The $99 Connexin-26 genetic test

For a limited time, personal genomics company, 23andMe, is offering their complete health and ancestry DNA analysis for only $99 (plus a $5 monthly subscription fee - offer ends 12/25/2010.) The regular price is $499, so that is a savings of $400. For $99, you can learn if you have a genetic predisposition towards many different diseases, or if you are a carrier of a gene that could result in a condition in your child.

Included in the 23andMe test is a carrier status report for two of the most common mutations in the gene coding for connexin 26. Hearing losses caused by mutations in connexin 26 are common, and might account for half of all cases of hereditary hearing loss that are not part of a syndrome. This type of hearing loss is almost always inherited in a recessive manner, which means that both parents have to have a copy of a deafness-causing connexin 26 variant. For more information on how common connexin 26 is relative to other causes of hearing loss and deafness, visit the Boystown site.

Part of a carrier status report from 23andMe

It is important to know ahead of time that 23andMe tests for only two of the most common connexin 26 versions - the 35delG version common among people of European descent (and present in approximately 1 in every 50 Europeans) and the 167delT mutation, common in people of Ashkenazi Jewish descent. They do not test for other variants, including the one that is common in Asia, or the many different but less common European ones.

If you buy the test, you will get a special test tube in the mail that you will spit into. After you spit, you mail that back to the company and they will analyze it and then let you know when the results are ready in about a month or so.

The ancestry component of the 23andme analysis can help you learn about your heritage and connect you with other 23andMe subscribers who share stretches of DNA with you and who are your distant cousins. People who are interested in researching their family history (as well as people who are adopted and don't know much about their history) can get some interesting information from this part. There is a discussion board at 23andme where people talk about results.

Matches to other 23andMe users by chromosome using the Ancestry Finder tool

If you have always wondered where you came from, where your hearing loss came from, or if your child's hearing loss is caused by connexin-26, this seems like a way you could possibly find out, and at the same time, learn more about yourself in general.

23andMe also has a blog where they post new findings in the field of genetics and describe how parts of their service work. Check it out at http://spittoon.23andme.com/. If you'd like to read about recent connexin 26 research, see our blog entry from February, 2010: New Connexin 26 Research: Does Hearing Worsen?

Update added 12/26/2010: The sale is over, but I just came across the following article, "How 23andMe Saved My Wife" about a cancer gene that 23andMe screens for.

Please Vote for the John Tracy Clinic

The John Tracy Clinic is in the running to win $250,000, through the Pepsi Refresh Project, to help create miracles - small, deaf children that never stop talking. *smile*

JTC did amazing things for our family and we will do whatever we can to help families walking a similar journey.

Please take a short moment (every day in December) to vote and then share this message with your family and friends.

http://www.refresheverything.com/jtc

Although we'd really love for you to support JTC, if there is something you feel you'd rather vote for, that would be okay too.

There are thousands of awesome ideas!

Thank you!

Article: "Music by People who Can't Hear"

Music by People Who Can’t Hear

Listening in.

* By Robert Sullivan

The Association of Adult Musicians With Hearing Loss held its first New York concert recently, at the Bruno Walter Auditorium in Lincoln Center, with a program entitled “Incredibly Musical and Significantly Deaf: More Music With Less Hearing.”
Charles Mokotoff, a classical guitarist who has a day job working in IT with the National Institutes of Health, was up first. Among his pieces was one by Edin Solis, a young Costa Rican composer whom Mokotoff communicates with via e-mail. Later, Jennifer Castellano played a tonal piece called Spectrum, Seven Preludes for Piano, which she composed after seeing Spectrum V, a painting by Ellsworth Kelly, at the Metropolitan Museum. It was reminiscent of the more percussive sections of Keith Jarrett’s 1975 improvisations in Köln, Germany, though more optimistic. If you had walked in during either performance, you wouldn’t have guessed that either performer was deaf or hearing impaired.

Click Here to read full article.

