“No Limits” Philosophy Drives
Boise Junior Miss
Susie Jones, Idaho Hands & Voices
“She was born with a smile on her face.” And that moment set the stage for the rest of Amy Stott’s life. Amy’s mom, Michelle has a picture from that special day of Amy’s birth when she surprised the doctor and her parents by starting her life with a smile. And when I met Amy nearly 18 years later, she was still smiling.
Amy has faced many challenges and adversity in her short life, but she works through them with grace and has surpassed her parents’ expectations in all that she has accomplished. Amy is a dancer, a pianist, an Honor Roll student, a public speaker, a piano teacher, 2008 Junior Miss of Treasure Valley, and an inspiration to many. Amy also happens to have a profound bilateral hearing loss, partial blindness, and equilibrium imbalance as a result of Usher’s Type I Syndrome. It’s obvious that Amy’s disabilities haven’t limited her participation in and enthusiasm for life.
What is Usher Syndrome?
Usher syndrome is the most common condition that affects both hearing and vision. A syndrome is a disease or disorder that has more than one feature or symptom.
The major symptoms of Usher syndrome are hearing loss and an eye disorder called retinitis pigmentosa, or RP. RP causes night-blindness and a loss of peripheral vision (side vision) through the progressive degeneration of the retina. The retina is a light-sensitive tissue at the back of the eye and is crucial for vision. As RP progresses, the field of vision narrows—a condition known as “tunnel vision”—until only central vision (the ability to see straight ahead) remains. Many people with Usher syndrome also have severe balance problems.
Who is affected by Usher syndrome?
Approximately 3 to 6 percent of all children who are deaf and another 3 to 6 percent of children who are hard-of-hearing have Usher syndrome. In developed countries such as the United States, about four babies in every 100,000 births have Usher syndrome.
Where can I get more information?
NIDCD Information Clearinghouse: Toll-free Voice: (800) 241-1044,
Toll-free TTY: (800) 241-1055,
Fax: (301) 770-8977,
Reprinted with permission from the National Institute on Deafness and other Communication Disorders website
Amy was diagnosed with her hearing loss at 18 months of age. Her family was living in California at the time, but moved to Idaho when Amy was three. For approximately three years after her diagnosis, Amy wore hearing aids and later a tactile aid, which provided vibrations in response to sound. When Amy was almost five years old her parents chose to get a cochlear implant for Amy to improve her ability to develop spoken language.
Amy’s diagnosis of Usher Syndrome came later, when Amy was about 11 years old. The discovery was somewhat of an accident. Amy explains that when she was ten, she admired her sister Suzanne so much that she wanted to have glasses just like her. So she tricked her parents into taking her to the eye doctor for an exam by claiming that she couldn’t see the board in school. Amy then tried to trick the doctor by deliberately failing the eye chart test. As part of the routine exam the doctor looked closely at her eyes and discovered early signs of retinitis pigmentosa, a symptom of Usher Syndrome. Further exams and genetic testing later confirmed that Amy had inherited the condition.
During those early years, Amy’s parents were very proactive. She was enrolled in the state’s early intervention program immediately after her hearing loss was diagnosed. The whole family learned sign language and supplemented the early intervention services with private speech therapy sessions. After moving to Idaho, Michelle continued to champion for services for her daughter within the schools and through private providers. Her advice to other parents of children with disabilities: “Don’t let anybody tell you ‘no’ and don’t set limits for your child. Never underestimate what any child can do. We were afraid Amy wouldn’t ever speak or hear, let alone dance, play piano, talk on a phone, or excel in school. But she’s exceeded all our expectations and continues to do so.”
Amy is the middle of five children, surrounded by David, 24, Suzanne, 21, Rachel, 15 and Jacob, 8. Amy began dancing at age seven, following in the footsteps of her mom, aunt and older sister. Suzanne is one of the coaches on Amy’s competitive dance team at school, along with her teammate, younger sister Rachel. Amy spent several years dancing at a private studio where she still regularly performs and rehearses ballet. Amy estimates that she spends more than 15 hours each week rehearsing and dancing. “I just love it!” says Amy.
Amy’s love for dance and music carries over to the piano, which she began playing at age eight. “I started all my kids playing the piano when they were in the third grade,” says Michelle. And even though Amy was deaf, the family expectation still applied and she insisted on doing it. She learned to play the piano and has a real talent and is now teaching her eight year old brother Jacob along with two of his friends.
