Eye Exam and Genetic Testing

Ben had an appointment with a pediatric ophthalmologist yesterday. He dilated Ben's eyes and performed a full examination. It is recommended that all children with hearing loss have an eye exam to rule out certain syndromes that are associated with both hearing and vision loss, which can be seen through retinal changes. We were overjoyed to hear that Ben's retinas looked perfect! Ben will continue to be seen by the eye doctor on an annual basis for continued monitoring, but for now we are so relieved and happy that Ben has perfect eyesight. We are anxiously awaiting the results of the genetic testing that our ENT ordered that tests for the three main genetic causes of deafness: Connexin 26, Connexin 30, and Mitochondrial abnormalities. Ben's blood was drawn and sent off several weeks ago, but we will still not hear the results for several more weeks. If it is determined that Ben has one of these genetic mutations, it will actually be good news because then we can rule out other syndromes and assume that his deafness is of an isolated genetic cause.

Also, Ben has a diagnostic ABR scheduled for Friday (also known as a BAER or an air, bone, and frequency specific Auditory Brainstem Response). Ben will be sedated for this hearing test which lasts a few hours, so please keep him in your prayers. While he had an unsedated ABR in February which gave us his diagnosis, it is recommended to have a sedated one at a few months older so that the audiologists can get more information. We will take both the results of the eye exam and the hearing test to our appointment with the cochlear implant surgeon at Johns Hopkins next month.

Way to Go, Abby!

Abby received a 4th grade Math League certificate after scoring well on a national math test. She was presented with it at her school's Awards Night. Keep up the good work, Abby! We are proud of you!

Trip to the ER

Ben's big brother Sam caused our family (and our entire street) some excitement when he rode his bike into the neighbor's mailbox one beautiful evening last week. All the kids on the street were outside playing and I was carrying Ben in the front pack when my daughters came running and screaming that Sam had fallen off his bike and there was blood "everywhere!" As Ben and I made our way to the end of the court, I found that they had not been exaggerating. He had split his lip and up through his cheek. Luckily, one of our neighbors is a dentist and was able to determine that all his teeth were OK (a true miracle). My mom came over to watch the other kids while I drove Sam to the ER and his dad met us there. We spent the next several hours waiting for the plastic surgeon to come. When he finally arrived, he gave Sam 14 stitches in his face and lip! The highlight for Sam was staying up until midnight. He was such a brave boy and we were so proud of him. He goes to get his stitches out in a few days and hopefully he will continue to heal nicely. We give thanks to God that our boy is OK and that it was nothing more serious.

Preschool Show

Ben attended yet another musical event featuring his siblings, this time Ella's preschool end-of-year songfest. Ella did a beautiful job singing the songs she has learned throughout the year. It was a great culmination of her very first year of school and she can't wait to go back in the fall for the 4 year old class!

Band Concert

Ben attended his oldest sister's band concert last week. Abby performed several pieces on the flute after learning to play this past school year, and will now advance to the Intermediate Band in the fall. Great job Abby!

Ben at 4 months

Ben had his four month old check-up last week and he already weighs 16 pounds! I guess that will happen when you eat all day (and night!) long. His pediatrician thinks he is doing great. He coos and smiles, and has just started laughing. His teacher through the county early intervention program for deaf and hard of hearing babies has started coming to the house once a week. She was amazed at her first visit how well Ben interacts and "talks." This, more than anything else, helped put my mind at ease. She has given us tips on interacting with a deaf infant, like responding with animated facial expressions any time he "talks" to us. To have conversations with him by speaking to him after he coos and babbles, and them waiting for him to respond to us. It is very important to give him positive visual feedback every time he vocalizes. Immediately after Ben's diagnosis we found it difficult to get and keep Ben's attention or gaze. He was easily distracted by lights, shadows, and other visual stimulation. I think that the early months are so driven by sound and voices for hearing infants. At times we felt at a real loss to interact with him. But once Ben was about three months, it all began to fall into place and we were able to interact much more with him. Today, he is engaged and interacting every bit as much as our hearing children did at this age.

His teacher also signs with him. We are all learning sign language by watching the Signing Time videos and I am considering taking a class. While it is our goal to eventually communicate with Ben by speaking, we know that there will be times in his life when he will not have his CI on (like just before going to bed, right after waking up, bathing, swimming, and in the event of a processor problem) and he will not be able to hear. And especially this first year before his surgery, we believe that getting some language into him in whatever way possible will be of great benefit to him. He really seems to enjoy looking at our hands and then back to our mouths, as if he was waiting for something else to experience.

His teacher has told us the goal for deaf infants is to become aware of the presence and absence of sound, so when the glorious day comes when he hears sound for the first time, he will have some understanding, however small it may be. We are trying to develop "pre-listening" skills and stimulate those nerves with his hearing aids. To that end, we have decided to begin auditory-verbal therapy at a private therapy center nearby, in addition to his county home visits. They will work on developing his listening and speaking skills, both now and especially after his CI surgery. Auditory-verbal therapy uses speaking exclusively and believes that children can learn to listen and speak with the proper early intervention during the preschool years. It requires the involvement of the whole family to reinforce what Ben is learning in therapy. Our goal is that after years of auditory-verbal therapy and an oral preschool program, that Ben will be mainstreamed in time for kindergarten. It is an ambitious goal, but we now know that it is possible.

