Lilyhear

New Shop on Etsy - Home Business

2010-02-22T23:54:00.002-05:00

Havent had time to post with this crazy new hobby / business.

Etsy Wooden Animals - Custom Wooden figures

Lily is doing incredibly well.

Here are some photos from the Holidays

First Cochlear Implant in Uganda

2008-06-06T23:22:00.002-04:00

Excellent story and video of NYU Doctor and the first implant in Uganda. Plus a lot more to this video - Josh Swiller and his brother and controversies.

Young Cochlear Implant from England

2008-06-06T22:46:00.004-04:00

Story from Britain

A baby girl who was born deaf can now hear after becoming one of the youngest people in Britain to have a cochlear implant.

Ava Pearson, 14 months old, can listen to bedtime stories and jiggle about to music thanks to the life-changing operation. She received first cochlear implant at 9 months and second cochlear implant at 12 months - Full article

What is a cochlear implant... Michael Chorost Video

2008-06-06T04:26:00.003-04:00

So many people have no idea what is involved with a cochlear implant.

I have gotten pretty good at explaining it but this 4 minute movie may help some people... Like the lady staring at my daughter in the restaurant, or the people who say those are nice hair bows and wonder what is that device?

http://www.exploratorium.edu/listen/lg_michael_video_sub.php

What does Cochlear Implant sound like
What is a cochlear implant

American Gladiator Brick Reilly NBC Full Episode

2008-06-05T00:04:00.007-04:00

NBC.com has the full episode of Brick Reilly Episode NBC makes you watch some commercials but they separate into chapters and you can skip around. It is the May 26th episode the one with the ridiculous looking "Wolf" guy grimacing.

Besides the first ever Cochlear implant athlete on network TV there is a interesting dynamic in the episode between the female competitor De and her loving husband who is not pleased that his cocky wife couldn't win - 54:54 mark is the most telling moment.

Check out this still - I am sure the ride home was not a pleasant one.

The really remarkable thing was Brick Reilly dislocating his shoulder in the final gauntlet and continuing. That is mental toughness and I think most would have stopped. I can say I never have cared who wins on American Gladiator but when he hurt his shoulder I was like oh man can he hold him off?
From Webmd Dislocated Shoulder I am sure they taped all the preliminary episodes to get the top 6? for the finals so I wonder if he had any time to recover and if they have already finished the show.

The fall on his shoulder.

His shoulder bulging from dislocation

Swimming with Cochlear Ziploc Food Saver

2008-05-25T22:49:00.010-04:00

Lily could hear at the pool today and it was great!

What a difference. Instead of fighting me as she swims, I was giving her instructions in a non yelling voice- "like close your mouth", "use your arms", "open your eyes underwater", I will hold you up"...

Today I said "paddle like a dog" and she had never thought about dogs swimming I think but said "I am a puppy"

Without hearing, swimming with Lily is more like a contact sport were I think she thinks I am trying to tickle her or chase her. Without her implant she also thinks "I can do this myself" when she really can't swim yet. And yes because she can't hear she gets very loud.

This year is better than last because she is 42 inches tall (not yet four years old) and the pool is 3 and a half feet deep(42 inches).... So she has learned to bounce off the bottom. She is also very close to being able to actually dive and swim.

We used the modified food saver and ziploc attempt as seen and detailed on a blog from Holland about Lottie Soffie -http://lotte-sofie.blogspot.com/search/label/Swimming

After 3 or 4 attempts in getting the speedo bathing cap on and holding the implant coil and processor in the right spot we were able to get the cap on properly. It did take my wife and I one to hold the magnet in place and one to get the cap on.

A ridiculous scene really.

I didn't get a lot of good photos because I was in the water with Lily, but I did get one of her waiting for "the bucket" which fills up and dumps ever 2 minutes.

The force of the water actually moved her cap (see photo) and the experiment was over... But if we had avoided the bucket everything would have continued to work.

A couple more photos

American Gladiator Brick Reilly Cochlear Implant

2008-05-25T22:29:00.003-04:00

This is great - awesome to see contender Brick with a cochlear implant. I guess he isn't wearing the implant under his helmet over the water. Maybe Cochlear can come up with a water proof version...(see Lily post) He was also being hit in the head by a 290 pound behemoth named "Justice" in the joust.

He is a tree climber and is dominating the other contestant. He even dislocated his shoulder and popped it back in and finished.

From his bio on NBC. Brick Reilly is a 27-year-old tree climber from Springfield, New Jersey. He was born deaf and has always deemed athletics an important escape from the realities of life. Though Reilly recently had a cochlear implant which gave him the ability to hear, he never used his disability as an excuse in his life. He works as a mentor for deaf children in the New Jersey area.
http://www.americangladiatorsblog.com/contenders/season-2-contenders

Cochlear Impant 16 year old dancer

2008-05-25T21:40:00.006-04:00

Nice article on girl from New Jersey. Annette Tavernese
Born to dance
Teen determined that being deaf will not keep her from achieving her goals

Great video too.

Video from last December

2008-05-14T22:48:00.008-04:00

This video shows how far Lily has come from March 2007 activation to December 2007 - She is answering her sister's question... What do you want for Christmas?

You Tube Hookup Cochlear Implant at Shands

2008-05-05T17:09:00.002-04:00

From March 2007

Non Updated Blogs are the worst

2008-05-05T16:31:00.006-04:00

Lily is doing incredibly well and coming up on her 4th birthday.

Lily and her cousin listening to a story by Grandpa

Turn on Day

2007-08-10T01:22:00.002-04:00

March 26th Lily got hooked up with her Nucleus Freedom Processor.

Looking back now we were all very excited about the big day. I think we all expected instant results.

They hooked up to the computer ran through some tests and turned it on. It didnt bother her at all. It was definitely not like the videos you see on youtube or other cases were kids go from hearing nothing to something. Lily has had some hearing, and she still has hearing in her left ear even unaided... So when the Dr. rang the bell... on her left side, well yes she heard that.

She wouldn't respond to her name or our voices but she did pick up Shhhh sound right away - so that was pretty amazing . And she definitely cannot hear high frequency sounds. She even said something right after using Shhh sound - something like I am spinning the bear. The Doctor said it was the best hook up she had ever seen.

Magic Ear Surgery Day Details / Photos

2007-08-10T00:26:00.000-04:00

Surgery date!

2007-02-12T22:21:00.001-05:00

Lily will have surgery on Tuesday, March 6th!
We found out this afternoon. That is 3 weeks from tomorrow. It is a relief to have an actual date! it will be at a surgical center instead of at the hospital. I am hopeful this means that they won't try to boss us around so much and will let us be with Lily as much as possible. -K

After a long break, a few updates...

2007-02-05T21:46:00.000-05:00

Whew. The holidays and a couple of big illnesses have kept me from updating our little blog here. Let's see if I can sum up the past six weeks. On Christmas day, Dr. Antonelli emailed us to say Lily's surgery was a sure thing and to look out for a call from his surgery scheduler sometime after the beginning of the New Year. I found out this person's name and called her the second week of January to find out the status of Lily's insurance approval. She gave me a standard response about how she has 35 patients at a time to get insurance approval for and how she works on them in the order she receives the cases and that she only works Mon-Fri from 9-4. I took that to mean that she would get to Lily's case when she got to it. She also said that at that time, she was already scheduling surgeries towards the end of February, which was pretty disheartening news. A week later, I wanted to call her again about Lily's status, but I figured I'd get the same response from her. I decided to have a purpose for calling and told her that if there was a cancellation list, that we wanted to be on the list. She said great and added us to the list. I found out from her that surgeries are performed on Tuesdays and Fridays. Tuesday surgeries are at a surgical center and Friday surgeries are at Shands hospital. (I wonder which place would be the most child-friendly and would allow me to be with Lily as much as possible. I guess that getting the surgery done ASAP is almost more important to me at this point and I am going to try my best to make things go our way no matter where we end up for surgery.) We continued to wait to hear about Lily's insurance approval and a specific date for her surgery. The last week of January, we come down with the second big illness in less than a month. We are coughing, feverish, snotty, and miserable. Chris's parents come for a visit and we pass our germs on to Gram. While they were here just a week ago today, the surgery scheduler calls and asks if we can be ready for surgery for this past Friday. My knees go numb and I say "Yes!". Lily is still coughing at this point but seems to be on the mend. I was hopeful that she would be well by Friday. The surgery scheduler says to take Lily in to see her regular doctor to listen to her lungs and check out her ears and that she would work on speeding up the process for insurance approval (which we still hadn't gotten yet!) and that we would talk again on Wednesday. Lily's cough worsened and she coughed so hard that she threw up all over the both of us Tuesday night. I decided then that we would probably cancel. This was disappointing but there was a positive to all of that- the insurance approval process was accelerated and we now have insurance approval. If there is another cancellation in the near future, hopefully we will be called again. Chris thinks we should send her flowers for all her hard work (and try to butter her up a bit too). So this is where we are- waiting. I imagine that we will hear Lily's surgery date sometime this week, which will likely be in March sometime. I just hope that we get another opportunity like the one we had last week...

A couple of recent news articles

2006-12-18T18:29:00.000-05:00

Here a some recent news items in regards to cochlear implants:

Baby becomes youngest cochlear implant recipient in Illinois

and this article:

Early cochlear implants get deaf toddlers talking
Media Release, Thursday 7 December 2006

Deaf babies and toddlers can develop normal language skills when cochlear implants occur between six months and two years, according to new research released by the University of Melbourne.

“This is the first study investigating the long term language progress of deaf babies and toddlers implanted with Cochlear implants,” said Head of the Department of Otolaryngology at the University of Melbourne, Professor Richard Dowell.

The results were revealed after a major clinical study of over 100 infants implanted between six months and two years of age was conducted at the Cochlear Implant Clinic at the Royal Victorian Eye and Ear Hospital.

