Anne, the eldest at forty-six, had undergone many surgeries to treat the physical problems that can come with Williams syndrome. She looked much older than her age, but she also conveyed a sense of wisdom and insight, and often seemed to be regarded by the others as a sort of adviser and honored elder. She favored Bach, and played some of the Forty-eight Preludes and Fugues for me on the piano. Anne lived quasi-independently, with some help; she had her own apartment and her own phone— though with her Williams loquacity, she said, she would often run up huge phone bills. Very important to Anne was her close relationship with her music teacher, who seemed to have a most sensitive ability to help her find musical expression for her feelings— as well as helping with the technical challenges of piano playing, which were exacerbated by Anne’s medical problems.
Even as toddlers, children with Williams syndrome are extraordinarily responsive to music, as I later saw at a Williams syndrome clinic at the Children’s Hospital at Montefiore in the Bronx. Here people of all ages go for periodic medical evaluation, but also to see one another and to make music with a gifted music therapist, Charlotte Pharr, whom they seem to adore. Majestic, a small three-year-old, was withdrawn and unresponsive to everyone and everything in his environment. He was making odd noises of every sort, but Charlotte began to imitate his noises, immediately catching his attention. The two of them began to exchange a volley of noises, which soon became rhythmic patterns, then musical tones and short improvised melodies. With this, Majestic was transformed in a remarkable way— he became fully engaged and even grabbed Charlotte’s guitar (it was bigger than he was) and plucked its strings one by one for himself. His eyes were constantly fixed on Charlotte’s face, drawing encouragement, support, and orientation from her. But when the session was over and Charlotte left, he soon reverted to the unresponsive state he had been in before.
Deborah, an engaging seven-year-old, was diagnosed with Williams syndrome before she was a year old. Storytelling and playacting were as important for Debbie as music— she always wanted a dramatic accompaniment of words and actions, rather than “pure” music. She knew by heart all the songs from her synagogue, but when her mother began to demonstrate this, she inadvertently sang a melody from her own childhood. “No!” said Debbie. “I want to do the song from my synagogue!” And she proceeded to sing it. (The songs from the synagogue are, of course, charged with meaning and narrative, the drama of ritual and liturgy— it is not coincidental that some cantors, like Richard Tucker, have become opera singers, going from the drama of the synagogue to that of the stage.)
Tomer, at six, was a strong, energetic boy, with a tough, outgoing personality to match. He adored drumming and seemed intoxicated by rhythms. When Charlotte demonstrated various complex rhythms, he got these instantly— indeed, he could simultaneously drum different rhythms with each hand. He anticipated rhythmic phrases and could improvise easily. At one point, the exuberance of drumming so overcame him that he flung the drumsticks down and started dancing instead. When I asked him about the names of different types of drums, he rapidly reeled off twenty different kinds from around the world. With training, Charlotte thought, he could certainly become a professional drummer when he grew up.
Pamela, at forty-eight, was, like Anne at the camp, the eldest, and she was highly, at times heartbreakingly, articulate. She became tearful at one point, speaking of the group home where she lived with other “disabled” people. “They call me all kinds of hurtful things,” she said. They did not understand her, could not comprehend, she said, how she could be so articulate and yet so disabled in other ways. She longed for a friend, for someone else with Williams syndrome with whom she could feel at ease, talk, and make music. “But there are not enough of us,” she said, “so I’m the only Williams in the place.” I had the feeling, as I had had with Anne, that Pamela had acquired a painful wisdom, a larger perspective, with age.
Pamela’s mother mentioned that she liked the Beatles, so I started singing “Yellow Submarine,” and Pamela joined in, bursting into a loud, joyful rendition and smiling broadly. “She comes alive with music,” her mother said. She had a huge repertoire, from Yiddish folk songs to Christmas hymns, and once she had started, there was no stopping her. She sang sensitively, always getting the emotion, and yet— I was surprised by this— she was often off-key, sometimes without any clear tonal center whatever. Charlotte, too, had observed this and had difficulty accompanying Pamela with her guitar. “People with Williams all love music,” she said, “they are all deeply moved by it, but not all of them are geniuses, not all of them are musically talented.”
