I must admit I’m overwhelmed by people’s interest in Matthew’s new cochlear technology. I mean, I’m sometimes interested in cool stuff about friends’ kids, but I didn’t think most folks really gave that much of a hoot about the mundane happenings of my kids.
But for some reason people seem to have a fascination with hearing technology, and so I thought I’d chat a little more in depth about our family and hearing loss.
There’s no childhood hearing loss in either of our families, just garden variety late-in-life loss for a few uncles. (My Great Uncle Joe, a character who lived into his late-80s, lauded not the guy who invented hearing aids, but the guy who invented the off switch!) So it came as a complete shock to learn that our first-born baby, six-week-old Ben, had not passed his newborn hearing screening, a standard test performed on newborns in RI. Apparently his screening results had slipped through the cracks, as parents are generally notified of such news before being discharged from the hospital. That blissful new parent euphoria instantly evaporated, and we jumped to action, learning everything we could about hearing loss. Fast-forward a few weeks –after testing at Boston Children’s Hospital confirmed the results – and little Ben was the youngest guy in the country at the time to use digital, programmable hearing aids. Back 14 years ago, such technology was not prescribed for such a young user, but we typically buck convention in such cases, and we wanted to be as aggressive as we could in assisting our little guy to hear.
What people who haven’t dealt with childhood hearing loss don’t think about is that without hearing, you don’t have an opportunity to develop speech and language. You can only express what you hear, and if you can’t hear, well, it’s difficult to develop meaningful language. We were advised not to teach Ben sign language in order to encourage his oral development. And because he’s a guy who wants to grab every cue possible to understand what’s being communicated, he also cultivated an outstanding ability to read lips (which used to come in handy in the bathtub, and is now quite useful at the beach and when cute chicks are talking across the cafeteria!).
Ben’s diagnosed bilateral (both ears) sensorineural hearing loss is in the severe-profound range in one ear, and profound in the other ear. An innate communicator, Ben took incredible advantage of the amazing technology combined with excellent speech and language therapy until he was around six or seven, and if you met him today you would have absolutely no idea that he has any hearing loss at all, let alone such a tremendous loss. His hearing aids are minute, and with his long hair, you don’t see them at all. The only tip-off is that he relies mostly on his right ear (which has more hearing), so he’ll tend to stand to the left of someone speaking to him. Periodically we need to remind his teachers that he suffers from such a loss since there’s no presenting evidence of it.
Given the fact that upon initial diagnosis, his audiologist didn’t want to give a prognosis about how –or if – he’d speak and whether he’d require education at a school for the deaf, I think it’s quite a tribute to Ben that he’s a top student and award-winning leader in the class of 300 at his mainstream middle school (which, not coincidentally, happens to feed into the top public high school in CT). One of his audiologists still adoringly uses him as a case study in presentations about the effectiveness of early identification and intervention. I guess I’ll forgive him for driving me to drink by pulling out his aids while buckled into his carseat and throwing them on the floor of the back seat, forcing me to choose whether to pull over and retrieve them or endure the nasty high-pitched squeal until we got to our destination. And oh yeah, that time I “let” him swallow the hearing aid battery forever lives in infamy.
Given our experience, you can imagine that with each ensuing baby, we were, um, rather anxious to know the results of newborn hearing screenings. There were no issues with the twins – and then along came Matty. We knew within 24 hours of his birth that we were facing a similar situation, but this time we were armed with knowledge – and spare equipment. We had tiny earmolds made, had Ben’s extra hearing aids programmed, and at eight days of age, we believe Matty was the youngest patient ever to use hearing aids. With a protocol already in place, we figured this would be a piece of cake. God, we thought, had obviously decided we had done such a stellar job with child #1, why not throw another one down the pike.
And God laughed. “Not so fast, Reisers. You figured out the first challenge; this time I’m tossing in a little twist to keep things exciting.”
See, it seemed that even though we were following the roadmap we had set with Ben, Matty just wasn’t developing meaningful speech and language. He suffered from ear infection after ear infection, and we rationalized that perhaps that was impeding his communication. Had a couple sets of tubes, had his adenoids removed, and we even scoped out a dude at the Lahey Clinic who did this cool OtoLAM laser procedure which is essentially tubes without the tubes. But still no progress.
The summer Matt turned four, I knew our window for programming his speech center was going to start to close soon. Even though on paper his hearing wasn’t too much worse than Ben’s and he should’ve been developing meaningful speech with hearing aid technology, it was clearly time to look into a cochlear implant. Matt’s hearing loss was clinically bad enough that if we hadn’t had this success story with Ben, it probably would have been recommended early-on that we explore the implant option. Given Ben’s success, however, everyone was reticent about going the surgical route unless it was absolutely necessary. And I remember sitting on the beach in Nantucket – listening to my little blonde rascal screech in frustration because he didn’t have enough language to communicate effectively – and right then and there coming to the conclusion it absolutely necessary.
(As an aside, at some point after Matthew’s birth, scientists developed a test to detect a particular gene mutation that causes childhood deafness, and lo and behold, our two boys do have the Connexin-26 mutation. Apparently David and I each carry the recessive gene, which is another one of those charmingly groovy gene mutations prevalent in Ashkenazi Jews. To compensate, at least we also each passed along to Matt a blue-eyed gene, a curly-haired gene and a wise-ass gene!)
So that fall, we took Matt straight to NYU, one of the top cochlear implant centers in the country, and learned he was an ideal candidate for an implant. The day before Halloween 2002, our brilliant surgeon performed the two-hour implant surgery on four-and-a-half-year-old Matty. He had to stay overnight with a pressure bandage around his head, but was released early the following morning. And those of you who know him won’t be surprised to hear that we drove back to RI later in the day and he proceeded to go out trick-or-treating that very night.
Okay, so what’s a cochlear implant? Basically it’s an internal piece (which was the part that was implanted back in ’02) that receives information from an external processor (essentially a microcomputer that looks like a big hearing aid) that Matt wears behind his ear. In a hearing person, sounds come through the eardrum, stimulating the tiny bones in the middle ear, which stimulate the hair follicles in the cochlea, which causes minute electrical signals to stimulate the auditory nerve, which alerts the brain to process the sound. (Amazingly all that happens in a less than a millisecond!) In Matt’s case, the external processor converts sound into electrical impulses that are transmitted to the internal implant, which then stimulates the auditory nerve via an electrode-filled fiber optic cable that has been implanted into his cochlea. While this doesn’t replicate hearing exactly the same as in a hearing person, it’s close enough to give the user access to a pretty wide range of soundwaves.
It’s a short process to get the implant up and running. A few weeks after Matt had the internal components implanted and the incision healed up, we returned to NYU to receive the external piece and program it to communicate with the internal piece. Because it’s a different way to stimulate the auditory nerve, it’s a strange sensation to a new user, and the audiologist actually activates only a few electrodes at a time over a three-day period so the user can get accustomed to the sound and stimulation. You know how it’s a shock to get in the car in the morning when you’ve left the stereo blasting the night before? Same thing. Turn up the volume gradually and you can tolerate more sound.
An implant user goes for testing and reprogramming (or “MAPping” as it’s called) of the external processor every few months so that he or she is always hearing optimally. Like with Ben’s hearing aids, they can put a variety of programs into the device that Matt can switch between, depending on the hearing situation – like a quiet room or a restaurant with lots of background noise or a music concert. It’s sort of like having a built-in equalizer.
Almost instantly following his implant activation, Matt began making huge strides in speech and language. Because he was already almost five (in his second year of mainstream preschool) and had missed out on hearing and processing so many meaningful sounds for his first four years, it was expected that it would take some time for his brain to learn to process expressive and receptive language effectively and get him speaking really well. So much vocabulary was new to him, given the fact that he hadn’t had access to so many sounds during his first four years. His enthusiasm to communicate was voracious though, and despite the fact that he hated every minute of it, he endured an intense speech and language therapy schedule during and after school four days a week. Instead of moving him right along to kindergarten, we took the opportunity to have him do a pre-K year at his preschool, and it was the greatest gift we could ever have given him. In that one year, the progress this kid made was mindblowing. He went on to kindergarten right there at the same private preschool/K program all his brothers had attended, and then moved on to the local mainstream elementary school for first grade, with super support in place to ensure he had access to all areas of learning. As the years have gone on, he has required less and less speech work, and currently he’s finishing up fourth grade – on grade level in every area, and with very little speech therapy or classroom support.
Since the implantation, Matt has had the only external processor available. What he got yesterday was the first upgrade in all these years. The technology is forward-compatible, meaning that it works with all previous implants, so there was obviously no surgery – just a visit to his audiologist and some training on the use of the new equipment. The technology is astounding. It’s almost like the difference between regular TV and high-def. It gives him access to an even wider range of sounds, particularly low frequency sounds. He’s able to plug a cord into the device and plug the other end directly into an iPod or iPhone jack. The new processor is bluetooth compatible, which will enable his equipment to communicate wirelessly with his teacher’s microphone in the classroom. And last, but not least, it comes with rechargeable battery packs. It quite adds up when you go through three high-power hearing aid batteries every two days!
What does Matt’s speech sound like these days? Pretty darn good! Because I hear him every day, it’s hard for me to assess his progress objectively, but folks who haven’t seen him in a while are always astonished by his incredible success. Although not perfect, his speech is universally intelligible with no deaf quality at all. He tends to overenunciate at times, but I’ll take that. And because he has that built-in wise-ass gene, his language is spot-on, including idioms and slang. For years, this impish character has said he’s goin’ to Hollywood, and we believe him (although we predict a short prison detour on the way).
This morning, we cabled him up to my iPod to test this cool technology, and it won’t surprise anyone that his first request was, “I’m Too Sexy” by Right Said Fred! (Sorry to plant that earworm for ya, kids.) You should’ve seen the giant grin he broke into upon hearing the first few pounds of the synthesized drum track. A miracle!
I will always recall the pity party many of our playgroup friends threw when we found out Ben was hearing impaired. Though we ourselves were never sorrowful, they all felt so sympathetic and were secretly relieved that their babies were “perfect.” Well, my great-grandmother used to say that if everyone put their troubles out on a clothes line in the morning, at the end of the day she’d take back her own problems in a heartbeat. I feel the same way. Eventually all these parents had to deal with some issue or other with their kids – we were just lucky enough to get a head start. Ours, thankfully, are treatable because of the wonders of technology, the work of excellent speech and hearing professionals and dedicated effort by our boys, who take it in stride and make it all look easy.