Interesting Article on Deaf Athletes and Strategies

Noise makers
How deaf athletes deal with noise as part of game strategies
By Carmen Renee Thompson
ESPN The Magazine

Tune it all out. That's the mental approach most athletes try to take in ­pressure situations. Screaming fans, trash-talking opponents, the drumbeat of feet on bleachers ... it's all just white noise. Or so they say. But what if you can't hear any of it? What if you can't hear a coach's instructions or your teammates calling for the ball? What if you live in a world with little or no sound? Nearly three out of every 1,000 American children are born deaf. And that got us thinking: What's it like for athletes who are deaf to compete at an elite level? We caught up with five of them, who shared their strategies for thriving in silence.

Click here to view full article.

Back To School: Helpful Information on Hearing Equipment

The following information was put together by this Mom and sent to school with her son on his first day of preschool. The child currently wears a hearing aid in one ear and Advanced Bionics CI (w/body worn processor) in the other. Feel free to use and modify it as needed!



My child is deaf in both ears. He wears a hearing aid in his left ear and a cochlear implant in his right ear.














Parts of the hearing aid:
• processor (hangs behind his ear)
• earmold (fits into his ear canal)
• plastic tube (connects processor to ear mold)
• microphone (at top of processor)
• red indicator light (on back of processor)
• controls (on back of processor; disabled for pediatric use)
• battery compartment (at bottom of processor)

Because the controls are disabled, the only way to turn the hearing aid on or off is to open or close the battery compartment.

To take the hearing aid off:
• Pull gently on the earmold, toward the back of his ear. (Don’t pull on the tube.) Remove the earmold from his ear.
• Slip the device off of his ear.
• Open the battery door by pulling at the little tab that sticks out.
• The red indicator light should go off.
• Store the hearing aid in the case provided.

To put the hearing aid on:
• Put a small drop of lubricant (Oto-Ease) on the earmold and smear it around.
• Work the earmold into his ear until it is fully inserted into his ear canal.
• Lift the processor over his ear so that it hangs behind it.
• Close the battery door.
• The red indicator light should come on.
• Ask my child if it sounds all right.

Feedback:
If there is a high-pitched squealing sound, this is feedback caused by amplified sound leaking out around the edges of the earmold and feeding back into the microphone.
• Push the earmold more firmly into his ear so that there are no gaps around it.
• Even if the earmold is seated properly, you might hear some feedback if he puts his hand (or another object, like a hat) up around the microphone. Adjusting the position of the object should eliminate the feedback.
• He can’t hear the feedback and it doesn’t bother him. It’s just a nuisance for others.

Protecting the hearing aid:
The hearing aid could be damaged if it gets wet or if it is dropped onto a hard surface. If it’s raining out, my child should wear a hat and/or be under an umbrella. (He can be in a light mist for a short time without protection.) It’s usually safe for him to wear it at a water table, as long as there isn’t a lot of splashing. He should never wear the hearing aid during swimming, bathing, or running under a sprinkler. Care should be taken that other children do not grab the hearing aid off of his ear or attempt to handle it.

Parts of the cochlear implant system:
• implant (under the skin behind and slightly above his right ear; you can’t see it)
• headpiece (plastic disk that attaches with a magnet to the skin over his implant)
• cable (runs from the headpiece down under his shirt to the processor)
• processor (worn in a harness under his shirt)

How it works (roughly!):
• Sound is picked up by a microphone on the headpiece.
• It is carried by the cable to the processor.
• It is digitized, processed so as to optimize speech perception, and turned into a signal that can be sent to his auditory nerve.
• This is sent back up the cable to the headpiece.
• The signal is broadcast across the skin to the implant, which fires electrodes that are inserted into his cochlea and stimulate the auditory nerve.

To take the CI equipment off:
• Slide the processor out of the pouch on the harness.
• Remove the headpiece from his head (the processor will start beeping).
• Turn the program dial on the processor to the off position (it will stop beeping).
• Gently guide the headpiece and cable out of his shirt. Be careful not to tug too hard on the cable.
• Put all of the equipment in a safe location.

