A story about Rachel and Jessica

Written by Melissa Chaikof in 2001. Updated in 2006.

When our older daughter, Rachel, was born in May 1987, little did my husband and I know what challenges the future held for us. When Rachel was all of a week old, I had already begun to suspect that she couldn’t hear. Our apartment was very small, and we kept her infant seat on our dining room table right near the front door. When this heavy door would slam, she wouldn’t startle. I voiced my concern to our pediatrician at our first office visit when Rachel was not even three weeks old, but he dismissed my fears, telling me that I was “an overly anxious new mother who didn’t understand how newborns react to sound.” By Rachel’s next visit at age two months, I insisted she be tested. While my husband and I had tried to mentally prepare ourselves for bad news, nothing could have prepared us for the agonizing two-and-a-half hour wait while the audiologist performed an auditory brainstem response (ABR) hearing test. At the end of the test, she confirmed our fears, telling us that Rachel had a bilateral severe-to-profound hearing loss.

The next two-and-a-half years had us on an emotional roller coaster as we tried to find the best approach to teach Rachel spoken language. We were living in Boston at the time, which was a bastion of Deaf culture. Thus, oral programs were not plentiful, but we very much wanted Rachel to be able to take advantage of all the opportunities the hearing world had to offer. At the age of three months, Rachel received her first set of hearing aids, body worn aids, which consisted of two boxes with wires running up to button-like earplugs. Because the microphones were on the boxes, Rachel had to wear the boxes on the outside of her clothing. To see one’s baby outfitted like this was very difficult, but we wanted her to hear.

When Rachel was ten months old, she cooperated for the first time for a hearing test and proved to have more hearing than we had initially thought. At the same time, she began to show definite responses to sound and received her first set of ear level hearing aids. We thought things were looking up. However, we began to see Rachel’s response to sound diminish shortly after that hearing test. Thinking we needed a better program, we traveled to the Helen Beebe Speech and Hearing Center in Pennsylvania to spend a week there learning about the Auditory-Verbal approach. We left so impressed by the children we saw there that we returned home with renewed enthusiasm and motivation and transferred to Jim and Lea Watson’s Auditory-Verbal Communication Center in Gloucester, Massachusetts. Unfortunately, Rachel’s diminishing responses to sound were a result of her diminishing hearing and not a result of her program. By the age of eighteen months she was totally deaf.

We were faced with a difficult decision at that point. We could attempt to keep Rachel oral, using visual and vibrotactile cues, or turn to sign. We chose the former, reasoning that we still had time to turn to sign if it didn’t work. Fortunately, while an oral, vibrotactile approach was a new challenge for Lea Watson, she agreed to keep working with Rachel.

When Rachel was two, Lea was speaking to Judy Simser, an AVI board member and Auditory-Verbal therapist in Ottawa, and learned of the cochlear implant. (AVI is Auditory-Verbal International. It is the association of Auditory-Verbal professionals, parents, and others who follow the Auditory-Verbal Approach to teaching language to hearing impaired children.)

While we knew of the implant’s existence, all the professionals we had encountered up to that point had told us that it was something to keep our eyes on for the future, but that most users heard only static at that point. However, Judy Simser told Lea of the remarkable progress she was seeing with one of her students who had received an implant. With her referral to the cochlear implant team at New York University (NYU) Medical Center, we made the trip in October 1989. They quickly identified Rachel as a candidate, introduced us to other children whose performance bowled us over, and scheduled Rachel for surgery eight weeks later. While the cochlear implant was not yet FDA approved for children but was in clinical trials, we decided to make the leap to facilitate Rachel’s development of spoken language. We also reasoned that, even if she only heard environmental sounds, it would be more than she currently had.

On December 21, 1989, Rachel became one of the first 200 children in the country to receive the Nucleus 22, the first multichannel cochlear implant available in the U.S. We were all guinea pigs at the time as no one yet knew what to expect from these children. Since then, though, we have never looked back. Whereas prior to her implant Rachel had only mouthed words, within two months post-implant, she found her voice. Soon, she began to demonstrate beginning language. With her newfound hearing, we could also return wholeheartedly to the Auditory-Verbal approach.

