Chapter 3: Cochlear Implants & Culture Wars, Part I
It took a month or two, but I began to shake off the sad. One day, I went up to Grace’s room to see if she’d woken from her nap. I opened her door, and there she was, sitting up in her crib, playing with a toy, and smiling. I felt in that moment the powerful truth in what Jason had said to me several weeks earlier: Nothing about Grace had changed. She was joyous. She was amazing. She wasn’t going anywhere. The only difference was that now we knew she couldn't hear. I was just going to have to adjust.
Jason says that maybe he owes his practical approach to dealing with Grace’s deafness to his mother’s pragmatism and his father’s reserved nature. Perhaps, he thinks, this helped him handle the pronouncement that Grace was deaf. From almost the very beginning, the option of cochlear implantation for Grace had a powerful champion in Jason. To him, it was not a matter of if Grace would get a cochlear implant, but how soon?
Audiologists are trained not to try to sway parents in any direction when it comes to making decisions about how to deal with their child’s deafness. They must have opinions about how best to proceed, but they remain (often frustratingly) neutral; objectively providing what they believe are balanced tidbits of information about all the options. I understand that diplomacy is key when dealing with such potentially controversial issues as cochlear implantation, or communication and education approaches. But audiologists are generally the first experienced professionals with whom parents come in close contact, when first learning the extent of their child’s hearing loss. While Jason and I were ruthlessly persistent in asking questions – Okay, implantation isn’t for everyone, but of the children you’ve seen, how have the kids with implants done? For a child like ours, with a hearing loss too profound to even measure, can hearing aids offer comparable benefits to cochlear implantation when it comes to accessing spoken language? – I often wonder how parents who are less bullish, less savvy (I have a masters degree in public health, and Jason and I both have professional backgrounds as researchers), would fare? How about families who are uninsured, don’t speak English, or who are mistrustful of the medical establishment?
Audiologists all seem to start this conversation with a huge spotlight on the “menu” aspect of things, making certain they try to provide an unbiased introduction to all the options, rather than saying, for instance, “In the case of your child, your family would either have to adopt sign language, or, should you choose to use hearing aids instead of cochlear implants, expect years of difficult challenges for your daughter, and potential isolation, as she tries to learn to speak a language she really cannot hear.” This would have been, in my opinion, an honest presentation of the potential futures for Grace at the time she was identified as being deaf. But no audiologist ever tread that deeply into the waters of if/then with us, no matter how many children they had worked with, no matter what they had pieced together into an opinion.
Learning about Cochlear Implants
In the beginning, it was difficult for me to imagine how much more potential cochlear implant (CI) technology had to offer than traditional hearing aids for a severely or profoundly deaf person. But the access to sound offered with a cochlear implant is orders of magnitude higher than with hearing aids. Especially when that person is profoundly deaf; most especially when it is a profoundly deaf young child.
We began the process of determining whether Grace was a candidate for a CI and received a video, produced by Advanced Bionics, one of the manufacturers of implants. The video documented the story of a little girl named Cecilia. Cecilia’s parents were not optimistic that she would be eligible for a CI, but she was, and her progress was fantastic. Over the years that she and her family were followed for the documentary, she grew into a confident, charming, unique girl, whose speech was easy to understand. She spoke with a slight slowness, but her articulation was great and her intonations lovely.
She had friendships with hearing children.
She learned to love to read.
She sang in her church’s children’s choir.
She was a success story. Of course, she was also an advertisement. We were reminded, over and over again, to manage our expectations while hoping for the best. Still, it didn’t seem this was something we could pass up. And we couldn’t help but begin to have a sense of cautious optimism. During visits from our parents, we pulled out the video and showed it to them; it was the first time they had the chance to see a CI in action. It gave them a chance to feel just how hopeful we might allow ourselves to be.