To put the CI equipment on:
• Run the headpiece and cable (they should stay attached to each other, and to the processor) under his shirt and out through his collar.
• Turn the processor on by turning the program dial from the off position to Program 1 (1 dot). The processor will start beeping.
• Watch the indicator light, located near where the cable plugs into the processor. It should blink red quickly, 3-4 times, to indicate that the battery is strong. If it only blinks once or twice, the battery is low.
• Attach the headpiece to his head by placing it against his skin behind his ear and feeling around until the magnet clicks into place. The processor will stop beeping. Make sure the cable is not twisted or kinked, or caught on his harness.
• If the system is working properly, the indicator light will blink green whenever it receives a medium-to-loud sound. Say “ba-ba-ba” and watch the light.
• Ask my child if it sounds all right.
• Put the plastic cap on the processor and push it down into the pocket on his harness.

If the processor starts beeping:
• If it beeps at a regular frequency, about once a second, this means that the headpiece has come off his head. Just put it back on.
• If it beeps intermittently, this probably means that the battery is making poor contact or is running low. First try wiggling the battery in its compartment to see if that seats it better. If the beeping persists, turn the processor off and then back on and watch the indicator light; if it flashes 3-4 times (in red, quickly), the battery is strong. If it flashes 1-2 times, the battery is getting low. Replace it with the back-up battery that we provide. (A single battery should last all day, but sometimes they don’t charge properly at night.) Another thing to try is to take the battery out and put it back in to see if it makes better contact.
• If it doesn’t seem to be a problem with the battery, it may be that another part of the system is failing (the cable or headpiece). If it’s early in the day, please contact us so that we can try to fix the problem and he doesn’t go without sound for a long time. If it’s late in the day, wait until we get there. If he seems to be hearing all right, let him continue to wear the equipment. If the equipment is clearly not working, take it off and store it until we get there.

Protecting the CI:
Just like the hearing aid, any part of the CI could be damaged by water or a hard blow, and the same care should be taken to protect it. If my child plays at a water table, he should wear an art smock to protect the processor under his shirt. There is also a slight risk of damage to the CI from static discharge, which can occur if he goes down a plastic slide or through a plastic tunnel. We don’t want to forbid him from using such play equipment, so we’re willing to take some risk. When possible, try to ground him when he gets to the bottom of a plastic slide by tapping his leg or hand (not his head), but we understand that this is not always practical when caring for many children at a time.

From Their Mouths...

CI AM A ROCK STAR!!! VOTE NOW!!!

Music Video for Do Something Battle of the Bands Raises Awareness of Importance of Music Education for Kids with Hearing Loss

Students enter unique video in DoSomething.org and the VH1 Save The Music Foundation Battle of the Bands contest to raise awareness of importance of music education while reducing stigma associated with kids with hearing loss; use Jonas Brothers tune as inspiration.

Jericho, NY (PRWEB) April 12, 2010 -- Following up last year’s first place award in the American Speech-Language-Hearing Association’s Better Speech and Hearing contest, Jericho High School graduate Mel Paticoff re-worked her video, “That’s Just the Way We Hear,” based on a Jonas Brothers tune, to create a new version for DoSomething.org’s Battle of the Bands contest highlighting the importance of music education. “That’s Just the Way We Hear 2” also aims to reduce the stigma associated with kids with hearing loss by demonstrating that they are able to speak, sing as well as hear with the assistance of hearing aids and cochlear implants. Two of the participants are deaf and use cochlear implants to hear.

Producer, songwriter and videographer Mel Paticoff feels strongly about the message of her video. "Teaching deaf and hard of hearing students to learn to love music can improve both their auditory listening skills as well as their social skills. This is definitely where music education money should be spent,” says Mel. As a Fontbonne University student in St. Louis, Missouri, Mel is studying to become a teacher of the deaf and has recently become a children’s book author. She has had years of experience working with deaf students both formally and informally. Her cousin, Julie Rosenthal, is one of the two deaf singers with cochlear implants in the video. Julie was late-diagnosed with her hearing loss at the age of two and spent several years undergoing intensive language therapy at an oral deaf school to learn how to talk.