In June 1991, when we moved to [our current home] for my husband’s new job, we transferred to The Auditory-Verbal Center (AVC) and began working with Mary Ann Costin. Rachel was four-years-old but still had the language of a two-year-old. What followed were six more years of hard work for us and for Rachel, trying to close the gap between her chronological age and her language age. Because she had had no auditory exposure to spoken language until almost the age of three, closing that gap was going to be difficult and would take a long time. When Rachel finally graduated from AVCA at the age of ten, she had caught up in all areas except vocabulary and auditory memory.

When Rachel was almost four, her little brother, Adam, was born. We held our breaths as, at all of five days old, the same audiologist who tested Rachel’s hearing tested his. This time, though, the outcome was very different. In all of two minutes, she pronounced his hearing normal in both ears. Previously the “experts” had all told us that Rachel’s deafness was most likely the result of a virus I had had when I was five months pregnant. Thus, we took Adam’s normal hearing as further proof of this theory.

When Rachel was almost eight-years-old, her little sister, Jessica, was born. She was not yet 24 hours old when her hearing was tested. Despite repeated attempts, the audiologist could get no response to sound in either ear. Rachel’s “viral” hearing loss had turned out to be genetic. Not only that, this time it was worse as Jessica never had any residual hearing. Although she was fitted with hearing aids at age three weeks, she never demonstrated any response to sound until receiving her cochlear implant.

Of one thing we were certain with Jessica, we didn’t want to wait two years to get her hearing and start feeding in language. Despite the reward of watching Rachel’s language develop, we knew the hard work it entailed and the difficulties she still encountered because of her language delay. Thus, we trekked back up to NYU, this time all the way from [our hometown], when Jessica was fourteen months old, as they had already implanted a twenty-month old. While FDA guidelines for implantation specified age two and up, because the device was now FDA approved, surgeons could use their own discretion to implant at younger ages. I will always remember sitting with Jessica in the surgeon’s examining room. He asked me, “So, when do you want to do this?” I answered whenever he was willing, hoping he’d say age eighteen months. I almost fell off the chair when he replied, “How about next month?” I remember beaming and telling him he had a patient! Whatever it took, we were back to commuting to New York for a few months to get Jessica hearing as early as possible.

Jessica’s surgery was in May, 1996, when she was 15 months old. At the time, she was either the youngest congenitally deaf child or second youngest (by a week) in the country to receive a cochlear implant, depending upon whose statistic we believed. Jessica received her implant when she was sixteen months younger than Rachel was when she received hers.

We jumped right in with Auditory-Verbal therapy at AVCA with Mary Ann as soon as Jessica received her speech processor. The speed of her progress soon amazed everyone. Within two months, she was already demonstrating a clear understanding of language. Her receptive language continued to progress at such a rapid rate that, just past her third birthday, it was already age appropriate.

The one remaining concern we had with Jessica was her expressive language, which lagged severely behind her receptive language. While Rachel’s receptive language always exceeded her expressive early on, the disparity had never been as great as Jessica’s. Being proactive parents, we couldn’t simply sit and wait for the expressive language to come. Having lived through this once before and having now seen so many other implant children at AVCA, we knew that something was not right. Consequently, we visited a pediatric neurologist, who diagnosed Jessica with oral motor apraxia, which-she explained to us is a scrambling of the signal from the brain to the mouth. She assured us that, with oral motor therapy, Jessica would be fine.

Mary Ann referred us to Sharon Wexler, an oral motor therapist, and we started oral motor therapy in April 1998. Within just four months, the difference in Jessica’s speech and expressive language was tremendous. Where before she might utter one highly unintelligible word, she began to speak in two to three word phrases with continually increasing intelligibility.

Rachel is now in the seventh grade in our public middle school. She is almost fully mainstreamed, receiving resource help only for language arts. She spent two years working intensively with her resource teacher to increase her vocabulary and close the remaining language gap. She has made honor roll every semester in middle school as a straight A student. In addition, she has run for and won one elected office at school and is currently running for another. Who would ever have thought that my totally deaf daughter would have the self-confidence, clear speech, and social ease to stand up in front of a large group of people and make a speech?

Jessica’s annual language evaluation at age 5.9 in November 2000 showed that her language scores, including her expressive language, are between ages 6.0 to age 7.9. Consequently, she will be completing her Auditory-Verbal therapy this spring. Despite the late start with her expressive language, she caught up quickly because all the language was inside waiting for her to figure out how to express it. Her voice quality is totally natural, and she enjoys singing. She has been and will continue to be fully mainstreamed in school.

While lobbying for additional state funding for the Auditory-Verbal Center recently, Jessica didn’t hesitate to speak to the state senators and representatives, walking up to each one who came to meet us, holding out her hand and saying, “It’s nice to meet you.” For the legislators, hearing was believing.

We are thrilled with and proud of both of our girls’ progress. However, we have seen the difference in ease of learning language that Jessica’s early implant has made for her. The early implant, in combination with Auditory-Verbal therapy, are a perfect match. Since Jessica’s surgery, surgeons have implanted children at younger and younger ages, including a six-month-old in Europe. In addition, the FDA has lowered their guidelines to age twelve months for one brand of implant. Because of Jessica’s progress, we have become vocal proponents of early implantation, so much so, in fact, that, when speaking to the cochlear implant audiologist at NYU the other day, he teasingly said to me, “If it were up to you, we’d do them in utero!” The sooner the better! It’s never too early to give a child hearing.

Update, February 2006
It has been a long time since I have updated Rachel’s and Jessica’s story, and much has happened in the last 5 years. Rachel is now 18 and a senior in high school, heading off to the Savannah College of Art and Design in the fall for a likely dual major in filmmaking and graphic design. Jessica is in the 4th grade.

Rachel
In March 2003, Rachel came to my room at 7:15 in the morning very agitated, saying that she put on her processor and heard only loud, painful clashing sounds. She had already changed the batteries and cables. I quickly got out her spare processor. She put that on and burst into tears, saying that it didn’t help. This was different from anything we had ever experienced before, and we all knew without saying it that she likely was experiencing a failure of her implant. I had our audiologist’s home phone number but had never used it before. I decided that this was enough of an emergency to do so and called her at 7:15 in the morning. She told us to meet here at her office at 8:00. Elliot works at Emory, across the street from the audiologist, so took her with him while I got the other two children out the door. I hadn’t even made it all the way to Emory before Elliot called to tell me that the audiologist had tried yet another processor as well as hooking her up to the computer, but nothing had helped.

We were very fortunate to be able to schedule Rachel’s reimplantation surgery for six days later. We opted to stay in [our hometown] for the surgery this time and not to return to NYU because there is a good surgeon here and because we feared the delay that going out of state and out of network would cause, and we wanted Rachel hearing again as soon as possible. She was in 9th grade in her first year of high school, and we didn’t want her falling behind.

Rachel’s tenacity during this period amazed us. We left the audiologist, and I expected to take her home, but she insisted on going to school even though she would be totally deaf. I called her resource teacher and explained the situation, and she quickly alerted Rachel’s teachers. Rachel attended school every day up until the day of her surgery, trying as best as she could to understand what was going on through lipreading alone. She said that, while she didn’t get much out of it, what she got was more than had she not been there at all.

Rachel’s reimplantation surgery went smoothly, although it did take 5 hours. She received a Nucleus 24 Contour and was excited to be getting the 3G processor, which she had been waiting for but which was not yet available for her Nucleus 22. Her recovery was marred by a severe migraine headache, which she woke up with when she came out of anesthesia. It kept both of us up the whole night following her surgery and didn’t start to ease until her neurologist phoned in a prescription for a new medication.

Rachel’s initial stimulation of her new implant was scheduled for nine days later. After 15 days of no hearing, we would welcome any hearing but kept our expectations low as we had heard from others that the readjustment would be difficult and would take time. We were pleasantly surprised to find that Rachel had some open set comprehension right from the start, although it was certainly far from where she had been with her Nucleus 22.