“Results have shown that implantation in an infant under 12 months has a significant impact on their language skills, “said Dr Shani Dettman of the University of Melbourne, who coordinated the study.

“The most important time for developing communication skills is below 12 months of age. This is a critical period for language growth,” Dr Dettman said.

“Prior to the introduction of early screening tests and cochlear implantation, profoundly deaf children experienced significant delays in language development,” she said.

“What we have found is that the children who received implants before 12 months of age had better language than the children who received implants between one and two years of age,” she said

“Until recently, most parents were choosing to go ahead with a cochlear implant when the child was between one and two years of age.”

“We now have definitive evidence to recommend that parents decide even earlier. This research is backed up by similar studies carried out in Europe and the U.S.”

The study involved a three month pre testing period of communication assessment, hearing tests and radiological scans, and then long term follow up years after the implant operation.

Dr Shani Dettman said the research will have a huge impact on the lives of the children and their learning capabilities.

“It is wonderful to watch the children begin to respond to their hearing, and learn to communicate. These children can joke, lie, tease and use language in all its forms,” she said.

“Ultimately it’s about providing more communication choices and better education, social and vocational opportunities,” she said.

“The deaf children we have in our early intervention program show great interaction with other hearing children,” said Program Coordinator of the Early Education Program for Hearing Impaired Children, Rita Corbett.

The major Cochlear implant device used around the world grew out of research at the University lead by Professor Graeme Clark. This device, manufactured by the Australian company Cochlear Ltd, is now used by over 80,000 deaf people.

MEDIA CONTACT
Rebecca Scott, University of Melbourne
Mobile: 0417 164 791

Sonia Aplin, Royal Victorian Eye and Ear Hospital
Mobile: 0412 887 170

More information about this article:

Rebecca Scott
Media Promotions Officer
rebeccas@unimelb.edu.au
Tel: +61 3 8344 0181
Mob: 0417 164 791

We are lucky in the sense that Lily's type of hearing loss has enabled her to acquire a great deal of speech. I have pretty high hopes that even though she is a "late" implantee in comparison to other deaf children, she has had the advantage of a progressive hearing loss and she will be able to catch up to her peers and will do as well as those children who are implanted at very young ages.

We have our answer...

2006-12-17T20:45:00.000-05:00

And it is a yes! The implant team has decided in favor of an implant for Lily. We don't know when it will be yet but hopefully very soon. When I have a chance, I will add more details about the decision. In the mean time, here is a nice, simple movie about cochlear implants and what they do:
http://www.youtube.com/watch?v=SmNpP2fr57A

Still no news

2006-12-11T15:22:00.000-05:00

We still haven't heard from Shands. I called on Friday afternoon to hopefully catch Dr. White but I ended up leaving her a voicemail message. Tried not to sound to desparate to hear their answer in my message to her. Here we are on Monday afternoon with still no call. I don't want to be a pest and at the same time I am frustrated that we haven't received the call. I am going to make Chris call her :P.

We will get our answer on Friday

2006-12-06T17:37:00.000-05:00

Dr. White called me today to let me know the implant team will be meeting tomorrow to discuss Lily's case. I think I sounded wishy-washy with her on the phone so I emailed her later to really emphasize that we are serious about this for Lily. I think the fear of doing the actual surgery is what made me waffle a bit. It is hard- Lily has made some pretty incredible progress since last summer.

We went to a support group meeting for families with hearing loss last night. Lots of school talk and how to deal with hearing loss in schools. Sounds exhausting.

Choices in deafness....

2006-12-01T12:25:00.000-05:00

A family that has a beautiful and very new infant son born a profound hearing loss has recently contacted us after reading our blog here. Their account of their journey thus far is here:
http://turnonmyears.blogspot.com/

Reading their story triggered a memory of reading a post a while back on the Listen-Up group about research regarding sign language for infant implant candidates. It is a pretty interesting way of seeing things:

I have heard Mary Koch speak several times and she is truly a pioneer. I have an enormous amount of respect for her. She rubs both the oral folks and the ASL folks the wrong way sometimes. But she knows her stuff! And she is saying something very new and very important and something that no one else is saying yet. And I think she has some really important insights that her very diverse background allows her to have.

I think what gets lost in the professional arguments about what she is saying is three things 1. the fact that language, communication and cognition need to be more important than communication mode. 2. You need to work with what you have at the time - if you have an implant, work on auditory - give the child the benefit of what the technology can do. Before the implant, work on auditory to prepare for the implant but work on sign to provide language, communication and cognition/understanding. If a two year old who has been deaf since birth has language and understanding of the world, then when they get an implant, you aren't starting from scratch. For example: a baby who understands and maybe uses the sign for "more" or "big" or "mine" will be able to simply switch modes to hearing the speech for those concepts. Compare this to starting from scratch with a 2 year old with an implant who doesn't know any of these concepts, you are having to build the auditory brain pathways plus the language plus the understanding of the concept. If you have a child who already has language and cognition you are only having to build the auditory stuff and therefore you can make much better progress. 3. And also that you need to follow the lead of the child. Some kids are going to be visual communicators and some will be oral communicators and as parents we need to figure that out, either by trying one and switching to the other or by trying both and seeing which one the child does best with. And to try to optimize whatever technology you are using, whether aids or implants. 4. The last and maybe first most important thing she emphasizes is 12 months growth in 12 months time. And if you are starting from behind, doing what it takes to get more than 12 months growth in 12 months time. These are VERY HIGH expectations.

I think it is important to note that Mary is NOT advocating sim-com or what is called "total communication" by most public school systems. She seems to me to be advocating more of an AV/ASL route which is different - VERY different. She is truly advocating a new paradigm or a new way of thinking about the oralist/signing conflict.

For example, she demonstrates what she calls the "auditory sandwich." It is a method she would use with a baby/young child who knew sign language and then got an implant. It is something she does while using an auditory verbal (AV) approach with a child who has an implant or aids. She would say the word, then sign it or show a picture and sign it and then say the word again. So the first and last things given to the child were auditory - so that would emphasize the auditory. But it would draw on the child's already built language and cognitive skills for instant understanding. Because the technology at this point in history truly gives the child access to the auditory modality. And if you want to build the brains auditory pathways you have to emphasize the auditory.

I think traditionally there have been three basic camps - oral/AV, ASL and "total communication/simcom." (ASL often gets lumped together with total communication/simcom because they both use "sign language" but in reality they are quite different) She is advocating a unique blending of ORAL/AV and ASL that is NOT the same as "total communication/simcom." For years ORAL and ASL were seen as two opposite ends of a spectrum or as mutually exclusive - pick one or the other. She is weaving AV and ASL together in a unique way. And she has a very well constructed model and understanding of both modalities and truly has something very NEW to say to ALL of us. And from the outcomes she is seeing, it has a lot more to offer our kids!

Here is a slide presentation by Mary Koch about this subject.

Here is the transcript that went with the presentation.

I guess reading about a child discovered so young with hearing loss made me think about what I might have done had I known all along that Lily was mostly deaf. I know I would still have pursued an implant for her. I started Baby Signs with Lily when she was around 7 months old just as I had done with Charlotte (of course, I assumed she was hearing like Charlotte). Charlotte made her first sign at 10 months for "milk". Lily made her first sign sometime after her first birthday and it was for "more" . Charlotte had about 60 signs at the height of her signing days and was stringing along 4 or so signs to tell little stories when she was about 17 months old. All of the signs (which were either actual ASL signs, Baby Signs, or several made-up ones) that Charlotte used gradually faded away by the time she was speaking in sentences. It happened without me noticing really. With Lily, it seemed that she did not take to signing as quickly as Charlotte did, although I imagine that with Charlotte having the auditory reinforcement of the words being spoken and signed to her that she caught on all the more quickly. Lily did eventually catch on though and probably had about 20 signs by this past May at 23 months, before we found out about her hearing loss and before we were encouraged to discontinue with the signs. I do not know for sure if this helped her with language acquisition or not but it makes sense to me that it is likely.

On an unrelated note, I can't believe that it is December 1st and we have not heard from the UF implant team. I was sure we would know by now. We did receive an email from a woman who received a cochlear implant in Tampa and her surgeon was Dr. Bartels of the Tampa Bay Hearing and Balance Center. His clinic offers all three brands of cochlear implants, rather than the one offered by UF. I can't even think about having to choose which brand of cochlear implant to go with. I just want a clinic to say that Lily is a candidate. After this long process of testing and getting all the right material together, this wait to hear from UF is starting to feel like we are waiting to hear if we have been accepted to our first choice college.

Oh, and I also corresponded this week with a mom with an 8 year old daughter with LVAS and a cochlear implant. As a 2-4 year old, her daughter had many behaviors that were similar to Lilys: night-waking, *extreme* tantrums, intense clinginess, obsessive pickiness. She says her daughter is now a joy to parent- I find a little comfort in that. What made things easier for her were preschool, adding some sign language, and doing some cued speech. These are suggestions that will take some thinking on. We are planning to be a homeschooling family but I am willing to consider preschool if it is something that Lily would really benefit from. I just don't think that there would be the type of program that I would like to see for her here. There is no school for the deaf unless you go to Jacksonville, or Orlando, or Tampa. There is a public school here that is the magnet school for kids with hearing loss but I have not heard many good things about the program. I will have to check it out for myself. The other suggestions, signing and cued speech, I guess I am open to them. I want to AV therapy some more time though. I have a few concerns about AV therapy. I worry that trying to listen all the time is just tiring for Lily. Here is an adult with LVAS and his take on Auditory Verbal therapy. I'm not sure what I think anymore about what is the right path for Lily. I thought I knew that it was strictly AV therapy but as time goes on, I see how frustrated she is becoming.