Teaching deaf and hard of hearing students to learn to love music can improve both their auditory listening skills as well as their social skills. This is definitely where music education money should be spent.

“That’s Just the Way We Hear 2” is open captioned for accessibility and showcases eight Jericho elementary, middle and high school students who play piano, guitar, drums and trumpet while singing an original song to the tune of the Jonas Brothers’ “That’s Just the Way We Roll.” Mel’s sister, Dani Paticoff, a vocal student at Long Island High School of the Arts, developed the base guitar and vocal tracks and displays her talented voice on several solos. The video’s view count has risen rapidly since it was posted on YouTube and at the Do Something website where voting takes place. The organization also featured the video in its email newsletter, citing it for “highlighting the power of music to raise awareness about different issues, break down stigmas, and impact the lives of the hearing impaired.”

Support the students in their endeavor to win and raise awareness about hearing loss and the importance of music education. View and rate the video online at http://www.dosomething.org/bands/entry/sophies-tales You can also show support by blogging, tweeting on Twitter and posting the link on Facebook. While the Battle of the Bands competition ends on April 16th, you can still help by continuing to pass the word on. For more information about Mel Paticoff, visit her website, Sophie’s Tales at http://www.sophiestales.com

Cochlear's Hearing Hero Award Goes To...

On behalf of all members of CiCircle, I would like to congratulate Miss Rachel Chaikof for winning Cochlear’s Hearing Hero award.

Rachel started advocating for others like herself at a very young age and has continued to devote much of her time to cochlear implant awareness. I can’t imagine having accomplished what she has in her short twenty three years. As a young college student, dealing with her own day to day hurdles, she still manages to dedicate time to CI awareness by maintaining cochlearimplantonline.com, moderating deafvillage.com, creating t-shirts, illustrating books, raising funds for various organizations, speaking to the public (not only in the US) and sharing her story. Along with everything else, Rachel continues to be an active member of the Cochlear Awareness Network.

The award is well deserved! As the winner, Rachel receives the opportunity to visit the Cochlear Americas headquarters in Denver, Colorado to share her experiences and meet with Cochlear executives and employees.

To read more about Rachel, visit her here.

Congratulations to all others that were nominated as well. Your devotion to the CI world is appreciated by many! More information regarding the Hearing Hero award can be found here, along with profiles of the other nominees.


...with love from the members of the Pediatric Cochlear Implant Circle

Size comparison of Nucleus 5 and Nucleus Freedom BTEs

If you're an avid CI family blog reader, you may have seen this already. Landon's (a.k.a. Buzz's) mom, Jen, posted some nice pictures on her blog comparing the new Nucleus 5 with the older Nucleus Freedom sound processor. There are photos both on and off Landon's ear (he's about 3 1/2 years old, BTW). Check it out: Landon's New Ears are Here

Also, in case you missed it a few months ago, here is a (captioned!!) video comparison of the Nucleus 5 and Freedom sound processors reviewed by an audiologist who also happens to be Nucleus user.

Congratulations Val and Rachel AND New Youtube CI Channel

Congratulations to two CI Circle family members, Val Blakely and Rachel Chaikof who were recently nominated for a "Hearing Hero" award given to "someone who has dedicated their time to proactively educate others on how Advanced Hearing Solutions can dramatically improve the communication ability and quality of life for those impacted by a significant hearing loss."


Val Blakely: Val has two children with bilateral implants, and she always has an upbeat, positive attitude. She's a loving, patient mom, and her knowledge of CIs and hearing loss is amazing. She is constantly coming up with ideas to reach more parents of children with hearing loss to offer them options and hope.

