Miles Microtia is combined with another condition called Atresia, the absence of ear canals. Exactly what this means can be different for every child. It can be just a closure in the external canal and skull bone where the normal opening should be. It can also include a fusion of parts internally or an absence of parts altogether. In most cases, you won't know until the child has a CAT scan at 2 1/2. At that age the internal structures are considered large enough to assess.
After the scan, the results are then sent off to the surgeon you have chosen to do the repair. The surgeon will then review them and let the parents or patient know, on a scale of 1 to 10 how likely a successful repair will be. Most surgeons won't even consider operating on anything below a 6 and that would mean the child or patient would then have a bone anchored hearing aid permanently. (Like the one Miles now wears, but attached by an abutment to the skull)
The surgery doesn't always guarantee that a hearing aid would no longer be needed, but it does help return the hearing to within normal levels, and with the repair of the external structure, an in-ear device could be used to help amplify sound. Occasionally revisions to the surgery, which are much less involved, can help correct any issues that may arise after the initial canal opening. These can take place months or even years following. We hope that technology will continue to advance and that techniques will only improve for the patients.
Just recently I have been seeing a lot of posts on our online support group referring to this type of surgery. The topic of conversation usually surrounds external repairs and answering general questions for new members. I have noticed more bilaterally affected children not being candidates for surgery in one or both ears lately. This has been very disheartening to me considering it is usually the unilateral ones that are turned down.
In some unilateral cases, the children have a syndrome call Hemifacial Microsomia. Which in short means that in addition to other possible problems, the bones on one side of the face or skull do not grow as fast as the other side, affecting the ear, eye, nose, and jaw. Eventually most of these kids end up having a jaw distraction to lengthen the bone in their lower jaw. This is not just a cosmetic procedure. If untreated, it could change their biting, eating and speech abilities in the future.
Because of the structural problems with HFM, the place where an ear canal would need to be drilled in Atresia repair, may not always be possible. A lot of times the facial nerve will be in the way or the skull is so misaligned with the internal structures the canal would end up on the cheek or jaw bone. All of these issues make a candidate inoperable. Again, these are almost always unilateral cases and sydrome related. But the few posts I've seen in the last couple of weeks have been for bilateral children.
I guess I just never thought of Miles not being a candidate for surgery. He did have a CAT scan when he was a newborn that showed promising internal structures, but he wasn't sedated so there was a lot of movement on the film. Being a newborn, the internal bones were still too small to see them in any detail, so we still have to wait for his CAT in another 1.5 years.
We instinctively explain both parts of the surgery to anyone who questions Miles condition as though they are both inevitable. I just assumed because his Microtia/Atresia is in isolation that nothing would prevent him from having surgery to repair it. Ultimately to us his lack of hearing is the most important repair over any cosmetic issues. I guess we have to expect the best and be prepared for the worst. It's hard to read about parents who never thought about it not being an option for their own children. They are so crushed by the news and only now are starting to research what to do next.
Miles doesn't know what it is like to hear the way we do, but I would someday hope that he would be able to.
