Emma Updates (January 2004 Archive)

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January 2004 Archived Emma Updates

1/31/2004-Here is a great description of the cause of Emma's hearing loss: Enlarged Vestibular Aqueduct.

1/30/2004-Emma is now waving "bye bye" when she hears us say it. She can have her back to us and when we say it she waves! She also tried to say bye bye in therapy today. I looked at the actual MRI as did Dr. Peter Roland. When we asked him about the cochlear dysplasia, he said that he really did not think it was that severe and that it actually seemed fairly mild. This means that FULL ELECTRODE INSERTION is highly possible!!! He did say that the enlarged vestibular aqueducts were rather enlarged and more so on the right side (explaining why Emma had more hearing on the left). The endolymphatic sacs are also greatly enlarged. But apparently he felt the risk of a "gusher" was not as bad as we thought. He said the chance of a "gusher" was about a 30% and that it would not keep this from being an outpatient procedure. We have now had three opinions from three of the four physicians in Dallas and all feel Emma will do great and that nothing in her MRI should limit her. All good news! Amy is continuing to work wonders with Emma by working on her listening and talking skills everyday. It is no wonder Emma likes to walk around saying "mama!" We recorded Emma talking on the computer the other night. Here is her saying "mama." This is actually some of her first words!!! A month ago we never thought this day would come!!! We feel as though a great weight has been lifted from our shoulders. I hated the thought of treating a medical problem without knowing the cause (and seeing it with my own two eyes). It had nothing to do with trusting the medical community (we are blessed to be near so many great professionals). It had to do more with acceptance of Emma's hearing loss for my own good (part of that denial thing again). But like I said, Thursday was the first morning I woke up without that hollow pit feeling in my stomach (since November 18, 2003). I awoke this morning again without it and had an extremely productive day at work. Things are getting better. As for Emma----she still does not know she is deaf and is the happiest little thing you have ever seen. At any given moment, she has a smile on her face and is always joking around and wanting to play. She has also come out of her shell and is becoming much more social and much less shy. Emma is deaf---but someone forgot to tell her.

1/28/2004-MRI Results: The MRI results are back and the results are (for the most part) good. The good news is that Emma's brain has no abnormalities to speak of. It also confirms that Emma should be a great cochlear implant candidate. But the results came back showing that Emma is missing part of the cochlea on both sides (Mondini's malformation or cochlear dysplasia). The report calls it "mild" but I think that is a relative term. The surprise was that the MRI also showed "massively enlarged vestibular aqueducts (EVAS)." This is the connection between the inner ear and the intra-cranial (in the skull) area. This means a few things:

1.) There is an increased risk of what is called a "gusher" at the time of surgery. This means that upon incision to the cochlea, where the electrodes will be placed, there could be a "gush" or leak of cerebrospinal fluid (the fluid the brain sits in). This means a slight increase in the risk of meningitis. However, people that have EVAS are predisposed to this slight increase in risk with OR WITHOUT the cochlear implant.

2.) Surgery will be a little more complicated. Since the inner ear is deformed, then the facial nerve may not be where it is normally meaning that there is an increased risk of facial nerve paralysis, although still rare in these cases.

3.) Enlarged Vestibular Aqueduct is an indication of a potential thyroid problem called "Pendred's Syndrome." From what I can tell, if she has it, it merely means that she might develop a goiter later in life and have to take synthroid. I can not find anything that says that this is a severe problem. The largest problem tends to be hearing loss. Given the family history of thyroid problems on both sides, this sounds like what I already have (given that I take Synthroid).

4.) Emma should still do great with a cochlear implant. The MRI showed a healthy (non-degenerative) cochlear nerve which means the connection from the cochlea to the speech areas of the brain are normal and an implant should function properly.

One other finding on the MRI was that Emma had a smaller than normal sized lateral semi-circular canal. Each side of your inner ear is supposed to have three semi-circular canals that lie at 90 degrees to each other. Their function is balance. Emma's other two are normal but the lateral ones are a little small. We have never seen balance issues in her so we (and our physician) are not that concerned about this finding. All in all the results have an overall positive finding. It could be a lot worse but it also could be a little better. We feel blessed that (more than likely) Emma will still be able to hear again with the cochlear implant. Oh yeah, EVAS tends to result in hearing loss AFTER BIRTH, earlier in her life. If so this means that the auditory part of Emma's brain probably received sound during the critical first 6 months of life. We always said that we thought that she once had hearing. This would explain that. Her auditory memory should still be there if this is the case. If so she should perform well with learning to speak and listen and she already is!!!