It took Rachel 10 months and many mappings to get her hearing back to where it had been with her Nucleus 22. For the remaining six weeks of the school year following her reimplantation, her school provided a C.A.R.T. reporter, which helped her greatly. Rachel’s determination to succeed and her maintenance of her excellent grades earned her V.I.P. awards from two of her teachers that spring.

We did not have very long to enjoy Rachel’s good hearing again. In February 2004, I received a phone call from Rachel’s school, telling me that Rachel was hearing static and could I bring her spare processor to school. I don’t know if it’s because I was anxious already having experienced one CI failure, but I had a bad feeling about this as I rushed to her school. I brought her spare processor also with a new cable/coil. She put it on, and it was a repeat of her N22 failure, as she and I looked at each other and she started to cry. I called the audiologist immediately on my cell phone, and, as luck would have it, the Cochlear representative was coming that day to do integrity tests on a few other patients, and she said that she would call her to see if she could fit one more in.

That afternoon, we went for the integrity test. It showed nothing wrong. However, during that day Rachel had not only continued to experience the constant static but also popping sounds and occasional drops in volume. We tried two additional processors, but nothing helped. Our audiologist spoke with Cochlear Corporation, and they said to go ahead and reimplant. Although this was not a total failure but what is referred to as a soft failure, it was not something that Rachel could live with long term as the constant static was not only annoying but also was affecting the clarity of her hearing.

A few months before this, bilateral implantation had begun to emerge as a new practice. Bilateral implantation was something that Rachel had been asking for for a few years. We had researched it and had become convinced that it was the way to go. First, as much as a 30% improvement in hearing in background noise was being reported. In addition, elimination of the head shadow effect was another advantage. To us, after having Rachel experience two CI failures in less than a year, a “spare ear” was another strong plus. We had already planned to have both Rachel’s and Jessica’s other ears implanted that summer but decided then that we did not want Rachel to have to go through two surgeries so scheduled her for simultaneous bilateral implantation two weeks later.

This time, the surgery took 8 hours. It was a very long day for those of us who were waiting. We had consulted with the neurologist prior to this surgery, and she instructed the anesthesiologist to put steroids in Rachel’s IV during surgery. This prevented Rachel from waking up with a migraine, and so the night after her surgery was a bit easier than the previous one, although Rachel was in more pain because she had incisions on both sides of her head and so could not find a comfortable position. Unfortunately, we did not know to continue Rachel on oral steroids after the surgery, and three mornings later, when the steroids from her IV had worn off, she woke up with the worst migraine she has ever had. We immediately started her on a six day course of oral steroids, which, fortunately, wiped out her migraine in about six hours. However, her recovery continued to be more painful as she had two incisions full of staples that were impossible to avoid lying on when she lay down.

Rachel’s original implant side was hooked up very quickly this time, only six days post-surgery. Her brother’s bar mitzvah was a few days afterwards, and we had all of our families, including Rachel’s out of town cousins, coming, and she wanted to be able to hear. The hookup, however, did not go smoothly. Our expectations were too high as we thought that since Rachel was going from an N24C to an N24C this time, she would not have the same adjustment she had going from an N22 to an N24C. The audiologist first tried Rachel’s last map, and it sounded terrible to Rachel and had her in tears. So, we had to start over again from scratch.

The next few months were very difficult. We were weary of having Rachel struggle to hear. She went from a child who always scored 85% – 90% on the test of single syllable words, which is one of the hardest tests, to one who scored only 56% to 60%. Fortunately, Rachel is a good lipreader. However, that did not help her on the phone or when her teacher’s back was to her. Her school once again provided C.A.R.T. reporting for the remainder of the school year, but that didn’t solve the problem of her diminished hearing abilities. Once again, though, Rachel’s determination and continued success in school earned her more V.I.P. awards that spring, this time from four of her teachers.