-K

Lily's Language Progess

2006-11-28T00:19:00.000-05:00

I have been meaning to write about Lily's progress for a while now. We had such a non-stop period of appointments that talking about Lily and how she is actually doing had to be put on the back burner. Lily has been in Auditory-Verbal therapy now for a little over 2 months. She is saying "me", "my", "you", "your", "I"- she has a good grasp of pronouns now. She is doing better with 2 step directions than she was initially. When we first started asking 2 step directions, like "Pick up the doll and give it to me", she would pick up the first thing she saw and it was hit or miss if she would actually give you anything. I guess this showed she was not used to really listening to what was being asked of her. She has pretty much mastered "in", "on" and "under" and on her own is demonstrating understanding of "behind". This is a good sign because it is exhausting to imagine working this hard with her on each and every abstract concept that there is in the English language. The stuff we are working on with her is the stuff that you take for granted when children acquire language naturally without any impairment. It is incredible when I think now how effortless it was for Charlotte to learn to speak. Children absorb language, from the time they are tiny, just from overhearing it. This is why hearing aids cannot be compared to eyeglasses- just because the sound is suddenly amplified does not mean that fixes the problem. There is a tremendous amount of work to be done to catch up hearing impaired kids on all that they miss from overhearing everyday conversation. We are now working on plural "s". I don't know how she is going to get this one and I think it is going to take a lot of work to get her to differentiate between "the dog" and "the dogs" when she can't even hear that final sound.

Lily's speech is somewhat clearer than it was before but you have to spend time with Lily to understand her most of the time. Here is something that you might hear her say: "Cah-leh wan wah moo-bem". Translated this means "Charlotte wants to watch a movie". So it looks like we will have to do speech therapy at some point to work on articulation with her. I think that the focus for now is to get her to expand her vocabulary as much as possible now and that cleaning up her speech will come later.

Lily's behavior is more and more trying as she gets older. She does not like to "perform" the required tasks during her therapy sessions and is getting bolder and wilder as each week passes when Janet and her student are here. Lily was very quiet and withdrawn for the first therapy sessions and fairly cooperative (as she often is in new situations with new people). And by withdrawn, I mean she buried her face into me and would have melted into my body if she could. But then, as her comfort level increased, she began to act like she does normally at home, as Chris says, like a feral child. What is difficult to do is to differentiate her behavior from her hearing loss. Would she act like she does even if she could hear or is her wild, inexplicable at times behavior soley due to her hearing loss? I can only draw on my experience with Charlotte when I try to understand Lily. Charlotte was not an easy baby/toddler by any means but I was able to figure out what she needed to be calm and comfortable and satisfied. That is something I have struggled with since Lily was a newborn- how to figure out what she needs in order for her to maintain emotional equilibrium. There seems to be something out of sync about Lily and I am searching for answers about that now too. Sensory integration dysfunction, while a controversial theory, is something I guess I have to explore at this point. It is not uncommon among children with hearing loss. Now the question is how to I pursue dealing with it....

Here is a description of kids with SID, many things sound just like Lily:

If they are oversensitive to touch sensations (tactile defensiveness), they will avoid touching and being touched and will shy away from messy play, physical contact with others, pets, certain textures of fabric, many foods, bumpy sock seams, etc. On the other hand, if they are under-responsive to touch sensations, they'll crave touching and being touched. These children will be fingerpainting their arms, stuffing their mouths with too much food, shouting indoors, turning up the volume and bumping and crashing into people and furniture.

If children are oversensitive or defensive to movement experiences, their feet will never leave the ground. They will shun playground equipment and object to riding in the car or elevator. They may refuse to be picked up. Or, if they are under-responsive, they may crave intense movement, and seem always to be in upside-down positions, swinging on the tire swing for long periods, and on-the-go constantly -- jumping, bouncing, rocking and swaying.

This will be my next focus after we find out what Lily's implant status will be...

-K

If they say no.....

2006-11-27T20:22:00.000-05:00

Still obsessing about the implant team's answer. Will they implant her now or will they make us wait until she loses further hearing? I have been researching other clinics to find out what determines candidacy. UF's policy clearly states a child must have a severe-profound loss in *both* ears. Lily's hearing is just slightly better than that. I found that the Listening Center at Johns Hopkins in Baltimore states this about implant candidacy, which seems less conservative and more flexible than many clinics:

Candidacy

Children and adults with an advanced level of sensorineural hearing loss in both ears may benefit from a cochlear implant. A cochlear implant may be considered for someone with a hearing loss that is severe enough to limit speech understanding, even with well-fit hearing aids. The inability to hear more than one half of amplified words provides a guide that helps define candidacy.

I am finding that geography plays a large role in how progressive implant centers can be. It looks like the implant centers to the east and to the west are more liberal than down here. I am not sure what that means for us yet and what it would take to get this done as soon as possible but I plan to start researching this now in case we have to switch gears very soon.....

-K

Assorted stuff...

2006-11-22T20:23:00.000-05:00

I found this link, http://www.sens.com/helps/helps_intro.htm , which has some demos of what it is like for a person with hearing loss. Demo 1 is a severity of hearing loss simulator. Lily's hearing in her "good" ear is the one labeled "HL5". No wonder Lily doesn't seem to care about music very much. Demo 5 has a cochlear implant simultation.

I discovered that there is a doctor in Jacksonville that does cochlear implants at a children's hospital and is not affiliated with UF. This doctor implants the brand "Advanced Bionics" (AB) implants. AB has focused on making music more enjoyable for implantees and has something called a 120 Virtual Channel external processor coming out in the spring. There are currently 16 to 24 electrodes that are implanted internally, depending on the cochlear implant company, but the AB technology works with the outer processor to create more realistic sound somehow. I think. I don't know much about the differences between the three companies, but I guess I will have to do some research. I did find this chart comparing the three implants, but it is slightly outdated and does not include the new AB Harmony. I am not sure if the doctor in Jax would even be an option for us though, considering Lily's abnormal inner ears. Dr. Antonelli is the one that doctors around the state refer their toughest cases to. This wait to hear if they will implant Lily is hard and we don't know the date of their team meeting. I just hope to hear by the end of the month.

I have joined a Yahoo group for LVAS and have learned quite a bit about Lily's condition there. A medical doctor with LVAS himself moderates the group and quickly answers questions and clears up misinformation. There was a recent discussion about Pendred's Syndrome and genetics and progressive hearing loss after these links were posted by someone from the National Institute of Health:

Enlarged Vestibular Aqueducts and Childhood Hearing Loss

http://www.nidcd.nih.gov/health/hearing/eva.asp

More About Childhood Hearing Loss and EVA

http://www.nidcd.nih.gov/health/hearing/eva-intro.htm

Genetics of Enlarged Vestibular Aqueducts (EVA)

http://www.nidcd.nih.gov/health/hearing/genetics.asp

Pendred Syndrome

http://www.nidcd.nih.gov/health/hearing/pendred.asp

NIDCD Research on EVA and Hearing Loss

http://www.nidcd.nih.gov/health/hearing/research.asp

A pretty interesting discussion followed. One person pointed out that the first article says that Pendred's Syndrome likely causes the progressive loss, but made no mention of enlarged vestibular aqueducts/non-Pendred's causing progressive hearing losses. Several people replied saying that their children do not have Pendred's but have had progressive losses so the article must be inaccurate. One mother replied with this, which has made a lot of sense to me out of some overwhelming information:

I've excerpted some passages below from the following genetic encyclopedia. One point this article makes is that there are many, many different possible mutations in the PDS gene most likely causing a RANGE of symptoms that include LVAS. We're all dying to know whether our kids fall into the genetic definition of Pendred Syndrome when the definition of Pendred Syndrome is really a symptomatic one. There were a set of symptoms, someone gave them a name, and medicine looked at the genetics later as the science advanced. The genetics showed that this range of disorders was a little more complicated than that particular set of symptoms.

http://health.enotes.com/genetic-disorders-encyclopedia/pendred-syndrome

"As of March 2001, genetic researchers identified at least 47 different types of alterations in the PDS gene among different families. However, four of these are more common than the others, and it is estimated that approximately 75% of affected people have these common changes.

" ... it is believed that changes in the pendrin gene actually cause a number of overlapping conditions. These conditions range from Pendred syndrome (i.e. sensorineural hearing loss [SNHL] and thyroid changes) to SNHL with EVA.

"Changes in the PDS gene have been found in people who have SNHL and EVA, even though they do not have thyroid changes that would have helped make a clear diagnosis of Pendred syndrome in the past. Thus, future genetic studies on large groups of individuals with SNHL will help researchers understand how common Pendred syndrome truly is, as well as the range of symptoms that are caused by changes in the PDS gene."

So that is it for the assortment of thoughts for now. I plan to get on here this weekend and talk about Lily's progress.

Let the waiting begin...

2006-11-19T23:13:00.000-05:00

With the completetion of Lily's sound booth testing last week and the receipt of the sedated testing results, all that is left now is waiting to hear news from the latest implant team meeting. I emailed Dr. Antonelli to ask him something I forgot to ask when we saw him last- what does Lily's cochlea look like, or rather, how many turns is she missing? A normal cochlea has 2.5 turns. Dr. Antonelli responded that Lily has between 1.5 and 2 turns in each cochlea, which is considered a mild deformity. I would be surprised if we hear something this next week since Thanksgiving is coming up, but I got the impression from him that we would hear something by the end of the month. I have been corresponding with a mom in New York who has a 2 year old son with Lily's exact level of loss and he was implanted in his severe-profound ear this past August. I am going to have to find out how she made this happen if UF turns us down. We have talked about going to Atlanta to try another implant center if UF says we need to wait until Lily loses more hearing in her left ear first. That would be rough since Atlanta is about 4-5 hours away, depending on how heavy your foot is. I would hope that they wouldn't want to do all of their own testing. It would be discouraging to have to start this process all over again. Hopefully, that won't even need to be a consideration.