The FDA recommendations on meningitis risks are as follows:

1.) Two-component electrode arrays should be withdrawn from clinical use. Only implant systems with single component, minimally traumatic electrode systems should be used. A cochleostomy should always be sealed with soft tissue at the time of surgery.

2.) Patients already implanted with two component electrode arrays should be considered as being at risk of bacterial meningitis and should be referred to specialists in infectious disease for advice regarding vaccination and antibiotic therapy for suspected infections.

3.) All patients with single component electrode arrays implanted in abnormal cochleas (common cavities, Mondini, CSF gusher, enlarged vestibular aqueduct etc) should be considered as being at risk of meningitis and should similarly seek advice from specialists in infectious disease.

4.) Implanted patients who develop symptoms or signs of acute otitis media should be treated as a matter of urgency to prevent the spread of infection to the cochlea.

5.) All cases where meningitis is suspected to have occurred should be reported to the competent regulatory authority in each country. The manufacturers of the implant system implicated in the infection should also be informed.

6.) Prospective patients or, in the case of children, their parents or care-givers should be informed of the risk of meningitis following cochlear implantation. They should be informed about the possible need for vaccination as well as the limited efficacy of vaccines to combat infection.

7.) An independent survey of all European centres should be carried out to determine the true incidence and impact of the problem.

1/26/2004-Emma continues to progress well in AV Therapy. It is amazing that 33 days ago, after our first AV session, I felt that this was going to be almost impossible----was I ever wrong!!! In these short 33 days, Emma has gone from unintelligible babble to MANY formed sounds and even saying "mama" as clear as can be!!! Tonight, Emma saw her favorite thing at AV Therapy--bubbles. She immediately started saying "mama" in an effort to stress what she wanted. 33 days ago this would have been nothing more than her pointing and grunting at whatever it was that she wanted. Once the bubbles were brought out, Emma kept going. Emma also repeated a lot of intonation (changes in pitch) tonight. After therapy, Emma "talked" up a storm all the way home. We heard "mama" "baba" "puh-puh" "up" and many more. Landon seems to be one of her best teachers as she loves to immitate anything that her big brother does. Emma is also showing increasing affection towards Landon. He is the first person she hugged when we walked in the door from her MRI last Wednesday. Still no results there. The therapy is fun. It is a great time to play and spend with the kids. It is hard to believe how far we have already come in such a short time.

1/23/2004-GOOD NEWS AGAIN!!!! Auditory behavioral testing revealed today that Emma is learning to listen more and more! We had the tests done at the Callier Center. Sound booth testing showed that (with hearing aids) Emma could detect speech at 35 dB!!!!!!!!!!! This is about the level of a whisper! With puretone audiometry (a series of tones played at various frequencies) she heard at about 50 dB across the board. This is an improvement from the 80+dB required without hearing aids!!! This does not mean that Emma's hearing has improved it merely means that the AV Therapy seems to be working in that it is teaching her to LISTEN and be more aware of sound. Emma was also enrolled in a study where Callier will be looking at the cortical response activity. To explain how this works, the brainstem (which DETECTS sound) never sleeps. It ALWAYS hears (unless there is hearing loss). The cerebral cortex (that wormy looking part of your brain) does sleep and this is where the brain INTERPRETS the sounds DETECTED in the brainstem. As a person develops this part of the brain in response to sound, it shows more activity and decreasing latency (the time between the sound stimulus and response). A "baseline" (or reference point) was established the first day Emma received hearing aids. It was performed again today to see how much more aware and how much more development has occurred in that part of the brain. We do not have the results of that yet but we should in a few days. While this news is exciting and it does mean Emma is becoming more aware of sound, it DOES NOT mean that her hearing has or will improve WITHOUT the cochlear implant. The folks at the Callier Center have been a HUGE resource. We are working closely with two audiologists that are familiar with Emma's case. They take the time to explain everything and interpret the results for us. They are also the only ones doing the cortical response test ANYWHERE IN DALLAS!!! The photo of Emma with all the electrodes all over her head is the cortical test.

