Going for the Bilaterals

Aiden had his last behavioral soundbooth January 12th. Well, obviously not his LAST, but his last one before his big day (which is just three short weeks away!). They tested each ear separately and the results were very similar to his last test -- he showed responses, with his hearing aids in, at 45db to 50 db across all frequencies. What is weird is he always seems to dip a little in the middle frequencies, his audiogram looks like the speech banana to a sort.

We took the past month or two and really looked at all the pros and cons of bimodal (one CI and one hearing aid) and bilateral (two CIs). We researched and read all we could on each. We know families whose child(ren) have been successful with bimodal and bilateral CIs. We questioned and questioned, but what we always came back to was that bilateral would be best for Aiden. We decided to wait and make the FINAL decision the day of this past soundbooth, but with no improvements, our decision had been made.

Here are just a few things we looked at when making our decision.

What we liked about bilateral CIs:

• Better localization and better understanding of speech in noise-- Kids learn so much from other kids and we believe that bilateral CIs will be a huge help in the classroom - for the noise and to help determine whom is speaking and from which direction.
• Better sound quality and more sound balance
• Less fatigue at the end of the day - I know at the end of any seminar I attend, I am worn out. Who would ever think listening would be such a hard task, but for a deaf/hoh child, even with cochlear implants, it can be very tiring!
• If one side breaks, there's the second one for back up.
• I also read many personal stories from parents or kids themselves saying that their child became more social with peers and participated more in class once they were bilaterally implanted. I believe this has to do with confidence. I can relate this to one of my fourth graders. I knew his hearing could fluctuate due to fluid/tubes. Whenever he was very quiet in class, stopped participating, and was very apprehensive if called upon, it alerted me to contact mom. Once the problem was corrected medically, he was a whole new student.

Some of the places we found helpful information on bilateral CIs are here, here, here, and here.

In this write-up, Is Bilateral Really Better?, this wonderful mom (and mentor to all us newbies out here), did an excellent job comparing unilateral vs. bilateral CIs. And, as she states in her article, bilateral CI's are not for everyone. There may be significant usable hearing in one ear, there may be insurance issues, or maybe medical issues that do not allow for two implants among other things. We are lucky that we have the choice between bimodal or bilateral, and bilateral is what we've determined is best for our baby.

What we liked about bimodal:

When we started seeing Aiden doing well with his hearing aids and learned his deafness was due to EVAS, we started thinking about just implanting one side. Here are factors we considered when making this decision.

• Use of residual hearing -- Through the great use of his aids and his learning to listen, Aiden has shown us that he does have some residual hearing, and therefore the sound of music and sound quality in general could be better for him. With CI's you hear electronically, with hearing aids you still hear acoustically (the difference was described to me by the analogy of a song played on an acoustic guitar vs. the same song played on an electric guitar). In a bimodal situation, it allows for all sounds of speech through the CI (hearing aids provide more lower frequency sounds than the higher ones), yet still allows for natural acoustic hearing through the hearing aid. One thing we had to consider though, was with his EVAS, his residual hearing can be lost progressively, or one day, completely gone.
• Future technological advances - This has always been a thought in the back of our minds, should we "save" an ear for future technologies? We are sure that one day there will be better technologies, but we decided we needed to stay in the now and the known. NOW is the time that Aiden is in his prime learning stages and what we know is that cochlear implants will provide him optimal learning capabilities.

When researching bimodal, this is one article I found interesting.

All in all, we decided that bilateral implants are a better choice for Aiden and our family. Therefore, on February 11th (with certification from our insurance, which we're still waiting on, please cross your fingers all goes through!), Aiden will get his cochlear implants. Our surgeon agreed to perform simultaneous implant surgery, which will save Aiden from having to go through a second surgery months from now. Recovery may be a little rougher (hard to sleep on either side), but again, we feel this is the best for Aiden and we can't be more excited (or nervous)!