I am going to try to get a chance to write about Lily's progress with AV therapy at some point this week. She really has made some remarkable progress since this summer. Right now, though, I need to be straightening up for tomorrow's therapy session here at home, bright and early at 9:00am!

-K

ASSR/ABR Results Arrived

2006-11-17T01:14:00.000-05:00

We received the results of Lily's November 1st ABR/ASSR in the mail today (or rather, yesterday, as it is now 1:00am on Friday). The report reads that Lily has a moderate-severe to profound sloping loss in her left ear and a severe-profound loss in her right. Pretty much what we already knew, although I had not realized that her left ear sloped into the range of profound. I never trusted the original audiogram done back in June by Lily's former audiologist (she was the one who missed her chance to tell us how potentially serious Lily's hearing loss was when she was 9 weeks old). I am sure there was some magical thinking on my part that this has all been just a big mistake and that really she is OK. But the ABR/ASSR is performed by a computer and is just about as objective a test as you can get for a small child and it clearly shows Lily is deaf. The projected audiogram from the ABR/ASSR is almost identical to the one Lily had in the soundbooth in June. I also had Lily's current and really wonderful audiologist test her left ear without her hearing aid this past Tuesday. (I also talked about the report written by Dr. White about Lily's testing at UF with Lily's audiologist. I understand it better now since it has been explained and am no longer worried about it.) Once again, there was an almost exact replica of that first audiogram. So now we have 3 different tests saying the same thing and at least there is no room for questioning what is her level of loss. Now we have to wait until we hear from the cochlear implant team. It is up to them to decide if Lily's residual hearing in her left ear is enough for her to get by in life. I don't think it is.

And yet another trip to Gainesville (and Orlando, too!)

2006-11-14T02:00:00.000-05:00

Last week was very busy. Charlotte had an apppointment last Tuesday with the orthopedic surgeon who fixed her arm on October 1st. He yanked out the two 3 inch steel pins from her elbow. Without out any anesthesia. It was pretty intense, to say the least. She didn't cry when she broke her arm in the first place but more than made up for it at this appointment. Not easy to see your child in so much pain and fear. I assumed that the doctor would at the very least numb the area in some way. Lesson learned was never assume anything and to call ahead to find out what a procedure will entail.

So on Wednesday, we left at 6:00am for Gainesville. Charlotte had an 8:45am dentist appointment and Lily had a 10:45am appointment with the ENT, Dr. Antonelli. We had hoped to hear the results of Lily's sedated ASSR/ABR testing from the week before and for a clue about whether or not she is an implant candidate at this appointment. The results had not yet been recieved (they are on the way in the mail, though, and I found out that the doctor who performed the ABR/ASSR is very meticulous and writes up his own reports following the procedure, rather than delegating to a resident). We did get to see the CT scan images of Lily's inner ears. It was clear, even to my untrained eye, that there is something abnormal about her inner ear.

We asked Dr. Antonelli a few questions regarding genetics. I have done a considerable amount of reading on the genetics of hearing loss (and more specifically, LVAS) and have a decent basic understanding at this point. With the underlying assumption that Lily's hearing loss is due to a recessive genetic cause, I asked Dr. Antonelli if he thought that it would be a good idea to do a CT scan on Charlotte to see if her inner ears are normal (I recently read about a 30 year old woman who had normal hearing her whole life and then begain losing her hearing rather suddenly. It was due to LVAS and she had a deaf brother with LVAS). Dr. Antonelli said it was probably not necessary to do a CT scan on Charlotte but to have her hearing checked yearly. He also then said that Lily's hearing loss is not necessarily genetic- it could be due to an assault on the developing embryo, either by drug or viral pathogen. I think this can be ruled out in my case though, because I was not sick in the critical time period and I took only a single Tylenol during my pregnancy around the 18th week. Here is a rather technical but helpful discussion of prenatal ear development:

the ear starts developing around the sixth week of gestation. The first and second arch give rise to the sixth helix. By the eighth week, the surface epiderm of the first pharyngeal groove starts to grow inward towards the middle ear. This becomes the epithelial layer of the tympanic membrane. By the 12 th week, the helix develops and by the 20 th week you have obtained full adult shape. It is not so later in life when the auricle obtains its full adult size. Moving on to middle ear development, around the sixth week of gestation, the malleolus and incus appear as a single mass. About a week later, the stapes arch appears and by the eighth week we begin to see the joints of the malleal, incudal, and incudostapedial joints . At birth, the ossicles are adult size and shape and only slightly grow postnatally. The mastoid area begins to develop around one year of age. Inner ear development is interesting in that it begins very early. In the third week, the neuroectoderm and ectoderm form the otic vesicle and it is not until the sixth week when the semicircular canals begin to take shape, as seen here, and the cochlea begins to form its basal turn. By the ninth week, the semicircular canals and the utricle are fully formed, the cochlea begins to take its turns, and by the 12 th week, the cochlea has a full 2.5 turns. That was the labyrinthine portion of the inner ear. The vestibular aqueduct and the endolymphatic sac are unique in that the labyrinth obtains its adult shape and size by mid fetal life, but the vestibular aqueduct and the endolymphatic sac undergo changes late in fetal life and undergo changes in the postnatal life up to age three. As you remember, the endolymphatic sac sits in the posterior cranial fossa. The posterior cranial fossa continues to grow postnatally and it tends to draw the vestibular aqueduct and the endolymphatic duct, thus giving it the long, narrowed, and J-shaped appearance. Around the fourth week of gestation, the vestibular aqueduct appears and is at its largest dimension at the fifth week. The vestibular aqueduct around the fifth week of gestation is short, stubby, and wide. Jackler proposed that some insult might occur around the fifth week, causing it to be arrested in development. Going on to further anatomy of the labyrinth; here we see the endolymphatic sac, which comes across attached to the inner ear with the endolymphatic duct. If you notice, the endolymphatic duct is connected to the utricle and saccule as well as the cochlea via the ductus reunion. This comes to important play when we talk about some of the mechanisms of how hearing loss occurs in enlarged vestibular aqueduct syndrome.

It was a bit confusing to have him focus on the fact that there could have been an environmental cause for her hearing loss. Everything that I have read points to recessive genetic causes. I think we will pursue the genetic testing anyway to satisfy our own curiosity. I would think that our extended families would want to know as well, since there is the potential that all of Charlotte and Lily's cousins could be recessive carriers for LVAS as well. It is not that I don't think that Dr. Antonelli knows what he is talking about in regards to the cause of Lily's LVAS and cochlear dysplasia, but I do think that a geneticist would be a better source of information.

I also asked if we should have genetic testing to rule out Pendred Syndrome. He said that when he first started seeing cases of LVAS with cochlear dysplasia, he would have all of his patients tested for Pendred's. He said that none of the tests ever came positive and so he stopped referring his LVAS patients for Pendred Syndrome testing. Once again, I think we will pursue it to satisfy our own curiousity. Pendred's is serious enough that I want to be able to say definitively that Lily does not have it.

That was about all our appointment entailed. We had high expectations that we would get an answer regarding the implant that day. Dr. Antonelli said that the implant team would meet again this month to discuss Lily's case and that we would get a phone call sometime this month. He needs to see the ASSR/ABR results and all other recent testing first. We got a written report in the mail of Lily's appointment with Dr. White from 3 weeks ago when we got home on Friday. Even though I thought she and I agreed that the testing seemed to be inaccurate and that Lily was just placing the puzzle pieces down after waiting a few moments and not necessarily when she heard sounds, it appears that Dr. White wrote up the testing session as though it was valid. I didn't even read the whole thing because it frustrated me so much. I need to reread it in a different frame of mind.

I can't end this post without mentioning that when we left Dr. Antonelli's office, we continued on to Orlando. We spent 2 nights at a resort at Disney World. It was just what we needed after all the crazy stuff we have had going on. We had a really great time. Lily and Charlotte had lots of fun at the Magic Kingdom and we are already planning our next trip......

Kicking and screaming

2006-11-05T21:18:00.000-05:00

So last week was pretty eventful. Lily and I drove down to Gainesville alone on Halloween evening while Chris and Charlotte stayed in Tallahassee. Her ASSR/ABR was scheduled for the following morning. It was the first time that I had ever spent a night away from Charlotte since she was born. It was a rough trip to do on my own but it would have been hard whether everyone came with me or not. Lily and I spent the night at a hotel. She slept all night without waking even once, thankfully, because she was not to eat or drink after midnight. I didn't sleep the entire night- I worried what I would do when she woke up and how I would explain to her that she couldn't have anything in her stomach. Lily becomes violently angry in her sleep when she is the least bit awakened by anything and I was afraid of how loud and furious her reaction to being away from home and being told she couldn't have a drink would be if she were to wake. The other thing I was worried about- how was she going to react to the anesthesia when she awoke from the procedure the next day.

Lily and I headed out of the hotel room at a very dark 5:30am. I had only a basic idea as to where I needed to go (and no map- duh) and I got so lost on the way to the hospital. There were some police officers hanging out in an alley way on the main bar strip near UF's campus and I flagged one over to get some help. It turns out I was only about a mile and half away from AGH Shands Hospital. I had been driving aimlessly in the dark and somehow managed to get near the hospital. We walked in at 5:55am and signed in. By 6:30, Lily and I were sitting in a surgery pre-op area. We were assigned a tiny space with room for a hospital bed and a chair and no more than that. We would sit in that tiny space for two more hours while waiting for everything to fall into place. There were 11 other tiny spaces like that one, all filled with other children awaiting various procedures.