1/21/2004-Today we went to Children's for Emma's MRI. We were told to be there at noon to fill out paper work. Her MRI was to take place at 1pm. However at 2pm we were told that there was an emergency regarding two critical newborns and that they would be delayed. Obviously we had no problem with this and we enjoyed the one-on-one time with Emma. She napped on my shoulder for about an hour and then at about 2:40pm she was given medication to sedate her. By 3:15pm the MRI was underway. It was tough seeing that little angel lying there lifeless but at the same time the excitement about the implant restoring some of her hearing was good. I must have prayed 3-4 times just while we were there! I told Amy that for some reason, I had an unusual sense of peace about this process. Amy said that she kept her mind off things by counting the pulses of sound the MRI was making. Anyway, after a 40 minute scan, we woke Emma up she downed 2 juice boxes, and was immediately her happy comical little self again. In the car home, she continued to say "mama." We should know some results by Monday. We are not expecting any surprises but we are planning just in case. I received info from the MEDEL corporation yesterday, and will have lunch next week with a representative from the Cochlear Corporation, who is also deaf. The next step in the medical process will be another Auditory Brainstem Response (ABR) to confirm her condition and tell us the ear that is the worst. That will be the ear that we implant. Tomorrow is more AV Therapy and she is progressing great there. Our new friends (Elliot's mom and dad) have proven to be extremely supportive and knowledgeable. We are so thankful we have found them!

1/19/2004-Emma will receive her MRI tomorrow at Children's Hospital in Dallas. This MRI will tell us if Emma's cochleas are malformed or ossified (unlikely). If the MRI shows that the cochleas are not so malformed then she should be a great cochlear candidate. Then the next test will be the ABR to determine the worse of the two ears. The worse ear will be implanted. We plan to have vidoe of Emma's AV sessions online within the week to show how far she is coming. Today marks one month since she received hearing aids and she is a totally different person. Emma is also showing more intonation in her voice which is unusual for a hearing impaired child. She seems to imitate all sounds she hears us say which shows that she still has some usuable hearing in her left ear. Her receptive language is still growing. Now she understands to wave when she hears us say "bye bye! We are excited about the CI surgery. It will probably happen towards the end of February.

1/16/2004-GOOD NEWS!!! Emma's cochlear implant surgery has been pre-authorized by CIGNA and it looks like it will happen in the next 45 days!!! Emma will have to undergo more testing to rule out things and determine which ear to implant. In the coming weeks Emma will be sedated three seperate times for an MRI, another ABR/OAE, and the actual implant surgery. It breaks our heart to have to put her through all this but we have to remain focus on the fact that this is what Emma would want 10 years from now. I am back from San Francisco and could not wait to see the kids. We immediately wrestled in the floor when daddy got home. Emma came up to me and hugged and grabbed me by the hand. She is such a ham! Things are progressing nicely and Emma is making great progress in AV Therapy. We think we have narrowed the implant selection down to the Medel device but still have many questions yet to ask and get answers on. We will update the site again soon. Next time I hope to start an audio clip library of Emma so everyone can start witnessing the progress Amy and Brenda (our AV Therapist) is making with her.

1/8/2004-Emma is "babbling" in more formed words. She is also beginning to talk to herself in front of her make-up vanity she got for Christmas. She is understanding more receptive speech (recognizing what we are saying). She now understands "no," "sit down," and when we are chasing her and playing we say "I'm going to get her!" and she takes off running. Amy asked her tonight if she wanted a yogurt and Emma immediately went to get a spoon and went to the fridge. We were not sure if it was a coincidence or not. I hope to post an audio file of Emma talking soon. Today, Emma looked at Amy and said "mama." She seems to know who "mama" is.

