Our Success with Cued Speech and Auditory Neuropathy— Deanne and Judd Grafe
My name is Deanne Grafe and my husband, Judd and I are the parents of two profoundly deaf children. Lexi is 6 years old and Isaiah is 5 years old. When Lexi was 15 months old, we took her in to see her pediatrician for a check-up. She had a checklist of milestones and Lexi had hit every one of them except for beginning to talk. Her doctor asked if she was at least saying Mama and I said no. She said I wouldn’t worry about it, but if she’s still not talking at 18 months, we’ll do some hearing tests. On the drive home, I started to get that horrible feeling in the pit of my stomach that something was very wrong. As soon as I got home, I set her down to play with some toys and ran and got some pots and pans. I snuck up behind her and started banging and she didn’t even flinch. My heart was in my throat at this point and I didn’t know what to do. I called my husband and he came right home and we did some more tests, some that she would respond to, but in retrospect, she probably felt the sound, rather that heard it. She was always very visual and we had hardwood floors so when we walked into the room she would turn toward us because she felt the vibrations from the floor rather than heard us.
We were lucky enough to live in Rochester, MN and we immediately called the Mayo Clinic and set up an appointment with Lee Fabry. She met with us the following day and ran an otoacoustic emission test, the auditory brainstem response test and did some testing in the booth. She was confused by the response and said she had never seen anyone with normal OAE’s and no response on the ABR’s, but she knew of some research that was being done by Dr. Charles Berlin at LSU Medical Center in New Orleans. She made some calls to him and he confirmed the auditory neuropathy diagnosis. We are so grateful that we were lucky enough to find an educated and qualified audiologist for our initial diagnosis. Lee went above and beyond that first day to help us. She did the research and found the information we needed. We’ve heard so many horror stories from other parents of AN kids that we realize how easy it would have been to mis-diagnose Lexi. If Lee hadn’t run the OAE test we would never have had the diagnosis so early. We also went to Boys Town National Research Hospital for a second opinion, which was confirmed and flew down to meet with Dr. Berlin in New Orleans. Dr. Berlin spent several days with us and was a huge help. He confirmed the diagnosis and gave us all the information he had at that point concerning AN.
After returning home, we felt relieved that we had a definitive diagnosis, but we were devastated and very confused as well. Auditory neuropathy was so new at that point, our children were the fourth and fifth diagnosed in the nation and no one knew what the best course of action was post-diagnosis. We were told not to use hearing aids because the aids might damage the cochlea (the OAE’s were normal meaning the cochlea was fine; amplifying sound to a normal cochlea through a hearing aid would most likely damage it) and that cochlear implants would not work for the same reason. We went home feeling lost and very uncertain about what the future would hold for Lexi. She was profoundly deaf and we could not find a way to get any sound to her.
It was an extremely scary and uncertain time for us and then Isaiah was diagnosed two months later at 3 months old. To have one child diagnosed was a shock, but to have two children, both profoundly deaf with this new and strange diagnosis, when neither of us had ever even met a deaf person was incomprehensible. How could this happen to us - it seemed so unfair went through our minds countless times. But the beautiful, wonderful, sweet children that we had fallen in love with the moment we held them were still the same beautiful, wonderful, and sweet children as before only now they needed our help, more than ever.
As parents, it was so frustrating to want to try to do the best for your child and to not know what the best was. We tried to gain access to sound for them with tactile aids, but they really didn’t help much, we also tried FM systems without much luck. After getting over the initial shock of the diagnosis, we began our homework on what we felt would be the best communication choice for our children. We read Choices in Deafness - A Parents Guide by Sue Schwartz and talked to parents and children that signed, cued and used the oral approach. We felt that Cued Speech would be the best alternative for our children. With Cued Speech they are exposed to English just like any hearing child would be. They have clear, concise access to every word we say with the aid of Cued Speech. To simplify it, Cued Speech is just a supplement to lip-reading. So many sounds look alike on the lips, but with the visual cues of Cued Speech, any ambiguity or hesitation is gone. You can say anything to them and they are able to understand. We are so grateful for everything Cued Speech has given our family. We’ve seen so many parents of deaf/hard of hearing children struggling to communicate with their children on the most basic of topics. Since we started using Cued Speech, communication has been easy and enjoyable. Another benefit of Cued Speech is you can learn the entire system in a few days, whereas with ASL it takes years and you will still be running to your dictionary looking up words. Cued Speech takes the challenge out of communicating with deaf children.