We had a really nice nurse assigned to us. She was very sweet to us. She was also a mother so she understood my tremendous anxiety about what was going to happen that morning. It was a long time to sit there and I had lots of time to think about what I was about to do to my child. Lily would be going under general anesthesia, with all the risks that entails (and not to mention emotional upset for the both of us), for a completely voluntary procedure. I certainly thought about just skipping the whole thing. But we needed clear answers about her hearing loss that were just not going to be obtained in the sound booth and I decided that we had come too far not to follow through with the procedure. I talked with the nurse about what it would be like when it was time for Lily to go in. She said depending on who was in charge that morning (meaning whoever was the head operating room nurse) would determine how things would go. She said that sometimes the head O.R. nurse will let parents put on scrubs over their clothing, take their drugged-up-on-Versed child into the O.R., place their child on the bed, and allow them to stay until their child is completely asleep.

I was counting on this being the scenario but when it came down to it, the head O.R. nurse on duty that morning was not the child-friendly type nor was he the least bit flexible. After Lily had Versed squirted into her nostril, which left her shrieking, kicking, and screaming in shock and utter despair for 5 minutes, I had to hand off my little girl to a stranger while he took her to a room I never got to see. She was goony-drugged up at that point and was no longer crying (she was leaning her head back and forth and looking at me with drunken eyes saying, "Whoa, Mama" by then). I walked as far as he would let me with her in my arms, tears streaming down my face, and I told her that I needed to go to the bathroom and I handed her away, as she looked back at me with a scared and puzzled look on her face,her arms reaching for me. It was a terrible feeling and I felt like I had betrayed her. I felt like I had not been strong enough and had not insisted enough that I be with Lily until she was asleep. I had tried my best and even tried reasoning with him that this was not surgery, there was not the worry about everything being sterile like there would be with a surgical procedure, this was just a simple hearing test. He had his rigid rules and he was not going to bend them for me. He said that she would not remember it anyway, I said she would bury it in her subconcious and that it was a matter of her trusting me to keep her safe at all times. He said that I must be the one suffering from separation anxiety. As you can see, we were fast friends.

I sat in a surgery waiting room for 2 hours trying to distract myself from worry. I had been under the mistaken impression that this would be a brief procedure, maybe 15 minutes per ear tops. I tried to watch the Ellen Degeneres show and the Megan Mullaly show to distract myself but the TV was muted so I could only guess what they were talking about. There was a mullet-sporting dude with a particulary vulger and absurdly funny t-shirt on there in the waiting room who provided a bit of needed amusement (I think Chris would be horrified if I typed out what was on the shirt so use your imagination :P).

At around 10:30, the pre-op nurse who had been so kind to us came rushing in to the room and sat next to me. I was terrified that something had gone wrong. It turns out that they woke Lily up before they came to get me (something I told them would be a big mistake) and she was completely out-of-control. I rushed back to the post-op area with the nurse to find Lily violently kicking, screaming, crying hysterically, thrashing, pulling at the IV in her arm, and just generally going crazy in the arms of another nurse. She was almost beyond reason and it took me 15 minutes to get her calmed down. I knew it would be bad when she was awakened from the anesthesia but not that bad. It was about an hour before they would give us the green light to go home. Lily guzzled a large milkshake on the way home and she stayed awake for the entire 2.5 hour ride home. She and I went to bed at 5:00pm that night and slept all night through to the next morning.

So that is all in the past and we now have an exciting appointment to look forward to on Wednesday. I don't know for sure but am pretty postive we will know one way or the other if Lily will be getting a cochlear implant soon. This is a big week for us- Charlotte gets the pins removed from her arm on Tuesday, we see Dr. Antonelli on Wednesday, and then we are off to Disney for a couple of days. We really need a little fun trip after the rough couple of months we have had.

-Kriste

Here is a cochlear implant propoganda-ish story about a family with 2 kids with Lily's condition, LVAS.

Here is an article supporting bimodal-binaural hearing and I plan to give to the ENT tomorrow.

Last minute trip to Gainesville today

2006-10-27T17:46:00.000-04:00

Lily and I drove to Gainesville today to meet with the cochlear implant evaluation team. We were scheduled to meet with them on December 20th, which seemed like an eternity to wait, so when the call came yesterday asking if we were available to meet this morning at 9:00am, I jumped at the chance. It turns out that we met with only one person, Dr. Lori White, and she was a very kind and knowledgable person. She was great with Lily and it felt like she really listened to what I had to say in regards to why I want an implant for Lily. She had some audiology students do a bit of soundbooth testing on Lily and it was obviously inaccurate. Thankfully, she recognized that and will wait to get further testing results from next Wednesday's ASSR. She asked that I get Lily's audiologist at FSU to do another round of soundbooth testing without her hearing aids in her ears so that we have several ways to "prove" Lily's levels of loss. Lily has become very accustomed to soundbooth testing and was faking everyone out by placing puzzle pieces in a puzzle after sounds were made in her headphones. I know her well enough to know that it was not a fair test and that she was just placing the peices down after pausing a few seconds. She would even pause and place a piece down when no sound was made.

We have just 2 more appointments to go to and we will know if Lily is a candidate for an implant or not. We see Dr. Antonelli on November 8th for a follow-up appointment and we may just find out that day what their answer is. Dr. White admitted that Lily is not a clear cut case, as she has a decent amount of residual hearing in her left ear at this point. But she agreed with me that hearing one only one side is a huge barrier to learning (read this man's account of life with one working ear!) and that two ears are better than one. They will also take into consideration that Lily's inner ear anatomy predisposes her to further hearing loss. And weirdly enough, the fact that Lily's cochlea is malformed could work in her favor eventually. She said they are a very conservative clinic and do very few bilateral implants because in a deaf patient with normal inner ears, they like to keep one ear available for future technological developments. When a cochlear implant is inserted, it destroys the cochlea and there would be no going back to it's former state. In Lily's case, her cochleas are already "damaged" so that would make her a more likely bilateral candidate if she should lose the rest of her hearing in her left ear.

Effects of Hearing Loss on Development

2006-10-12T13:56:00.000-04:00

American Speech-Language-Hearing Association (2005)

It is well recognized that hearing is critical to speech and language development, communication, and learning. Children with listening difficulties due to hearing loss or auditory processing problems continue to be an underidentified and underserved population.

The earlier hearing loss occurs in a child's life, the more serious the effects on the child's development. Similarly, the earlier the problem is identified and intervention begun, the less serious the ultimate impact.

There are four major ways in which hearing loss affects children:

1. It causes delay in the development of receptive and expressive communication skills (speech and language).
2. The language deficit causes learning problems that result in reduced academic achievement.
3. Communication difficulties often lead to social isolation and poor self-concept.
4. It may have an impact on vocational choices.

Specific effects:

Vocabulary

* Vocabulary develops more slowly in children who have hearing loss.
* Children with hearing loss learn concrete words like cat, jump, five, and red more easily than abstract words like before, after, equal to, and jealous. They also have difficulty with function words like the, an, are, and a.
* The gap between the vocabulary of children with normal hearing and those with hearing loss widens with age. Children with hearing loss do not catch up without intervention.
* Children with hearing loss have difficulty understanding words with multiple meanings. For example, the word bank can mean the edge of a stream or a place where we put money.

Sentence structure

* Children with hearing loss comprehend and produce shorter and simpler sentences than children with normal hearing.
* Children with hearing loss often have difficulty understanding and writing complex sentences, such as those with relative clauses ("The teacher whom I have for math was sick today.") or passive voice ("The ball was thrown by Mary.")
* Children with hearing loss often cannot hear word endings such as -s or -ed. This leads to misunderstandings and misuse of verb tense, pluralization, nonagreement of subject and verb, and possessives.

Speaking

* Children with hearing loss often cannot hear quiet speech sounds such as "s," "sh," "f," "t," and "k" and therefore do not include them in their speech. Thus, speech may be difficult to understand.
* Children with hearing loss may not hear their own voices when they speak. They may speak too loudly or not loud enough. They may have a speaking pitch that is too high. They may sound like they are mumbling because of poor stress, poor inflection, or poor rate of speaking.

Academic achievement

* Children with hearing loss have difficulty with all areas of academic achievement, especially reading and mathematical concepts.
* Children with mild to moderate hearing losses, on average, achieve one to four grade levels lower than their peers with normal hearing, unless appropriate management occurs.
* Children with severe to profound hearing loss usually achieve skills no higher than the third- or fourth-grade level, unless appropriate educational intervention occurs early.
* The gap in academic achievement between children with normal hearing and those with hearing loss usually widens as they progress through school.
* The level of achievement is related to parental involvement and the quantity, quality, and timing of the support services children receive.

Social functioning

* Children with severe to profound hearing losses often report feeling isolated, without friends, and unhappy in school, particularly when their socialization with other children with hearing loss is limited.
* These social problems appear to be more frequent in children with a mild or moderate hearing losses than in those with a severe to profound loss.

What you can do

Recent research indicates that children identified with a hearing loss who begin services early may be able to develop language (spoken and/or signed) on a par with their hearing peers. If a hearing loss is detected in your child, early family-centered intervention is recommended to promote language (speech and/or signed depending on family choices) and cognitive development. An audiologist, as part of an interdisciplinary team of professionals, will evaluate your child and suggest the most appropriate audiologic intervention program.

©2005 American Speech-Language-Hearing Association.

Getting back to the new "normal"...

2006-10-06T20:18:00.000-04:00

Things were a bit off-kilter this week since Charlotte's injury occurred but we are slowly getting back to normal. I emailed Dr. Antonelli to ask what I needed to do to get the appointment for the ASSR scheduled. The next morning, the person I had been having a hard time getting a hold of to arrange Lily's appointment finally called. She talked to Chris (I was at FSU with Lily for her AV session) and gave him a date of November 1st for her ASSR. Chris did not ask her what time the procedure will be but I sure hope it has been scheduled for first thing in the morning. Lily will have to have no food/water for 6 hours before the procedure. While I am relieved that we finally have a date for the procedure, I have 2 reservations about this date- one is for superstitious reasons (a few coincedental family accidents happening on this day: sister badly burned as a toddler, brother broke arm falling from tree, I fell and knocked out my permanent front teeth at age nine) and the other because Charlotte is over-the-moon excited about Halloween this year and is so excited about going trick-or-treating. We can trick-or-treat while we are in Gainesville, I imagine. So we will forge ahead with the November 1 date...