Part of the Waiting Game

Last I wrote, Aiden was diagnosed with EVAS/LVAS (enlarged/large vestibular aqueduct syndrome), which I still don't completely understand .. in fact, that whole appt. went so fast and so has all the time since then. I've joined an LVAS group and have asked many questions to professionals I have already built relationships with, all of whom told me that yes, my baby could have failed his newborn screening. Yet it still doesn't make sense to me, since they say this is not congenital and that hearing loss in LVAS kids is typically not detected until well after birth.

So here's some of what I've learned:

• The vestibular aqueduct is a narrow bony canal that runs through the skull, connecting the inner ear (by the cochlea) to inside the skull. In short, the tube within this canal is filled with fluid which typically flows away from the organs of the inner ear. With LVAS, the fluid flows back into the hearing and balance organs, causing balance and hearing problems.
• This is a syndrome ... it is genetic, it is hereditary. They have traced the gene to be responsible for LVAS, but still have a lot of questions about the syndrome.
• LVAS occurs sometime after birth and is the result of abnormal postnatal or early childhood development (this is why I question why Aiden failed his newborn hearing screen? Could he truly fail it that fast? Most kids with LVAS are not diagnosed until after 12 months.)
• People who have LVAS are predisposed to the development of hearing loss (wouldn't this mean it's congenital then that at some point in their life they would have hearing loss??)
• It can be associated with Mondini (where the cochlea only develops 1 1/2 turns instead of the normal 2 1/2 turns - Aiden has a full 2 1/2 turns). They do say too though that hearing loss is greater if you have LVAS and do not have any other cochlear deformity.
• It can also be associated with Pendred syndrome - (We will need to get him tested for this although he shows no other signs of this syndrome).
• If you have LVAS without any other cochlear deformities, the hearing loss can be subject to sudden drops in hearing ... which may explain why Aiden failed his ABRs - was this a point where his hearing was at a sudden drop?
• The hearing loss can be fluctuating. It can be sudden by the bump of a head, it can continue to fluctuate over time, or continue to be progressive over time.

So where are we at? Aiden is still a candidate for cochlear implants. Our surgeon tells us that LVAS kids are typically prime candidates for cochlear implants depending on the severity of their hearing loss. Aiden's testing seems to just get better and better, with his last aided testing at 45 to 50 db, across all frequencies. THIS IS AMAZING, BUT ...

... is this his highest he will go?

...will his hearing fluctuate more and to a point he reaches even higher db's or continue to decline?

... is this the best we're going to get?

We know we can't turn up his hearing aids any more because there's a point that sounds become distorted. Without his aids Aiden is hearing about 80 db. So his aids work very well for him. If we turned them up any more though, he'd actually lose the "good" sounds. He's at his peak.

So, we're in a holding pen right now. We're playing the waiting game. We have everything done as far as CI candidacy goes and it's all a go. Except the most important part ... the audiogram. With his most recent audiogram, 5 years ago, he wouldn't be a CI candidate. We're on the borderline. Will it get better? Will it stay the same? Will it get worse? Do we just take the next one and run with it? Do we wait even longer for more consistent results? WHAT DO WE DO NEXT?!?

Aiden's daddy and I still feel very strongly about implanting Aiden, on at least one side (although we've been prepared for bilaterals for some time now), and before he is 12 months old. Even if his aided tests went up 5 to 10 db, we're still dealing with a fluctuating hearing loss, we're still dealing with the constant wondering of whether Aiden is receiving all sounds of speech, and we're still dealing with the fact that he could completely lose all of his hearing at any given moment. We want him to have access to ALL speech sounds and learn these sounds when they are easiest accessible to him ... in his prime learning years ... NOW.

