My Life With Usher's Syndrome: A Personal Perspective
(Source: Boystown Research Registry)
My brother and I are afflicted with a genetic disorder, Usher Syndrome, the progressive degeneration has affected both our sight and our hearing. We both have had moderate hearing loss since birth. My brother´s visual loss has been more gradual than mine. At age 43, I am legally blind (have been for the last five years). This is my view of life with Usher syndrome, a psychological adversary that I subconsciously joust with everyday of my life.
I was born in a military hospital in Oceanport, New Jersey, on October 14, 1950. I have been blessed with a loving , supportive family. My dad was a 42 year career military man. He is a gentle, good-natured man despite his massive six foot four, 219 pounds. I guess I was 4 or 5 years old when I knew I wanted to be a soldier like my dad with one difference, I wanted to be an officer! My mom was the greatest! She tirelessly took my brother, Ray, and me to the best doctors and medical facilities. The way she "worked people" was magic and her influence on my life aspirations has been invaluable. My older sister, Judy, was a true scholar, intelligent and articulate. She spent many hours playing games with me and was an inspiring role model for academic achievement.
My hearing loss was diagnosed around the age of five. I started having noticeable problems with night vision around 8 or 9 years of age. Retinitis pigmentosa (RP) was diagnosed and we were informed that I would be blind by the time I was 11, obviously and fortunately for me, a miscalculation. Any real cognition of how my life and career choices had been diminished did not hit home. I was too young to understand.
My family´s love and concern has always been there as a support for me. Their only failing was they may have loved and supported too much and demanded too little. Despite my parent´s better judgement and the recommendations of the school, I was allowed my way in refusing a special education program. Of course, I wanted to be normal, to be accepted by my peers. It is apparent now that the school authorities and my parents were correct. But that is with hindsight, and no one knows if it would have made a difference.
One day, I was taken out of my grade school class by my teacher after refusing to put on my hearing aid. In Gestapo fashion, I was marched to the nurse´s office where the hearing aid vest was strapped over the front of my chest with a long wire hanging down. I was taken back to class; my classmates all stared, some laughed. I was sure I would be a freak for the rest of my life. The disgrace was too much; I jumped up and ran home crying. I never wore a hearing aid again until after I graduated from college. If my family had insisted that I wear my hearing aids, I might not have wasted so much effort on denying I had a problem. This incident was the beginning of a very long period of self-imposed limitation to my academic achievement, all for the sake of being accepted by my peers.
My family was made up of high achievers in academics and in sports. If I could not reach the heights in academic endeavors without my hearing aids, I would in sports. I was "All State" in track and football in high school, but I made everything more difficult for myself by pretending to be "Mr. Normal Guy". Since I would not wear hearing aids, I could not hear the starter when running track. I always had to compensate for being a few milliseconds slower than the others off the blocks. One time I almost ran off the track because I could not see the lanes. Numerous athletic scholarships for football and track were offered, but I could not accept them because of my increasing visual loss added to my hearing loss.
As the RP progressed, I also lost out on many personal, social experiences. Due to the severe night blindness, I could not drive at night. One of my most embarrassing moments occurred when I took a girl to a daytime movie. My eyes could not adjust to the sudden change in light when we walked into the theater. I could not see anything and was stumbling all over the usher who said, "What´s wrong with you? Are you blind?" I quit going to parties at night and avoided other situations that required my eyes to adapt to a change in light.
My ambition to become a military officer died when I realized I could not meet the vision and hearing requirements for candidates to West Point. It was devastating when I realized I had the intellectual abilities, probably more than some of my friends who were applying to the service academies and could pass the physical requirements. Fear of new and unfamiliar surroundings kept me close to home after graduation from high school. I resigned myself to a marginal existence academically and socially. I dropped out of college twice and worked part-time before eventually returning and obtaining a degree in biology.
My first job was as a chemical analyst. One day my boss pulled me aside and said "You can´t hear well. You need to get your hearing tested." That´s when I started to wear hearing aids. When my color vision became too poor to be able to discern the changes in color of the chemicals, I had to look for another job. I worked in management for a retail company for 2 1/2 years until we moved to Nebraska. For the next 11 years, I continued to hide my vision problems while holding a supervisory position. I started on nights and worked really hard to get moved to the day shift so I could drive to work. Eventually I had to surrender my license because my visual fields became too restricted to drive safely at all. When I left my job, I was legally blind.
My wife is the real reason for my career successes; she is the first person to make demands of me. She and her family provided the first break in my wall of denial. They are sharp, frank, and blunt in their criticisms. One day, my mother-in-law openly mocked my slurred speech; it was the first time I could hear how I sounded to others. After that I forced myself to speak up so I could hear myself and monitor my own speech for clarity. I never would have been able to hold a management position without clear speech.
It was one of the most difficult things for me to do, but with my wife´s encouragement and push, I went through the rehabilitation program at the Nebraska Services for the Visually Impaired for mobility training, learning braille, etc. She seems to know when to support and when to push. She believes in me and has fostered my belief in myself.
Currently, I am a graduate student in adult counseling. I do not try to hide my problems from my professors, and I am helping my fellow students to understand what a dual sensory impairment is. I want to use myself in helping young people with disabilities. I wasted so much time trying to deny the reality of my problems. I want to support others in facing the realities of their situations so that they can maximize their potentials.
A key in the discussion of the influence of my experiences with Usher syndrome is that it is a part of me whether or not I like its presence. I want people to understand it is truly awful, but I am still alive and have something to contribute to society. People with Ushers do not need people to think, "How terrible, this person is deaf and blind; there is nothing to do." What we need is for people to say, "This person is deaf and blind. What can I do to facilitate his achievement of his full potential?" I have allowed the reality of being blind/deaf to take its place as a singular reality in my life. No longer do I have to deny my full potential as a human being. I have learned that life and people are good, and I value myself.
Lawrence W. Lee
. Lawrence is a 43 year old Afro-American with Usher syndrome. He is President of Exceptional Students at Creighton (ESC) University in Omaha, Nebraska, & a board member of the National Federation for the Blind of Nebraska (NFBN).
Date Originally Created: Summer of 1994.
The information presented here first appeared in publications of the Boys Town National Research Register for Hereditary Hearing Loss, the National Institute on Deafness and Other Communication Disorders (NIDCD), Hereditary Hearing Impairment Resource Registry (HHIRR), or the Boys Town Research Registry for Hereditary Hearing Loss.
The Boys Town Research Registry for Hereditary Hearing Loss
The Boys Town Research Registry for Hereditary Hearing Loss (Registry) is designed to foster a partnership between families, clinicians and researchers in the area of hereditary hearing loss/deafness through three primary functions. First, the Registry disseminates information to professionals and families about clinical and research issues related to hereditary deafness/hearing loss. Second, the Registry collects information from individuals interested in supporting and participating in research projects. This information is used to support the third function of the Registry - matching families with collaborating research projects.
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