Happy Holidays!

It's been awhile since I posted. The semester came to a crashing conclusion with overmuch grading, and between that and preparing to fly to Wisconsin, where my parents live, we've been pretty busy.

One of Ben's favorite presents so far is a beautiful orange blanket that his Great Aunt Kathy knitted for him. Whenever he sees it, he says "oynee, oynee, oynee!!" (That's his version of "orange".) We snuggle under it while reading books. Speaking of books, he got a ton of them, and his current favorite is "Is Your Mama a Llama?".

He also got several airplane-related gifts, in keeping with his current airplane obsession. Usually about every fifteen minutes or so his thoughts turn to airplanes, and he'll point to the nearest window and say, hopefully, "appuh?" It's been very cloudy lately, both here in Wisconsin and also back home in New York, so we haven't had much luck spotting appuhs. He was very excited to ride in two different appuhs to get here, although we spent far too much time in airports waiting for weather-delayed appuhs.

We've been celebrating both Christmas and Hanukah. He really likes lighting the menorah every night and playing with his dreidel. Christmas morning was almost too exciting, and we couldn't "opah" the presents quickly enough for him.

Dadda's Eye!

Ben put together his first two-word sentence tonight! He pointed to Neil's eye and said, "Dadda eye!" We were flabbergasted!

I think it was mostly accidental -- he was thinking about both "Dadda" and "eye," and they just came out together. But of course that's the way it usually goes with language and other developments. He does something by accident, we dive in there with the praise and adulation, and with repetition it becomes purposeful and meaningful.

On Thursday, we went to BHSC to get Ben's new ear mold (for his hearing aid), and while we were there the director of the oral-deaf program gave him a language assessment. (We had requested this. We were pretty sure that he was doing very well, but we wanted to make sure we weren't missing anything.) She was very impressed. He is either at or beyond all language milestones for his age, even for a typical hearing child.

Can we build it?

Yes we can! Ben is a regular Bob the Builder groupie these days. It all started when the pediatrician's nurse gave him a Bob the Builder action figure as a present. He thought this was pretty cool. Then we discovered that we have the "Sprout on Demand" channel, which lets us watch any of a number of PBS and BBC kids shows whenever we want. So we watched some Thomas the Tank Engine and Bob the Builder episodes. He liked Thomas, but he absolutely adored Bob. Now whenever he needs a Bob fix, he'll point to the TV and say "Bob Bob Bob!!"

Well, specifically, he loves the Bob theme song. After that, the actual show itself can't compete with whatever toys or books are lying around near him. Neil and I are more into the show than he is. But at the end of the show, when the music starts up again, Ben tunes back in. Yesterday, he was completely absorbed in a book, his back to the TV. The theme song started, and Ben bolted upright, his mouth wide open. He turned to the TV and watched the credits roll, listening to the music with a look of transcendent glee on his face.

Doctor-induced wagging

You know how Ben wags his finger whenever he hears the lines "The mama called the doctor, the doctor said...." from "Five Little Monkeys"? Well, this morning Neil said to Ben, "We're going to the doctor's office to get a flu shot," and Ben started wagging his finger! So he hears and understands the word "doctor" even when it's in a completely different context.

In other news, Ben is really making progress with expressive language. There are days when he's a bit on the quiet side, or he reverts to old patterns of vocalization (primarily vowels, or the same few repeated consonant sounds). But on most days he's pretty talkative. His most impressive word so far is apple, which comes out like "aaaapp". He also says Mama, Dadda, bye-bye, kitty ("gedda"), can ("guh"), Bob (as in Bob the Builder -- he has become a devout Bob fan), more ("mo-mo"), and bubble ("ba-ba"), and he makes brave attempts at a variety of other words. He can also tell you what sound a horse, sheep, or lion makes. Yesterday he was saying "knee" and pointing to his knee. When we ask him to make various consonant sounds (e.g. "ma-ma", beginning with m, b, g, w, n, or d), he is pretty good at imitating us. He still prefers repeated syllables rather than mixing up different consonants, so the speech therapist is currently working on what is called "variegated" babbling. Also, he seems to be a little behind in what the experts call "jargon", which is babbling with adult-like inflection ("Ba ba GA ga wa goo?"). However, I think that in most respects his expressive language is either right on target or even a little ahead. And his little voice is so sweet and lyrical!