So ... next steps include:

• Continue to work daily with the Ling 6 Sounds (ahhhh, oooo, mmm, eeeee, shhh, ssss), which by the way, he responds to all of them in therapy except the "ssss" sound.
• We hope that by working with the Ling 6 sounds on a daily basis will truly condition him to listening for these sounds and prepare him for the next soundbooth, which is on January 12th. I'll post a video soon of us practicing these sounds.
• After the soundbooth, we will meet with the surgeon again to verify candidacy and decide on a surgery date, which, if all goes well, will be in February (Aiden will be 11 months)

That's where we're at with cochlear implants. It's the waiting that's the hardest part. Before the whole LVAS diagnosis (and his last soundbooth), we were looking at bilateral implants by January. Now we're trying to figure out what's going to happen next. I have faith though and I feel a peace about me that everything is going to work out the way it is supposed to. I still anxiously await my baby's hearing birthday, but I know, it's right around the corner. Until then we continue with what we have to do to help our baby hear.

I found an AVT (auditory-verbal therapist) that I really like, so we continue to see her once a week. She used to work for Johns Hopkins (plus, trained under one of my AVTs from Texas!)then started her own AVT business, yet still works closely with our whole team.

Maryland ECI comes out one time per week. A couple weeks ago they came out and evaluated Aiden for his motor skills and language skills. I am happy to announce that Aiden was at 7 to 8 months for both receptive and expressive language!!! This is phenomenal! He's on track with fine motor skills, but behind with gross motor skills (which he tested at 5 months). He is still not sitting completely by himself. He is on his way, yet still has that whole balance issue. It will come though. He definitely gets around - by rolling. ECI will come out at least once a week for both speech and PT.

So, we're underway, trying not to watch the clock (or calendar) and just enjoying this holiday season.

Better Results in the Soundbooth ... AGAIN

I know this should be so exciting to me, and believe me, it is. We had a full day at Johns Hopkins today, starting with our psychological and ending with what I thought was just going to be a choose your implant device discussion. Well, they started with another sound booth ... which I'm always glad to walk into, but then threw our decision for "simultaneous bilateral implants before 12 months" off a bit.

Here is his audiogram and then next to that the speech banana, which I explained when Aiden got his first soundbooth test. At this time he was just over three months old and showed responses with his hearing aids at 75db and I was like my daughter in a candy store. I couldn't contain myself that my baby heard something! That was 5 months ago. So to think now that he is hearing sounds at 45 to 50 db and definitely hears some of what his mommy says to him is just UNBELIEVABLE! From 75db at 3 months to 45 db at 7 months. Actually, from being told he "probably wouldn't get any benefit from hearing aids" to learning to listen like no other!

UN ... FREAKIN' ... BELIEVABLE!

NOTE TO NEW MOMS ... NEVER GIVE UP!

Okay ... to explain his audiogram for family & friends ... the left side shows decibels (how loud something is ... a whisper is at around 20 db and normal conversation levels around 45 to 50 db). The bottom axis shows the frequency level ... the more energy it takes to make a sound, the higher the frequency ... with deaf/hard-of-hearing people it is typically harder to hear high frequency sounds than lower).

Okay ... so look at Aiden's audiogram, the "S"s show his "Binaural" responses - that is with both hearing aids on - he was hearing between 45db and 50 db from frequencies between 500 to 4,000. This includes normal conversational levels.

Then, the O's and X's show his responses without hearing aids ... O's show his right ear responses and the X's show his left ear responses ... which, as you can see, both ears are pretty much the same from 65-70 db at lower frequencies and then drops to 75 to 80db at the higher frequencies. So without his aids, he could possible hear a dog barking or lawn mover.

All of these responses are to sounds though that he would probably hear only about 50 percent of the time. Think about it, as normal hearing people, we can make out what we don't hear based on everything we do hear ... for hard of hearing/deaf people it is so much harder to do this ... it's harder to "fill in those blanks".

Now look at the speech banana next to his audiogram. This shows what a person can hear at different db's and frequencies. So you can see that even with Aiden hearing with aids at 45 to 50db, he's still missing out on hearing (and therefore speaking and responding to) the important sounds of speech, such as the /p/, /k/, /h/, /g/, /th/, /s/, /f/, /z/, /b/, /d/, etc.

