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.