Does anyone else have a diagnosis of this? It seems to be very rare and we are wondering how other people with this diagnosis manage. The 'classic and unmistakable' symptoms are deafness, ataxia and anosmia. My husband was only diagnosed after being admitted with Bell's Palsy and the neuro took another look at his MRI from 3 years ago!! My husband is so disgusted at this mis-diagnosis that he now refuses to go for tests to find a possible way to stop the progression - there is no cure for this condition, but sometimes it can be stopped from getting worse. So, if anyone else has these symptoms or has been diagnosed please communicate; it would help to know we aren't alone with this horrid thing.
My Father was misdiagnosed with SS for over 10 years. The symptoms progress from hearing loss, to balance disturbance, to dementia, etc. He is 63 yo and if you met him you would think he was 80. It is very rare so it will be difficult to find someone to treat it. But, the first thing is to try to find the source of the bleeding. My father had a tear in his spinal cord lining and that was surgically repaired. Now they are attempting to treat him with Ferriprox---a drug that removes IRON from the brain.
Last edited by moderator2; 03-19-2009 at 04:09 PM.
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Hello- thank you so much for replying. It's a comfort to know we are not alone with this. It's also somewhat helpful knowing that it's so slow progressing. I totally agree that having the tests to find the bleed and (perhaps) stop it would be the best course of action; however my husband feels that a negative result would rob him of any hope at all, and thus he would rather 'wait and see'.
He's had ataxia for over 3 years now, and deafness for a bit longer, and sense of smell diminished gradually; but it wasn't until he developed Bell's Palsy that his neuro consultant took another look at the orignal (3 year old) MRI and found the SS!!
I wish you all peace and hope for the New Year, and again, thank you so much for responding.
Thank you for responding - I would love my husband to have some treatment, but he won't see the neuro as he feels this will take away any hope he has that things won't get worse!!
I have not heard of Ferripox, but will do some research and thank you for this information.
I hope you are coping with this horrid thing. My husband has probably had it for about 4-5 years as he was going deaf long before the ataxia became a problem. He is still active and working, but walking is a major worry. Digital hearing aids have helped with the hearing thankfully.
It would be nice to correspond with you, if only for mutual support and to know we are not totally alone with this.
I wish you well, and thank you for responding.
My Father had a cochlear implant to help with the hearing aspect. That was done at Hopkins,too. The symptoms seem to appear in a certain chain starting with hearing, ataxia, walking, then smell/ taste, bladder issues, and dementia. At this point, we mainly see dementia if he is sick with some kind of other virus/ cold/flu. We know he is getting the flu a day or two ahead because he will be very confused. He has been using a cane for the last few years. Your husband seems still to be in the rather early stages, so the Ferriprox might prevent worsening of the condition.
Hi Julia, My husband also has SS. He was diagnosed in 06. One of the problems I have is that no one knows anything about it. The neurologist knew what is was but had never seen anyone with it.
He has lost most of his hearing, his gait is very unsteady, and has cognative problems. They seem to be worsening, seems to be having a personality change. One of the latest is that he focuses in on one thing and then is obsessed by it and irrationally acts on it.
It is very frustrating that no one has any answers.
Last edited by moderator2; 02-24-2009 at 02:22 PM.
Does the drug reverse some of the symptons or just slow them down? My husband has SS and it is progressing. I notice more signs of cognative impearment and of course the walking and hearing. I never know what he will do next. Hard to deal with all of that.
I will see what I can find out about the drug also. It is so hard to find any answers. We don't have any idea how long he has had SS. Started have serious signs starting a few years ago, and was diagnosed in 07. Will keep in touch.
Supposedly, the drug has showed some success at reversing the symptoms--but mainly the hope is that it stops the progression. My Father is a few years ahead of where it sounds like your family members are right now. The latest challenge is loss of bladder / bowel control. My Father was diagnosed in September 2007 and we were told that the bleeding probably began as far back as the mid 1980's for the iron toxicity to build to the level where he would have the symptoms that he has. We also have problems with physicians not having any idea what the condition is...we are lucky enough to be within a hour of Hopkins, so that has been a good resource.
Marcia, I just saw your posting today. I have SS and am about to undergo Ferriprox treatment with Dr Levy at Johns Hopkins. I don't know how it will all turn out but I feel I have nothing to lose by trying. I'm 55 and the 1st sign of SS was in 1989 when I lost my sense of smell and hearing went bad. Since then I get tired easily, shake and have muscle spasms, numb feet and stiff muscles. How old is your husband? Is he still able to work? I am able to work full time, but am exhausted at the end of the day. Jeff