Monday, June 3, 2013

The Latest Edition of "The Results are in..."

So, after another ridiculous hullabaloo with the ENT's office, we finally got the MRI results today.  I'll spare you all the details because, well, they really don't matter.  What matters is that his ear anatomy looked fine.  I actually spoke to the doctor on the phone, who read me the radiologist report, and told me that his ears' anatomy looks normal.  Yay?  <----- That question mark is intentional.  I really want to be excited about this news.  And I am, seriously, I am thrilled it looks normal, but the bottom line to me is, what does that mean then?  I asked him a ton of questions, and I'm not sure I'm completely satisfied with all the answers, but at least we have an insight as to what his ears look like to start us in a direction.  Here is a loosely translated "transcript" from what I remember of my Q&A session with the ENT during recess this morning:

Me: OK, so if his ear anatomy looks normal, what's causing his deafness?
Dr:  It could be the nerves inside his cochlea.
Me: So, you can't see that on the MRI?
Dr: No, we were just looking at the auditory nerves.
Me: OK, so as long as the auditory nerves look normal, he'll be a candidate for the cochlear implant?
Dr:  Well, there's still a long list of tests and hoops we need to jump through, but yes, his anatomy looks like he'll be a candidate.  You and your family will need to meet with psychologists for screenings.  It will be a big deal for him to go from not hearing anything to hearing some sounds, so we have to make sure everyone is psychologically ready.
Me:  You know he's one, right?
Dr: Yes, but you all will need to be screened as well.  You'll meet with social workers, etc, etc.
Me:  So, if the nerves inside the cochlea were damaged, is there any way to see that or do you know what might have caused that?
Dr:  No, there's no way of knowing.  It could've been congenital.
Me: You mean like he was born deaf?  Could it have been because he was so premature those nerves just didn't develop properly? Or could it have been ototoxic medicines?
Dr:  Maybe, but there's no way to know.  It could've just been random congenital deafness.
Me:  But there's no history of hearing loss in our family at all.
Dr:  It can be random.
Me:  Could it be neurological?  Did the radiologist mention anything in his notes about his brain in the images?
Dr:  He just mentioned that he does have an enlarged ventricle.
Me:  Well, he was diagnosed with mild hydrocephalus, but the neurosurgeon said it didn't need to be followed, and he could just have a large head.

And so on...

So, now I'm wondering.  What the heck???  What does this all mean?  The great news is that it seems like he will most likely be a candidate for a cochlear implant, but I just don't know what to think.  If his anatomy looks normal in both the CT scan and the MRI, shouldn't we start seeing a neurologist?  Of course this is just me thinking out loud (well, out loud with my keyboard) because I know I'm going to be asking for another neurology referral as soon as I see our pediatrician next week.

I wish I had more answers for everyone, believe me!  But we will work to get to the bottom of all this and see what we can find out.  Dave and I are thinking that in addition to the neurologist, it's probably time to start seeing one of the audiology centers we've heard of in LA (House Institute) because they seem to be the best, we're just going to have to face the music with the insurance not covering any of the hearing stuff. 

Thank you all for continuing to pray with us for Ryan's hearing as we get this all figured out.  What a road we'd never imagine we'd have to walk down!  But your love and support has certainly guided the way, and I know that MRI was a miracle.  To hear the words, "The anatomy looks normal..." was such a blessing and I knew it was all in God's hands.  I'm praying now that He can continue to unravel this mystery and point us down the right path to getting our little man hearing whatever he can as soon as possible.  I'm going to share this blog post all over the place in the hopes that by some miracle, someone who knows something about audiology can maybe send me a message or leave a comment and let me know their insight. :)

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Kayla Smith said...

Most children with hearing loss are born to parents with normal hearing. In fact, most "genetic" causes of congenital hearing loss are from recessive genes - meaning that there may not be a history of hearing loss in the family.

You can see an audiologist for an ABR (auditory brainstem response). Instead of looking at the structure of the inner ear (what the MRI did), it will look at the nerve's response to sound, giving you and the audiologist an idea of the function of the nerve.

As far as your little one's prematurity - the cochlea (the inner ear) is one of the first things to fully form in a little baby in utero.

The enlarged ventricle can sometimes be a cause of hearing loss, but not necessarily. The radiologist, doctor, and audiologist will need to communicate to see if that's a factor.