There is a vast difference between the ways in which hearing aids and cochlear implants function. Imagine your ear: sound is captured in the elegant shallow cup of the outer ear, and travels inside until it gets to the inner ear (the cochlea), where it shivers the tiny cilia-like hair cells that line its spiral shape. There are thousands of these hair cells inside a normally functioning ear, which pick up the acoustic message and send their sound signal to the auditory nerve, to be interpreted by the brain. The large number of hair cells, and the amazing biological nature of them, enables an extraordinary amount of nuance and variation in the sounds we, the hearing, can hear. We can distinguish between a grown person’s voice and a child’s. We can hear the range of notes on an octave. We can identify hundreds of thousands of things just by listening to them.
Babies learn very early on to begin to make sense of sound. Before they are one year old, they begin to understand that the barking they hear comes from a dog. That the rumbling they hear comes from a passing truck. They turn their heads toward the direction of sound – their parents’ voices; footsteps in the hallway, birds and airplanes overhead. They can subconsciously tune out inconsequential noises, and develop an inherent sense of alarm when a noise startles them. We learn to make sense of the things we hear long before we can actually explain what they mean. Sounds become a natural part of the every day world, and serve an important function, as they help us to navigate through that world.
For many deaf people, the inability to hear stems from a lack of healthy, functioning hair cells; this is called sensorineural hearing loss. A person with this type of hearing loss in both ears has a bilateral sensorineural hearing loss. This is the type of hearing loss that Grace has. The lack of functioning, healthy hair cells, bilaterally, left Grace with essentially no access to sound whatsoever. She is profoundly deaf.
Hearing aids work by taking whatever sound IS being captured and perceived by the hair cells, and amplifies it. Often, someone with a moderate or even severe hearing loss can navigate in the hearing world very well using hearing aids. But for profoundly deaf people, like Grace, there is no measurable sound input in the first place, so hearing aids offer no meaningful benefit. Even if you could amplify what she was hearing one-hundred fold, one hundred times zero still equals zero.
Cochlear implants work completely differently.
To insert a cochlear implant, a surgeon feeds a thin wire, called an electrode array, inside the cochlea. Attached to the wire are several electrodes. The surgeon also shaves out some of the bone in the skull, to make room for the magnet and the receiver.
A CI works by capturing acoustic sounds with a microphone that is built into the external part of the equipment. These are converted to electrical signals within the speech processor. The electrical signal is then transmitted up a cable and through the skin to a receiver, and then travels to the electrode array inside the cochlea, where various electrodes are stimulated depending upon the specific electrical signal being sent. The auditory nerve responds to the signal and provides information to the brain.
Eligibility
There were countless visits to different doctors and centers that first year. There was a list of things we had been given by our audiologist, Jill C, and the doctors we saw, at the Listening Center at Hopkins– procedures to undergo, letters to be written from doctors for insurance coverage, and on and on, that we had to take care of before the eligibility process for implantation was considered complete. I took Grace for what turned out to be a quick and easy trip to Children’s Hospital for an EEG (which showed her heart was just fine, and that she had not inherited her dad’s slightly irregular heartbeat). While we were in the waiting room that day, a woman and her mother, who had brought a child in for reasons I don’t recall, asked me questions about Grace, who was sitting up in her stroller, watching us talking. Because we were in the cardiac center at a children’s hospital, they asked with genuine concern if she was okay. I explained that she was deaf; that the doctors wanted to make sure there were no health issues before moving forward with Cochlear Implant surgery. They were taken aback that she was deaf. “She looks so healthy and happy,” they said. “She is,” I said.
It was one more day that I was reminded, as I had been so many times that year, that we were the lucky ones. Compared to so many of the children we saw, as we entered the doors and walked the halls of hospitals, we were unbelievably fortunate. We were not wheelchair bound, in need of a transplant, living with life-threatening illness, or struggling with significant cognitive challenges.
Part of the eligibility process included outfitting GRace with hearing aids to determine, through observation and audiogram, whether in fact she received any real benefit from them, before proceeding with such an invasive procedure as a cochlear implant. Only children who were too deaf to benefit significantly from the amplification offered by hearing aids were considered candidates for CI surgery.
Hearing aids.
