Monday, May 3, 1998
Seeing clues to hearing loss
Condition is linked to distinct features
BY DEBORAH KENDRICK
The Cincinnati Enquirer
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Group support
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The mix of traits caused by Waardenburg syndrome varies from individual to individual, even within families. While many members of one family may be deaf in one ear, there may be only one person affected in another even though many display the visual characteristics.
Kelly Spataro and Dr. Alice Kahn are seeking to identify other families with Waardenburg syndrome, for the purposes of establishing support groups and - or a national foundation. For information, contact Dr. Kahn at (513) 529-2508, or e-mail at kahna@muohio.edu.
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When Kelly Spataro was a child, she remembers the neighbor who, on Halloween night, would pull her into the living room so all guests assembled there could admire her ''brilliant blue eyes.''
She grew accustomed to her mixed blue-brown eyes attracting attention but thought of it as little more than a personal quirk.
When she noticed the same eye coloring in her second child, Neil, at 4 months, she realized that the mixed eye color must be a dominant gene.
Nothing, however, prepared her for the pronouncement from a Children's Hospital audiologist that Neil, at age 4, had profound hearing loss on his right side. The Hyde Park mom stumbled into the parking lot, dazed, knowing from her work as a special education teacher that ''profound'' meant severe.
Shortly afterward, a doctor astonished her again by saying, ''I'll bet your eye color is related to his hearing loss.''
Called Waardenburg syndrome (after the Dutch eye doctor who first identified it in institutions for deaf children), the gene responsible for Mrs. Spataro's beautiful eyes and her son's partial deafness is a dominant gene and reveals itself in easily detected characteristics.
Pigmentation of skin, eyes, or hair, and facial features such as wide set eyes are often quick giveaways of the Waardenburg gene.
The hearing loss alone is the invisible characteristic - and the reason that Mrs. Spataro has been driven to seek the support of other Waardenburg families.
For the last five years, she has spent hours poring over library stacks, questioning medical professionals and searching the Internet for information. She now knows that if she spies a stranger with wide-set eyes, a white forelock, prematurely gray hair or differently colored eyes, chances are there is hearing loss somewhere in the family.
Dr. Alice Kahn, an associate professor of speech pathology at Miami University, has long researched the connection between minor craniofacial anomalies and hearing loss.
''If I see that someone has a very receded chin and the ears are slanted back,'' Dr. Kahn cites as example, ''I know they probably have a hearing loss . . . because when they were an embryo, those jawbones and ear bones were developed at the same time.''
Dr. Kahn is seeking a grant to conduct a visual screening to identify Waardenburg individuals. Since WS characteristics are very striking, they are hard for the trained eye to miss.
''At the very least,'' she says, ''people should be aware that if you have this syndrome, there is a risk that people in your family will have the hearing loss.''
She has identified at least six Waardenburg families in Cincinnati, and suspects there are more. Her aim is to teach others coming into the fields of audiology and speech pathology to observe faces, heads and necks as diagnostic indicators of hearing and other sensory disabilities.
Meanwhile, Mrs. Spataro has learned that her son's hearing loss will probably progress no further. Neil, 9, has learned to use an FM assistive listening device in school, and to maximize use of the good hearing on his left side. ''He talks every day about when he grows up and has children,'' Mrs. Spataro says.
Obviously he now puts his hearing disability in the same category of ''personal quirk'' that his mom once did her eye color.
Deborah Kendrick, a Cincinnati free-lance writer, is a nationally recognized advocate for people with disabilities. Write: Deborah Kendrick, Cincinnati Enquirer, 312 Elm St., Cincinnati 45202; e-mail: 71340.473@compuserve.com.
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