* * *
WILLIAMS SYNDROME IS very rare, affecting perhaps one child in ten thousand, and it was not formally described in the medical literature until 1961, when J. C. P. Williams, a New Zealand cardiologist, published a paper on it; the following year, it was independently described by Alois J. Beuren and his colleagues in Europe. (In Europe, therefore, people tend to refer to Williams-Beuren syndrome, but in the United States it is usually known as Williams syndrome.) They each described a syndrome characterized by defects of the heart and great vessels, unusual facial conformations, and retardation.
The term “retardation” suggests an overall or global intellectual defect, one that impairs language ability along with all other cognitive powers. But in 1964, G. von Arnim and P. Engel, who noted the elevated calcium levels that seemed to go along with Williams syndrome, also observed a curiously uneven profile of abilities and disabilities. They spoke of the children’s “friendly and loquacious personalities” and “their unusual command of language”— the last thing one would expect to find in a “retarded” child. (They noted too, though only in passing, that these children seemed to have a strong attachment to music.)
Individual parents of these children, similarly, were often struck by the unusual constellation of strengths and intellectual disabilities their children displayed and found it very difficult to find appropriate environments or schooling for them, as they were not “retarded” in the usual sense. In the early 1980s, a group of such parents in California discovered one another and came together to form the nucleus of what would become the Williams Syndrome Association.1
At much the same point, Ursula Bellugi, a cognitive neuroscientist who had pioneered research on deafness and sign language, became fascinated by Williams syndrome. She had met Crystal, a fourteen-year-old girl with Williams syndrome, in 1983 and was intrigued and charmed by her, not least by her ready improvisations of songs and lyrics. Bellugi arranged to see Crystal every week for a year, and this was the start of an enormous enterprise.
Bellugi is a linguist, albeit one as much attuned to the emotional powers of speech, and all the poetic uses of language, as to its formal linguistic character. She was fascinated by the large vocabularies and unusual words that youngsters with Williams syndrome would use, despite their low IQs— words like “canine,” “abort,” “abrasive,” “evacuate,” and “solemn.” When asked to name as many animals as she could, one child’s first responses were “newt, saber-tooth tiger, ibex, antelope.”2 And it was not only a large and unusual vocabulary, but all communicative powers that seemed highly developed in these children, especially in contrast to IQ-matched youngsters with Down’s syndrome. Those with Williams particularly showed a special feeling for narrative. They would use vivid sound effects and other devices to convey feeling and heighten the impact of what they said; Bellugi called these “audience-hookers”— locutions like “All of a sudden,” “Lo and behold!,” and “Guess what happened next?” It became increasingly clear to Bellugi that this narrative skill went with their hypersociability— their longing to connect and bond with others. They were minutely aware of personal details, they seemed to study people’s faces with extraordinary attention, and they showed great sensitivity in reading others’ emotions and moods.
They seemed strangely indifferent, though, to the nonhuman in their environments. Indifferent and inept— in some cases, childre
n with Williams syndrome were unable to tie their shoes, to judge obstacles and steps, to “get” how things were arranged in the house. (This was in striking contrast to autistic children, who might fixate on inanimate objects and seemed indifferent to the emotions of others. In some ways, Williams seemed to be the exact opposite of severe autism.) Some children with Williams syndrome were utterly unable to put simple Lego blocks together— toys that IQ-matched children with Down’s syndrome could easily assemble. And many children with Williams syndrome were unable to draw even a simple geometric shape.
Bellugi showed me how Crystal, despite her IQ of 49, had given a vivid, quirky description of an elephant, but the drawing of an elephant she had done a few minutes earlier bore no resemblance to an elephant or, indeed, to anything at all; none of the features she had painstakingly described had actually made their way into the drawing.3
* * *
OBSERVANT and often puzzled parents, while noting the problems and difficulties of their children, also noted their unusual social attention and friendliness, their reaching out to others. Many were struck by how, even as infants, their children listened with great attention to music and began to reproduce melodies accurately by singing or humming, even before they could speak. Some parents observed that their children would be so completely absorbed in music that they were unable to attend to anything else; other children were extremely sensitive to the emotions expressed in music and might burst into tears at a sad song. Others would play their instruments for hours each day, or might learn songs in three or four other languages, if they liked the melody and beat.
This was very much the case with Gloria Lenhoff, a young woman with Williams syndrome who learned to sing operatic arias in more than thirty languages. In 1988, Bravo, Gloria, a documentary about Gloria’s remarkable musical abilities, was aired on public television. Soon after, her parents, Howard and Sylvia Lenhoff, were surprised to get a phone call from someone who had seen the documentary, saying, in effect, “That was a wonderful film— but why did you not mention that Gloria has Williams syndrome?” The viewer, a parent, had identified Gloria right away from the characteristic facial features and behaviors of Williams syndrome. This was the first the Lenhoffs had heard of the syndrome; their daughter was thirty-three.
Since then, Howard and Sylvia Lenhoff have been instrumental in bringing awareness to the condition. In 2006, they collaborated with the writer Teri Sforza on The Strangest Song, a book about Gloria’s remarkable life. In this, Howard described Gloria’s musical precocity. At the age of one, he said, “Gloria could listen to ‘The Owl and the Pussycat’ and ‘Baa Baa Black Sheep’ over and over again— rhythm and rhyme delighted her.” In her second year, she became able to respond to rhythm.
“When Howard and Sylvia played their records,” Sforza wrote, “Gloria grew excited and focused all at once, pulling herself up in the crib, holding onto the railings, and bouncing up and down…keeping time to the beat.” Howard and Sylvia encouraged Gloria’s passion for rhythm by giving her tambourines, drums, and a xylophone, which she played with to the exclusion of all other toys. By her third year, Gloria could carry and sing a tune, and by her fourth year, Sforza noted, she was “ravenous for language…greedily picked up bits of Yiddish, Polish, Italian, whatever she heard…absorbed them like a sponge, and started to sing little songs in other languages.” She did not know these languages, but she had learned their prosody, their intonations and stresses, by listening to records, and could reproduce them fluently. Already then, at four, there was something extraordinary about Gloria, prefiguring the opera singer she was to become. In 1992, when Gloria was thirty-eight, Howard wrote to me:
My daughter Gloria has a rich soprano voice and can play on the full-sized piano accordion almost any song she hears. She has a repertoire of about 2,000 songs…. Yet, like most individuals with Williams syndrome, she cannot add five plus three, nor can she get along independently.
Early in 1993, I met Gloria and accompanied her on the piano while she sang a few numbers from Turandot, which she performed, as always, with brilliance and an impeccable ear. Gloria, despite her deficits, is a dedicated professional who spends most of her time perfecting and expanding her repertoire. “We know she is ‘retarded,’ ” her father says, “but in comparison to her and others with Williams syndrome, are not most of us ‘retarded’ when it comes to learning and retaining complex music?”
Gloria’s talents are extraordinary, but not unique. At much the same time as her talents were emerging, another unusual youngster, Tim Baley, was showing a similar picture of striking musical abilities and fluent speech, along with severe intellectual impairments in many other respects. His musicality, and the support of his parents and teachers, allowed him, like Gloria, to become a performing musician (in his case, a pianist), and in 1994 Gloria and Tim joined three other musically talented people with Williams syndrome to form the Williams Five. They had their debut in Los Angeles, an event that led to features in the Los Angeles Times and on NPR’s All Things Considered.
While all this delighted Howard Lenhoff, it left him dissatisfied. He was a biochemist, a scientist— and what had science to say about the musical gifts of his daughter and others like her? There had been no scientific attention to the musical passions and talents of people with Williams syndrome. Ursula Bellugi was primarily a linguist, and though she had been struck by the musicality of people with Williams syndrome, she had not made a systematic study of it. Lenhoff pressed her and other researchers to investigate this.
