Question about Ushers Syndrome Type 2

[Emma Handford]

Hey there,

I suffer from Ushers Syndrome Type 2 and I'm 24 years old.

I've had a nagging question for a while now and wondered if anyone who
is a part of this group could answer it?

My vision loss currently is in the shape of a doughnut, a polo mint
shape. I can see within the polo and outside the polo, but the rest
just gets filled in by my brain.

For instance, I'm looking at a bush. All I can see is the bush, but
actually there is a bird sitting in it, I won't see the bird if it
lies on the polo. But I would see the bird if I moved my eyes around
so that the doughnut shape no longer covers the bird.

My question is however, I know that the eyesight is failing, but I'm
not sure in what way. For instance, my sister suffers from the same
condition, but is losing her vision in what I believe is the normal
way - tunnel vision.

So does anyone know of, or suffering from the same thing? What I would
really like to know is what happens after this? Will the doughnut just
grow? Both sides, so that the center of my vision fails before the
peripheral? Or will it be a case of I end up with the central vision
being the last thing to lose?

Preferably of course would be that my peripheral vision goes first so
that I am left with my central vision last. Having this polo mint
shaped blind shape however has made me ask the question, what next?

Hope someone can help!

Many thanks,

Emma

[Mark]

Hi Emma,

I'm not an opthalmologist, but I know a number of great ones. I've
forwarded your question on to them. I'll let you know when I hear
back from them.

On Oct 6, 12:05 pm, Emma Handford <emma.handf...@googlemail.com>
wrote:

[Mark]

Hi Emma:

What you describe is indeed fairly common in Usher 2. We call it a
mid-peripheral scotoma or a ring scotoma (from the Greek for "area of
darkness"). You describe it very nicely....when we test your visual
field,
we find that you can see the spot as it moves in from the periphery,
then
you lose the spot, and the spot reappears near the center of vision.
It is
extremely important that you know of your "blind spots".....some
patients
don't realize that they have them until they have some kind of
accident.

In our experience, and in the published literature, the progression in
the
field is from the outside in. As you lose field in the far periphery,
the
outer seeing region becomes a ring, then a peripheral island and then,
possibly no peripheral vision. But I think it is fairly same to
assume that
your central vision will be the last to be affected.

Here's to a way to stop the progression before it comes to that!

Cheers,

David Birch

On Oct 6, 12:05 pm, Emma Handford <emma.handf...@googlemail.com>
wrote: