Music, the brain and Williams syndrome: rare disorder offers insight into the genetic basis of cognition (focus on Genes and Genomics)

1. Reference
Music, the brain and Williams syndrome: rare disorder offers insight into the genetic basis of cognition (focus on Genes and Genomics)
By Brendan A. Maher
The Scientist
November 26, 2001
For Dr. Lee Bartel – Music and the Brain 2122H
A Summary, Review and Response
Lani Sommers

2. Summary
Williams syndrome is a neurodevelopmental disorder caused by a deletion of about 20 genes of chromosome 7. People with Williams syndrome have characteristics such as pixie-like features, upturned nose, small chin, protrusive ears, stunted growth, heart problems, poor visuospatial cognition, sensitivity to loud noise, gregarious personalities and an average IQ of about 60.

Almost all patients with Williams Syndrome have an extraordinary connection with music. Even though people with Williams Syndrome have short-lived attention spans they will spend hours listening to or making music. There is some research which illustrates a high occurrence of perfect pitch and excellent rhythmic ability among this group of people. For example, one boy with the syndrome was able to tap a 7/4 rhythm with one hand while keeping 4/4 time with the other.

Audrey Don, a neuropsychologist at the Good Samaritan Hospital in Seattle, was one of the first researchers to explore the relationship patients with Williams Syndrome have with music. She administered several musical tests of tones and beats to people with Williams syndrome and found that “musical ability matches verbal ability and was higher than the Williams’ children overall cognitive abilities.” She also found that the patients had very strong emotional connections to music.

Other research done with patients with Williams Syndrome showed that these children did as well as normal musically trained students, however, they were able to offer “creative completion” to some of the test rhythms.

Further to this research, Howard M. Lenhoff, professor emeritus, School of Biological Science, University of California, completed a study linking Williams syndrome to a high occurrence of perfect pitch. This condition normally occurs in one out of 10,000 people in Western populations. In the case of patient’s with William’s syndrome the incidence rises to 1 out of 1000.

Usually, a person has to study music before the age of six in order to develop perfect pitch. Many of the Williams Syndrome subjects started studying music much after this critical period yet have still been able to develop perfect pitch.

In relation to brain studies, research has shown that in patient’s with Williams Syndrome the neocerebellum seems to be connected to their phonological working memory, a kind of “short-term memory for sounds.”

Music for patients with Williams Syndrome seems to be what helps them to have a sense of normalcy and fulfillment in life. There is much to be learned from the connection these children have with music and its affects on their brain function and development.

3. Reflection

The article, “Music, the brain and Williams syndrome: rare disorder offers insight into the genetic basis of cognition,” by Brendan A. Maher was interesting; however, it could have been more informative and specific when making reference to the brain. I felt that all of the ideas about Williams Syndrome, Music and Brain Development were superficially connected and more detail was required. That being said, this article was an effective introduction to the description of Williams Syndrome and the occurrence of music proficiency, including the incidence of perfect pitch in children suffering from the syndrome.

The description of Gloria Lenhoff, the 46 year-old lyric soprano singer who can sing nearly 2500 songs in more than 25 languages in a perfect accent was very effective in setting the tone for the article as an introduction to the disease. It is absolutely amazing that a person can be so incredibly skilful in one aspect of their life can be so deficient in another because of a chromosome deficiency. The fact that 1 in 1000 patients with Williams Syndrome have perfect pitch is an incredible statistic; however, Maher fails to delve into the details of this matter adequately or effectively. He touches on a few musical research projects and studies using MRI to examine patients with Williams Syndrome but the results of these studies seem to be superficial and inconclusive in the context of his paper. The title of the article would indicate that the reader is going to find some hardcore data about Music, the Brain and Williams syndrome, but instead, the reader finds very little concrete evidence about the three topics. Maher gives excellent background information about the syndrome and the affinity the people suffering from the syndrome have for music however; this article is better suited as simply an introduction to the syndrome, rather than a study in regards to music and the brain.