Dr. Peters' office is working hard on getting Emma's Cochlear Implant surgery approved from insurance. It has already gone to medical review and as of 6pm today there is still no word. They DID approve 60 one hour visits PER YEAR (not per condition as we first thought) to Dr. Peters' Auditory Verbal Therapist, Linda Daniel. We also found out that we can really go (out of network) to whoever we want (granted the surgery is approved by CIGNA) and only pay a maximum of $1900!!! It is our understanding that if we stay with Dr. Peters' (in-network) and it is approved then, more than likely, we will pay NOTHING!!! But nothing is set in stone yet and we have not even gotten approval yet. We are praying that CIGNA will come through for us. Of course all of this is thrilling news!!! Now if we just get the word that CIGNA will pay for the surgery then the barriers to every option for Emma to hear and communicate will be removed with the exception of her and our hard work. We are intensely researching cochlear implants from the two companies that make them. Our choices are down to the Cochlear Nucleus Device and the Medel Combi 40+ device. You can see an animation of how normal hearing and a cochlear implant work here.

We also found a new friend on the web that we are corresponding with. His name is Elliot. He just turned 3 and was implanted at 9 months. His website shows his remarkable progress on video and it is obvious that he hears without reading lips or expressions in the videos. He is another inspiration to us. Another great website is by 16 year old cochlear implant recipient Rachel. She recently had her implant fail after 13 years and had it successfully removed and upgraded to the most recent technology. She reports that it works so well that she heard her brother yell up the stairs that they were leaving.

We are so excited (and yet terrified) of the potential for Emma to get a CI. She is still not approved as a candidate as further testing (an MRI) will tell us. We will return to the Callier Center for more cortical testing later this month. This will tell us how much the speech and hearing perception part of the brain has improved since getting her hearing aids on 12/19/2003. Apparently, sound goes from the cochlea, through the auditory nerve, to the brainstem, and then the signal is transmitted to the cortical part of the brain where it is interpreted. This is all measured using encephalography (monitoring of brain waves).

1/6/2004-WOW!!! Emma is really responding to sound and speech with her hearing aids!!! We can tell her "no" on something in somewhat of a normal level voice and she reacts! She knows two words now in her "receptive speech" and those words are "no" and "sit down." Since Emma loves to climb you can imagine those becoming the first two receptive speech words she recognizes. She also made amazing strides today in verbalizing. I worked with her and her puzzle after dinner tonight and, while three days ago she would only tell me the duck said "wa wa wa," tonight she told me that the dog said "oo! oo! oo!" and the cow said "mmmmm." She also decided again tonight that she would read the bedtime story to me. I read to her every night and as I read I point to the individual words as I say them. Tonight she was opening the books, pointing to the words and saying "ba ba wa wa ma ma." She is obviously hearing us with the aids. She also can not wait to get them in and when we take them out at night she is trying to stick her pacifier in her ear like that might help. I also worked with her on "mama and dada" using a photo of me and Amy. An hour later, I said "mama" to her and she pointed to Amy in the picture. So I think her receptive speech is really coming around. That comes before the "expressive speech" which is what we are so eagerly waiting for. Her progress seems to be pulling me and Amy out of our latest "slump" of feeling down and depressed. I was not planning to update this site but once or twice a week but Emma is making new strides so fast everyday that I have to keep posting the newer updates so that we can log all of her achievements. Emma is also making more formed sounds that we have ever heard her make in her life. It is very obvious that she wants to talk and vocalize and I now know it will happen!!!

1/5/2004-It was back to work today for Amy and me. It feels good to get my mind on other things than Emma's hearing loss. I took Emma to AV therapy tonight. In the car on the way I handed her the duck piece of her animal puzzle. I immediately heard from the back seat, "wa wa wa." Then we got to AV therapy and it was like hearing your baby say their first words! Brenda (our AV Therapist) was showing her the bottle of bubbles. Brenda and I would "talk to the bottle" to make the bubble blowing wand appear. We would say "bababa" to make the wand appear and when it was Emma's turn she did her part by saying to the bottle, "bababa." I had to fight back the tears. I knew then we had made a breakthrough. All the way home (an hour in Dallas traffic), Emma decided to practice her new found skill and chatted with me all the way home. She said, "bababab, "wawawawa," "mamamamma," and "da dah." Needless to say that I did not fight back the tears being in the car. I can see how we are actually going to be excited and looking forward to AV Therapy every week now!