Amy’s talents were showcased in the 2008 Boise Valley Junior Miss Pageant. Amy’s friends encouraged her to enter the competition, and she’s grateful to them for pushing her. “It was so much fun! I had the time of my life on the stage,” recalls Amy. She and 22 other high school juniors from throughout the Boise area competed in five different categories. For academic, talent, fitness and interview portions, winners were selected in each category, and composite scores were used to decide the overall winner. A fifth category titled “Be Your Best Self” was also part of the competition. The winner of that category was selected by pageant staff based on overall attitude and accomplishments. Amy impressed the judges, audience, contestants and staff (many of whom didn’t know she was deaf) with her poise and skills. Not only did she win the title of Boise Valley Junior Miss, she also won the talent competition, the fitness competition and the “Be Your Best Self” award. Amy chose to play piano for the talent portion rather than dance because, as she states, “I put my emotion more in piano.” Amy went on to compete in the state Junior Miss Pageant in northern Idaho, where she was a crowd favorite.
So what inspires Amy? What makes her push ahead when faced with challenges? Well aside from her parents, Amy credits a few people and events.
Her best friend, Katie, has been her cheerleader, champion and confidante since they first met as neighbors almost 15 years ago. Katie learned sign language and played the part of Amy’s interpreter if needed. She even participated in speech therapy sessions to give Amy practice listening and talking. Even though Amy doesn’t use sign language anymore, relying more on her implant for listening, Katie and Amy occasionally tell secrets in sign. “They’re really bad in church!” says Amy’s mom. Amy asked her parents to move her to the neighborhood school when she was in fourth grade and leave the school with a center based DHH program so that she could be with Katie. Amy says that this decision marked a turning point for her in her quest for independence.
Many professionals helped Amy and her parents along the way, but one individual in particular has made a lifelong impact on Amy, and that is her former speech therapist Patty Heinz-Unger. “She taught me everything,” says Amy with admiration. “She encouraged me and emphasized how I could do anything. She believes in me.”
Michelle recalls that Patty was the one who taught Amy about self-advocacy, which convinced Amy to be in charge of her own IEP meetings. “Amy would plan the agenda, run the meetings, and Rick and I would just sit in the corner as she explained to administrators and teachers what her needs were and how they could help her.” Patty also helped Amy rehearse important speeches for school, practice interview questions for the pageant, and prepare for driver’s ed testing. “Patty incorporated Amy’s life into speech instead of the other way around,” recalls Michelle.
One of the speeches Amy practiced with Patty resulted in her election to the Student Council in Junior High. Preparing and delivering a speech to the entire student body was a daunting task, but Amy pushed ahead with confidence and her trademark “no limits” approach to life. She was elected to the student council and she was voted “Most Valuable Person” by the faculty and staff of the Junior High.
Amy used a sign language interpreter in her elementary and middle school years. When entering high school, she made the decision not to use an interpreter. Her parents were skeptical of this choice, but they knew their daughter was driven to succeed and had the confidence to ask for help if she needed it. “I just felt like I was wasting the interpreter’s time, because I wasn’t watching her,” recalls Amy. She notes that her teachers have been supportive with closed captioning and preferential seating. She’s not afraid to ask for clarification and checks in often with them about due dates, details, and steps needed to complete projects. Amy readily admits that assemblies are both loud and boring, so she doesn’t work too hard to listen and her friends keep her up to date if needed. She has been enrolled in honor’s classes since eighth grade and has maintained a 3.9 GPA.
Right now, Amy’s vision loss isn’t too limiting. She describes her visual field as “donut-shaped,” with the donut being the part she can’t see through. So when looking straight ahead or when using peripheral vision Amy can see pretty clearly. She recently discovered, however that she has trouble seeing her arms when extended in front of her at certain points, which has made dancing more of a challenge. Disturbances in balance are another symptom of Usher’s, but Amy has considered this just another challenge that won’t hold her back and has learned to compensate.
Amy will graduate from Mountain View High School this spring and plans to attend Brigham Young University in Provo, Utah. She’ll most likely pursue a degree in counseling or psychology to accomplish her goal of helping other people. “I’m not sure what I will do,” say Amy, “but I know that I want to make a difference.” Most would agree that she’s off to a great start. ~
Editor’s note: Susie Jones, M.S. CCC-SLP is a member of Idaho Hands & Voices and the mother of a child with hearing loss.