With Cued Speech we were able to get language in to them so we were able to communicate freely with Lexi and Isaiah. We also talked to them all the time and tried to get as much language as possible into them. We were not seeing much progress with our local school district because of their lack of experience working with profoundly deaf oral children, so we contacted Beth Walker from Alabama, a woman well known for working with profoundly deaf oral children and she used Cued Speech. We flew her in for a 3-day in-service for the teachers and us. It helped us a great deal, but we didn’t see the type of progress we had hoped for and we felt like we had become speech therapists and teachers of the deaf, instead of parents.
I’ll give you a little history on Lexi: At 17 months of age, after 1 month of cueing, she understood 15 words, at 18 months, after 2 months of cueing she understood 45 words and she was understanding phrases like Give Isaiah his pacifier. At 20 months after 4 months of cueing she understood 190 words, at 22 months, after 6 months of cueing she understood 370 words. At 24 months, after 8 months of cueing she understood over 1000 words and at 36 months of age, after 19 months of cueing she understood over 2500 words. At 2 1/2 years she knew her alphabet, all the colors and her numbers 1-10 and was already starting to recognize written names of common people and objects. At 2 1/2 her receptive language was that of a 4 year old hearing child according to the Ski*High Language Test and she has consistently been a year or more ahead of her hearing peers receptively since then.
Since Isaiah was the second child, I wasn’t able to spend as much one-on-one time with him as I would have liked, but having two deaf children in diapers under the age of two, I kept very busy. I also didn’t keep as detailed records as I did with Lexi, but at 16 months of age, Isaiah understood 40 words, at 21 months he understood 290 words and at 30 months he understood over 500 words. When Isaiah was 13 months old and Lexi was 26 months old, I was videotaping them to document their language acquisition and I asked Lexi, Where’s the TV remote. Lexi just saw Where’s the TV and pointed to the TV, but Isaiah turned around and picked up the remote. I almost fell off my chair. We had never said This is a remote , he just learned it by seeing us cue to each other. It was amazing how naturally they picked it up.
When Lexi was 3 and Isaiah was 2, both were on track as far as receptive language because of cued speech, but we were not seeing any real progress with expressive language. We did more research and decided we needed teachers trained in working with profoundly deaf children and helping them learn to talk. We decided to fly to Saint Louis and check out the oral schools that we had heard so much about. We toured and met with the teachers at CID, St. Joseph’s and the Moog Oral School and we fell in love with the teachers and staff at the Moog Oral School. It just seemed like the perfect fit for Lexi and Isaiah. Although Moog didn’t use Cued Speech, both children read lips so well, we felt they would do fine without it and the teachers at Moog would be able to help them with speech production and intelligibility.
We decided that I would move to St. Louis with the kids and Judd would stay in Minnesota with the family business, which couldn’t be moved, and we would try to make this work. It was a hard for everyone emotionally and financially, but Judd drove or flew every weekend to see us and it was well worth the sacrifice. In the first few months, we saw more progress than we had in two years in Minnesota and the kids loved it. They were happy and enjoying school and all their new friends. The teachers at Moog were amazed at their lip-reading abilities and their huge receptive language. Lexi’s teacher’s struggled to find words that she didn’t know. When she was four years old, they were doing vocabulary on clothing items and the only word her teacher could find that she didn’t know was cuff. When other children were learning circle and square she was learning trapezoid and hexagon. At 4 years of age she knew her numbers up to 100, was sight-reading, and doing simple addition and subtraction. She could write all her letters, numbers, and the names of herself, Mommy, Daddy, Isaiah, Grandma and Paris, and her close friends and teachers from memory. When Isaiah was four he knew all his colors, most of the alphabet, his numbers 1-10, and could tell you anything you want to know about Thomas the Tank Engine and all his friends.
