I have always had many dreams and fears for my family, but the thought of having a deaf child never crossed my mind. I can’t help but compare our lives to Mr. Holland’s Opus, a movie where a professional composer and music teacher has a son who unexpectedly turns out to be deaf. In our case it was the musician and worship leader whose daughter is profoundly deaf. What are the odds? Interestingly enough the odds are slim and thanks to SJ’s recent genetic testing we know just how slim they are.
Originally I didn’t think SJ had genetic hearing loss because like I said, it’s not in our family at all. We all had theories about what the cause was, but nothing seemed to match up. I had to come to terms with the realization that we may never know why. Then after connecting with another mom whose son is deaf she told me that her son’s hearing loss came from something called connexin 26. She said it is extremely recessive and they have no history of it in their family. Her son’s hearing loss is progressive and it is most common in Caucasians. It sounded an awful lot like SJ’s hearing loss. We had the testing done over a month ago and I recently got a call from the doctor with the results, which showed that it is in fact a result of Connexin 26! I can’t say I am thrilled about this one way or the other, but I am definitely relieved to finally have an answer.
Here is a quick genetic lesson for anyone who might be like me and need the dummie’s guide to Connexin 26. It turns out that J and I are “carriers” of this recessive hearing loss gene. 3% of people are carriers, but of these 3% most of them will never know and it won’t matter because it doesn’t affect you at all. However if two carriers have a child then they have a 1 in 4 chance that their child will be deaf, a 1 in 4 chance that their child will have normal hearing, and a 50% chance that their child will be a carrier because the child receives a gene from each parent. I love this illustration that I got from raisingdeafkids.org
So most people have two “big D’s”, but J and I both have a big D and a little d. This means one of my parents and one of J’s parents do too.
We know SJ has 2 little d’s as shown in the diagram above, but the question is what about my sons!? Well, Z we know can hear fine and since the loss happens early on he is in the clear. Whether or not he is a carrier we will probably never know. E passed the newborn hearing screening perfectly in both ears. We are supposed to bring him in to have hearing testing every 3 months until he is 3 to see if it progresses. The Doctors are pretty certain that he is fine though because with this type of hearing loss there is usually some indicator at birth even if it is not severe. On the other hand, we can have E tested for connexin 26 and just know for certain whether or not he is deaf. This would save us a lot of stress over the next 3 years so I plan on getting him tested. This simply means getting some swabs of DNA from the inside of his cheek, at least that is what they did with SJ.
The next question is if we have any other kids will they be deaf if so does this mean that we are finished having children. These are serious questions. We always knew we wanted a big family and we knew this would not be our last pregnancy. Having this hereditary hearing loss doesn’t change anything. We don’t know what the future holds. We may not have anymore kids, it’s in God’s hands, but if we don’t it would not be out of fear of having a deaf child. If we do then it will always be a 1 in 4 chance that the child would be deaf. J JOKINGLY says if it’s one in four then we will have one more hearing child. Granted, it is a lot of hard work and it will be a LONG time before I feel like we are at a place to add another person to our family.
Speaking of a long time from now, my mind even began to wonder what this would mean for my grandchildren. Since SJ is two “little d’s” does that mean her children would be deaf? I don’t think so. As far as I know SJ’s children are guaranteed to be carriers because they will receive the deaf gene from her, but they couldn’t possibly have connexin 26 hearing loss unless she married someone who is a carrier and then they would have a 3/4 chance? If she married a connexin 26 deaf person then all of their children would be deaf? I put question marks because I don’t understand all of this and I could do a lot more scenarios, but if SJ were older I could just see her rolling her eyes saying “Mom. Seriously?”
So there you have it. It’s Connexin 26. At least now we can stop worrying that we did something to cause SJ to loose her hearing. As it turns out J and I are just more alike than we ever thought. What are the odds?
I'm glad you have an answer. Like you, I was wondering about grandchildren as well! 🙂