Wednesday, March 23, 2011

Auditory Neuropathy… maybe not.

Today was Blair’s ABR (auditory brain response) test in the OR at All Children’s Hospital in St. Petersburg. We came all the way down here to see Dr. Berlin’s team. We’ve been planning this trip since the fall, but this visit to Dr. Berlin has basically been two years in the making – since I talked to him that first time two years ago. Dr. Berlin has been a leading researcher in Auditory Neuropathy (also called Auditory Neuropathy Dys-synchrony – AN/AD – which better describes problem) for over 15 years – since it was discovered.

I was nervous about the procedure at first because the preop instruction nurse told me over the phone that Blair would have to be intubated. It turns out she was wrong, and Blair did great for the whole 2 hour procedure on just oxygen! (PACU was a little rough with a veeeery sleepy, crabby Blair who couldn’t keep her oxygen saturation up well enough to leave. But we eventually got out of there – after a popsicle, juice, and two chocolate ice creams!)

st petersburg 032311 (5)editThe fashionista wasn’t too crazy about the gown. She wanted her “heart dress back on.”

Dr. Berlin had told us before the procedure that it could take 30 minutes if they got bad results on the test and 2 hours if they got some good ones. About an hour into the procedure they came out into the waiting area and nearly gave me a heart attack. They had GOOD news though – Blair does NOT have AN in the left ear. She has a moderate to mild sensorineural loss that can be treated with a hearing aid in that ear. She already has hearing aids, so they actually boosted the gain up a little on the left one to better accommodate the loss. It was set really low before because she had been diagnosed with Auditory Neuropathy in both ears. (This AN thing is so confusing, and after two years of mommy research and dealing with it with Blair, I still myself don’t understand it totally.)

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Why is it good that she doesn’t have AN in the left ear? Well, AN is characterized by bad timing in the firing of the auditory nerve, causing words to be garbled or heard with static (like tuning in an football game in an AM radio station – you hear the game but you also have a static over the announcer’s voice). AN is hard to treat with hearing aids because they amplify the static and don’t allow for better discrimination of speech. Cochlear implants are becoming the treatment of choice for these kids because even those that hear pretty well and have developed speech are suffering socially in school because due to “interference” they can’t keep up in conversations among friends. Our decision whether or not to do a cochlear implant is most of what brought us to Florida. I’ve been in touch with Dr. Berlin for two years now, and we felt it was time to have him look at Blair. I just did not feel totally good about going with a CI (and taking away her natural hearing in that ear that is implanted), and we were getting somewhat conflicting opinions at home, so God led us to the right place!

st petersburg 032311 (9)Dr. Berlin won her over from day one with his magic blow-in-the-hand-and-make-the-quarter-disappear trick. She was checking behind everyone’s ears for it! Dr. Berlin came to the RMH the day before the procedure to meet us and then he escorted us all day today. It was SO nice.

Since Blair only has AN in the right ear, we can try to work with the left (no static – yay!) and see if we can get good progress with a correctly programmed hearing aid. For the right ear, which does have AN, she can wear her hearing aid… or not – it might actually be better if she didn’t so that she can get a good clear signal from the left. We’ll have to talk more about that later.

st petersburg 032311 (11)Daddy’s girl in PACU. The nurse could not get over her paci obsession. It’s bad, really bad.

We still have some stuff to discuss and a lot of work ahead of us. We have to address the hearing aids to make sure they’re the ones she needs (at least for the left), a repair that these need for the time being, a NEW pediatric audiologist at home (which will be further from home than the one she has, but the drive will be worth it), possibly getting her an FM system for the left ear that will put sounds right in her ear from a mic we can wear, and probably more things I’m not aware of! We are thankful, thankful though for the wonderful audiology team here at All Children’s – the hospital and other staff are fabulous too – and so thankful that God led us to come here. We feel blessed to have gotten this new diagnosis and pray that the new treatment brings great developments in Blair’s speech.

st petersburg 032311 (29)editSliding later at a park on the water.

st petersburg 032311 (28)Oh goodness, dirty hands!

God is good! I’m still amazed that the original diagnosis was not completely correct – even after 3 ABRs at home. We are just so thankful that we are here and didn’t make a rush decision to implant.

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I’ll follow with more details! Thanks so much for your prayers!

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Loving riding on daddy’s shoulders and touching every sign!


Jen O'Daniel said...

I'm so glad you found Dr. Berlin and his team! Sounds like he's been great to provide some clarity to Blair's situation! I've been thinking of you today! I'm so happy to hear about that left ear and that you can use it to help you work with the AN in the right. You are an awesome mom, and Blair is a lucky girl to have you.

Linsey said...

Go Blair and go Dr. Berlin! So happy for y'all!!!

Unknown said...

Ash, what great news! You are a great mama! I just tear up looking at those pics of Blair...she is so precious and happy!

KatieG said...

Yay! I'm so happy for your results! Praying for you guys. Blair is so lucky to have you as her mom.

AA said...

Wow, Ash! It sounds like it was the perfect trip for clarity and a path. So happy for you guys that it's not in both ears. I was thinking about you guys a lot and so happy that prayers were answered. Go Blair! What a blessing Dr. Berlin is! Have a safe trip home!

Amy said...

Awesome news! Blair looks thrilled :)
i know you are feeling better now that you are getting some clear answers....praying for continued guidance and discernment!

Amy Odom said...

Ashlee, so glad to see that your trip brought you some much needed answers! I love, love the pictures! Blair is such a beautiful child. Hope y'all have a safe trip back home :)

jmluckie said...

Glad for the good news.We'll keep praying.

Jen said...

Cried so much reading this news...
We need to chat soon!!!!!!

Molly @ View From a Saddle said...

I love that last picture of her on brad's shoulders!! so cute!!
I'm glad Dr. Berlin was as awesome as you thought he'd be!

CHERI said...

I certainly don't understand a single thing about what's going on, but sounds like you are pleased with the results; therefore, so are we.

Cow Pies & Mud Pies said...

Hi~I just came over thru CowCamp Tales.
Your daughter is beautiful and I'm so sorry she has had to have surgery. I will pray for ya'll to find exactly what you need for her. I will also believe for her healing! I will be back to visit soon.