First ear question from a friend

A little girl in our playgroup asked Trey about his ears. She asked why one was bigger then the other. I just simply told her that he was just born that way. Trey immediately repeated my answer then he started to talk about him getting his bigger ear. Great thing about kids is that they don't care once they get an answer. She said oh and continued to play. Trey is to young to care right now that he gets these questions. I am not bothered by the questions, just a little sad that we are finally getting to the point that kids he plays with now notice. I hope his surgery goes well and he will be spared from having to answer numerous questions about his ear. I am sure there will be some just hoping to keep it minimal. I will teach him to be honest and polite and that people are naturally curious and that it is okay for people to ask as long as it is in a kind manner.

Did we know about microtia before Trey was born?

I have been asked this question before and have heard others wonder the same thing on the yahoo group message board. Did we know before Trey was born, sorta. We had a 3D ultrasound performed when I was 27 weeks pregnant at one of those private ultra sound places. The tech noticed the ear but was not able to tell us since it was for "entertainment" purposes only. Thankfully she informed my OB of what she had seen. I was not told of the possible ear issue until I saw my doctor 2 weeks after my ultrasound. I got my doctor to agree to further ultrasounds but by that time Trey was head down and laying on his left side, the ear in question could not be seen. I went through many emotions at the thought something was wrong and we wouldn't know for sure until the birth. I cried that first night. I looked at the 3D images and convinced myself they were wrong of course they look just like his ear. The interesting part is a week before my due date I saw a show on plastic surgery and it was about a boy who got a new ear with Dr. Reinisch. I then realized this was ours and Trey's fate but I at least knew there was something that can help and that gave me hope. Knowing ahead of time that something might be wrong was hard but a gift, I was able to deal with my emotions before hand and come to terms that we may have to face things we weren't planning on. I was not however prepared for the jaw issue and the words syndrome. But once we got a grip on what it was exactly we were dealing with I became so thankful it wasn't worse but sad Trey was going to have to deal with things you never want for your child.

Microtia options

So in regards to his left outer ear, we have 4 options. The first one is of course do nothing. Second is prosthesis. A third option is rib graft surgery which is done at age 6 or 12 depending on the doctor and the technique used. The forth option is surgery with a medpor implant. All options are good options, not one option is better then another just different. Important factor is when deciding on surgery is to go with a doctor who is very experienced in this surgery. I asked each of our local plastic surgeons who they would go to if it were their child it helps to know who other surgeons think are the best. Rib graft is the surgery most commonly used, many surgeons are not familiar with medpor so they will not recommend it since it is relatively new like the past 15 plus years.

I bring up the part about medpor because that is our choice for Trey. For us medpor can be done at age 3, I personally like the medpor ears over rib graft. I also like that it is not as invasive as rib graft since they do not use the childs rib cartilage. We were opting for prosthesis but we didn't like that the color fads in time and it has to be replaced every 2-3 years. I know of families who have chosed each of the choices and they chose what is best for them and their child. Some wait until the child can make the choice and others make the choice for their child, it all depends on what factors are important to you and only you. That is actually what makes this all hard, there are no right or wrong choices, each has pros and cons. We have no crystal ball so we go with what we know at the time.

CT scan

So this is just a post to write what we learned about getting a ct scan. First try to do it without sedation. The scan takes no more then 5 minutes and if they can't lay down still, the nurses will burrito wrap your child. Trey was wrapped and screaming most of the time during the scan, but I would take that then having him sedated which is what we went through with the ABR. It might sound traumatizing to the child but really its not, he was calming down right before it ended but since the scan itself was so fast the crying didn't last long at all. I am sure some kids won't cry, no pain just laying down on a bed that moves a little. I was able to stand next to him the whole time. They also used toys to distract him while the scanning was going on but that is because we had it at a hospital where they have child life specialist whose job it is to help kids get through such medical tests. We also requested a copy of the scan and got a 3D image as part of the report on a cd. It was interesting to see how things are underdeveloped but sad at the same time.

Finding support and information on atresia options

The hardest part is finding out nothing can be done until Trey is older. As a parent I just wanted to do what I can and as soon as I can but I have had to learn patience which isin't the best thing for me. Thankfully I found a yahoo group support site for microtia and atresia. Since many kids who have microtia and atresia also have HFM this group site provided so much needed information on all levels that we needed.

We found out our choices in how we can address these issues. For the atresia we could do a BAHA (soft band or surgically implanted), canalplasty(creating new canal and fixing middle ear) if a good candidate or of course nothing. To begin this process you have to do an ABR test to make sure the affected ear has only conductive hearing loss, if the nerve is not functioning not much can be done. A ct scan can be done to determine if atresia repair surgery(canalplasty) is an option. Very complicated surgery and the best doctors will review ct scans for free. CT scan should not be done before age 2 1/2 or you risk having to do it all over again, child needs to be big enough for the inner and middle ear parts to show up. Unfortunately the ct scan showed Trey definitely is not a candidate. Not surprising to us, he had less then 20 percent chance to be a good candidate because he has HFM/Goldenhar (side note, we prefer HFM but officially he is diagnosed with Goldenhar, the geneticist who diagnosed him considers HFM to be the same as Goldenhar while others differentiate Goldenhar for when the eyes have dermoid cysts or skin tags, all of which Trey doesn't have)

We can do a BAHA (bone anchored hearing aid) that could be used to provide some hearing since his inner ear (the nerve) works. There are 2 options with that, one is a soft band type that goes on with the use of a band that sits on the skull, no surgery and it can work well, just that it is very expensive and our insurance will not pay for it. They would pay however if we had the same device surgically implanted.

We have elected to wait and see if it would be useful or needed before we do that surgery. One doctor in town would have done the BAHA surgery already, I kinda freaked out about the permanent of the surgery and that the doctor didn't seem to care if the posts goes to far into the skull and rests on the outer layer of the brain. Infections are not uncommon with the posts of the BAHA so I am just not ready to go for it. We tried a softband that was loaned to us by the audiologist but Trey would not keep it on for any longer then a few minutes, of course he was only 18 months old and he knew he had complete control over if he would wear it or not. Maybe in the future we could try it again.

The begining of Trey's story and ours.

When our son was born we began a new journey into parenthood that included the journey of dealing with Goldenhar/Hemifacial Microsomia with microtia and atresia. What all that means for our son in plan English is that one side of his face did not develop normally so his left side appears smaller and his external and middle ear did not develop on that side. We had no idea what i all meant except that he looked different so our journey began.

Starting at a week old we had medical tests to make sure his eyes, kidneys, spine and heart were fine and thankfully they were. So all we have to address is his lack of external ear, unilateral hearing loss and a smaller jaw. We see an ENT doctor 1-2 times a year to make sure his good ear is working fine. We had to go do and ABR hearing test to verify the hearing on both sides after he was a year old which meant he was sedated. If only we knew to do this test when he was an infant where they do the test while the baby sleeps so no meds to knock them out. We didn't like our first ENT who wasn't much help to us but love Dr. Mancuso our current ENT. We also see a craniofacial team that will address the ear and jaw issues. We know Trey will require jaw distraction surgery and lots of orthodontics work. So we also see a dentist who is part of the craniofacial team.

So far Trey is a happy 2 year old who has not shown any problems. His speech is normal and he is happy and very very active 2 year old. He is social but can be reserved especially in noisy environments which is not surprising since he can not locate sound well. Around age of 2 he started noticing the differences in his 2 ears but did not seem to care which I would also expect do to his age. Sometimes he doesn't hear us when we try to get his attention and other times I am sure he DOES hear me but ignores me, again age appropriate behavior.