Not all people with Williams syndrome are as musically talented as Gloria— few “normal” people are. But virtually all share her passion for music and are extraordinarily responsive to music on an emotional level. Lenhoff felt, therefore, that there needed to be a proper arena, a musical arena, where people with Williams could meet and interact. He played a crucial role in setting up, in 1994, the camp in Massachusetts, where people with Williams could socialize and make music together, and receive formal training in music. In 1995, Ursula Bellugi went to the camp for a week; she returned the next year, accompanied by Daniel Levitin, a neuroscientist and professional musician. Bellugi and Levitin were thus able to put together and publish the first survey of rhythm in such a musical community, in which they wrote:
People with Williams syndrome…had a good, if implicit, understanding of rhythm and its role in musical grammar and form. It was not only rhythm but all aspects of musical intelligence that seemed to be highly and often precociously developed in people with Williams syndrome.
…We heard many stories about infants (12 months) who could match pitch with a parent playing the piano, or toddlers (24 months) who could sit down at the piano and play back their older siblings’ piano lessons— such anecdotal accounts demand controlled experimental verification, but the similarities among them— and the sheer number of them— lead us to believe that Williams syndrome individuals do have a much higher degree of musical involvement and “musicality” than normals.
That the whole panoply of musical talents could be so strikingly developed in people who were deficient (sometimes severely so) in general intelligence showed, like the isolated powers of musical savants, that one could indeed speak of a specific “musical intelligence,” as Howard Gardner had postulated in his theory of multiple intelligences.
The musical talents of people with Williams syndrome differ from those of musical savants, however, for savant talents often seem to emerge full-blown, to have something of a mechanical quality, to require little reinforcement by learning or practice, and to be largely independent of influence by others. In children with Williams syndrome, by contrast, there is always a strong desire to play music with and for others. This was very clear with several young people I observed, including Meghan, whom I watched during one of her music lessons. She was clearly much attached to her teacher, listened to him carefully, and worked assiduously on suggestions he made.
Such engagement manifests itself in many ways, as Bellu
gi and Levitin found when they visited the music camp:
Williams syndrome individuals had an unusually high degree of engagement with music. Music seemed to be not just a very deep and rich part of their lives, but one that was omnipresent; most of them spent a great proportion of the day singing to themselves or playing instruments, even while walking to the mess hall….When one camper encountered another camper or group of campers involved in a musical activity…the newcomer would either join in immediately or begin swaying appreciatively to the music….This consuming involvement with music is unusual in normal populations….[We have] rarely encountered this type of total immersion even among professional musicians.
The three dispositions which are so heightened in people with Williams syndrome— the musical, the narrative, and the social— seem to go together, distinct yet intimately associated elements of the ardent expressive and communicative drive that is absolutely central in Williams syndrome.
* * *
GIVEN SO EXTRAORDINARY a constellation of cognitive talents and deficits, Bellugi and others started to explore what their cerebral basis might be. Brain imaging, along with, more rarely, autopsy reports, revealed remarkable divergences from the normal. The brains of people with Williams syndrome were, on average, twenty percent smaller than normal brains, and their shape was quite unusual, for the decrease in size and weight seemed to be exclusively at the back of the brain, in the occipital and parietal lobes, whereas the temporal lobes were of normal and sometimes supernormal size. This corresponded with what had been so clear in the uneven cognitive abilities of those with Williams— the devastating impairments of visuospatial sense could be attributed to the underdevelopment of parietal and occipital areas, while the strong auditory, verbal, and musical abilities could, in general terms, be attributed to the large size and rich neuronal networks of the temporal lobes. The primary auditory cortex was larger in people with Williams syndrome, and there seemed to be significant changes in the planum temporale— a structure known to be crucial for the perception of both speech and music, as well as for absolute pitch.4