That summer, I decided to take Rachel back to our audiologist at NYU to see if he could help her. We went for two lengthy mapping sessions over a three day period and spent the time in between sightseeing. The NYU audiologist, Bill Shapiro, discovered that Rachel’s threshold levels were set incorrectly. He found that she has something called T-tails, which means that from the point where she would first perceive the beeps for each electrode up through several increases in the threshold levels, Rachel would not perceive an increase in loudness. Bill set her T levels not at the point where she first perceived the sounds but at the point where she first perceived them getting louder. It made a huge difference in Rachel’s hearing. We were thrilled. Testing showed an almost 20% increase in Rachel’s hearing on the test of single syllable words so that she was up to 75% – still not back to where she had been with her N22, but it was much better. We also had to keep in mind that Rachel was only four months post reimplantation.

For Rachel’s next mapping, we went back to our original audiologist, who tried to map Rachel the way that Bill had, but it did not work, and Rachel’s hearing dropped again down to 60% – 66%. So, back to NYU we went for a very long day of flying out very early in the morning, going for a mapping, and flying home that evening. It was well worth it, though, as immediately Rachel’s scores went up 20% to 85%. She went from being barely able to use the phone with a familiar voice in quiet to calling me on her cell phone without using her telecoil at the end of her school day while walking down the very noisy main hallway of her school and didn’t miss a thing.

At this point, we have resigned ourselves to traveling back and forth to NYU for Rachel’s mappings as it makes such a difference for Rachel. Understandably, she doesn’t want anyone else to touch her. She and Bill have a system worked out now and go through her mappings in only an hour or less an ear. I liken it to the old grandmother who is a master cook but who has none of her recipes written down. Even though someone tries to watch her and write things down, it never comes out the same. That is how we feel it is with Bill mapping Rachel. Others may try to do what he does, but it is never as good. We just hope that he keeps practicing for many many years to come.

Rachel’s bilateral CI was hooked up three weeks following implantation. Although she had never heard in that ear, she could imitate two of the Ling sounds, ah and oo, right from the start. Her progress with her second ear was slow though. She said that she could tell what she was hearing was speech but that it was all just a bunch of unintelligible mumbling.
It has been almost two years now, and, although Rachel is older than the recommended age for bilateral implantation, she has realized tremendous benefits. First, her hearing in background noise jumped up tremendously, between 30% and 60% depending upon the noise setup. Second, the clarity of her hearing is noticeably improved with two, as is her distance hearing, and she reports that sound is richer and fuller and that she enjoys music more with two CIs. Her hearing with her new CI alone is not equal to that of her original. She seems to get about half of what I say the first time and can almost always get it upon repetition. With lipreading, she could get by with her new ear alone if she had to.

Rachel has been in the art magnet program at her high school and, through it, discovered a love and talent for filmmaking. She won her high school film festival’s awards for best documentary and best overall film two years in a row, finishing her first film, a documentary about life with a cochlear implant, after her first CI failure. Her second film was about a very inspirational little girl who not only has a CI but who also has physical disabilities. Because of her hearing, Rachel is able to edit dialogue and integrate music into her films. Her goal is to become a documentary filmmaker, telling stories that inspire others.

Rachel also has been studying French since 9th grade and is in AP French this year. In 11th grade, she received her school’s award for excellence in foreign language study. This award is given to one junior or senior each year out of about 800 students total. Recently, her French teacher entered her in a competition sponsored by the Foreign Language Association of Georgia. This was an oral only competition, testing the students’ conversational skills. Rachel participated in the advanced level and won a rating of superior from the judges, which is the highest possible rating.

Rachel heads off to college in September. The Savannah College of Art and Design, or SCAD as it is known, has one of the best filmmaking programs, and we are glad that she will at least not be going too far from home for the next four years. SCAD also has a campus in the Provence region of France. Rachel plans to spend at least part of her junior year there, combining her loves of art and French. We cannot believe that she will be going away to college. While at times when she was little, the therapies, helping with homework, etc., all seemed endless, now, looking back, it all seems to have gone by too quickly. While I will never forget all the hard work, time, and money that it took over the years to get Rachel where she is, it was all so very worth it. The proof is in the wonderful, independent young woman whom Rachel has become.