We have a follow-up appointment with Dr. Antonelli on November 8th. I have lots of questions since learning about Lily's EVA. I plan to ask how to go about doing genetic testing. We need to find out if she has Pendred Syndrome or not. I have been doing more and more reading about EVA and all the possible issues that this involves. I also want Charlotte to be tested to find out if she is a carrier- I think it is important for her to know this. It is strange to think that one of my parents passed this recessive gene along to me and that is has silently lurked in my family through the years. We know very little about my grandfather's background since he was adopted so don't know if there is a history of deafness there.

One more month and we will have more answers....

Now things are even more interesting...

2006-10-02T12:17:00.000-04:00

Charlotte broke her arm yesterday evening. She has a Supracondylar Fracture of the Humerus. I don't know what we will do with the month of October in regards to all of our appointments, but maybe it will be best for us to take the month off anyway. Charlotte is doing well, just hates the cast.

All October appointments are out the window...

2006-09-30T12:22:00.000-04:00

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)...

2006-09-30T11:18:00.000-04:00

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....

2006-09-30T11:02:00.000-04:00

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....

2006-09-26T17:32:00.000-04:00

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

2006-09-21T21:49:00.000-04:00

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

2006-09-15T21:11:00.000-04:00

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

2006-09-08T18:31:00.000-04:00

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....

2006-09-04T08:15:00.000-04:00

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

2006-09-03T20:23:00.000-04:00

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

The day after...

2006-08-29T21:42:00.000-04:00

There is a lot to say about our appointment yesterday but not enough time to write it out yet. The abbreviated version is that Dr. Antonelli has agreed to take Lily on as a patient at this point and he and his team will do a study on her. There will be lots of testing to be completed and then he and his team will have a meeting to discuss her implant candidacy. I liked him. I was so worried that I wouldn't like him but he was nice.... -Kriste

3 days and counting

2006-08-25T18:20:00.000-04:00

There are three days left until we go to see Dr. Antonelli at UF Shands. Part of me is terrified that he will say that she is not a candidate. The other part of me is terrified that he will say she is a candidate. The surgery seems scary and it is hard to imagine them shaving off half of Lily's pretty blonde curls and making a permanent, quarter-sized hole in her skull.

Part of the process in getting ready for this appointment has been gathering up copies of all the evaluations that Lily has had and of all the audiological testing. In an earlier post, I described my memory of Lily's early tests. It turns out I was pretty close. Lily did pass the newborn screening in her right ear and it was because she was too awake that testing on the left ear was incomplete. It was 3 weeks after the newborn screeening (not 6 like I thought) that we were at the audiologist. The report written by the audiologist says that Lily passed the ABR in her right ear. It says with more description what parts of the ABR she passed in her left ear but does not come out and clearly say that she fails in her left ear. The OAE says she passed in her right ear and that further testing is necessary for her left ear. There is some terminology and a few graphs that I am unable to interpret at this point. (I recently did an audiology quiz on a site for audiology students and I did pretty well. Not an expert by any means but I know a lot more about ears than I ever thought I would.) I am hoping that Dr. Antonelli will be able to explain to us what he thinks about those tests from 2 years ago.

It is very likely that we will never find out why Lily has hearing loss. There is only one conclusion to make about Lily's hearing when you compare those early tests to Lily's most recent ones: her hearing loss has been progressive. There are many possible causes of progressive hearing loss, such as Unknown Nonsyndromic Genetic, Syndromic Genetic (like Enlarged Vestibular Aqueduct Syndrome) , Meningitis, congenital Cytomegalovirus (CMV), Neural degeneration, Teratogen (Chemotherapy), Low birth weight/premature, and Perilymph Fistulas. I haven't explored all of these causes yet and am not sure if I ever will. Some are easy to rule out, like meningitis, Perilymph Fistulas, and chemotherapy. She has never been on any antibiotics (of which some are ototoxic) and has never had much more than a minor cold or stomach bug. If Lily is a cochlear implant candidate, then we may find some answers. Or not.

I talked on the phone today with the mother of a 15 year old girl here in Tallahassee who has bliateral implants. Her daughter received her first implant at age 2 and was, at the time, the youngest person in Florida to receive an implant. Children as young as 6 months are receiving implants in some parts of the country these days. Anyway, we are meeting with this family on Wednesday evening next week. It is my hope that we will have Deaf/Hard-of-hearing role models for Lily as she grows up. Maybe this girl will turn out to be one of them.

Oh, and we are still waiting for Lily's hearing aid but I have recently had a lot of help from our service coordinator/Program Director of Early Steps. She tells me that I am keeping her on her toes. She has been talking with Lily's audiologist and with someone from the State of FL and it looks like we will likely be able to get even more financial help with Lily's aid. We have been told to hold off until after our appointment with Dr. Antonelli because they think that he will want to aid both ears initially. I am not sure why there has been such a struggle in getting this aid. Surely there are other parents of children with hearing loss that have been through this process. 1 in 600 children in Florida each year are born with hearing loss. There were 3900 babies born in Tallahassee last year. That means roughly 6 kids per year, which means about 18 kids 3 and under that are served by the Early Steps/SHINE program. I just don't understand why it seems like they don't know what to do with us. Maybe I ask too many questions....

-Kriste

Cochlear War

2006-08-19T21:13:00.000-04:00

I have been almost forcing myself to read the other side of the argument regarding cochlear implants. This argument comes from the Deaf community. It seems important to understand their views, since they have actually know what it is like to live the life of deaf person, and I have no firsthand knowledge of this. For Lily's sake, I have sought out the deaf persepective. I came across this site, Cochlear War, which has an open letter to parents who are considering implanting their prelingual children. It was an eye-opening thing to read and gives me lots to think about in this week before our appointment with the cochlear implant surgeon.

There is a lot to read in that letter and I have read it a couple of times now. In my last re-reading, I picked up on this part, which I missed the first time around. Makes me feel better somehow...

We don’t believe that a cochlear implant should be installed in prelingually deaf babies and young children. If a child already has experience in hearing and speech before losing her hearing, and has become profoundly deaf as a result of sickness or trauma after learning how to speak, or was born hard-of-hearing but lost her hearing gradually, we have no objection to the implant, because the child is simply reconnecting with her previous mode of communication. That child already has a basis for spoken and aural language. An implant will enable the child to build on the existing foundation.

On the opposite of the spectrum, this story came out about a little girl in Boston who was just bilaterally implanted: Special Ear Implants Help Girl, Born Deaf, To Hear. There is a short video to watch and you can hear her converse just like a hearing person. I wonder how hard she had to work to speak that clearly. I wonder if we can pull off Auditory-Verbal Therapy in a way that won't make Lily need therapy as an adult.
- Kriste

The background on Lily's hearing loss...

2006-08-15T14:07:00.000-04:00

There is a bit of background on Lily's hearing loss that we haven't mentioned yet and is an important piece to this story. Lily was born at The Birth Cottage on the bed in the Hydrangea Room on June 26, 2004 at 9:15pm. It was a normal birth, although much harder than Charlotte's birth (that is probably because Lily had a 15.75" head!). Because we were not at the hospital, Lily did not have a newborn hearing screening right away. Chris switched jobs not long after Lily was born and we were in insurance limbo for a few weeks.

We traveled to Tallahassee Memorial Hospital when Lily was 6 weeks old for her to finally have the newborn hearing screening. Her hearing was tested with the ABR test, or Auditory Brainstem Response. Her right ear was checked first and she quickly received a "pass" for this test (remember- this is the ear that has the profound loss!). She was deeply asleep for this ear. When it came time to switch to the left ear, Lily woke up a bit. Babies need to be asleep for this test to be accurate. We went ahead and tested the left ear even though she was not fully asleep. It took forever for this ear and the test administrator had to keep adjusting the wires that were attached to Lily's head because she was wiggling so much. After a long trial, the tester indicated that she didn't think we were going to get a good reading that day and she was going to have to refer us to Audiology Associates for further testing. I was glad to go, as I had a 2 year old Charlotte along with me and she was ready to go as well. The tester said she thought that Lily's abnormal results in her left ear were likely due to Lily's squirminess and that there was nothing to worry about.

The appointment was set for us with Audiology Associates for a date 6 weeks later. Since there was no emergency on their part to get us in as soon as possible, I still had little concern about Lily's hearing. I have not yet seen Lily's complete file so I am not positive about what tests were given to Lily that fall day in 2004. I can only guess at this point what tests she was given that day based on what I now know about audiology. I believe that the audiologist tested her ears first with the Otoacoustic Emission Test (OAE). She did not share the results with me and I didn't know anything about audiological testing then so I didn't know what to ask. Since Lily's left ear was the questionable one from the newborn screening, the audiologist started the ABR test on this ear. Once again, Lily was very difficult to test. She was squirmy and restless and her normal cranky self. Each time Lily squirmed, the test had to be reset and started over from the beginning. We were there for over 2 hours. Finally,the audiologist told me simply that the results were "inconclusive" and that it was hard to get a good reading due to Lily's restless behavior. She did not tell me that there was something wrong with Lily's ears (something that she could tell from the OAE), she just told me that further testing would be necessary in a few months when Lily was older and could be sedated for testing. That did not sit well with me- I did not like the idea of sedating my infant for a test that didn't seem necessary. I blew this test off. The audiologist missed her chance to tell me how important it was for us to find out definitively if Lily had hearing loss or not. Telling me that the results were "inconclusive" was just not enough information. Had she explained to me that sometimes babies are just born with unexplainable hearing loss and had given me the pamphlet I later saw this past June in the ENT's office about infant hearing loss and signs to look for, I might have taken all of this more seriously. Believe it or not, we are still seeing the very same audiologist.