Patty-Cake

Here's Ben rolling out pie crust dough on Thanksgiving morning. What a hard worker!

It's raining, it's pouring....

We just got back from spending Thanksgiving with Neil's parents. My mother-in-law Hedy observed this gem yesterday morning: Ben was listening to his Peter Paul and Mommy CD while playing with toys. At one point, he started tapping his head. At first Hedy couldn't figure out why. Then, a second later, the lyric "bumped his head and he went to bed" came up!

See, the amazing thing about this is that even while he was distracted by other things and the music was in the background, he heard it well enough to recognize what song was playing and remember what lyrics came next!

No, we didn't teach him to do this. This was apparently his natural response to the song.

How do you get a CI, anyway?

Well, first you have to be deaf. The FDA guidelines currently require that you have severe-to-profound bilateral sensorineural deafness, that you do not receive benefit from hearing aids, and that you're at least a year old. Well, phooey on them. The FDA guidelines are just that -- guidelines, and not legally binding.

Ben does indeed have severe-to-profound bilateral sensorineural deafness. The hearing loss classifications (mild, moderate, severe, profound) are determined by hearing thresholds, as given in decibels (dB). (Actually, they are given in dB HL, i.e. hearing loss decibels. Nobody has been able to give me a straight answer as to exactly what these are, and how they are related to the regular decibel system, which essentially tells you how loud a sound is. Specifically, the number of decibels is the logarithm of the power of the sound. But I digress.) For example, if you hear nothing below 90 dB, then you are profoundly deaf. This is all complicated by the fact that almost nobody has the same degree of loss over the entire frequency range. Typically, a person has better hearing at low frequencies than high ones. In his left (better) ear, Ben hears "low" frequencies (low for the purposes of speech perception, namely 500 Hz) at around 65 dB. Higher frequencies he doesn't hear until around 80-90 dB. So the hearing loss in his left ear is severe-to-profound. The loss in his right ear is profound.

The "sensorineural" part means that the hearing loss is due to problems in the inner ear, auditory nerve, or higher levels of auditory processing, as opposed to an obstruction or abnormality in the outer or middle ear. In Ben's case, the culprit is the hair cells in the cochlea.

Some people have a "reverse slope" hearing loss, meaning that they hear better at high frequencies. (Leah, if you're reading this, I'm thinking of Nolan here.)

The FDA used to require a profound hearing loss for CI candidacy. Then it was discovered that profoundly deaf people with CIs performed better than severely deaf people without them, and it seemed a bit unfair to deny the latter group the benefits of a CI. In fact, I think that people with CIs often perform better than people with moderate hearing losses; however, at that point, the benefits may not be substantial enough to warrant the costs and risks of surgery.

You need to undergo a trial period with hearing aids in order to demonstrate that you do not receive (sufficient) benefit from them. Ben was first fitted with aids back in January of 2008. After a couple of months, we were increasingly confident that he was getting some meaningful sound in his left ear, but it seemed like there wasn't much going on in the right. This was verified by a number of hearing tests in Buffalo and at the NYU Cochlear Implant Center, where we went for candidacy evaluation in June. We took advantage of the hearing in his left ear to pump as much sound and language into him as we could, and I think this early exposure has been very beneficial to him. But it was pretty clear that the amount and quality of sound that he was getting with the aids was insufficient for spoken language development over the long haul.

Another thing you need before CI surgery is a CT scan or MRI, to look for structural abnormalities that might complicate the surgery or render the implant ineffective. Ben had a CT scan in May.

Now, Ben was implanted at 8.5 months. Increasingly, CI surgeons are implanting before 12 months. Some implant centers still refuse to do this. One of the reasons that we travelled to New York City is that the NYU Center has done many implantations at less than 12 months. We are absolutely convinced that Ben's rapid and easy progress with hearing and language with the CI is largely due to his early implantation, and the extra four months of hearing this bought him at a crucial period of development.