So, is he hearing somethings? Definitely. Is he hearing all he needs to acquire ALL sounds of speech? NO. Is he hearing enough to only need one implant or maybe, just possibly maybe, only hearing aids ... still checking this out. My gut still wants at least one cochlear implant. My big question now truly is will he be just as successful with one than he would be with two?

Honestly, truly, I wish he was just deaf OR could hear enough to know he just needed hearing aids. Seriously, wouldn't it just make it all that much easier?!? Don't get me wrong, I'm on cloud nine that my binky boy is hearing as well as he is. He's moved up to the "moderate to severe" hearing loss category and is on his way to being successful. He wants to listen. He loves to hear. He continues to use new sounds every week (we were so excited to hear /r/, /m/, and /b/ from his loud mouth this week!) He will succeed. He already shows it.

Another Day, Another Appointment

All these appointments we have the next couple of weeks are definitely good, actually they're great ... they drive me crazy, but I know there's a means to an end and it brings us that much closer to Aiden hearing!

So here's what is going on this week:

• Monday - Yesterday, ECI came out, except here they are called ITP (Infant-Toddler Program) up to the age of 3, and are then called ECI (Early Childhood Intervention) from 3 on. Anyway, Aiden's new TOD (Teacher of Deaf) is amazing! We clicked right away and she's right on path with our goals for Aiden! She talked to me about all the possibilities for Aiden in the state, what our expectations are for him, what this program will provide him, and how she will advocate with me for anything I believe as his parent, that he needs! We met and talked and laughed for two hours. We're going to have fun together!


• Tuesday - Today was a lax day, Aiden's dad had his first government holiday so we took Aiden shopping at BabiesRUs to look for a new stroller. Are the MacLaren's really THAT GREAT?? Seriously?!?
• Wednesday - Tomorrow Aiden and I get to venture out on a new trail to the Columbia area where we will meet with and interview an Auditory Verbal Therapist. Now, we left THE BEST in Texas, so it's going to be VERY HARD to measure up to what we've come from. BUT, not only was this person recommended by one of my AVT's in Texas, but is also known and recommended by our new audiologist AND our new TOD! BONUS!


• Thursday is a full day of appointments up at Johns Hopkins. First we have to go in for a (get this) TWO HOUR psychological eval ... don't ask 'cause I have no clue. I think it's to make sure Aiden's daddy and I don't have these crazy expectations or feelings. We know our son is deaf, we know he is going to do AMAZING with CI's because we know WE are going to get the help needed and work VERY HARD with him to make him succeed! See, that only took all of 15 seconds! : ) After our psych eval, we meet with Aiden's audiologist for D-day ... to select THE DEVICE! This is a whole other blog I can't even get into right now.


• Oh yeah ... and then Thursday night we have a CI support group meeting that takes place in the county we live in! Our TOD told me about it (along with providing me at least 3 names and numbers of people in my immediate area whose kids have recently gotten CI's or whose mommy's use the AVT we're talking to tomorrow!).


• Friday ... Aiden and mommy chill and have fun day. PERIOD!

Then, next Monday we meet Aiden's surgeon. At this meeting we're going to talk to him about simultaneous implants and getting the implants before he is 12 months. Hell, my chunky monkey is already over 20 lbs at 7 1/2 months, so he's ready! I cannot believe we're to this point! Where has the time gone?!? My eyes tear up just thinking about it!

Other than that, we're all still having fun (minus the fact that I'm dying to get out for dinner & wine with girlfriends)! Ryan told me last night, that although he misses his friends, he really loves it here. Kailyn had a field trip today to the Baltimore Aquarium which she said was "pretty neat". This past weekend we went and checked out the Baltimore Harbor and took a walk through some beautiful nature trails. (And NO mom, I promise, I don't walk these paths alone!)

To close, here are some pics from our weekend ... my kids have NEVER stomped through fall leaves like this before (I think I was more excited than they were)!

He Loves To Listen!

