The Real Explanation of The Eyes Plus The Ears
I’ve got what’s called RP or Retinitis pigmentosa, if you don’t want to click on the link. With the hearing impairment, it’s called Usher Syndrome.
The disease is genetic. My parents don’t have it, my grandparents don’t have it, and none of their brothers or sisters have it. They gave it to me via recessive genes, and it just happened to pop up in me. The rest of my siblings are normal, except for Paul. He’s more hearing impaired than I am, but we wonder whether he really does have Usher’s since his vision is still normal. He’s twenty-two.
There are several types of Usher’s; my type is defined by my non-degenerative hearing loss and my degenerative eyesight. My ears have always had a cookie-bite line on the what-you-can-hear graph. Without my hearing aids, I can hear low sounds best and cannot hear high sounds unless they’re right up next to me. The two best examples are airplanes and microwaves. I can hear the microwave’s low hum when it’s warming something, but I can’t hear the beep when it’s done unless I’m staring into the low-resolution view window. I can’t hear my little brother as he runs screeching through the house.
Without my hearing aids, I can hear you if I can see you clearly and if you raise your volume just a little. Even with my hearing aids, I depend a lot on reading lips. I generally can’t understand the lyrics in an unfamiliar song unless I’ve got the text in front of me, someone’s singing along, or the song is simple and repetitive. I really like instrumental music because I don’t have to work as hard to try to make sense of what the song means.
In general, my vision is just bad. I’m already legally blind because of my left eye. There is some vision in it, but what’s there is in patches and what’s there isn’t very good to begin with. In my left eye, I can see vague patches of color and large sweeps of movement. If you hold up a number of fingers and I cover my right eye, I can’t tell you how many fingers you’re holding up and I’ll be lucky to be able to see your hand at all.
The right eye is much better but it’s affected by the RP. In that eye, I have trouble seeing far away things, which is one reason I shouldn’t ever be allowed to drive. A lot of driving depends on signs, and if I can’t see them until they’re just about to zoom past me, I’d be constantly turning my head to see the signs and missing the traffic around me. I don’t think that’s an effect of the genetic disorder.
The RP has eliminated most of my peripheral vision, which makes me horrible at sports. I can’t see a ball flying at me until it’s right in my face or unless it happens to fly straight at me. I’m a typical girl in that I’m horrible at sports anyway, but the vision just makes it worse. I was often picked last at team games when the captains got to hand-choose their teams. I tried softball and soccer and didn’t stay more than one year at either of them. I stuck to cheerleading for two years; I quit volleyball in my second season because I realized that the team was sacrificing its quality by making sure I had the chance to hit the ball.
I have horrible night vision, and it’s getting noticeably worse. I use a cane at night even in familiar territory so I don’t have to stare at the ground to make sure I don’t trip over anything. The cane lets me look up at cars that might be in the road or branches overhead. Sometimes I still doubt it’s usefulness, but when it’s pitch black and I’ve just stepped outside, sometimes I wish I have it.
Some of the time, I have a Just’In instead. He’s become really adept at describing the terrain just ahead of my feet. It’s the same thing a cane does, but he’s warmer. And he smells better.
My eyes wiggle or tremble when I’m tired. It’s called strabismus, and it has to do with the muscles the move the eyes to and fro. My first eye doctor did a surgery to fix it in my left eye, but it has either returned or never was fixed fully.
When I’m concentrating really hard to see the details of something, there’s one spot close off the center of my right eye, closer to my nose, that seems to have the best vision. It hasn’t translated to field vision tests, but It’s been captured on film since I was a baby, and it translates to a very peculiar look. You might see it someday.