Monday, March 30, 2009

Does He Hear or Not?

Tomorrow we have a hearing impairment teacher coming through the infant and toddlers program. It was actually quite a battle to even get this initial consultation to occur, despite the state audiology department's specific request for this to begin ASAP.  The ironic thing is both program departments are run by the same state agency. Isn't that the best of bureaucracy and government. The main reason they are pushing so hard is the inherent potential for hearing loss or deafness with Elias diagnosis of FA. Since there has been no official determination on his hearing abilities either way, because of testing issues, they want us to begin early intervention for the worst case scenario. We are still working with the hospital to have the level 3 hearing test completed during one of Elias surgeries in the coming months.

Admittedly, we are a bit apprehensive for the final results. We have wondered since the beginning about his hearing because of his right ear, which is not fully developed. We always anticipated diminished hearing, but lately it is appearing more and more like Elias might be deaf. There are days that we feel as though he can hear and others we are uncertain. The most convincing that he is deaf is coming behind him while one of us holds him so he does not know we are there. We have made loud noises, clapped loudly and done other activities with zero response and no startling. That is concerning and leads us to anticipate the loss of hearing. On the bright side, it certainly has not prevented him from doing many things so it is not an overwhelming concern. Tomorrow will be interesting and a little anxious, but we are positive with this intervention good things will come out of this.


  1. Hoping for peace with whatever the testing shows - although it seems like you already know. Just remember, none of Elias's medical issues define him: he is an extraordinary boy because of his heart and spirit (and his parents), and he will continue to be exactly that after this round of testing.

  2. My son Sebastian (FA-D2) is hearing impaired on both ears (not completely deaf, but severely hearing impaired). We had a suspicion very early (2-3 months old), and the tests confirmed this. Some of the tests were very tedious (I am not sure of the spelling) and only dragged the choice of hearing-aid longer. The doctors could not tell exactly why Sebastian could not hear, only that he indeed - and very fast - needed an hearing aid. At first they tried 'normal' aids, ear-hangers they are called here. As his ears were VERY small they had to make some very special and custom-made ones for him (more time lost there), and we could see no sign of him having any use of them. They were very complicated to use, and fell out (he was 5 months old at the time) all the time. At 6 months old we went to the audiologists again, and one of them happened to have a BAHA-softband hearing aid in her drawer. It is a bone-conducting hearing aid, which you just put around the head of the child as a headband. The box turnes the sounds into vibrations, and - if the inner ear is functioning - makes the child hear. It was very easy to try on Sebastian, and the second he got it on the head he froze in his movements. Since then he has been hearing extraordinarily well - with the aid. He speaks fluently and has been speaking since the age of two. I would recommend to try on the BAHA-softband straight away (maybe even before all the tests). I know of quite a few FA-kids who uses this bone-conduction hearing-aid, as they have trouble getting the sound through the ear-cannals, which - in Sebastians case - are partly or totally closed. You audiologist certainly knows of bone-conducting hearing aids, and might have one in the drawer, which you could try on. Then the tests could be done afterwards, to find out exactly where the 'trouble' might be. Just an idea as we ourselves could have done without the long test-waiting-time with Sebastian. If you want more info on Sebastians hearing-impairment please do not hesitate to contact me (via Sebastian la Cour Marker Rasmussens homepage). Take care, Kirstine

  3. It sounds similar to emma. She has no right ear canal and a tiny left ear canal. We did the same type of hearing tests! We would come up from behind and do clapping and got no response. She has moderate to severe hearing loss. It appears the only problem is her ear canals. Her inner ears works great with a very slight delay. Sorry I'm so behind on all this! You've probably updated about this and I haven't gotten there yet. I have your blog on my google reader and I'm slowly making my way through the last ten entries... :-)


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