Marion Andrea Schmidt
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Preventing tragedy, negotiating normalcy
The changing meaning of Usher syndrome, 1960–1980
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Usher syndrome – congenital deafness and progressive vision loss – is often portrayed as one of the most tragic disabilities imaginable. This notion certainly drove psychologist McCayVernon, who in the late 1960s initiated a campaign to raise awareness with the goal of the eventual eugenic eradication via early screening, counselling, and, if necessary, ‘therapeutic’ abortion. Over the course of the 1970s and 1980s, the definition of Usher syndrome changed, not least because of the input and experiences of people living with it. In the 1960s, professionals defined it as a severe psycho-neurological disorder that caused psychiatric and emotional disturbances alongside the hearing and vision loss. Encounters with individuals with Usher syndrome who lived independently and were successful professionals challenged this paternalistic definition. Patient advocates shifted attention from eugenic prevention to improving quality of life, yet also used genetic motives to argue for their identity and worth. By the 1980s, both advocates and professionals focused on the emotional impact of living with deaf-blindness for the affected individuals and their families.

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Eradicating deafness?

Genetics, pathology, and diversity in twentieth-century America

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