Val volunteers a great deal of her time to on-line efforts. She writes a popular blog about her two kids with cochlear implants called deafkidscanhear.blogspot.com, she moderates the DeafVillage.com news aggregator site, and she comments frequently at the CI Circle Yahoo group for parents of kids with cochlear implants. Together with me and two other Cochlear parents she helps edit the CI Circle News blog (http://www.cicircle.blogspot.com/). Val also writes the Alabama blog for children with hearing loss with another mom (bamaears.blogspot.com) and often mentions cochlear implants on Facebook.

This is probably just some of what Val does -- she probably does a lot more I don't even know about! And she does it all without being pushy about implants, teaching or communication methodology or implant brand.

*AND*


Rachel Chaikof: I would like to nominate Rachel because she is an awesome role model for my son, Max who also uses a CI. Rachel's devotion to helping others learn about the option of listening and speaking and benefits of the Cochlear Implant is amazing. I have benefited from her website: http://cochlearimplantonline.com/site/immensely. I have learned so much that helps me directly impact my son's life. I also felt pride when she set out with her pals to create http://www.deafvillage.com/. I am in awe and amazement of her abilities, her talents, and her devotion to educating our world, and empowering those like my Max!

Recently, Rachel has undertaken a new endeavor. She has created the first official youtube cochlear implant channel, where she has just begun assembling an impressive collection of many of our Pediatric Cochlear Implant Circle kids- click here to meet our kids!

*Hats off to you both!*

New Connexin 26 Research: Does Hearing Worsen?

Researchers at Children's Hospital Boston and Harvard Medical School have just published a new study on children whose hearing loss is caused by connexin 26 mutations. They wanted to know if hearing loss caused by connexin 26 remains unchanged or worsens. They monitored the hearing of about one hundred children for about 10 years to see what happened. Read on to learn what they discovered.

Background: Connexin 26 is a protein made in the cochlea. (It is coded for in our DNA by a gene called GJB2.) The exact purpose of the protein is not known for sure, but it is thought to help regulate the balance of potassium in the supporting cells. If the potassium balance is wrong, the supporting cells die, and there is evidence to suggest that if they die, the sensory hair cells -- those that pick up sound vibrations and stimulate the auditory nerves -- die also.


A cell-level view of the sensory part of the cochlea (from www.nature.com) (You don't need to try and understand this picture if you don't want to -- it's just for decoration.)

Connexin 26-caused hearing loss is typically inherited in a recessive fashion. In this case, each parent, usually unknowingly, has one healthy and one mutated copy of the gene. If a child inherits a mutation-carrying copy from both parents, the child will have hearing loss. (More details about how hearing loss is inherited can be found in the booklet you can download here.)

Connexin 26-caused hearing loss is common and could account for 10% or more of all cases congenital, or "born with", hearing losses. In other words, if your child was born with hearing loss (and you haven't yet had any testing to find out the cause) the chances are 1 in 10 that it is caused by connexin 26. If your child does not have any kind of syndrome that is responsible for their hearing loss, such as Waardenburg, the odds that it was caused by connexin 26 are even higher, like 1 in 5:


Causes of hearing loss from Boystown's www.babyhearing.org website.

The Research Study The new research described here was carried out by a group of geneticists, otolaryngologists, and audiologists. A total of 126 children were included in the study. Of these children, 33% already had profound hearing loss when their parents agreed to take part in the research. Their hearing loss could not get any worse, so its progression couldn't be studied. The rest of the children had hearing losses somewhere in the mild to severe range when they first enrolled in the study.

Does the hearing loss progress? This was the first question the researchers asked. They found that in just over half of the cases (56%) the answer was yes, the hearing loss did get worse. The rest of the children seemed to have stable hearing levels over time.