And your baby being 1 is actually important. The sooner that your baby can get some auditory input, the better. Since the cochlea is the first the to form, typically developing full-term babies are born with about 4 months of listening experience.

An audiologist and speech pathologist can work with you and your family to jump through the "hoops" the doctor was talking about.

They won't want to fit your baby with a cochlear implant if he can benefit from hearing aids. Unnecessary surgery on one so little isn't something we like to do!

And the doctor is on to something- very often, there's no known cause of the hearing loss. It's called "idiopathic" - a fancy word for "We don't know."
Ototoxic medications can also be a factor.

There are "risk factors" for hearing loss and one of them is prematurity. This doesn't necessarily mean that it causes the hearing loss, it's just known as something that frequently co-occurs with hearing loss.

Check out the John Tracy Clinic in Long Beach (I mentioned this on facebook). It was founded by the actor Spencer Tracy and his wife for their son, John, who was deaf. Now it is one of the largest providers of information, support, and services for families of kiddos with hearing loss. They'll get you in touch with professionals, other families, and provide you with information that you may want.

This is an overwhelming time for you. Being stressed is perfectly normal. Being confused is normal, too. If you have any questions - facebook me!

(Sorry this is so long!)

Janice Hoffman said...

Hi there - I'm friends with Jessica Haight, and she send me your blog post because my son is profoundly deaf in one ear, and we spent 2 years jumping through hoops to figure out what the problem was! So, I definitely can sympathize with all the questions, even though my son has perfect hearing in one ear. We were instructed to do an Auditory Brainstem Response test (ABR), not an MRI, which we did back in Dec. and that's how we found out that his loss is sensorineural, not cochlear. I think I'm a bit confused by your post - so his cochlea is formed properly, but it might be his nerves? I'm not sure I read your post right. If so, that's what my son has as well, it's called Sensorineural loss. We were told a CI wouldn't help him because the cochlea reacts to sound, it's his nerves that don't process it. I won't keep throwing my son's info at you if it's not similar to what you're going thru! But feel free to e-mail me, if you want more info or to chat about this process. It's hard!

I wish you all the best, and hope you get some answers soon! Hang in there!

Anonymous said...

Hi Jen,

Courtney passed along you blog post to me. I have two children with bilateral sensorineural hearing loss. I hope to help you make sense of some of this, and I know how confusing this can all be. You are correct that it's good news Ryan's anatomy of the ear is normal. Sometimes abnormalities will point to progressive hearing loss. There's more hope for his hearing to remain stable with normal ear anatomy. You don't want a cochlear implant unless his hearing loss is classified as profound or deaf (no hearing at all). My son (also a Ryan ;) ) has severe hearing loss and just wears hearing aids. The reason for this is that the CI surgery would destroy any natural hearing he has on his own. By getting children fitted for aids as babies or young children, you'll dramatically increase their success with speech and language. Keep in mind that he is not deaf if he has some hearing, any hearing at all. There is a difference between being hearing impaired and deaf.
This was the first big thing I learned. My son spent some time in the NICU at birth so I assumed those early antibiotics caused his hearing loss. Then, I had another hearing impaired child. It's so frustrating not to know the absolute cause for the hearing loss, but it is very possible that it's genetic. If two people both carry a recessive gene for hearing loss, you can easily have a hearing impaired child. We also have no history of hearing loss in our family. I'd be more than happy to help you with any other questions. My kids are 8 and almost 12 now so I've been navigating this world of HI for some time now. Ask Courtney for my info. if I can help you out. :)--Jacqui

Anonymous said...

Think of it this way... inside the cochlea there are little hair cells. Their job is to dance every time we hear a sound. It's that "dancing" that sends the electrical signal through the auditory nerve to the brain. Sometimes those hair cells lay down and die (and in the case of genetic hearing loss don't work properly). The risk factors for causing them to die are numerous of which your so has many. So did he have a perfect little cochlea with perfect hair cells when he was born? Likely yes, but you can't rule out genetics either.

The thing about AN (auditory neuropathy) is that it'll give you an absent ABR despite some "useable" hearing, hence the hearing aid trial. Present OAEs with an absent ABR indicates auditory neuropathy. Unfortunately absent OAEs with an absent ABR does rule it out.

Ask your questions to your audiologist. He/she will be more than happy to answer them. There is no question he/she hasn't heard. No question is too dumb. Ask away. They'll guide you through this.

Anonymous said...

*doesn't rule it out.

Sorry, that's what I get for typing using my phone.