We also got the pre-certification process rolling today for Emma's Cochlear Implant. We are scared that CIGNA is going to shoot us down. I am even more terrified that Emma will get a Cochlear Implant and then CIGNA will deny or limit her AV sessions. We are already having trouble getting those covered. We did find out that they did grant us 60 hourly AV sessions with Dr. Peters' AV therapist. But it will take much more than 60 visits. Given that most CI kids require 1-2 hrs per week from implantation until kindergarten. For Emma that means about 300 visits. We think insurance will cover some beyond the 60 and we can always appeal but we do not know. We also plan on buying an AV system for the house. We will wear wireless microphones around the house and it will transmit an FM signal directly to Emma's CI. It will virtually keep us less than 3 feet from her ear no matter where she is in the house.

Emma is being her same comical silly self. She is happier than ever and is really beginning to claim her independence. She goes to get a spoon every night from the drawer and then goes and tugs on the fridge to get a yogurt-----exactly like her brother did at this age. She refuses to let anyone feed it to her. She has to do it herself. She also likes music and seems to hear it with the hearing aids. She dances and claps in her car seat everytime we play Shania Twain in the car! Her brother got her liking Shania. Today was a great day. The speech therapist, Melissa, is coming out from ECI tomorrow, Susie Jasper (deaf educator for the school district) will be coming on Wednesday, Emma will go back to AV therapy on Friday and Landon has to get his final re-check on his arm Friday as well. Emma will have four appointments a week until she is three years old---over a year away. Then she has to tolerate us working with her on learning to talk when she is not at an appointment. But we do get to play as she learns. Today gives me every confidence that Emma will hear (again) and speak someday (and probably sooner than I think). She has already exceeded my expectations.

1/4/2004-It is a new year and Emma seems to really be responding much better than we anticipated with the hearing aids and AV Therapy. She has been to two AV sessions and we have been working with her on the "Ling Six Sounds" principle of AV training. We just returned from Christmas with my family. While at Granny and Granddaddy's house, Emma played AND HUMMED "ring around the rosie" with her cousins. We also worked with her on an animal puzzle. I picked up the duck on the puzzle and, while covering my mouth, said to her, "duck says 'quack quack quack'." Emma immediately responded by saying, "wa wa wa." On our return home we also worked with her on saying "mama," to which she repeated "mamamama." We have continued to work with her on the animal puzzle and she is making more and more "formed" sounds than we have ever heard. We also noticed a change in Emma's personality in the past 3-4 weeks (since hearing aids). She has become increasingly outgoing and much less shy. She will let others hold her now and is increasingly showing affection. Her little comical spirit is brighter than ever and she has to be one of the happiest little ones I have ever seen! Another development in Emma's progress is that now she seems to hate it when we take her hearing aids our and she can't wait to put them in once awake. We have seen her go as far as grabbing them off her dresser and attempt to put them in herself. While we are encouraged and thrilled at this progress, we realize that we have a long road ahead and many more up and down days.

Time seems to be flying by more than ever since her diagnosis. We are documenting all progress, successes, and setbacks in a daily journal. The website has become increasingly busy. We logged some 800 hits in December and January is already busy with many hits from as far as Egypt, Denmark, Australia, and Poland. The website has become my "therapy" for helping us get through this. Things are improving (or we are becoming more emotionally numb---sometimes I am not sure). Things are still overwhelming. It seems that Emma's hearing loss has been on my mind 24/7 since diagnosis. If it were not for Ambien, there would be little sleep. It seems I can no longer get on the computer without doing research on Emma's condition. Our other "therapy" has been visiting the sites of other hearing impaired kids and seeing how far they have come.

Landon still says he is going to be a doctor when he grows up. When asked what kind of doctor he says an "ear doctor so he can fix 'Nemma's broken ears." He found a Frank Netter Anatomy of the Heart book I had. It is EXTREMELY in-depth cardiology and has drawings of various heart procedures. He loves to look at it. He now knows that the electrodes attached to someone during an EKG are called "leads." He surprises us more and more everyday with his level of intelligence for his age. Sometimes we have to remind ourselves that he just turned four.