what cause you to become Ddeaf/Hhoh?

My Mother had very high fever and German Measles when she was pregnant with me. I was born deaf.
 
Doctors, as usual, make all kind of guesses which they want us to think are gospel. At first, my sister's deafness was attributed to our mom being exposed to the measles when pregnant her. Mine was blamed on so much shooting, even though I've worn ear protection since I began competitive shooting at age 10. My brother's deafness was said to result from a head injury, and my mom's was attributed to advanced age.

Now they think family genetics. It's like they draw reasons out of a hat.
 
my hearing in my right ear went from normal to severe-profound when I was 5 years old suddenly after undergoing proton beam radiation as treatment for a brain tumour. at least I think that's what happened.

since I was 7 or so, middle ear infections (otitis media) in my left ear led to the development of a cyst (cholesteatoma), basically a pseudo-tumour, which was discovered when I was about 16 years old. The infections caused a progressive hearing loss from when I was about 8 years old (mild, wore an FM system occasionally in class) to 16 years (severe, and profound in very high frequencies). after I had a mastoidectomy at the end of 2005, the hearing in my left ear dropped into the profound range (97 to 120+ dB). It might have also worsened from sound exposure.

Was CIborged on May 26th this year (right ear... residual hearing is still useful in left ear)
 
RedFox said:
I am deaf because of a version of long QT syndrome. It's the homozygous form of long qt syndrome type 5.

Getting one defective copy of the KCNE1 or KCNQ1 genes is enough to have heart problems with LQT5. Having two defective copies gives the homozygous version that also gives you deafness. That version is known as the Jervell and Lange-Nielsen syndrome. It is the KCNQ1 gene 90% of the time and the KCNE1 one for the other 10%. I don't know which one it is for me. I've been down by like 100dB since birth.

These genes code for parts of the voltage gated potassium channels in the cell membranes of cardiac muscle and inner ear cells. The channels are made of proteins encoded by a bunch of genes to carry K+ ions out of the cells to repolarize them.

The treatment I got is taking the beta blocker metoprolol and an operation. The beta blocker cuts the kidneys' production of the hormone renin, Renin causes the release of the hormone angiotensin that makes blood vessels contract, increasing blood pressure. So reducing renin release cuts blood pressure.

The operation I got 14 years ago involved cutting a branch of nerves that were near the left side of the collarbone and connected to the left sympathetic nerve trunk, one of the two trunks of sympathetic nerves going down from the head parallel to the spinal cord.

That had the side effect of making my left pupil smaller than the other one with the left eyelid drooping and my left hand drier. Here's a picture of someone with eyes like that. I attached a picture of my eyes.

I also had an older deaf brother with the same genetic disease. He didn't get treatment because he died 15 days before his 6th birthday. About half of untreated people with JLNS die by the age 15. After he died, someone who studied the disease heard about it so I got checked for it and put on the medicines and got the operation in 1994 at the Strong Memorial hospital in Rochester where they study the disease.
Click to expand...

Redfox, my son, 7, has JLNS. I would love to speak with you!!!!
 
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