Guest guest Posted January 9, 2000 Report Share Posted January 9, 2000 hi lauren; all i can say is that in orion's case, his hearing was lost due to his mito. if i understand (and remember) it right, his auditory nerve was eventually affected, which caused his brain to not receive the signals properly, thus causing the hearing problems. in his case, an aid would not have been helpful, because his ear was fine-- it was the nerve that was not functioning. he had a similar problem with his eyes-- optic disc hypoplasia-- which meant that (because of the mito) his optic nerves never developed properly, and he was blind from birth. i had asked about doing a transplant from me-- giving him one of my optic nerves, so he at least had partial vision, but it wasn't possible. in my heart i guess i should have known it wasn't possible, but i was desperate to at least do SOMEthing for him-- i know you all know the feeling. strange that you mentioned that sometimes your son seems to hear fine-- this was the same way with orion. his BAER scan (spelling) showed he was deaf, and an opthomologist diagnosed his optic disc hypoplasia, but (and this is not just a hopeful mother speaking) he very often responded (of course, never in front of the drs) to audio and visual stimulation. when i mentioned it to the drs, their explanation was that he only SEEMED to respond to the stimuli-- that he was not totally blind, but could only see movement, light, and shadows. thus, he was only responding to the light, etc, not the actual " sight " (for lack of a better word). they, however, were not with him on a daily basis, as we were. they had no explanation for why he responded to audio stimuli. i think they thought that i was just seeing what i hoped to see in him... but others had commented on it, so i know it was not all in my head. sorry i can't really offer any advice-- i just wanted to share my experience (it's not often that i get to do that, so i jump at the chance *smile*). julia orion's mommy september 18, 1998 to february 3, 1999 familial erythrophagocytic lymphohistiocytosis and mito In a message dated 1/9/00 12:04:01 AM Eastern Standard Time, sfitzger@... writes: << Does anybody know about hearing loss related to mitochondrial disorder? My almost 3 year old son seems to have lost his hearing sometime last summer. Before that he used to talk a lot, listen to music and understand most of what is said. Now he acts very autistic and does not respond to his name. I wanted to make sure that this wasn't autism as opposed to hearing loss, so I snuck up behind him with a pot and a spoon and clanged it really loud in his ear: he didn't turn around or flinch or anything. The strangest thing is that somedays he seems to hear okay and other days he doesn't hear at all. Does this sound familiar to anyone? If it is mito related hearing loss, can hearing aids help? I know that he has an ear infection at present, but I doubt an ear infection would cause hearing loss like this. Thanks for the input. (mom to 33 months...developmental delay, nystagmus, optic atrophy, no diagnosis) >> Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 9, 2000 Report Share Posted January 9, 2000 Hi , My daughter, Jill, is ten years old and she started to have hearing loss just last year. She was seen by an audiologist and the ear itself is fine. It seems to be a processing problem in the brain. The brain is not processing the signals it receives from the ear properly. As with your child, Jill sometimes hears just fine and other times she is not hearing very well. This was first picked up on an Evoke Potential Test which is a test that measures the brain's response to auditory and visual stimulation. My own unprofessional theory is that the brain is sometimes working to process the information and sometimes it can't. I have found that Jill will sometimes being yelling at us and I think she can't hear herself talking. Other times she seems to hear quiet conversations in the background very easily. Has your son ever had an Evoke Potential test? It may help determine if his responses are due to autism or hearing loss. I wish you good luck and hope you will find the answer. Take care. Hearing Loss > > > > >Does anybody know about hearing loss related to mitochondrial disorder? My almost 3 year old son seems to >have lost his hearing sometime last summer. Before that he used to talk a lot, listen to music and >understand most of what is said. Now he acts very autistic and does not respond to his name. I wanted to >make sure that this wasn't autism as opposed to hearing loss, so I snuck up behind him with a pot and a >spoon and clanged it really loud in his ear: he didn't turn around or flinch or anything. The strangest >thing is that somedays he seems to hear okay and other days he doesn't hear at all. Does this sound >familiar to anyone? If it is mito related hearing loss, can hearing aids help? I know that he has an ear >infection at present, but I doubt an ear infection would cause hearing loss like this. > >Thanks for the input. > > (mom to 33 months...developmental delay, nystagmus, optic atrophy, no diagnosis) > > >--------------------------- Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 10, 2000 Report Share Posted January 10, 2000 Thanks . No, we haven't had a brain evoked test yet, just two behavioral tests which were inconclusive. We tend to avoid any test that requires sedation because my son has a history of obstructing under sedation. However, if I knew that a hearing aid would help any hearing loss he has, I would be willing to do it so that I could help him. I feel so sad, because he used to LOVE music and now he doesn't even notice it. Yet he is so hyper and full of energy (he has to be on the move constantly), that I wonder if he is just too busy to notice. Thanks for the info. My hunch is that it is also a processing problem with my son, as his ears have been checked and they are fine as well. Thanks, McGarry wrote: > > > Hi , > > My daughter, Jill, is ten years old and she started to have hearing loss > just last year. She was seen