Are we doing the right thing???? This has been a huge question in our minds ... recently. There was no doubt in the very beginning when we were told Aiden would probably not have any response with his hearing aids. Since then, we have seen Aiden respond so well with his hearing aids. But, are they good enough? Will they give him what he needs to hear and acquire the sounds of speech needed to be successful in the hearing/speaking world we so want him to be a part of? These questions flood our minds every day. Every day we wonder are we doing the right thing for our baby.

Before we left Texas, they turned up his aids ... he's getting bigger and therefore can take on more. He responded ... he definitely responded. He's been responding and today in the sound booth he proved it ... he loves to hear. He didn't only respond to spoken language, but to what I call the boring "static/monotone" sounds. He responded to the "mmmm's", to the "ahhhhh's", and even to the "shhhh's". He showed responses not only through the infamous "binky" tests, but TURNED HIS HEAD TO ALL THE SOUNDS! Continuous responses at 50 to 60 db. Flat line ... all the way across all frequencies. His audiogram shows pretty much a flat line between 50-60db. My baby is definitely hearing something and all I could do was smile and cry.

All the countless hours of ...
...him pulling his hearing aids out and us putting them back in,

...nonstop talk and singing and narration,

...of making sure we talk to him within his "hearing bubble",

...giving him every bit of auditory information we can.

All the countless hours we just want to STOP talking, but keep on going ... he is responding ... and every minute of our time has been worth it. The soundbooth confirms what we see at home, but always question, was that a response?!? Aiden wants to hear ... his auditory nerve has such integrity, and we are doing something right!

So we've questioned ...

Does he still need cochlear implants?

Will hearing aids be enough?

Will his hearing keep getting better or will it get worse?

Can he learn to communicate in a hearing/speaking world that we want him to be a part of with just his hearing aids?

Should we implant one ear or two?

How could he have no response at 120 db unaided six months ago, yet test so much higher with hearing aids?

Aiden loves to hear ... his brain is getting ready for listening, and he loves it.

We know that Aiden will not receive all the sounds of speech with just his hearing aids. Could he learn to speak with just his aids? Probably. Will it be at the same level he would get from implants? Not at all. We don't want him to struggle any more than he has to already. Our goal is for him to be mainstreamed by kinder. or first grade and feel the least bit of difference possible from his peers. Either way, Aiden will always have to wear devices on his ears, whether it is hearing aids or cochlear implants. At his hearing levels, we know that the implant will give him opportunities to so many more sounds, at an age that is imperative to development for spoken language. We know in our hearts what the right answer is.

We have gone round and round with questions in our mind if we are doing the right thing for Aiden. This is a choice we are making for him, yet a choice I believe will benefit him for the rest of his life. We are empowering him with the fullest potential to hear and speak. We feel in our hearts that this is the route to take. Aiden wants to listen ... he hears and speaks to us everyday! He shows us he wants more. He shows us he is ready to take it all in. That he is ready.

I can't keep dwelling on the question to implant or not. I've questioned myself, I've done my research, asked my million and one mom questions to the professionals, and have done my own "mommy diagnosis" with my son. We're moving forward ... and there's no doubt, he's going to continue to amaze us each step of the way!

Cheers, Tears & Lots of Chills

It's been some time since I've posted, and SO MUCH has gone on. I just have to say that I am a very blessed person, along with God, my daddy's looking out for me right now, and I feel it! Where do I start?!? I could really post about so much with Aiden, but I'll keep this to one of the biggest steps we've been waiting for ... not just his MRI results, but even more chilling after.

The first part of this is MRI results, but you have to read on! I have been through a lot the past few years, and I want everyone to feel my happiness in the most recent news I received. Not that I feel "I deserve it", but I feel my boy deserves it. But I do know, in the past couple of years, we have always kept our faith. I believe and I continue to pray for God to lead my way, for Him to take on my problems and help me each step. And I never give up on Him. NEVER. And He has taken care of us.

