Identified more than 40 years ago, Williams syndrome occurs in an estimated one in 20,000 births worldwide. It arises from a faulty recombination event during the development of sperm or egg cells. As a result, almost invariably the same set of about 20 genes surrounding the gene for elastin is deleted from one copy of chromosome seven, catapulting the carrier of the deletion into a world where people make much more sense than objects do. Despite a myriad health problems and a generally low IQ, children with Williams syndrome are loquacious, sociable, and irresistibly drawn to strangers.
To determine the extent to which this behavioral profile is universal across culture, the researchers settled on two vastly differing environments: the United States and Japan, whose cultural differences are said to be aptly summarized in two proverbs: In America, “The squeaky wheel gets the grease,” while in Japan, “The nail that stands out gets pounded down.”
Using a questionnaire developed by Salk researchers, Bellugi and first author Carol Zitzer-Comfort, a professor at California State University in Long Beach, asked parents in the U.S. and Japan to rate the tendency of their child to approach others, their general behavior on social situations, their ability to remember names and faces, their eagerness to please other people, their tendency to empathize with others’ emotional states, and the tendency for other people to approach their child.
Despite the differences in upbringing, in both countries children with Williams syndrome were rated significantly higher in global sociability and their tendency to approach strangers than were their typically developing counterparts. But cultural expectations clearly influenced social behavior, since the sociability of normal American kids was on par with Japanese Williams syndrome kids, whose social behavior is considered out of bounds in their native country.