In my mind, Lily was a colicky, very crabby, hard to soothe baby. That was why she didn't get normal test results. She didn't have a hearing problem. How could she have a hearing problem? We don't have any known risk factors. She was not a preemie -she was a 4 days late 9lb4oz baby. There was no birth trauma- she was pushed out in 25 minutes with no drugs. As a matter of fact, I did not take anything harder than an occasional Tylenol during my pregnancy with Lily. She has never had an ear infection, we have no known relatives with childhood-onset deafness, she never had any high fevers. The only thing close to a pregnancy complication was that Charlotte had Roseola when I was 25 weeks pregnant with Lily. Charlotte endured 105 degree fevers and febrile seizures with this illness. I came down with a cold or some virusy thing towards the end of Charlotte's bout with Roseola. But it seems unlikely that this would be the cause since babies are generally protected from most illness in utero.

I will admit that the questionable test results planted a seed of doubt in my mind about her hearing. At each well-baby check-up, I would ask our family doctor his opinion. He also agreed with us that it was not a great idea to sedate a tiny infant. He also agreed that since she had one ear that easily passed the newborn screening, even if there was something wrong with the other ear, she would get the sounds that she needed from her "good" ear and that we could do further audiological testing with her when she was older and no longer needed to be sedated for testing. He even did an old-fashioned tuning fork hearing test on her when she was about a year old because I brought the hearing worry up at her 1 year check up. She responded to the tuning fork being struck behind her back, but I now know that this is a common way for unknowing family doctors/pediatricians to check for hearing loss. Deaf kids are incredibly good at picking up on visual cues. She probably noticed my eyes following the doctor behind her or she watched to see what Charlotte was looking at or she noticed subtle movements that the doctor made.

So that pretty much explains the history of Lily's hearing loss. The doctors believe that her loss has been progressive, although we are not sure about that. Lily has from day one been a difficult baby. When she was a day old, I had this feeling that there was something off about her, although I never would have guessed hearing loss. There was something about the way her eyes darted all over the place instead of focusing in on me that seemed strange. She stared so intensly at lights when she was tiny and could not fall asleep if there was any light in the room, not even the lights from the clock on the VCR. One of the signs of infants with hearing loss is being unable to soothe your child by voice alone- there was no soothing Lily by speaking softly to her. It is hard to look back at pictures of Lily as an infant. There is a blank look on her face that I recognize now as the face Lily makes when she is not sure what is going on around her because she cannot hear. As Lily got older and more mobile, it seemed as though she had an invisible steel helmet on. She felt very distant to me and so unbelieveably stubborn. Now we know why.

-Kriste

Maybe next week will be the week...

2006-08-12T09:00:00.000-04:00

It looks like we have been reassigned and the program director of CHS has taken our case. She has been out of the office for a week so this explains last week's continued delay with the new aid. Maybe this upcoming week will be the week when everything falls into place. From what I understand, CHS must get a denial from our insurance company regarding hearing aid coverage. I was told by our very first service coordinator that all they needed was a verbal denial but it appears that this is no longer true and there must be a written denial (and I hear that this is a new rule imposed by the state). From what I have learned from a Yahoo group for parents of children with hearing loss, hearing aids are denied by insurance companies everywhere and across the board. Seems silly to need a written denial for something that insurance companies are known to deny.

The Dora doll has made all of the difference. Lily has consistently worn her aid since Dora joined the household. She has even asked to wear her aid a couple of times! Maybe she is starting to realize that she has been missing out all this time...

-Kriste

Bilaterally implanted Miss America and more hearing aid waiting

2006-08-10T23:03:00.000-04:00

So it has been a month and we are STILL waiting for Lily's hearing aid. It was July 10 when we choose Lily's pink Oticon hearing aid. Our new service coordinator, whom we have never even met, has resigned, and with her resignation comes more waiting for the proper paperwork to go through to get a little financial help with Lily's aid. In reality, Lily needs 2 aids and if we could just get our hands on the first aid then I could talk to the audiologist in person about why she does not think it is important to aid Lily's right ear. Even with a loss as great as hers in her right ear, she would still benefit from stimulating the auditory nerve. It is frustrating because sometimes it feels like we are dealing with people who don't really care every step of the way.

A new Dora the Explorer doll that can only be held when she is wearing her hearing aid seems to have made the difference today. Lily wore her aid for most of the day. It felt terribly manipulative but we are desperate to get her to wear her aid.

In other news, Heather Whitestone, the former Miss America, has been bilaterally implanted. Here is a story that came out this week about it:

Life -- now in 'surround sound':Ex-Miss America has 2nd implant to aid hearing
By Chris Emery August 8, 2006

Just before doctors turned on her new bionic ear yesterday, Heather Whitestone McCallum saw that her son, John, 6, had lost a baby tooth as he ate a sandwich.

When the device was activated, McCallum got to hear John explain what happened. "A seed in the bread took it off," John said, holding the tooth out to show his mother, who smiled and nodded.

"I like his voice," said McCallum, who underwent cochlear implant surgery six weeks ago at the Johns Hopkins Center for Hearing and Balance in Baltimore and returned yesterday for the activation. It was the most sound she had heard through her left ear since it was damaged by a bout with haemophilus influenza that left her deaf in both ears when she was a child.

McCallum, 33, is an Atlanta resident who in 1995 became the first Miss America with a disability. She had her first cochlear implant, in her right ear, in 2002, and is one of a growing number of people with little or no hearing who opt to have both ears augmented with the devices, which convert sound waves into electrical impulses and transmit them directly to the inner ear.

Strange sounds
After the first operation, McCallum used a hearing aid in her left ear in concert with the implant in her right ear. Some sounds took her a while to identify. Once, sitting in a car with her husband, she heard something she could not identify. "That was me sniffing," he told her.

Other sounds, such as her sons' screaming when they wrestle, were disconcerting. "They weren't in pain, but I had never heard it and didn't know that," she said.

Six months ago, when McCallum completely lost hearing in her left ear, she decided she wanted to get an implant in that ear, as well. "Suddenly, I misunderstood what people were saying to me," she said. "People had to struggle to communicate with me, even my boys."

Having two implants will allow McCallum to hear in "surround sound," an important ability for a mother raising two children, said Dr. John K. Niparko, the Hopkins surgeon who put in McCallum's new implant. Hearing with both ears helps people pinpoint where noises are coming from and focus on one speaker in a noisy room, Niparko said.

He said about 10 percent of the 100,000 people worldwide with implants have them in both ears.

Cochlear implants, developed in the 1970s, now consist of two parts. One is surgically implanted under the skin near the ear, and the other, which is detachable, rests on the ear and is held to the skin by a magnetic attraction between the parts.

Digital signals
The outer portion of the device captures sound waves, converts them into digital signals and beams them across the skin to the internal portion.

From there, minute electrical signals travel down a wire into the cochlea, the snail-shaped portion of the inner ear, where they stimulate the nerve cells connecting to the brain.

When implants were developed, they could stimulate only a few frequencies of sounds in the cochlea. McCallum's implant, on the other hand, can transmit up to 24 frequency bands. "There is a spectrum of sound that is missing, but it is much better than nothing," said Niparko.

The operation, including purchasing the device, costs about $35,000 and is covered by many insurance plans, and by Medicare and Medicaid, he said. People who understand fewer than one-third of words spoken to them might qualify for the implants, he said.

Yesterday, Hopkins staff members first calibrated McCallum's implant by plugging it into a computer and stimulating different tones of sounds inside her ear. Jennifer D. Yeagle, an implant audiologist, tested the 24 frequencies' bands of sound to see which ones McCallum could hear.

"She seems to be hearing the high frequencies better," Yeagle said. "That's because those are the ones her brain hasn't heard in the longest time, so it's noticing them more. As [the neurons] wake up after all these years, they will adapt to what they are hearing."

Later, when the computer was disconnected and the external portion first connected and turned on, McCallum said people's voices sounded high-pitched.

"You sound like a 3-year-old girl," she told Yeagle.

A few minutes later, she said voices were starting to sound more normal and turned on her older implant along with the new one.

"They have their own sound when they are apart, and a different sound when they are together, " McCallum said.

"When they are together, I love it."
________

-Kriste

Trip to Gainesville setup

2006-08-08T00:50:00.000-04:00

Kriste emailed Dr. Antonelli one morning and got a reply 30 minutes later.

Since then she got an out of town referral form and we will be making the 300 mile roundtrip to Gator land. technically I think it is a 2nd opinion form... No one like him in Tallahassee and Shands one of the best.

We will be at Shands on August 28th. We hope that Dr. Antonelli will consider Lily a cochlear implant candidate. She is likely a borderline candidate since she has some residual hearing in her left ear.

-Chris

Blues Clues, Scottish Rite and "Mine"

2006-08-07T23:31:00.000-04:00

Lily said - "I want mine." (sounds like "I wah my") today - referring to a bowl of cut up melon. Her sister had a bowl too. She also has picked up on "in" and "on" concepts. like put my clothes "on". put your foot "in."

Lily saying "Cheese" for camera

She cannot stand her hearing aid - I will say Lily do you want your hearing aid? And she will run away and say - no hear, no ear, no horsey..."Hearing" sounds like "horsey" I guess. (Lily's version of the word horsey sounds like "whforfin" but we know what she means)

No new hearing aid yet... since Children Home Society rep left and didnt send in paperwork... apparently they will pay $500 of cost... why just $500 and why doesnt insurance cover ? Luckily we have credit cards and at this point what is the difference between $2000 or $1500?

Lily loved the Blues Clues movie on last night... Charlotte loved it more but it caught Lily's attention for a good stretch.

Good News for Tallahassee - (I copied whole article bec it will soon disappear)- Kriste met the girl and mom in the article on August 1 at the library.