Does the type of mutation determine whether it will progress? Many different hearing loss-causing mutations have been discovered in the gene for connexin 26 since it was first discovered in 1997. Earlier research showed that these different mutations can have different effects. Those that cause the protein to not be made at all are the most damaging and usually cause more severe hearing loss. Those that cause it to still be made but in an altered form usually cause less severe hearing loss. What was not known is whether certain kinds of mutations are more likely to cause hearing loss to worsen. This new study revealed that there is no correlation between the particular mutations a person has and whether or not the hearing loss will worsen. In other words, there is no crystal ball. However, for the children whose hearing loss levels did worsen, changes were detected on average 13 months after their hearing loss was first discovered. (A graph in the article shows a pattern of fairly rapid progression over the first year or two followed by a gradual leveling off.)

Why might some individuals' hearing losses progress while those of others with the same mutations stay the same? The researchers wrote that they don't know, but that there could be additional, as yet unidentified, modifier genes or environmental factors involved.

What does this mean for parents? While it would have been nice for parents to have a more definitive prediction of whether or not their child's hearing loss would progress based on which connexin 26 mutations they have, at least knowing that the odds of it being stable are 50:50 is something. Also knowing that progression, if it is going to happen, would likely be seen in the first year or two after the initial diagnosis gives parents at least some time-frame over which fret. Inevitably, our (genetically programmed??) individual human natures will determine just how much sleep we lose over this knowledge.

The full article is published in the Archives of Otolaryngology - Head & Neck Surgery (free full text available):

Audiologic Phenotype and Progression in GJB2 (Connexin 26) Hearing Loss, MA Kenna, MD, MPH; HA Feldman, PhD; MW Neault, PhD; A Frangulov, BS; B-L Wu, MMed, PhD; B Fligor, ScD; HL Rehm, PhD, Arch Otolaryngol Head Neck Surg. 2010;136(1):81-87.

Lydia is the parent of a child with bilateral cochlear implants and hearing loss caused by connexin 26. She is also an audiology graduate student.

Deaf Children and Reading

Reading and Deafness: One Mom’s Journey

When I first found out that Neal was deaf, three things were in the back of my mind. 1) I’m going to have to learn sign language; 2) Neal will never attend a regular school; and 3) He’s not going to read above a 4th grade level. (My husband had just finished a mainstreaming course as part of his teaching credential and been given the “fact” that deaf people don’t usually read above a 4th grade level). Of those three, the last one weighed most heavily on me.

I consider reading to be one of the most important skills in life. It was a plan of mine to instill a love of books and reading in my children from infancy. I gave them books as toys so that even before they could read them, they would consider them a fun thing to have around. I chose books that were nearly indestructible and didn’t take them away if my children used them as chew toys. I was even quoted in a parenting magazine on the subject when my daughter was a baby. It was nothing less than devastating to think that my son might be robbed of the opportunity to enjoy reading.

I needed to remedy the problem. I didn’t run to the computer to research the subject. I didn’t even take time to question if the statement might not be fact. Instead I sat down and thought about it. I asked myself, “Why would a deaf person have trouble learning to read? They can SEE the words after all.” I imagined myself reading and then it struck me. When I read, I hear the words going through my head. I sound out any words I don’t know. What if I didn’t know what sound was? What if I couldn’t “hear” myself think? Others could give you statistics or lead you to articles that endeavor to explain why the average reading level in deaf people was thought to be 4th grade, but this was enough for me. It was probably one of the reasons that I was so open to learning about the use of technology to provide access to sound for my son, and why he ended up with bilateral cochlear implants too. Of course that wasn’t the only reason, and of course it wasn’t all just up to me, but it is a good example of how important reading was to me.

So it became a quest for me to make sure that Neal broke that stereotype. In the year between his diagnosis of profound deafness to the time that he finally had access to sound, I used the sign language that we had to read books to him. He showed interest and I made it part of our day every day. Once he did have access to sound, I started using books to help teach him new language. At the urging of the teachers and therapists at his preschool, we made our own “experience” books with pictures and descriptions of everyday activities. I read books to him paying only minimal head to what the words in the books actually said, instead using the words that he was working on at that time that made sense with the pictures. I used the same books with new words added or substituted as time went on and Neal had more language. I read to him for at least an hour per day. We would read up to 20 picture books before bed sometimes. When he was about five years old, I started reading chapter books to him (the Junie B. Jones series by Barbara Park in particular). I could still modify the language as need be (giving Junie B. language much better than she had in print so that he wouldn’t pick up her habits). I found that he could do it. He could listen even without pictures. Soon we moved on to other chapter book series, and before Kindergarten was over, we had read the entire Chronicles of Narnia.