Our insurance company was not so enthusiastic, though. After initially telling us over the phone that they had no minimum age requirement for CI surgery, they then refused to approve the procedure, on the basis that implantation before 12 months is still considered "experimental." We found this out a few days before we were supposed to head back to NYU for the surgery. Maybe I'll give the full story of our insurance battle in a future post, but the short version is that we launched an expedited appeal, our surgeon intervened on our behalf, and eventually we were approved. Our surgery date had to be pushed back a little over a week, but that and a boatload of stress were the only costs to us.

So Ben went in for CI surgery on Friday, July 11. The operation went beautifully and quickly. We spent one night in the hospital, and he was discharged around noon the next day. He recovered very quickly. The device was not activated until July 31, because the incision site needs to heal completely. I'll give a full account of activation another time. This post is already quite long enough!

Blocks in Box

My mother got Ben a subscription to the magazine "Babybug," which Ben loves. The latest issue has a little story about Ned (a toddler) and his mother putting various things into boxes. Yesterday, Neil got out Ben's box of animals and also a bag of alphabet blocks. Ben was standing around pondering these things, and Neil idly commented, "Ned puts blocks in the red box," a direct quote from the Babybug story. Ben alerted to this, walked over to the magazine sitting on the coffee table, flipped through it until he found the story about Ned, and then held it up and showed it to Neil.

We introduced Ben to books very early on. We would hold him on our lap and flip through a board book, pointing to objects, sometimes reading some of the words. At first he didn't pay attention for long, and we didn't press the matter. But we continued to read books with him, modeling how to hold the book, how to turn the pages, etc. And by the time he was three or four months old, he loved books -- he would turn the pages himself, stare intently at the pictures, and follow along as we pointed to the words while reading. As he got older, books became a great way to introduce vocabulary to him, especially after his CI activation.

No More Monkeys....

Ben likes the book "Five Little Monkeys Jumping on the Bed." Often at mealtime or when he's going down for a nap, we'll recite some of the story to him, and here's what he does:

So five little monkeys jumped on the bed.
[starts tapping his head, in anticipation of...]
One fell off and bumped his head.
The Mama called the doctor, the doctor said
[starts wagging his finger as if to say...]
"No more monkeys jumping on the bed!"

Still working on that receptive vocabulary list. We're up to almost 200 words now!!!

Here's a cute anecdote from earlier this week: Ben was standing behind the sofa, where we keep his little rocking horse. He couldn't see me or the teddy bear that was on the sofa. I said, "Ben, can you get your big blue teddy bear and put it on the horse?" A second later, Ben marches out, looks around briefly, gets the bear, takes it back behind the sofa, and places it very precisely on the horse's back. (The intense gravity with which toddlers perform every task is just delicious!) Oh, did I mention that he's deaf?

Eine kleine (more) Ben-musick

Ben's favorite CD is "Peter Paul and Mommy" by Peter Paul and Mary. A couple of the tracks are live performances, and one has applause afterward. Ben always claps along. His favorite song right now is "I'm Being Swallowed by a Boa Constrictor" (lyrics by Shel Silverstein). As soon as it starts, Ben reaches down and touches his toes ("Oh no, oh no, he swallowed my toe, he swallowed my toe!").

My Aunt Kathy made me a cassette of this album when I was very young, so I grew up listening to it. I'm often very moved by the songs even now. I got a copy of the CD a few years ago, and when I was expecting Ben, I really looked forward to sharing it with him. ("And if you take my hand my son, all will be well when the day is done....") I remember playing the CD shortly after we found out he was deaf, and feeling devastated that he would never be able to listen to it. How utterly and completely wrong I was!!!

Music Appreciation!

This'll be quick -- I have to take over Ben-care in three minutes from my husband.

One of Ben's tricks is that when we start singing "When You're Happy And You Know It," he starts clapping his hands. One of his baby toys plays that melody (among many others). The sound quality is pretty poor -- rather tinny. Today Neil noticed that whenever the toy starts playing that song, Ben claps his hands -- and not for the other songs! So he's clearly recognizing the melody.