Putting hearing aids on a deaf five month old is kind of a bad joke. The earmolds have to be perfectly placed inside the ear, or else they generate the horrible, high-pitched squeal of feedback. This happens constantly with a baby who is always moving, often tugging, and rapidly growing. And, when she was five, or six, or seven months old, Grace was far more interested in sticking the hearing aids in her mouth or pulling them apart than she was in keeping them stuck in her ears. I learned later that the batteries are (of course) highly toxic. Great.
The audiogram – the test they do to see how much she is hearing, and in which frequencies – showed perhaps some small response with the hearing aids, but on a baby so little, it was really hard to tell. Grace couldn’t say, “Hey, I heard that.” Or even raise her hand, or a toy, when she heard a tone. They would try to measure whether she had heard anything with the hearing aids by watching her behaviors – Did she look up? Turn her head? Smile? Cry? And if she did, was it because of a sound she heard? And if she didn’t, was it because she couldn’t hear it or because she wasn’t particularly interested in giving us an indication to let us know she had heard it? The test findings were inconsistent – sometimes we’d see a tiny response to a very loud, low-frequency sound, sometimes not. This is not surprising. But consistently, we were getting no response at all in the higher-frequency range where speech resides. The only real question wound up being, When wearing hearing aids, was she hearing nothing, or just next to nothing?
Anecdotally, we were led to understand that how much benefit Grace got from the hearing aids would be directly related to how willing she would be to leave them in – if they were providing a significant amount of amplification, that was enabling her to hear significantly more, she was far more likely to leave them alone. Based on how often and how diligently she messed with them, it was hard to imagine she was getting much of anything.
Our quality of life, once she’d gotten her hearing aids, actually decreased. The aids did nothing for her in terms of hearing anything practical. They were constantly screaming feedback at us; she was relentlessly yanking them off and putting them in her mouth. On almost every car-ride with her between her fifth and eleventh month, I spent more time reaching around back - not just to give her bottles, pacifiers, and toys, but to pick up pieces of the hearing aids that were feedbacking on the floor - than I did facing forward. At first, I would pull over and put them back together and stick them back in her ears. Then, there came a point when, once she’d pulled them out, I just picked them up off the floor, turned them off, and stuck them in my bag. Finally, I gave up completely and took them out of her ears when I was strapping her into the carseat, before I even started up the car. I began to worry that if this was how it was with hearing aids, how much worse would it be with all the added cords and separate pieces of a cochlear implant?
The audiological team had hoped that even if the hearing aids didn’t help a great deal, they might at least offer some improvement, at least on one side. If that had been the case, we would have had some useful information. It would have helped us make the choice, if and when it came time to implant, about which side to select for surgery; the side with less or no benefit from amplification would be the “deafer” ear, and so long as the structure inside that ear was compatible with the implant (no ossification, no oddly-shaped cochlea, etc..) this deafer side would be the side to implant[1]. But although on one or two audiograms it appeared she might be getting a little bit (I should say, a tiny amount) of benefit on the right side, the tests were inconsistent – there was even one test that contradicted these findings completely, when Grace outperformed on the left. We did gain one thing from the hearing aid experience, tough. We learned for sure that they could not help her in any meaningful way, and that she was “deaf enough” to be eligible for a CI. She had passed that test, anyway.
[1] The process of implantation was thought to destroy any “residual” hearing; that is, the surgical means of implantation, particularly, the process of feeding the wire and electrodes into the cochlea, will eliminate just about any hearing the individual has prior to the procedure. Thus, choosing the “deafer” ear makes sense for several reasons; it does less damage by preserving the better ear, and, as mentioned above, may allow the individual to get some access to acoustical sound in the stronger ear, either through the use of a hearing aid on that side, or even unaided. However, although the eligibility criteria set forth by implant centers and insurance companies are constantly evolving, at the time of Grace’s surgery, a person would have to have severe-to-profound hearing loss in both ears to be considered a candidate for implantation. Someone who had enough hearing, even in one ear, to get by without a hearing aid would not have been considered a good candidate.