A few months ago Joanna and I noticed that Natalie wasn't responding very well when we interacted with her. We'd call her name or ask her a question and it would be hit or miss if we'd get a response. We weren't sure why she wasn't responding very well. Our hypothesis was that her hearing must be struggling significantly. She has a history of ear tubes and infections so we took her to an ENT to have it checked out. In the past it seemed like she couldn't hear well and we'd have her checked out. So we figured they would do the same testing and maybe say it was an infection or a need for tubes again. This time, at a new triad ENT they did a pretty intense round of tests and noticed that her middle ear wasn't responding. They can test both the inner and outer ear separately to determine how much sound is getting into the ear and how. She was hardly hearing anything through the middle ear. So we were sent over to a specialist today at Baptist in Winston and they did a CT scan to check out the condition of her ears. They concluded that she has extreme Conductive Hearing Loss in the middle ear. The middle ear couldn't function any worse. The middle ear is made up of 3 small bones called the Stapes, Incus, and Malleus. These bones move back and forth as the ear drum lets in sounds. If they can't move then very little sound gets in. They are theorizing that Natalie's Stapes bones (the smallest ones connected to the cochlea) have fused to another bone and are no longer moving and as they should be. This means very little transfer of sound from her outer ear to her cochlea (inner ear). They don't know what caused this but think it MIGHT be connected to her arthrogryposis (bone irregularities). But as usual, since it's Natalie, they can't say for sure.
So what can be done? Hearing Aids are the solution. Sometimes surgery can fix Conductive Hearing Loss but not in her case. They aren't damaged they are fused. Apparently surgery can fix a damage but not a fusion. The aids will essentially serve as an artificial middle ear. It should greatly improve her hearing. Possibly even be the best she's ever had. She was fitted today and as soon as insurance approves the order it will be about 3 weeks until they arrive. As expected, she picked out purple sparkles as the color for the aids. They are very cool technologically and can even be connected to bluetooth and used as earbuds. So she's gonna be fancy fancy.
There were some interesting things the doctor noted. One, he couldn't tell how long she has had this hearing loss. He said that this type of hearing loss does not happen quickly. Meaning she has most likely been struggling with her hearing for a very long time. There is the possibility that she has NEVER heard completely. She has never fully passed an ear test due to what we assumed was inability to respond (facial and verbally). Sometimes the tests would be inconclusive because couldn't respond during the test. The specialist doctor told us that many regular docs are very bad at testing kids with disabilities. He said that the testing she's had before probably weren't given thoroughly. But that is speculative and there is no real way to know. The Second interesting thing he noted was, the part of the ear that is malfunctioning in Natalie is located very anatomically close to her main facial nerve. She has no function of her facial nerves so her lack of use in her ears and face could be related. This idea is purely speculative but gives us something to think about.
So the bad news is; she has to have hearing aids and will always need them. The good news; the aids should help significantly and her middle ears won't get worse (because they're already as bad as they can get). If her hearing were to decline further it would be due to a completely different issue. So that's the silver lining I suppose.
ALSO...Natalie was recommended to receive a power wheelchair last year. She still has mobility but struggles to keep up with her peers and others. She can crawl faster than she can walk and some days refuses to walk because it's become hard for her. Her back has gone through so much and she does a lot of therapy to keep up. The chair will help her get around when she needs it and will help her move as fast (or faster) as anyone else. She won't need it all the time but she'll use it quite a bit. It will help her gain some independance and prepare her for the potential of needing a wheelchair as she grows into adulthood. Initially she was denied approval for the chair but after a second review it was approved. So some time within the next 2 weeks she'll be getting her Natalie-sized purple power wheelchair. The company came out last year and sized her. We'll be constructing a ramp on the side of our house to accomodate the weight of the chair and let her get in and out of the house. We have been researching a lift/carrier for the car but haven't exactly figured that out yet. She's excited about her chair. I can almost guarantee she will figure out how to use it inappropriately and jump it off of things, etc. She's a mad woman haha. We'll post plenty of pictures when it arrives.
So that's all for now :-)
