Friday, January 1, 2016

ANSD and CIs

Cece was diagnosed with Auditory Neuropathy Spectrum Disorder (ANSD) when she was a few months old. The simplified message for me with her diagnosis is that her ears are fine, but that her brain scrambles sounds.

We started a journey of observation and noting when it seemed like her brain was processing sound and when it was scrambling the sound.  For quite a few months, it appeared that her brain was processing sounds and things were going well.  When Cece was 12 months old, she said about 15 words.  Well, the beginning sounds of 15 words.  Bah for ball, buh for bug, mama, dada, Eh for Ellie, and things like that.

Cece has been in an early intervention program called PIP since she was 3 months old.  PIP is through the Utah School for the Deaf and Blind, and also associated with Kids on the Move.  It's funny, but I don't even know what PIP stands for.  (Ha!  Just googled it and PIP stands for the Parent Infant Program.)  We meet with our Auditory Verbal Therapist (AVT) Lori twice a month.  Lori has done a lot of developmental checklists with me (Cece is always on track or ahead on everything except receptive and expressive language).  We have played lots of games to test Cece's hearing and train her to respond to sounds.  One of Cece's favorites early on was when Lori or I would shake a rattle.  Cece would lift her head in response to the sound, but she could never tell where the sound was coming from (above, below, right side, behind, etc).

. : snapshot of paperwork from Cece's assessment when 19 months old : .

Another favorite, when Cece got a little older, was to play the 6 Sounds Listening Game.  (And that is totally my own title.)  Apparently, the building blocks of the English language are the sounds ah, ee, oo, mm, ss, and sh.   I put together a toy purse with objects to represent the sounds.  I would fly the airplane and say "aahhhh!"  Hold the play food to my lips and say "mmmmm."  Monkey = ee, doll = sh, ghost = oo, and snake for ss.  (The plastic snake didn't make it past our last toy clean out.  He went to live in the circular file.)  Cece engaged in this game often, and would repeat the sounds ah and mm.  She would sign monkey for the monkey toy, and, to this day, will hold baby dolls to her shoulder and pat their backs (what I always did while saying sh).  Cece never repeated any of the other sounds besides ah and mm.  And really, she mostly liked playing with the purse, taking the toys out and putting them back in.

When Cece was 9 months old, we started doing monthly Sound Booth testing with the USDB audiologist.  She started wearing her hearing aids when she was 11 months old.  Last spring, Cece was consistently hearing on the sound booth tests in the 20-45 decibel range.  That is normal hearing -  mild hearing loss.  Then, last summer, Cece's hearing responses worsened.  Instead of hearing and responding most of the time, she started responding to sounds very minimally.  She would go many days in a row with no response to sounds.  Then it started being weeks in a row.  At this point, I cannot remember the last time she responded to sound.

A few months ago, I switched Cece from the Listening and Spoken Language track (LSL) in the PIP program to the American Sign Language track (ASL).  Luckily for us, Lori, in addition to being an AVT, is also fluent in ASL.  (She is deaf and got a cochlear implant as an adult.)  I have been signing with Cece since birth, but have really amped up my efforts in the past year.  In filling out forms for PIP in October, I counted up that Cece uses over 150 signs and she understands more.

When Cece was 18 months old, we were able to join the PIP ASL toddler playgroup.  Every Thursday, Cece and I get to play with Kristi.  It is set up like preschool, with free play, circle time, arts and crafts, even a snack.  Kristi has taught us many signs related to the activities we are doing, as well as any others I want to know.  She is super patient with my subpar fingerspelling skills.  I am especially bad at understanding when Kristi is fingerspelling to me.  Kristi is really good at lip reading so usually I do not have to spell very much.

A couple of months ago, we signed up for PIPs Deaf Mentor program.  Ariana comes to our house every Friday and teaches the whole family ASL, and this will be for the next 3 years.  Grandma has been able to come to a few lessons, and that has been fun.  Ariana is also extremely patient with fingerspelling and helping me communicate in ASL.  Cece and Lynlee usually do not engage much with the lessons, but Ellie is soaking it all in.  She remembers words week to week that I have forgotten.  The lesson that got lots of attention from Cece was when Ariana did animals.  Cece loves animals and knows lots of animal signs.

. : snapshot of lesson with Ariana : .

ASL is Cece's language.  She gets that it is her language and it is so amazing to me to watch her "babble" in signs.  She still is obsessed with Baby Signing Time and the Signing Time programs and asks to watch them every day.  Cece often surprises me by using signs that she has seen on the shows but that I don't remember using with her.  For example, at playgroup there is a large rug on the floor with pictures.  We often play a game where Kristi or I will sign things like, "where boat" and Cece will run and stand on the boat.  A few weeks ago I was testing her with new words and she found the pig, bee, and other objects just fine.

We are committed as a family to becoming fluent in ASL and having that be Cece's first language.  I also want her to be able to learn English, through reading and writing, and also through hearing it spoken.  We have decided she will get cochlear implants, and her surgery is scheduled in 2 weeks.  After much prayer and discussion, we have decided to do bilateral sequential cochlear implants, which means Cece will get one ear implanted now, and if all goes well, she will have the other ear implanted in a few months.

The success rate for implantation with ANSD is high (which is why our insurance authorized bilateral implants with no fight from us -- a big shock!), but there is a higher failure rate with ANSD recipients than sensory neuro hearing loss recipients.  There is really no way to predict if Cece's brain will be able to process the information from the CI because of the way ANSD affects her brain.

We have gone back and forth on doing a bilateral simultaneous implantation or a bilateral sequential.  A major con to doing sequential is that the second ear often does not do as well as the first, having lost a few months of learning.  A major con to doing simultaneous is if the implants are unsuccessful and Cece went through all of the surgery and extensive therapies for nothing.

What it came down to, for me, was this:  I can live with the regret when (if?) the implantation is successful on one ear, and her second ear has lost some months of hearing in stereo.  I cannot live with the regret if the implantation is unsuccessful and we put Cece through everything and destroyed both of her ears. 

Cochlear Implants can be controversial.  I do not believe Cece is broken and needs to be fixed.  I do want her to have access to all the tools that I can give her.  Hearing is a sense.  Learning how to speak because she can hear sounds is a skill.  ASL is a language.  I want her to be able to utilize all of these things.

And, music.

I want Cece to be able to experience hearing music.

We have done a lot of research over the past couple of years on CIs.  I have talked with and read accounts of people with CIs -- the good, the bad, and the ugly.  I have been resistant to the idea of getting them because for so long, Cece was doing well and responding to sounds.  But then, as Lori puts it, her ANSD "kicked in" and everything shifted.  Her sound booths show no response to sound, which is the equivalent of a profound hearing loss.  

So this is where we are at.  I want Cece to have it all.  The ability to be in the Deaf world and the hearing world.  To be fluent in ASL and spoken English.  To continue with our ASL efforts and to attend ASL preschool when she turns 3.  And, if everything goes well with her cochlear implants, to be able to process sounds and learn to speak.

Please pray for Cece that, first of all, her surgery goes well and she recovers quickly (surgery on January 15).  And then we need lots of prayers that she will do well with her therapies after surgery and that she will learn how to hear with her CIs.


Linder said...

We will keep you all in our prayers. You are a wonderful mother!

Kristel said...

Cece is so fortunate to have you as her advocate. I love that you are committed to both ASL and the hearing world. It doesn't have to be either/or!


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