FSU speech and hearing clinic awarded $20,000
Civic group pledges future endowment totaling $500,000

By Diane Hirth
DEMOCRAT SENIOR WRITER

Jennifer Koch's daughter Karen Moore lost her hearing at age 3 and a half and was deaf for a year before receiving a cochlear implant in one ear.

In her year without sound, the little girl regressed to a 16-month-old's language level.

However, with the help of auditory therapy at Florida State University's L.L. Schendel Speech and Hearing Clinic, the 8-year-old has been catching up. She will be in a regular second-grade class this fall at W.T. Moore Elementary School.

"I love my clinic," Koch said.

The clinic on FSU's campus received a $20,000 check Wednesday from the Scottish Rite Foundation of Florida. The money will be used to increase the number of area children with speech, language and hearing disorders who are diagnosed and treated.

The Scottish Rite Foundation's commitment to the FSU clinic goes beyond the one-time contribution.

"When we come, we come to support this forever," said Robert Goldsmith of Jacksonville, Scottish Rite Foundation of Florida president.

His group will raise money for a $500,000 endowment to support the clinic's work with children in Gadsden, Leon and Wakulla counties. With adoption of the FSU clinic, the Scottish Rite Foundation will be associated with 14 such clinics in Florida.

Stephen Berry of Tallahassee, who is associated with the foundation, said, "It's a dream of ours" to support the clinic's work with children.

Part of FSU's Department of Communication Disorders, the clinic is a teaching and research tool in addition to a service for about 300 children and adults a year. About 18 faculty, 75 undergraduates and 100 master's and Ph.D. students are in the department. The clinic also works with schools, health care facilities and private clinicians.

While some clients pay through their private insurance or programs like Medicaid, "We also provide services at low or no cost," said clinic Director Juliann Woods. Thanks to the $20,000 gift, "That's where we're able to expand," she said.

"There's a critical link between language skills and reading and ultimately school success," Woods said.

One goal of the clinic is to create summer camps for children with communication disorders.

"I hope to create an autism camp," graduate student Nikki Greenwald said.

Florida State University's L.L. Schendel Speech and Hearing Clinic provides diagnostic and therapy services for children and adults with speech and hearing disorders. Call 644-2238 or see http://speechandhearing clinic.fsu.edu for more information. Fees are charged only to clients with the ability to pay.

To contribute to the Scottish Rite Foundation of Florida endowment fund for the clinic, call Robert Goldsmith at (904) 378-8957.
--------

-Chris

Appointments, appointments, and more appointments

2006-08-07T21:34:00.000-04:00

The real whirlwind of appointments and phone calls began in early June.

We were assigned a "service coordinator" through Children's Home Society. Probably not important enough to mention but the the service coordinator was actually assigned by the SHINE program, which is an off-shoot of the Early Steps program, which is an off-shoot of the Children's Home Society. Our initial service coordinator promised many wonderful and helpful things, but in the end, was not really able to deliver all she promised- most likely due to having too many cases and not enough time (and I imagine she was not paid enough either).

Actually, she has already left her position and a person equally as overwhelmed has taken over her cases as of mid-July. The service coordinator is supposed to be someone who helps with coordinating appointments for a family, attending appointments if need be, and just generally a helpful guide through the early days of discovering that your child has a disability.

Here is a quick run down of all of our appointments thus far:

April 13- Appointment with FSU's First Words Project, where we discovered for sure that Lily was language delayed. Also was noted that the "quality of Lily's utterances" was very nasal and unusual.

May 25- Lily has OAE hearing test. Audiologist recommends more testing at a later date. I leave not knowing the name of the test, just that the results were far from normal.

June 8- Lily and Charlotte and Kriste see Dr. Roberts, an ENT, at Tallahassee Ear Nose and Throat. The only ENT center in town. It was a nightmarish appointment with Lily and Charlotte battling over lollipops during the entire appointment. I left knowing that Lily likely had a "moderate" hearing loss based on the OAE- that was devastating enough news. I also left thinking that I didn't want to see that ENT again as he had no rapport with children - He yelled down the hallway - "I am going to need a holder!" He had to hold Lily down physically just to check in her ears... a task done without difficulty by our family doctor, and audiologists.

June 15- Lily has a soundbooth hearing test, which gives us a more complete picture of Lily's loss. This is the first time I see Lily's audiogram and hear the words "profoundly deaf in one ear and moderate-severely deaf in the other". Really devastating news.

June 20- Lily has an evaluation at the Children's Home Society to qualify her for services. It is a formality though as she already qualifies due to her level of hearing loss. The first sentence of the evaluation was "This is a large child" Her weight 38.6 pounds, length 38 inches...97th percentile."

June 22- Lily has a 2 year check up with our family practitioner. We tell him the news. We hope that he will suggest to other parents to be more aggressive if they have a concern about their child's hearing (there is more to this story but will share in a later post)

June 26- Lily's 2nd birthday and appointment with a new ENT. Since the first ENT appointment went so badly, I thought it might be wise to meet with another one in the practice. This one had better rapport with Lily but he was not very helpful. He didn't tell us anything we didn't already know and he didnt make any suggestion that Lily was a cochlear implant candidate. He told us that further testing , MRIs, genetic testing really wasnt necessary and that we don't really want to know why Lily is deaf. If we find an answer, it won't be something we want to hear he said. He also did not suggest a 2nd hearing aide to stimulate the right ear and he said "see you in 4 months".

June 29- More hearing testing and confirmation of what we already knew- Lily is deaf.
We are given a loaner aid today with no instruction on how to use the aid or take care of it. A mold of Lily's left ear is also taken. The audiologist informs me that there is no need to aid Lily's right ear, as it is non-functional. We hear later that this is not true, that the auditory nerve needs to be stimulated even if only loud, environmental sounds are heard.

July 6- A representative from the SHINE program came our house to explain all the options in deaf education. Options ranging from ASL to Total Communication to Auditory-Verbal Therapy are presented. We ultimately choose AVT, which we hope will give Lily the most normal life possible.

July 10- Lily has an appointment to receive her new ear mold. We chose pink and white swirls. The audiologist gives us a different loaner aid until Lily's real aid is ordered and received. She calibrates this loaner aid in Lily's ear (which was loud and scary)and Lily is terrified. From then on, getting the aid into her ear is nearly impossible.

July 14- The SHINE representative returns and Kriste shares with her what communication method we have chosen. Auditory verbal. We hope that this is the right choice for Lily. Some days I see it as the only choice. There is an ongoing debate over Sign Language vs. AV - and whether a person can fit in to the deaf community if they get implant or use AV...or if you are deaf and don't know sign language you cant communicate with other deaf people. Right now with her limited hearing she can say many words so we feel like this is the way to go.

July 21- We meet Janet Kahn of FSU for the first time finally. Childrens Home Society covers cost until age 3 (we hope!). We spend almost 3 hours at FSU as Lily is tested to determine her language capabilities. Lily keeps her hearing aid in the whole time and does pretty well. The deficiences are evident in following multiple step directions, pronouns, prepositions.

July 31- Lily's first AVT session. It will be mid-September before we have another session because of FSU schedule and it is now up to us to keep the momentum going.

-Kriste

Hearing "banana"

2006-07-27T00:56:00.000-04:00

This is something I most people never have to think about...the hearing "banana" and the frequency of certain letters. The X's mark Lily's hearing ability.

But this does explain why Lily liked the bagpipes at the springtime parade and the sounds of a piano and also why she can't hear f s th - sounds also impossible to lip read because just air coming through your lips...

Since the news - we have been speaking, reading directly into Lily's "good" ear, talking louder, and she has a loaner hearing aid (until the new one comes in) but she has only worn for a few long stretches.
-This is a recent article in the washington washington post by Josh Swiller and really gives insight into hearing loss

-Chris

Hearing aid

2006-07-27T00:42:00.000-04:00

So in the past month with numerous not very helpful audiologist appointments we have secured a loaner hearing aid..

Now getting a 2 year old to do anything is hard... especially putting something in your ear... Lily has worn it for a few long stretches and then she says ouch, ear, hear and yanks it out.

But it is definitely hard convincing someone who has functioned quite nicely without hearing very much at all to use this device...

Adventures in Hearing

2006-07-26T22:27:00.000-04:00

Blogging how strange - I really don't have a desire to share my life with the world... but I do this now with the mission to help others in our situation and to help our daughter.

what "situation" you ask...

I could start at the beginning but I can get into that later. For reference Lily just turned 2 years old. About 6 months ago, we took her to the doctor... what is wrong her, is she okay...Is there something in her diet that she is allergic to? She was always on the go, she was/is nonstop, doesn't want to sit in one place, she is stubborn, easily frustrated and she doesn't say many words. She won't sit still for books, she won't be entertained by videos or tv, why is she so loud.

She is fine, we were told by our doctor. From family and others we got the "her older sister" is just talking for her, (her sister is 4), we got the stories - "my brother/sister/uncle didnt talk at all until he was 3" or "lots of kids dont talk", she is a late bloomer, your other daughter was just unusual...

Still concerned we took her in for an evaluation at the First Words Project in Tallahassee, Florida, part of Florida State University's Department of Communication Disorders. They did a communication and symbolic behavior scales development profile.

From report - "Lily was presented with situations that tempted her to communicate. She was active, interactive, and adorable throughout the evaulation.... She frequently displayed shifts in eye gaze between people and objects, and shared emotion by smiling." "Lily used gestures and vocalizations to communicate... and she displayed a high rate of spontaneous communication.

- later on in report - "when asked Lily did not identify familiar people, objects, or body parts. She was ready to be done."
Here was her scoring
social communication 75th percentile
speech 25th percentile
Understanding and play <10 percentile.

We were told to look into audiological testing...

Here is the somewhat shocking test result from 6-15-06

no hearing in the right ear (profound) and 60 decibels in the left(moderate to severe)...