Dance Recital

Ben was happy to attend his sisters' dance recital yesterday.
Ella performed a ballet and Grace did a tap dance. They both did a beautiful job and were greeted with bouquets of flowers from their parents and grandmothers. Thankfully, Ben slept through most of it.

Ben has hearing aids!

Ben received his loaner hearing aids last week and it has been going well. He has not seemed to mind wearing them. They do not seem to have made a difference as far as we can tell, but we will continue to put them on him each day because we know that any possible sound will help stimulate the nerves and it will also get him used to wearing something behind his ears. He will have hearing tests done while wearing them next month. He will wear these hearing aids until his cochlear implant surgery, hopefully by the end of this year.

Background on Ben

Our baby Ben was born January 10, 2008 after a wonderful, uncomplicated pregnancy and delivery. He is our fifth child, and his older brother and sisters had fallen in love with him every bit as much as his dad and I had. Our family was so very happy. Ben failed his newborn hearing screening (OAE) the day after he was born, and then again the following day right before we checked out of the hospital. We were told not to worry as it was probably just fluid in his ears, and made an appointment to return to the hospital in one month for a follow-up test. At his one month checkup at the pediatrician, he again failed an OAE, but we assumed it was just inadequate testing conditions. The next day was his scheduled follow-up at the hospital and we were sure it would all be worked out at that appointment. I took him to the Audiology department of the hospital and he was given an OAE, which he failed. Then we were moved to another room for an automated ABR. I was told that this test was much more extensive and would take into account any fluid in the ears. Wires were attached to his head and little earphones were placed on his ears while he slept in my arms. He failed the test. Now I was getting worried! The technician told me that I needed to get Ben a full diagnostic ABR test (air, bone, and frequency specific ABR) also called a BAER test. They were backed up for the next 3-4 months. I was given the number for another hospital to try to get an appointment sooner, and we were able to get in with this other hospital the following week.

On February 22, when Ben was 6 weeks old, my husband and I took Ben for his diagnostic ABR. I still really felt that he could hear us, and that if he did have a hearing loss, it was probably just a mild one. The ABR was performed unsedated, so after nursing Ben to sleep, I held him in my arms for 2 1/2 hours, as he slept off and on with wires attached to his head. I could tell by the looks on the audiologist's face that something was wrong. A student came in to review the results as the test was going on. When it was over, I wanted to scream, "Just tell me what it is!" But I stayed calm on the outside, while inside I was freaking out. She went to talk to her supervisor and then got Ben's dad from the waiting room. Once they returned, she told us that the test had shown "at least a severe hearing loss in both ears". After reviewing Ben's test results, the ENT we saw the next day told us that he would classify Ben's hearing loss as profound. He had shown no response during the 2 1/2 hours of testing. Our baby was deaf.

Ben's dad and I were completely shocked and heartbroken. How would we communicate with him? What would his life be like? We assumed we would all learn sign language and Ben would attend special schools. I remember there was a pamphlet in the audiologist's testing room, that said, "When Hearing Aids Aren't Enough: The Cochlear Implant." I didn't take it because, thanks to my limited exposure to CIs in the media, I had a negative impression of the surgery, and hoped my son wouldn't need it. Over the next several days of research, I discovered that the cochlear implant could be the miracle device that had the potential to radically improve my deaf son's life. I stumbled upon a blog (www.turnonmyears.blogspot.com) of parents whose child, Drew, had the same diagnosis as Ben, and he was now learning to listen and speak because of the cochlear implant surgery. I felt like I was given a road map out of the pain and confusion. I emailed Drew's mom and she immediately responded by asking how she could help. She gave me invaluable advice that I will always be grateful for, and through Drew's blog, I linked to other wonderful blogs and web sites that gave me information. I saw that there was hope that we would be able to communicate with Ben by speaking and that he would have just as bright a future as our other children. Now we pray that Ben will be found to be a suitable candidate for the cochlear implant surgery. I have visited oral deaf schools and an auditory verbal therapy center. I have joined the CI Circle forum on yahoo. I have made contact with other parents of deaf and hard of hearing kids. We have visited two CI Centers to discuss the candidacy process and the surgery. Ben has begun county Early Intervention home visits with his Teacher of the Deaf. Each day I have tried to make an appointment or make a phone call or read a book or make an email contact that will help me bring Ben closer to hearing.

Many people have told us that we were lucky to receive Ben's diagnosis when he was so young. At the time, part of me wish I didn't know, because I felt like my happiness had been stolen so soon, and that there wasn't much we could do about any of it for a while. But I now realize how fortunate we were; how much we owe to the Universal Infant Screening program in our state, to the blogs of other parents who have been down this road before us, and to the professionals who have helped us along on this journey. Ben will finally get his hearing aids today, just a few days before he turns four months old. The CI process can now formally begin. Please pray with us that Ben will be able to receive and benefit from cochlear implants, and that we make the right decisions for our son as we go about this journey to hearing.

First Post

Our son, Benjamin, was born on January 10, 2008. He was diagnosed with profound bilateral hearing loss at the age of 6 weeks. This is a record of our family's journey to get Ben to hearing, as well as the adventures of our life with our five beautiful children.