Both Lexi and Isaiah were making good progress, but after the first year, Jean Moog, the director, pulled us aside and said that at some point their expressive progress would plateau, unless they gained access to the auditory channel. It was around that same time that we heard of two auditory neuropathy children that were implanted before they knew they had auditory neuropathy and it worked. We were very excited, but at the same time apprehensive. We consulted again with Dr. Berlin and he said that before we considered and implant we should contact Dr. Arnold Starr at the University of California, Irvine to rule out any possible neurological problems. We flew to California to meet with Dr. Starr. He conducted some deep tendon nerve reflex tests to rule out any problems. With that information in hand, we consulted with Dr. George Facer and Dr. Jon Shallop at the Mayo Clinic about the possibility of an implant for Lexi and Isaiah. They were wonderful and were able to get insurance approval for us within the week and scheduled the surgery date the following week. We were so excited that our children may now have the possibility to experience sound and would hear our voices for the first time, but also scared about being one of the first and about the risk that any surgery entails. Judd and I had often wished for the option of a cochlear implant for our children and now it looked as if it could happen. We decided to implant Lexi first, since she was already 4½ and we knew how precious those first 5-6 years are for language and speech development. We decided that if it worked we would schedule Isaiah’s appointment the following day. I have to say that, throughout all the medical testing, procedures and different schools, that we have been blessed to meet and work with wonderful, caring, dedicated and truly great doctors, audiologists, and teachers. We are continually amazed at the unselfish giving of people’s time and energy to help our children succeed. Without all the wonderful support that we’ve received from everyone mentioned in this article our children would not be doing as well as they are and we are eternally grateful.
During surgery we all were so anxious and worried, but the surgery went very smoothly and there were no problems. During surgery Dr. Shallop had conducted nerve response telemetry tests, which indicate whether or not the implant worked and the response was wonderful. We were so relieved, because we didn’t know if implants would work with AN kids, but we knew that we had to wait 4 weeks to know for sure if the implant was a success. Lexi recovered very quickly after surgery, in fact the hardest part was trying to keep her in bed to rest. By the end of the first day after surgery she was back to her old self. We had told her that she was getting an implant to help her hear and she was excited about that, but she would have to wait for a few weeks until it was turned on.
Ann Peterson at the Mayo Clinic Implant Center helped us on the day of her initial stimulation. It was an exciting and emotional day for everyone and Lexi’s eyes lit up when she heard her first sounds. She said, I heard that and smiled and continued to let us know every time she heard something. It truly was a miracle and we were all in tears on and off throughout the day. Lexi was able to hear our voices for the first time in her life. We’ll never forget that moment. At one point the sounds got too loud and she cried, but recovered quickly and didn’t want to take it off that night. She did get sick of us saying Lexi over and over, though, to make sure it still worked – we couldn't quite believe it ourselves. We scheduled Isaiah’s surgery the following day and he received his implant the following week. His NRT’s were also positive and his initial stimulation was similar to Lexi’s although he was much more laid back and a little harder to test. His eyes got as big as saucers when his implant was hooked up, he just didn’t know what to think. When Isaiah’s implant worked we were even more excited and I think they could hear our screams of excitement on the next floor. Isaiah loved his implant from the beginning, which surprised us, because when we had tried the FM system we could never get him to keep the aids in this ears. It was obvious the implant was helping him hear. He always put his coil on as soon as it fell off and never took it off himself. Lexi on the other hand would often pull her coil off when there were loud sounds or whenever there was running water, which was a bad frequency for her. But the things that they heard just astounded us. Within the first few months, they were both picking up on very soft sounds, like the crunching leaves beneath their feet, the creak of doors, birds singing and the rain falling; it truly was a miracle. These were two children who you could stand behind and scream their names and they wouldn’t even flinch and now they were hearing whispers.
We were thankful and relieved that our children would have access to sound knowing how much easier their lives would be, but we also realized that giving them sound was the easy part, that they still had to learn how to interpret that sound and make sense of it. They were in effect newborns and their hearing age was now zero. We knew that it would take hard work to help them make sense of their new hearing world. They continued for the next year at the Moog Oral School and it was amazing how much easier everything was for them both. Lexi’s speech intelligibility cleared up dramatically and Isaiah was responding to so much auditory information. His speech was not progressing as we had hoped though and we later found out he had speech apraxia in addition to the Auditory Neuropathy.