by an audiologist and the ear itself is fine. > It seems to be a processing problem in the brain. The brain is not > processing the signals it receives from the ear properly. As with your > child, Jill sometimes hears just fine and other times she is not hearing > very well. This was first picked up on an Evoke Potential Test which is a > test that measures the brain's response to auditory and visual stimulation. > My own unprofessional theory is that the brain is sometimes working to > process the information and sometimes it can't. I have found that Jill will > sometimes being yelling at us and I think she can't hear herself talking. > Other times she seems to hear quiet conversations in the background very > easily. > > Has your son ever had an Evoke Potential test? It may help determine if his > responses are due to autism or hearing loss. I wish you good luck and hope > you will find the answer. Take care. > > > Hearing Loss > > > > > > > > > > >Does anybody know about hearing loss related to mitochondrial disorder? My > almost 3 year old son seems to > >have lost his hearing sometime last summer. Before that he used to talk a > lot, listen to music and > >understand most of what is said. Now he acts very autistic and does not > respond to his name. I wanted to > >make sure that this wasn't autism as opposed to hearing loss, so I snuck up > behind him with a pot and a > >spoon and clanged it really loud in his ear: he didn't turn around or > flinch or anything. The strangest > >thing is that somedays he seems to hear okay and other days he doesn't hear > at all. Does this sound > >familiar to anyone? If it is mito related hearing loss, can hearing aids > help? I know that he has an ear > >infection at present, but I doubt an ear infection would cause hearing loss > like this. > > > >Thanks for the input. > > > > (mom to 33 months...developmental delay, nystagmus, optic > atrophy, no diagnosis) > > > > > >--------------------------- Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 10, 2000 Report Share Posted January 10, 2000 I don't have personal experience with hearing loss issues but do know of a family in town with two little ones with mito that both experienced progressive hearing loss. They were told it was a very common symptom of the disease. Actually, some of you may recognize them...remember little Sam from the series in the Exceptional Parent on mito? He is now over 5 and has a little sister... -- The s , , Adelaine and Baby? Ann Arbor, MI http://www.mich/com/~jaj Quote Link to comment Share on other sites More sharing options...
Guest guest Posted March 19, 2000 Report Share Posted March 19, 2000 Hi Alice. I just got your e-mail...I dont' know why I just noticed it now. Your post was very encouraging, as well as the posts of all the others, and I am going to insist that see an ENT sometime next week. His neurologist wants to run a brain stem auditory evoked potential. And while this may be necessary at some point, I don't understand why she and the other doctors don't see the correlation between when started getting effusion and infections with his hearing/speech problems. The two seemed to occur simultaneously. All I know is that used to sit in his room for hours listening to music. Now he doesn't even notice if it's on. He doesn't understand words he once did and his speech has deteriorated. Is this hearing-related? I don't know. But if something like tubes or a cochlear implant will help him, we sure need to find out asap. Thanks so much! Best, A wrote: > > > Hi , > > As hearing issues seem to be my " specialty " I thought it was appropriate for > me to answer your question regarding . > > The physician has no excuse for not checking every possible reason for his > obvious hearing loss. The brain needs the stimulus of sound and the > lessening can in fact contribute to eventual permanent damage to hearing. > If you require a referral to an Otololaryingologist....request one > immediately. A qualified physician needs to rule out every possible cause > for hearing loss. It cannot be assumed that it is neurological. I would > guess right now that he needs ear tubes but even if it is more extensive > than that, he deserves a chance to have every hearing option there is. > > I would question the physician who has not researched this thoroughly as I > am deaf and know what it is like to live in a silent world. Perhaps he > needs to spend a day trying to read lips. My experience with many medical > personel has been a very sour one at times when it comes to working with the > hearing loss issue. Most of them simply do not understand. > > Hearing loss and/or deafness is worse than being blind according to Helen > Keller. A blind person loses their association with things...a deaf person > loses their association with people. I can share the fact that I have never > known anything more isolating. > > There can be many tube surgeries if necessary. More than one of my > grandchildren has had this surgery and three of them have had it many times. > In fact, in the last two months, all three of them have had new tubes put > in. The youngest is 2 1/2 and he had his fifth set put in on Wednesday. > This child has multiple disabilities and has Malignent Hyperthermia as well. > He did just fine. > > So please, save his hearing and call today. I'm on a crusade to prevent > deafness. I hope you are not offended by my strong concerns. > > Please let us know how he is doing. I am extremely interested. > > Alice > Mitochondrial Disorder, Diabetes, Deafness, Cochlear Implant > > ------------------------------------------------------------------------ > GET A NEXTCARD VISA, in 30 seconds! Get rates > as low as 0.0% Intro APR and no hidden fees. > Apply NOW! > http://click./1/975/1/_/368657/_/953292997/ > ------------------------------------------------------------------------ > > Brought to you by www.imdn.org - an on-line support group for those affected by mitochondrial disease. Quote Link to comment Share on other sites More sharing options...
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