During this time, I also did what most parents do. I started teaching him the alphabet and the sounds that the letters make. I hung an alphabet chart by his bed and we went through it every night naming the letters and their sounds. I went on to teaching Neal sight words, the words kids need to “know” without sounding them out. He was almost through the first 100 of those by the time he started Kindergarten. I really feel that knowing a certain number of sight words was a big help to him. It gave him some self confidence that he would someday be able to read a book to himself.

In Kindergarten he did start reading short picture books to himself. I had him read out loud to me and try to impart to him the importance of tone while reading, so that he ended up being very good what I would call “non-robotic” reading. His reading flowed well. One book I used to help with this was called Hug by Jez Alborough. It has nothing but the word “hug” in its pages, but I taught him how you could make it have slightly different meanings depending on how you intoned it. I also asked him questions constantly about what he was reading and had him make predictions about what might come next, etc.

By first grade, he was already above most kids in his class in reading skills. He read his first chapter book to himself and passed a reading comprehension quiz on it that year as well. I think that was when I first knew he was going to be ok. He would read at whatever level his brain was made for and being deaf was not going to hinder the process.

Neal is now in 4th grade. He recently took a test that placed him at a 12th grade reading level. He was told by the test administrator that he had the highest score she had seen in a 4th grade student. I’ve had several school personnel comment about that accomplishment to me. I doubt that any of them realize what an achievement it really is though. Sure, any parent would be proud of it, but for me it represented the accomplishment of a personal goal for my child. He would not be leaving 12th grade with a 4th grade reading level. Instead he would be doing the exact opposite.

I still read out loud to Neal to this day. I think reading out loud to your child is at least as important, if not more so, than having them read to themselves. I still ask him questions as we go along, but these days it is just as likely to be Neal who asks me a question or reveals his suspicions about where the story is going (and he’s usually right). I have always read books that are slightly above his language level so as to have new language to offer him while doing it. He also reads to himself and has described himself on more than one occasion as a “reading maniac.” He even has his own book review blog these days, which he is quite proud about.

In the end, none of the three things that I first worried about with Neal being deaf have turned out to be true. He doesn’t need me to be fluent in sign language to communicate with him. We still know a few signs and the alphabet, and maybe someday he’ll decide to learn more. But then again maybe Spanish or French will have more appeal to him. He has attended a regular, mainstream classroom since Kindergarten. No different than his hearing sister. And he most certainly will not be reading at a 4th grade level as an adult.

Having a child with a disability sure does open your eyes to what other stereotypes and misperceptions might be out there in the world about other groups, and what a gift that is!

Written by Rhonda Savage (http://www.mamasavage.blogspot.com/)
Neal's blog (http://savagereads.blogspot.com/)

Cochlear Implants in the Middle of Nowhere

Hello from eastern Montana a.k.a. the middle of nowhere. I am the mother to a four year old, bilateral cochlear implant user and despite having absolutely zero cochlear implant resources in our area, my son continues to excel.

Many of you may be living our same experience, some, not so extreme. We travel 10 hours one way, to Denver, for mapping and Auditory Verbal Therapy (AVT) sessions. There is a CI Center in Western Montana, but nowhere in Montana, North Dakota, or Wyoming can you find experienced pediatric cochlear implant professionals. We rely heavily on online resources, self-education, and we take in as much as possible when we visit our AVT every six months. Our family does not strictly follow the AVT principles, but we continue to receive much support from the AVT at our clinic because of her experience in cochlear implant rehabilitation.