They both made so much progress throughout their first year post-implant that we decided that we would move back to Minnesota the summer after their second year in St. Louis. After we moved back, Jean Moog let us know that she would be opening a new oral school in the Twin Cities and we were thrilled. We moved from Rochester to the Cities that summer and Lexi and Isaiah enrolled in Northern Voices. Isaiah adapted very well to his new school, loved his teachers, made new friends and continued to make good progress.
Lexi on the other hand seemed bored and it was obvious she was ready to be mainstreamed. In fact, a few months after her fifth birthday, she had taught herself to read and in kindergarten was reading at a second grade level and was chosen by her teacher as the best reader in her class. Lexi is now in first grade and she is reading at the fifth grade level although her comprehension is closer to third grade. She was recently assessed by her speech teacher at school using the TOLD-3: Test of Language Development, which is normed on hearing children and the results are as follows:
Relational Vocabulary Oral Vocabulary Picture Vocabulary Grammatical Understanding Grammatic Completion
95th percentile >99th percentile 50th percentile 84th percentile >99th percentile
Isaiah is still attending Northern Voices although we are considering mainstreaming him next year if his speech continues to improve. In addition to attending an oral school, he has speech therapy 3 times a week with a therapist trained in working with children with apraxia and we have seen great progress with his speech. Isaiah is now 5 years old and is talking up a storm, he is always so excited to tell us all about his day and is enjoying school and all his friends. He is beginning to sight read and sound out words, loves counting and anything dealing with numbers and math. Both children have always done so well receptively that our main concern has been intelligible speech, which is why we chose the oral schools. Lexi no longer needs the intense language and speech work and Isaiah is now progressing along the same lines as Lexi. We continue to cue at home to Isaiah so his receptive language keeps growing and to aid in his speech production with the apraxia (it really helps to have a visual representation of the sounds), but we don’t cue to Lexi anymore unless her implant is off at bedtime, in the bathtub or at the pool. She just doesn’t need it anymore. Both of their auditory abilities are amazing. We can talk to them from behind doors and they understand us. They can hear whispers now, the implant is truly a miracle. Although if there is a lot of background noise, they lose a lot of their auditory discrimination abilities and then they depend on lip-reading or cueing.
I feel our family is a good example of how well Cued Speech and the oral method work with auditory neuropathy children because we’ve gotten different results with each child. Lexi is an exceptional child, she loves to learn and is excited by new challenges. She watches intently and is always asking for more information. Isaiah on the other hand is all boy. He loves to run, jump and wrestle and isn’t all that concerned with watching us. We’ve been told it’s the testosterone. Since his implant his attention has improved dramatically and our main goal now is to improve his speech intelligibility, which is another benefit of Cued Speech. We can cue the exact sound that he’s making and then redirect him to the correct sound by cueing it. Cued Speech has worked well with both types of personalities and temperaments. I do want to clarify that this method has worked very well for our family, but each child is different and what works for one child and family may not work for another.
Cued Speech is not a very well known option, but it is gaining in popularity because of reading levels. The average child using sign language graduates with a third grade reading level. The average Cued Speech child graduates from high school with college level reading abilities. Many children who switch from ASL to Cued Speech make-up 2-3 years in their reading levels in one school year. Our experience with Lexi and Isaiah proves this point. I could go on and on about how much Cued Speech has meant to us and how much easier it has made our daily lives, it has allowed us the ease of communication with two precious children we love very much and every family deserves that.
Writing this article has brought back a lot of memories and I remember so many days when Judd and I would wish If only they could talk and now it seems we never can get a moment of peace. Although our house is a noisy one, filled with talking and laughter, we couldn’t be happier. If anyone had told us when Lexi and Isaiah were first diagnosed that they would have done as well and come as far as they have we wouldn’t have believed them. These were the same children that people had told us would never learn to talk and that we were wasting our time. I just want every parent out there, who may be struggling with many of the frustrations of daily life to realize that it does get better and it does get easier. It just takes time, hard work, and believing that your child can do anything that they want to do. With all the wonderful doctors, teachers and audiologists, as well as the ever-changing technological advances in cochlear implants, there really are no limits to what our children can achieve. Lexi and Isaiah are proof that miracles do happen.
We can be reached at our web site through email at GrafecueX2@aol.com.