible, which to me is through play and acquiring language from listening to the world around her.

If Lily does not qualify for a cochlear implant we may change gears completely in regards to how we choose to communicate with her. It seems too hard on her (and by this I mean that I really think pushing AV therapy on her when she understands so little could break her spirit and that is the last thing I want to do) to continue on the AV path if she does not receive more access to hearing. We will likely switch to a Total Communication education for her so that we may fill her up with language as quickly as possible. I feel like we have already lost so much time. This choice means that Lily’s entire future will be changed dramatically and her choice of education, employment, and relationships with others will be very limited. This is obviously not what we want for our little girl.

I heard back from him almost immediately but he was not able to reply with anything other than a request for a form that gives him permission to discuss Lily's case with me via email. I have since faxed in that form and have not heard back from him. I honestly don't expect him to reply. He is a very busy person- I'm just glad I had the chance to express myself more clearly.

On a different note, Lily and I met with the most incredible family yesterday. We were originally to meet last week on Wednesday but we met yesterday instead. The mother, father, and 15 year old daughter were kind enough to share their experience of life with a deaf child with us. Our stories have some similarities although we have a much easier path to follow than they did 15 years ago, mostly because Lily has some residual hearing and knows what sound is and their daughter had never heard any sound until the age of 2, when she received her first cochlear implant. She had a long way to go but all the hard work has paid off. She is extremely bright and articulate and they are so wonderfully supportive of her. I hope that we will continue to meet with them. I would love for Lily to have such a great role model in her life.

-Kriste