CHEERS: We went in and spent two hours with our CI surgeon/ENT this week for our MRI results. All in all, his results are GREAT. No abnormalities in structure. The hearing nerves are in place. Everything looks "normal". Which is good. Which is great. It doesn't answer our question about Aiden's hearing loss, but we're okay with that. Kidney ultrasounds, check, good to go. They did find an "arachnoid cyst" on his brain, but actually, the report said "suspected arachnoid cyst". Not definite, just suspected. Our Dr. said this is not uncommon (1 out of 10 to 15 MRI's show this), but is still referring us to a neurosurgeon to make sure this is nothing to worry about. I'm okay with this, I have that feeling that it is okay. His brachial cleft sinus, this will have to be removed, preferrably before he's a year old, for scarring and surgery remembrance issues. He also had a little bit of fluid in his ears. If the fluid doesn't clear up, then they'll put in tubes and remove the brachial cleft all in one surgery. We'll figure this out after a couple months of letting the fluid clear up with some antibiotics. All in all, MRI GREAT, brachial cleft, as long as it's not draining and infected, good to go, but get it removed before preschool age (I figure if he doesn't need tubes, they might as well do this surgery with the CI surgery).

Side note to parents on ear fluid: I took Aiden in to our pedi. about a possible ear infection, no fever, he just kept tugging at his ears. They said, yes there is some fluid, but no ear infection. I told them, he can't keep fluid because of his path of getting CI's. They wouldn't put him on an antibiotic and told me not to worry about it unless he started running fever. I knew I was heading to the ENT a few days later and sure enough, our ENT said no fluid allowed, what-so-ever, and put him on an antibiotic (and he still does not have an infection, yet this fluid is not clearing up). So, even this fluid doesn't clear up, they will put tubes in, because fluid is not allowed prior to any CI surgery!

THIS IS WHERE IT GIVES ME TEARS & CHILLS ...

We then discussed our move to Maryland. Our Dr. said he recommended we go see Dr. John Niparko, at John Hopkins Listening Center (which I HAVE known since day one is where I wanted to go), and that he would have his office contact John Hopkins and fax all of our info. there. I felt GREAT! (I have been having a hard time with leaving my wonderful team here, but hearing this, just reitterated this move was meant to be).

On the way home I got a call from our ENT's office. The people up at John Hopkins wanted me to call them directly. No problem, because I have called & emailed many times, trying to get appts. when they kept telling me they couldn't do anything for me until they had all of his audiograms plus some. I was more than happy to call and actually hopefully get somewhere. I called and left a message, feeling, once again, I wouldn't hear back.

I heard back. Within a half hour. She got Aiden's history, his audiograms came in the mail THAT SAME DAY (which I had sent 2+ weeks prior), and then , after chatting a bit, she said to me,

"Great. I'm going to schedule your family a surgery consult with Dr. Niparko."
"You mean, for him to actually do my son's surgery?" I replied.
"Yes, he'll be your s0n's CI surgeon assuming everything works out."

TEARS. SMILES. LOTS OF TEARS & SMILES (OH YEAH. AND LOTS OF CHILLS). You don't understand. I didn't understand. Five months ago I was told my son was deaf. As his mother I looked up every resource on the Internet and what I came up with, for whatever reason, is that I WANTED this doctor TO IMPLANT MY BABY. Why? I don't know, what I read IMPRESSED ME, it helped me in a time of need. Then this job literally fell into my husband's lap ... he didn't look for this Maryland job after I told him about this doctor, it just happened. Then my ENT (who I would love to do Aiden's surgery as well) recommends us to this surgeon when we tell him we're moving. Then we GET ON SCHEDULE with this DR. that I first wanted for my binky boy's surgery. The surgeon I wanted BEFORE I EVER KNEW WHAT WAS AHEAD OF ME IN THIS WORLD I'M COMING TO KNOW AND LOVE, is going to give my boy cochlear implants!!!

HOW AMAZING IS THAT?!?