Benjamin's Story
Determining the Benefits and Challenges of Cochlear Implants for Children with Combined Hearing and Vision Loss
Children Who are Deaf-Blind with Cochlear Implants
Benjamin was born the fifth child out of six in November 2003. His birth was a few weeks early. The pregnancy itself was healthy. I ran the entire pregnancy, doing several 5Ks and two half marathons. He weighed only 5 lbs. 4 oz. We thought he was healthy, they only took him to the nursery to give him a bath. Within a few hours, we realized that they suspected something else.
After many tests and ruling out of different syndromes, Benjamin was diagnosed with CHARGE Syndrome. CHARGE affects approximately 1 in 10,000 live births. It is a clinical diagnosis of a specific set of birth defects and medical problems. A gene, the CDH7, was isolated in 2004 and can be found with blood tests. Because not all people with CHARGE will have a detectable change in the gene, CHARGE remains a clinical diagnosis. Rarely does a family have more than one affected person. It is not related to sex, race, nationality, religion or socio-economic status.
It was apparent early on the Benjamin had some issues. He has a small eye on the left and a keyhole pupil on the right. There is only a small retina on the left and a partial retina on the right. He would react to light but it was hard to tell how much usable vision he would have. He had a mild heart defect, a pulmonic valve stenosis, which would not need to be fixed right away. He was having reflux and difficulty swallowing. His ears were set low and did not have much cartilage, looking very wafer like. He did not pass several hearing tests. Within a few weeks of his birth, he had a fundoplication of his stomach and a feeding tube inserted. We took him home for the first time when he was 40 days old, in time for Christmas.
When Benjamin was 3 months old, he got his first set of hearing aids. We had to use double sided toupee tape to keep them on. He didn’t seem to like them and wore them inconsistently. Once he realized how to take them out, he took them out quite a bit. When he was 4 years old, he was starting to hit some typical milestones and I knew it was time to look into a cochlear implant. My husband had been ready for the cochlear earlier than me.
I was scared to death. What if it didn’t work? We would lose all usable hearing on that side. And which side to pick? The one with the better nerve for better chances? But then we lose more of the usable hearing. We started through the process at Cincinnati Children’s Hospital and felt like we were working with a great team. Dr. Daniel Choo gave us the support and information we needed.
I knew it was time, but I didn’t feel any less scared. Except for my kids, I don’t have a lot of family. My parents live only a few hours away, but they do not really comprehend all that is involved. My best friend let me vent quite a bit, and being a social worker, she always knew what to say.
Benjamin was scheduled for surgery on January 22, 2009 and activated in March. He tolerated the surgery well and his post op period was uneventful. It seemed like it was working and he was hearing something.
Funded by the U.S. Department of Education, Office of Special Education-Technology and Media Services for Individuals with Disabilities (CFDA 84.327A). Grant H327A080045; Project Officer, Maryann McDermott. Opinions express within are those of the project/author and do not necessarily represent the position of the U.S. Department of Education.
Through Children’s, Benjamin receives aural rehab through the audiology department. He has been going there for several years. He began receiving speech therapy after the implant. He already receives PT/OT with Children’s. He had received some speech therapy for feeding purposes, but since he had not been progressing with oral foods, that has been put on hold. At school, he receives PT, OT, speech, vision and hearing therapy, plus adaptive PE. He has an aide with him and is mainstreamed for music, art and gym class.
At times, Benjamin does not keep the implant on. It is a struggle. We took the batteries out for about 3 months and made him wear it so that he would learn to leave it alone. About 6 months ago, we turned it back on and have been turning it up very slowly. He seems to be tolerating it better now. There are actual times when he will stop and appears to be listening to something.
Since the implant, he is more vocal, even when he does not have it on. Although he does not use words, he makes apparent happy and unhappy noises. He has a sound, “ning”, that he uses for “no”. We know it is “no” because of his facial expression and he will be pushing away what he does not want. He searches out his siblings more when he wants to play. He does not walk yet, but can crawl around the house and knows his way around. He communicates by reaching for your hands and pulling you to what he wants. For example, if he is in his wheelchair and wants out, he will grab for your hand and place it on the seat belt. If you do not unbuckle him, he will grab your hand again and place it palm down. He seems to interact more with other kids at school also. We are giving him total communication now. We speak, sign and the school has been introducing Braille.
It has been a long slow road. We knew he would not just start talking and catch up quickly. That it is so slow is at times very frustrating. I did not think he would take it off as much as he does. I thought that he would leave it on better than the hearing aids since it is not in his ear. Having to start back over with the mapping, while necessary, was a set back none the less. We knew that there would be extra appointments and that part we do not mind. We want to give him every chance to make this work.
The one thing about Benjamin is that he is consistently inconsistent. What he reacts to today may not get the same response tomorrow. His therapists are aware of this and they work on the same activities repeatedly and then try to bridge his learning to the next step.
Getting an implant was a tough decision for me. His previous procedures and surgeries were needed. I knew that the hearing aids were not working for him and that this was his chance for usable hearing, but there were so many risks too. Losing residual hearing, no matter how little it was, was still a loss. I knew that I couldn’t give him new retinas but I could give him this. I work in an emergency room and I met a man that had lost his hearing as an adult due to a head trauma and had gotten an implant. He answered so many questions for me and I knew he had been sent to me to allieve my fears.
Benjamin is a fairly happy guy. He loves to take things out of boxes and baskets and make a mess. He has the best giggle and belly laugh. He figures things out in his own way and lets his needs be known. I am hoping that he will begin communicating better, either with sign language or orally. I think that the more he wears his implant, the better he will be able to interpret his world and figure out how to live in it. His brothers and sisters help him out quite a bit and I think that is good for him.
If I had to do it all over again, I think I would choose to do it sooner. I had to make sure that this was going to be the best thing for him. Now that I know, I would feel better about it. At this time, we are not considering getting the other ear implanted.
