Bobby'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
Bobby joined the world in May of 2005. He was a month earlier than we expected, but we were overjoyed to welcome our first child into our lives! The pregnancy had been uneventful and there were no indications that his arrival would hold anything unexpected… until it did.
Bobby was quickly whisked away by doctors and nurses, and we were told he was having some trouble breathing and would be taken to the NICU. His breathing improved, but it was noted that he had a number of congenital anomalies and genetic testing would confirm that Bobby had a chromosomal abnormality. He was missing a small piece from the short arm of his 6th chromosome, a deletion at 6p25.3.
Of course you all know what that means, right? You don’t?! You mean you have never heard of a 6p25.3 deletion? Well, neither had we until we got the news from the geneticist. As we learned more and more about his deletion, we learned that he was missing many genes, but one gene in particular was typically linked to eye abnormalities and hearing loss.
Before leaving the NICU, Bobby failed his newborn hearing screen. We followed up with an audiologist after discharge, and it was determined he had a profound loss in both ears. He was referred for further testing, but that was trumped by open heart surgery at four months old.
A few months after heart surgery we were ready to start the process of getting hearing aids, but that process was yet again trumped, this time by an unexpected issue with his right eye.
At birth, Bobby was determined to have some eye abnormalities, but nothing the ophthalmologist thought was too concerning. He was diagnosed with a precursor to glaucoma, but otherwise the eyes were considered healthy. But in early December in 2005 his right eye started changing from blue to brown, and then the white part developed a yellow tinge.
A visit to the ophthalmologist would reveal that Bobby had developed a vitreous hemorrhage in his right eye. While the cause was not immediately determined, it was later assumed that there had been a previously undetected abnormality in the vessels of the eye and the hemorrhage was triggered by the pressure that built during one of Bobby’s severe reflux episodes. We were advised to wait and see if the hemorrhage resolved, but it did not. In early February of 2006 we headed to Philadelphia for a second opinion and it was advised that Bobby’s eye be removed.
After that we were finally ready to focus on hearing. Hearing aids did not work for Bobby. His first set were programmed to match his ABR results, but he reacted to those as if they were too loud. Another set were programmed lower, but he showed little response. Soon we began to investigate a cochlear implant. I wasn’t convinced it was right for him. There were times when it seemed as though Bobby could hear better than his tests said he could. And the idea of destroying any residual hearing scared me.
As we researched and discussed implantation with the doctors and sought out second opinions, the evidence that finally convinced me that this was the right decision were his CT and MRI results. We saw that his cochleae and hearing bones were malformed, but that the auditory nerve was present. We now knew that even if he was “hearing” it was likely that what he was hearing was unclear, which is likely why he hated the amplification of hearing aids. If we bypassed the damaged areas of his ears with the cochlear implant, his brain would get a clearer sound that it would be better able to interpret.
Bobby had his implant surgery in December of 2007 and was then activated in January of 2008. We saw an immediate reaction - albeit small - but our boy heard something! We were thrilled.
Since then, the journey has been LONG. Our biggest challenge now is expressive communication. Bobby is still non-verbal, although we believe he is hearing and comprehending much of what he hears. But, since his cognitive development is still that of an 18-month old, he doesn’t always listen to what we are asking of him.
We have tried various forms of expressive communication. Bobby still has not picked up on signs. We’ve tried pictures and some technology, but nothing seems to stick with him. And as therapists and professionals change in his life, so do their ideas about how best to teach our boy to communicate. I know that as his parents, my husband and I have a say, but honestly, it is not my area of expertise so I have deferred a lot to the professionals. His school recently obtained two iPads and some other technology that they are going to try with him, and we are excited about that. His two year old sister also loves to sign, so we’re hoping that maybe if we keep signing with her that he’ll want to join in and start signing too. We know that more is going on in that cute little head of his. Someday we will find a way to get it out!
Parenting Bobby is like solving a puzzle everyday. He is unique, like any child is, but he doesn’t fit neatly into a “box” as so many doctor's and other professionals would like him to. Being a part of this project has introduced me to more parents of children who fall outside of the typical categories, who are constantly searching for ways to help improve their child’s quality of life, and who are seeking answers from the best resources out there. If you have a child who is vision and hearing impaired, and you are considering a cochlear implant, then these are the experts you want to contact. I am so glad that we did!
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.