Jessica
Jessica’s performance with her Nucleus 22 has always amazed everyone. She hears at least as well as children with the newer technologies. However, we still wanted to pursue bilateral implantation for her, especially because she hears so very well that she cannot read lips at all. After watching Rachel experience a CI failure, we knew how critical it was for Jessica to have a spare ear since she would not have Rachel’s lipreading skills to fall back on. Thus, she received a Nucleus 24 Contour in her other ear in May 2004 when she was nine years old.

Although Jessica was younger than Rachel when she received her bilateral CI, her initial reaction to it was worse. She hated it, saying that all she could hear was “ssss.” Fortunately, though, as soon as she put her Nucleus 22 processor on, she said that it masked the sound from her new implant and so was fine. For the first two weeks, we left Jessica alone, letting her wear both processors together to get used to the new one. Her brain adjusted rapidly, and we were able to begin therapy with her new ear alone at that point. I wasn’t exactly sure what to do with her as she was much older than a toddler starting Auditory-Verbal therapy and already had a solid language base. However, we muddled through together, working on auditory discrimination tasks. Interestingly, she did better on short phrases and sentences than on individual sounds. Cochlear Corporation came out with software, “Sound and Beyond,” that we purchased to help both Rachel and Jessica learn to use their new CIs. It helped Jessica greatly, and by five months post-hookup, she was already scoring 60% with her new CI alone on the test of single syllable words.

Although Jessica does not have T-tails as Rachel does, she is still difficult to map as she tends to give many false positive responses for her threshold levels, and figuring out the legitimate responses takes time. Thus, in June 2005 I opted to take her to Bill at NYU with Rachel. He tried a different approach with Jessica, telling her that he was giving her a beep and asking her if she heard it. This eliminated the false positives, and we got a great map on Jessica’s new ear. Her progress after that was very rapid, and she now hears as well with her new ear as with her original ear.

Our goal in implanting Jessica bilaterally was to give her a spare ear. Anything else would be icing on the cake, and we got a lot of icing. She already heard better than average in background noise, scoring 90% in a 10 db signal to noise ratio with the noise to her unimplanted ear, and 60% on the hardest test, a 5 db signal to noise ratio with the noise to her implanted ear. Normal hearing for this latter test is considered to be 80% or above. With her bilateral CIs, Jessica now scores 84% on this test. She also reports that she can understand well now both in her computer lab and in the gym at school, two situations where she struggled more with just one CI.

Jessica’s speech now is also very clear. She has had speech therapy off and on since finishing the initial therapy for her apraxia. Most recently, we returned to the wonderful oral motor therapist who treated her for her apraxia, and she has experienced another jump, finally using her Ls and Th’s in her speech and slowing her rate of speech most of the time, which is what causes her to lose some clarity. She is such a chatterbox and so often has so much to say that she talks a mile a minute, which is when we have to tell her to slow down.

Jessica has attended a nearby public elementary school since 2nd grade. While it is not the school that we are zoned for, we are very fortunate that Jessica has been able to attend this school for two reasons. First, our elementary school is not very good, whereas this one is. Second, this is the school that has the hearing impaired resource teacher for oral hearing impaired children, which is why Jessica has been able to attend the school. Ironically, it has not been Jessica’s hearing, speech, or language that have caused her any difficulties because she hears and speaks so well and has excellent language thanks to her early CI. However, she does have some minor learning disabilities that are associated with her vestibular issues. At the private school she was in for kindergarten and first grade, she could only get 30 minutes of resource help a day, whereas at this public school she has been able to get 60 to 90 minutes from a wonderful resource teacher, and she has done very well in school. Next year, we likely will put her back in her private school because we like their hand-on curriculum and smaller student teacher ratios. However, we have been very fortunate for Jessica to have had the opportunity these past three years to get the extra help that she needed.

Adam
Although this is a story about Rachel and Jessica, I would be remiss not to mention their brother Adam, who is four years younger than Rachel and four years older than Jessica. Although he was born with normal hearing, he has been affected by mild learning disabilities and vestibular issues that run in my husband’s family.  His fine and gross motor skills have always been poor. He spent 21?2 years in occupational therapy with Jessica and now uses a laptop in school. He, too, is a real success story, and we are very proud of all three of our children and of what they have overcome.

E-mail Melissa.