He has another toy (an octopus) that hangs off his high chair tray, and when you pull on it, it plays "Sailing, Sailing." When it's hanging off to the side, he can't see it. Sometimes when he's not paying attention to me, I'll reach down and pull on the octopus so it plays the song -- and he'll listen, and then look down and point at the octopus. This is even when the TV is on and there is other noise in the environment.

More on Ben and music another time ....

A couple of tidbits....

1. Neil taught him this one: When we sing "Ba-ba-ba-ba-ba" (to the tune of "Shave and a haircut"), Ben responds with "Ba-ba!" It's too funny.
2. Ben has a friend named Cal, who is about 6 months older. When you ask Cal, "What sound does a horse/cow make?" he makes the appropriate sound. So yesterday, when Ben was having fun making some growling noises, I asked, "What sound does the lion make?" (Coincidentally, we had a book opened to a page with a lion on it.) When he growled, I said, "Very good!" and he picked up on it. So now whenever we ask about the lion, he growls! (Thanks, Cal, for the inspiration!)

Halloween!

Here's Ben dressed as a pumpkin (what else?). Halloween was a lovely day here -- sunny and warm. We took Ben for a walk down the street so that he could see the trick-or-treaters (and also so that we could show him off to the neighbors, who descended on him with much ooohs and aaahs).

On Saturday we went to a birthday party for Ben's friend Jackson, who is just a couple of weeks younger. There was a good crowd of kids there, and Ben had a wonderful time. In fact, he did a great job of hearing and responding to our voices, even in a noisy environment, which was very encouraging! Jackson's parents put on a CD and everyone danced to the music. Ben loved that.

Ben is saying "Mama" and "Dadda" very clearly now, and he's working on "mo-mo" for "more". He's starting to repeat the sounds we make. For example, we were talking about the baby gate, and I said, "Ben talk -- ga-ga-ga-gate." He responded with "ga-ga-ga". He has an "n" babble that he likes to make that sounds like "neng-neng-neng". One of these days I'm going to compile a list of all of his receptive vocabulary. I've been keeping a hearing journal (on paper) ever since his CI was activated, and I have most of his vocabulary recorded there -- it's just a matter of getting it all onto one list. So look for that soon!

How did we find out?

Ben was born at 8:21 pm on Friday, October 19, 2007. It was a terrific day -- everything went smoothly, and we were so thrilled to hold him in our arms.

The next day, he was given a newborn hearing screening. He failed. Strictly speaking, he was "referred" -- meaning that the machine showed no response and he would need follow-up testing. (They don't like to call it "failure".) The nurses assured us that there was nothing to worry about. He might still have some gunk in his ears from birth. He probably squirmed and disconnected the electrodes. We had plenty of other things on our minds and we were more than willing to believe their assurances, so we took their advice and didn't worry.

On Sunday, they tested him again, and again he was "referred". Again they told us not to worry, and again we were happy to comply. We were discharged that day and told to come back in a week for another test. In the meantime, we convinced ourselves that he was reacting to sound and that everything was fine.

By Wednesday, we were having problems with latch-on and so we made an appointment with the lactation consultant at the hospital. She was terrific; she showed us exactly how to handle the situation, and with a few weeks of careful training, Ben became an expert nurser. While we were there, they tested his hearing again. You can guess what happened.

So we called and made an appointment for a more comprehensive hearing evaluation at a facility in Buffalo. They couldn't get us in until December. While we were still bravely trying to follow the nurses' advice and not worry, we knew we couldn't keep it up for that long. Fortunately, our college has a hearing and speech clinic, and one of our colleagues offered to perform a hearing test within a few days. We took him in, but he was too squirmy and noisy to get an accurate reading. We brought him back in a few days later. He failed.

By now, we were no longer in denial. We knew something was wrong. Our colleague sat with us in the quiet testing booth while it started to sink in. I couldn't concentrate on everything he was saying, but I remember that he used the phrase "cochlear implant". I think I had heard of this before, but it was completely off my radar screen and I didn't know anything about it.

