"Mustache!"

Sean's FAVORITE movie, The Lorax is by far his biggest obsession! I'm sure it's because the mustache resembles that of Poppy's! Or it could be the upbeat music that he can now hear and likes to move to!
This picture below just goes to show you how obsessed he is! Movie playing on both the iPad and iTouch!

"STOP!"

If I hear Sean say, "stop," one more time, I'm gonna scream!!!  LOL!  "Stop," is his favorite word to say these days and boy is he one stubborn, independent, toddler!  We had to laugh tonight, when reading and singing before bed, Sean decides to take off his ears and say, "stop!"  I guess he didn't like my singing of, "I've Been Working on the Railroad!" 

I've been so behind in updating the blog, probably because we are so busy "learning to listen" and speak!  Sean is like a little parrot, attempting to repeat every word and phrase!  Some words are pretty clear, others will come with time!  I will do my best to get some more video footage up of our little guy, especially some footage of him saying, "STOP!"

Congenital Deafness

We have been requesting genetic testing for a year now, unfortunately insurance kept denying our request.  Thanks to our wonderful and persistent team, we were able to get genetic testing results, giving us more information on Sean's hearing loss.

Sean has the three following syndromes.  The last one, Pendreds, is a new diagnosis.
All three are complicated disorders, but are often interconnected in people with hearing loss:

Enlarged Vestibular Aqueduct Syndrome (LVAS/EVA)- An abnormality of the inner ear, which causes hearing to fluctuate, hearing is progressive, and causes issues with balance and vertigo. 


Mondini malformation- Sean's cochlea did not fully form correctly in utero.  This was observed by Dr. Moody in April, on Sean's CT Scan. 


*Pendred Syndrome- Genetic testing came back positive that Sean has Pendred Syndrome.  Receiving this news means that in essence it is deafness associated with thyroid disease.  As Sean gets older, we will need to monitor his thyroid, as he has a good chance of developing a goiter.

Pendred syndrome is autosomal recessive. Of course I had to do a little research on what this means, but basically, Sam and I are carriers of a gene for hearing loss and Sean ended up with both recessive genes, causing hearing loss.  1:4 chance of hearing loss, IF both parents are carriers of the recessive gene causing hearing loss.  Below is a cute picture of how to easily understand this genetic stuff, plus a great link too!

      http://raisingdeafkids.org/hearingloss/genetics/inheritance/recessive.php

Awesome week of "Audition only" during speech therapy

Sean was on fire this week listening and repeating Ling 6 sounds, colors, and animals! 

Coincidence?

I think not!

A weekend on the water would not be complete without eating the crabs we caught.
Poppy and Blake cooked the crabs and I prepared the table for our feast!  Newspaper from 12 years ago was in the shed to be used for crab picking!

Look what article was on the front of the Health section as I prepped the table!!!

3rd mapping and booth testing today!

It's hard to believe that we were back at CHKD this morning for our 3rd mapping!  Thankfully these last 2 weeks have been much better than the ones prior!  For the most part, Sean is doing a great job of keeping his processors, aka "ears," on and is now seeing, or should I say, "hearing" the benefits of the processors!
Our appointment today was at 8:30 a.m., which was a bit stressful getting into Norfolk during rush hour, but ended up being the best appointment yet!  Our Audiologist, Mike, had Sean go in the booth to play a listening game of "Hi bunny!"  Testing how well Sean is hearing with one processor at a time.  Each time Sean heard the "noise," he would turn and a bunny would light up. Then  Sean would say, "Hi Bunny!" It was the cutest darn thing seeing him so excited about testing back in the booth!  Blake was a big helper, as he quietly sat in a chair behind Sean.  Mike even let Blake do his own "animal show" by pressing the buttons making the sound booth animals move and groove!!
After booth testing, it was on to make changes to Sean's processors.  The #3 and #4 maps he had the last two weeks we will keep and then add two new ones that will allow for us to turn Sean's sound up a little more!  Should sound be too loud, we can always back the sound down some.
Thankfully I am not feeling as overwhelmed by this whole process and starting to see Sean "Growing by leaps and sounds!" 

"Monkey See, Monkey Do"...

More like..."Blake do, Sean do?!"

Toddlers imitate what they "see and hear" other children and adults doing.  Prior to our second mapping, Sean wasn't hearing much of anything.  D/HOH toddlers, rely so heavily on their sense of sight, that often they see something done once and they will imitate it!  It's scary how quickly Sean picks up on EVERYTHING he sees us do and at the same time, it's pretty cool seeing the wheels turning in his head. 
It's quite comical watching Sean imitate "animated" facial expressions and body language that he has previously seen us doing! On the other hand, he has started this "whack and point," where he will hit us and point at something to be seen.  Guess the last few months of "tapping" Sean to get his attention has come around to bite us in the ass! LOL.  So now that Sean has Sound, we rarely have to tap him and will now be teaching Sean that, to get "our" attention, he simply has to call our name and point! LOL.  I have to admit, it was pretty darn funny when he swatted me on the cheek and pointed to what was on TV.  Of course, I didn't let him know that!!

AVT (Auditory-Verbal Therapy)

Every Monday afternoon, we travel to CHKD for Auditory-Verbal Therapy (AVT), with Miss Barb.  Today Sean had an awesome AVT session, demonstrating that the CI's are working and that he is making great progress, in such a short amount of time "hearing!"

Q: What is Auditory-Verbal Therapy (AVT)
A: Auditory-Verbal therapy is a specialized type of therapy designed to teach a child to use the hearing provided by a hearing aid or a cochlear implant for understanding speech and learning to talk. The child is taught to develop hearing as an active sense so that listening becomes automatic and the child seeks out sounds in life. Hearing and active listening become an integral part of communication, recreation, socialization, education and work.

LVAS/EVA

What in the world is LVAS/EVA, the cause for Sean's hearing loss?

Large Vestibular Aqueduct Syndrome LVAS, also known as Enlarged Vestibular Aqueduct (EVA) is.  This is what Sean has and unfortunately causes several issues with hearing.  Hearing fluctuates (day to day, hour to hour), hearing is progressive, and any bumps to the head, ear infections, or changes in barometric pressure can cause a drop in hearing.   I guess we can say that it's been a blessing that Sean's hearing has been wiped out, requiring him to get CI's now.  In addition to hearing loss, LVAS/EVA causes vestibular issues...balance, vertigo, and tinnitus.  Another part of this RARE 
Thanks to technology I have been in contact with a NC family who's daughter also has LVAS/EVA (Enlarged Vestibular Aqueduct Syndrome).  We met online through the LVAS/EVA support group.  Sarah is the same age as Sean and is doing very well with her hearing aids.  It's been great having another mom to talk with who totally gets the frustration of having a child with hearing loss, due to LVAS/EVA.  While in Greensboro, NC at our family reunion, we had the pleasure of meeting face to face and boy were Sarah and Sean awfully cute together!  LOL!