Anyone else with Ehlers-Danlos Syndrome?

#1

Does anyone else have a hearing loss related to Ehlers-Danlos Syndrome? It’s an unusual syndrome, unusual to have this particular variant and unusual to have this level of loss from it, so I find it really hard to get hearing aids to suit me.

Quick tour for the unfamiliar, EDS is a connective tissue disorder where the collagen that makes up the body is not formed properly, causing joint dislocations due to inadequate tendons and ligaments, poorly formed muscles, lax bowel, bladder difficulties, etc. In the ear the main problems are the ear drum being too lax so it doesn’t vibrate up to speed and takes more powerful sounds to cause it to do so at all and that it’s actually possible to dislocate or subluxate (slightly misplace but not push all the way our of joint) the small bones inside the ear, leaving you suddenly and usually reversibly deafened.

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#2

Thought this link might be helpful for anyone researching Ehlers-Danlos Syndrome - http://www.ehlersdanlosnetwork.org/typesofehlersdanlos.html.

Thanks.

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#3

Have you considered something like a BAHA. Not sure what the loss is beyond the conductive component but if the loss is due to the components of the middle ear, it might be worth looking into.

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#4

I am currently researching the Baha after realising I heard 100x better when a speaker was lying against my bed frame and I put the back of my head on the wood then putting it to me ear! The problem is really with getting agreement to go ahead because testing doesn’t really show up the nature of the problem. According to normal qualifying thresholds I don’t havea large enough air-bone gap, because all they are looking at is the difference in audibility threshold. They are not measuring the quality of the sound, just the quantity. I don’t have a stable and consistent 30dB air-bone gap at present therefore I do not meet the Baha criteria.

The problem is EDS is not so much with the sound not arriving at the other side of the middle ear at all, but that it is seriously warped by the time it gets there. Frequencies can change and clarity is cut off significantly. Because it’s a rare condition to start with and then all the specialists are rheumatologists because that’s its major manifestation there really isn’t such a thing as a specialist in EDS and hearing loss. If there were one then I might well be able to get that person to agree to a Baha or a middle ear implant system, but first I have to send out psychic waves for someone to start studying EDS and hearing loss and how they relate to each other.

And then find someone who would like to do surgery in a condition where surgical complications are rife. sigh

I’d love to get hold of a Baha on a band to see what it’s like, and if it’s good then I would start trekking around the country looking for someone who will implant one, but I can’t get a trial version because they only give those to peopel who have been accepted for the operation, which won’t happen unless I have a successful trial!! I figured when they said there was a trial version on a band they meant you tried it for a few months or something, but it turns out that procedure in the UK is for you to agree to the surgery in principle first, then when you go in to discuss the surgery you get to wear a test band for about 10 minutes in an audio booth, or if you are lucky you can wander around the hospital with it on for an hour or so, and then you must agree to the surgery and hand back the demo. OMG!

There is also the risk just now that I’d have to wear a body-worn Baha because they are not really designed for you to have much SN loss. I wouldn’t consider it unless I can meet the criteria for the head-worn version.

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#5

You might want to think about Auditory Neuropathy as a cause of your hearing loss or at least the non-conductive part. Unless that was ruled out during the tests for your CI. " There are other conditions involving neuropathy of peripheral nerves that have been associated with hearing loss and Auditory Neuropathy Spectrum Disorder. These include Leber’s optic neuropathy, Stevens-Johnson syndrome, Ehlers-Danlos syndrome, and Charcot-Marie-Tooth (CMT) disease, also known as hereditary motor sensory neuropathy. " (http://www.asha.org/aud/articles/CochlearImplantsANSD.htm)

LCL

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#6

Haven’t came across. Googled it and found some Symptoms of Ehlers-Danlos syndrome which includes:
• Double-jointedness
• Easily damaged, bruised, and stretchy skin
• Easy scarring and poor wound healing
• Flat feet
• Increased joint mobility, joints popping, early arthritis
• Joint dislocation
• Joint pain
• Premature rupture of membranes during pregnancy
• Very soft and velvety skin
• Vision problems

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#7

Yes. Yes. Yes–I have hearing loss related to EDS. I have hearing aids but have reached a point where they are not helping much anymore. I am consulting with an audiologist who is in touch with a doctor about Cochcular implants.

Do you have experience with these implants per chance, or know if they would be helpful for this kind of hearing loss?

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#8

I am wondering what a BAHA is.

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#9

Bone Anchored Hearing Aid.

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