Some families that lack proper resources in their area choose to relocate. We chose not to leave our comfort zone and decided we’d do our best to educate ourselves, our local Speech Language Pathologist (SLP), our local school district, and family and friends, all of whom will contribute to our child’s future education.

Besides the lack of experienced cochlear implant professionals, another huge downfall of living in the middle of nowhere is encountering the unforeseen problems that may arise with the equipment. If maps need tweaking, we can’t just schedule an appointment and go. There was a time when we had to mail a processor to be re-mapped a few weeks after returning home. Thankfully, while at our appointment, we discussed potential problems and the proper way to resolve them. Also, we always need to be financially prepared for an emergency trip and have a travel plan in place. Internal failure or head injury could happen at any time.

After learning of our child’s deafness, we first started learning American Sign Language (ASL). When you have no ASL or Signing Exact English (SEE) educators and you want to teach your child sign language, you, the parent, will be responsible for teaching yourself through books, online sources, videos, or other deaf adults. Our family turned to online resources, such as Lifeprint.com, and we fell in love with the Signing Time videos. In our case, we also had an outreach consultant from Montana School for the Deaf and Blind that taught adult ASL courses.

When we found out that a cochlear implant was a reality for our little guy, we immediately started seeking out oral deaf resources. Our first saving grace was the many blogs written by other parents of CI kiddos. From there, we found an abundance of other online resources, such as the CICircle and Listen-Up Yahoo groups. The amount of information provided by other parents is, at first, overwhelming, but an invaluable tool and very inspiring.

On the activation day, we had our first experience with the Auditory Verbal Method. Many families begin these services, or similar services, shortly after finding out about their child’s hearing loss. By doing so, the family is prepared and knowledgeable by the time the child has access to sound via hearing aids or cochlear implants. Our AV therapist modeled different activities we could do at home and sent us home with some “homework” to complete before our next session.

Other resources suggested early on that we find helpful are the John Tracy Clinic Distant Education Courses and The Listening Room , provided by Advanced Bionics and Dave Sindrey, a certified AVT.

An integral part of our journey was our visit to the John Tracy Clinic International Summer Session. There, children participate in a spoken language preschool designed to identify their strengths and needs and parents participate in an education program to learn about hearing loss, auditory-verbal techniques, emotional support for families, and educational services for their children. We left there feeling armed and equipped to face the world at home where no CI resources existed.

Every week, we attend therapy with our local SLP (Speech-Language Pathologist). There is always a parent, grandparent, or other family member in attendance with the child. We are not there to only learn and progress, but to teach her what we have learned throughout our journey. We know our child better than anyone and we’ve been there every step of the way, paying close attention to all the little details. Our SLP communicates regularly with our AVT and other oral deaf educators, welcomes and uses resources provided by our family, and also uses many activities printed from The Listening Room. Communication among the members of your child’s team is vital, no matter the distance.

As preschool and IEP (Individualized Education Plan) time approached, we found communication even more important than before. We had many consultations with our AVT over the phone, we put our district in touch with our AVT, critiqued the local preschool setting, and spent many hours online researching IEP laws. It is very important to have a good relationship with your child’s educators. Share all of your knowledge and tools regarding cochlear implants and most importantly, communicate regularly. For many teachers in rural places, it will be the first time teaching a child with a cochlear implant.

Our AVT, highly educated in oral deaf education, offered to do an in-service via Skype for our district since no one here has experience with cochlear implants or oral deaf education. On the CiCircle forum, there has been mention of other families participating in remote AVT sessions instead of driving the distance and participating in person. There is not an AVT in every city or even in every state. Click here to find an AVT and ask if they’d be willing to provide remote AVT services via Skype or another web-based conferencing application.

We have found a wealth of knowledge online and we continue to share it daily with our son’s educators and therapists. For more resources relating to hearing loss, please click here.

Despite where you live or the number of available resources, you, the parent or provider, will always be your child’s most important teacher.