USHER SYNDROME AWARENESS; Congressional Record Vol. 161, No. 121
(Senate - July 29, 2015)

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[Pages S6128-S6129]
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                        USHER SYNDROME AWARENESS

  Mr. WYDEN. Mr. President, I wish to bring attention to a genetic 
condition known as Usher syndrome. Usher syndrome is the most common 
form of combined deafness and blindness in the United States, impacting 
as many as 50,000 Americans, not including their families, friends, and 
communities. It is estimated that 82 percent of those afflicted by 
deaf-blindness are unemployed. In the United States, the annual 
economic cost of blindness alone is estimated at 145 billion dollars.
  Usher syndrome results when there are mutations in genes that are 
important for the function of both photoreceptors in the retina and 
hair cells in the cochlea, or inner ear. To date, 11 genes have been 
identified that can cause different subtypes of Usher syndrome when 
mutations take place. These mutations usually lead to a deficiency of a 
protein that is critical for the health and function of the retina and 
cochlea. Usher type 1 individuals are born deaf and then learn, often 
before adolescence, that they are also losing their vision. Usher type 
2 individuals are born with moderate to severe hearing loss and then in 
the prime of their adolescent lives are told that they are losing their 
vision. Usher type 3, usually diagnosed during adolescence, leads to 
the slow loss of both hearing and vision.
  Life with Usher syndrome requires constant adaptation to the loss of 
vision, caused by retinitis pigmentosa. First is the loss of peripheral 
vision, when the rods are impacted resulting in the loss of night 
vision and the onset of tunnel vision, which shrinks over time to the 
size of a pinhole. Once the rods are gone, the cones atrophy. Color 
vision and the ability to read lips are lost, further impacting the 
hearing impaired Usher syndrome individual's ability to communicate 
with others. Often, central vision fades and the person is left 
completely blind.
  During this time--for which there is no prediction of how long the 
decline to total blindness will take--individuals with Usher syndrome 
are constantly adapting to remain aurally and visually connected. For 
the hearing loss, hearing aids, cochlear implants, American Sign 
Language, closed captioning, assistive listening devices, and tactile 
sign language are among the adaptive strategies used. For the vision 
loss, glasses, magnification, high contrast on computer screens, screen 
readers, audio descriptive devices, braille, canes, and guide dogs are 
used to compensate for the increasing blindness.
  To accelerate research, the Usher Syndrome Coalition is raising 
public knowledge by launching ``Usher Syndrome Awareness Day'' on the 
third Saturday in September. The theme centers on the autumnal equinox, 
which marks the start of days that contain more darkness than light--a 
powerful metaphor for the threat of Usher syndrome. This will be a 
global event that starts on one side of the world--Australia--and runs 
around the globe to the farthest point before the international 
dateline in Alaska.
  Like many, I too have a personal connection with Usher syndrome. A

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former congressional fellow in my office, Moira Shea, has Usher 
syndrome. She, with her guide dog by her side, took the Metro and came 
to work every day. In addition, she wore hearing aids, read lips, and 
overcame whatever adversity came her way. Today, Moira has lost her 
sight but not her vision--of finding treatments and cures for Usher 
syndrome so that no other generation has to go through what she and 
tens of thousands of other Americans have.
  With the acceleration of research, it may be possible for Moira and 
the thousands of others afflicted by this genetic disease to regain 
sight. The technology is there. The Casey Eye Institute at Oregon 
Health & Science University has started the first human study of gene 
therapy for Usher syndrome. I am proud that the first clinical trial 
for Usher syndrome is being conducted in the state that I am privileged 
to represent.
  I am committed to working with my colleagues to raise awareness about 
this devastating disease, and I applaud the hard work of the Usher 
Syndrome Coalition to make Usher syndrome research a higher priority at 
the National Institutes of Health.