At this point it seemed like he had some sort of hearing loss, but the test our colleague performed (an otoacoustic emissions test, or OAE) couldn't tell us the degree of loss. The place in Buffalo would perform a more comprehensive test (a diagnostic ABR, or auditory brainstem response test), and it turned out that they could reschedule us for November 15.

So, on Thursday, November 15, we took Ben for his diagnostic ABR.

It wasn't good.

It was very bad.

He was profoundly deaf in his right ear, and severely-to-profoundly deaf in his left. We knew he was deaf, but to have it confirmed and to have it be so bad....

After the test, we stood in the rain in the parking lot, and we cried.

The Daycare Question

We're trying to sort out what to do about daycare next year. We were working under the assumption that we would enroll Ben in the oral-deaf daycare and preschool up at Buffalo Hearing and Speech Clinic. Their daycare program is for kids from 18 months to 3 years, so Ben would start there next summer. The upside is that they have a terrific program. The downside is that it's an hour away from where we live. So it was going to be pretty difficult to get him up there and back with both my husband and me working full time, but we had figured out some possibilities for handling that.

At Ben's last programming appointment at NYU, the communication evaluation team said that he was doing great and that if he continues on this track, he would be better off in a mainstream daycare (where he would have hearing role models) rather than a special one. This is great news, of course, but we're trying to decide what to do about it. In so many ways, it would be a lot more convenient to have him here in town with us, assuming we can find a high-quality daycare program that can take him, rather than driving for two hours every day (especially through those south-of-Buffalo "persistent snow bands"). On the other hand, we don't want him to fall behind on language development. The program in Buffalo has the expertise and structure to monitor his progress and address any delays or problems that become apparent.

Furthermore, it's not going to be easy to get a good daycare placement for him here in town. There's a great facility on campus, and we got him on the waiting list before he was born (not knowing that he would have special needs), but they don't take children under two. We're not comfortable with the idea of dropping off a toddler who wears thousands of dollars worth of equipment on his head at any daycare. Not only would the staff need to be vigilant about the equipment, but we would want for them to give him the same language-rich environment that we've been providing at home. Most daycare programs do a fine job of getting the kids through the day, but it's an awful lot to ask of their overworked, underpaid staff to meet Ben's needs. Good daycare is difficult to come by, especially in our small town.

1st Birthday!!!

Ben turned one yesterday, October 19. He had a terrific day, and was inundated with far more presents than can possibly be healthy for him. We had a party in the afternoon, and some of his friends from play group came with their parents. It was very noisy, and Ben was clearly having trouble understanding us. Hearing in noise is very challenging for CI users. We switched the program on his processor to one that is supposed to help with that by narrowing the input dynamic range. Even with this, he was less responsive to what we were saying than he usually is. Nevertheless he was interacting with the other kids and having a good time. I got a little teary-eyed when "he" (I) blew out the candle on the cake and we all sang Happy Birthday -- probably a pretty typical "mother of the birthday boy" reaction.

Even though it was a great time overall, I still felt bad to see him struggle to hear. Explaining how this fits into the emotional big picture, and where we are on the psychological journey of coping with his deafness, requires a longer entry than I have time for right now. The short story is that we've gotten to the point where we actually take pride in this characteristic that sets him apart from other kids, and of course we're very proud of how well he is doing. We now look at the future very optimistically. There's every reason to believe that he's going to get a mainstream education and that he might do very well academically, that he'll enjoy music and possibly learn to play an instrument, play sports, have close friendships, and in general participate without much constraint in the hearing world. At the same time, we know that he'll occasionally struggle and suffer in ways that a hearing child wouldn't. Yesterday was really the first time I'd witnessed this first hand, and even though he certainly wasn't suffering in any way, it was another brick in the wall of reality, and as such it weighed heavily on me.

But that sensation passed quickly. We all had a wonderful day, full of friends, family, cake, toys, and interesting bits of packaging and wrapping paper (which make for far better playthings than the toys themselves).

Update: Neil posted some birthday videos on youtube:
Singing Happy Birthday:
http://www.youtube.com/watch?v=AcMp3wu_xds

Eating some cake:
http://www.youtube.com/watch?v=EvuYY4fOSZg

Taking a ride on Thomas the Tank Engine:
http://www.youtube.com/watch?v=WiPLDTpxYZc

What caused it?

Ben's deafness is due to mutations on the Connexin-26 gene. It turns out that both my husband and I are carriers of this mutation (who knew?). Any child we have has a 25% chance of being deaf. Ben is our first and only child, so you could say we hit the jackpot on the first try!

The good thing about a Connexin-26 diagnosis is that it's non-syndromic. Sometimes deafness is just one of many symptoms of a syndrome (like Alport's or Long-QT), and often the other symptoms don't appear until later. For example, with Usher's Syndrome, blindness usually starts developing in the pre-teen years. So you're always waiting for the other shoe to drop. With Connexin, there aren't any other shoes. Ben might develop other problems later, but he's at no greater risk than a non-deaf child.

Another good thing is that Connexin kids tend to do very well with cochlear implants. Their hearing anatomy is all present and intact. The only problem is that the hair cells along the basilar membrane, which translate mechanical sound energy into nerve impulses, are defective, due to a lack of Connexin protein.

We had mixed feelings when we got the results of the genetic testing. On the one hand, we were glad to have a definitive cause, and on the whole a Connexin diagnosis is the best. Also, I could stop worrying that I had done something wrong during pregnancy, which was a relief. On the other hand, we both felt a little sad to think that we had given him a bad gene. Then a friend pointed out that probably most people are carrying around at least some mutated genes, and that we probably gave him lots of really good genes, too.

The CI equipment

Here is Ben last Monday, October 13. My husband takes him to a play group every Monday morning, and this week it met at a local pumpkin patch and petting zoo. Ben had a blast. He loves pumpkins (and anything orange).

You can see the equipment for his cochlear implant. He has an Advanced Bionics HighRes 90K implant, and his sound processor is programmed with Fidelity 120 software. Right now he's using the Body Worn Processor (BWP), which is in a purple pouch attached to his belt. There's a cable which goes up to the headpiece, which sits just behind and above his right ear. The headpiece attaches to his head with a magnet. (There's another magnet just under his skin, on the actual implant itself.) There's a microphone on the headpiece. Sound enters through the mic and travels down the cable to the sound processor, where it is digitized and the signal is processed in all sorts of fancy ways. Then the signal is sent back up the cable to the headpiece, where it is transmitted by short range radio to the implant under the skin. The implant then activates a sequence of electrodes along a wire that has been inserted into his cochlea. There are 16 electrodes spaced along the basilar membrane, corresponding to 16 different frequencies. When an electrode is fired, it stimulates the auditory nerve at that point directly, bypassing his inoperative hair cells. By firing the electrodes simultaneously in various combinations, it can produce the effect of more than just those 16 frequencies. In theory, there are 120 frequencies he can perceive. Some users are able to discriminate all or most of these. Who knows exactly what Ben is hearing right now, and how it compares to the "natural" sound that I hear. It doesn't really matter -- for him, this is natural sound.

The most important goal is good speech perception, and traditionally this has been the only priority for CI sound processing -- to optimize speech. At this point in time, all three CI manufacturers (Advanced Bionics, Cochlear, and MedEl -- the only three approved for use in the US) produce terrific speech perception in most users, so two of them (AB and MedEl) have started trying to improve the perception of music, which has traditionally been a sore spot with this technology. They do this by monkeying around with the way the signal is processed after it has been digitized. I'll say more about Ben's love of music in a future post!

Hello, world!

Why am I starting a blog? Well, everyone else is doing it....

My son Ben, who was born deaf, will be turning one in a few days. He has a cochlear implant in his right ear and a hearing aid in his left, and with this combination he is doing beautifully! He loves music, knows almost 100 words, and is starting to say "Mama" and "Dadda" (or approximations thereof).

It's been an exciting journey so far, and there's so much more to come, so I decided to share it with others. Besides, like I said, EVERYONE else has a blog. I am alarmingly behind the times -- I don't even have a CD player in my car! I'd better do something before I become a committed Old Fogey, beyond hope of rescue.