| |
Book Review
*Published in Volume 117, Issue 5 <http://content.jci.org/117/5>*
/J. Clin. Invest./ *117*(5): 1117-1117 (2007).
doi:10.1172/JCI32009.
Copyright © 2007, The American Society for Clinical
Investigation
The strangest
song: One father’s quest to help his daughter find her
voice
by Sforza Teri, Lenhoff Howard,
Lenhoff Sylvia. Prometheus Books. Amherst,
New York, USA. 2006. $24.00. ISBN: 978-1-59102-478-1
(hardcover).
Review by Christopher L. Karp
Division of
Molecular Immunology, Cincinnati Children’s Hospital
Research Foundation, and University of Cincinnati College
of Medicine,
Cincinnati, Ohio, USA.E-mail:
chris.karp@chmcc.org
The high degree of interindividual
variability in musical abilities and proclivities is not
well understood. Indeed, the genetic, environmental,
developmental, and neurocognitive substrates of musicality
(itself a concept difficult to define operationally) remain
quite obscure. That said, such differences are easily
appreciated, in both senses of the word. On the other hand,
the often obvious differences in cognitive abilities
associated with neurodevelopmental disorders frequently
have pernicious consequences for affected individuals and
their families. In /The strangest song: one father’s quest
to help his daughter find her voice, Teri Sforza tells a
story that melds different worlds of individual experience
and social response — that of an extraordinarily musically
talented, intellectually impaired woman with Williams
syndrome (WS).
WS, a multisystem disorder occurring in about 1 in 7,500
live births, arises from hemizygous deletion of a 1.55-Mb
region containing some 28 genes at chromosome 7q11.23.
Physical manifestations include dysmorphic (“elfin”) facial
features, supravalvular aortic stenosis and other
connective tissue abnormalities, and growth retardation.
Characteristic personality traits include hypersociability
and heightened empathy (generally described as
“delightful”), along with nonsocial anxieties.
Developmental delay and cognitive impairment are the norm,
with low IQ scores and severe deficits in spatial and
arithmetic cognition and in conceptual reasoning. However,
WS appears to be associated with relative preservation of
abilities in language, face processing, sociability, and
music.
This mix of cognitive abilities and disabilities has proven
irresistible as a human model for examining the links among
genes, neurodevelopment, cognition, and behavior. Defining
the genetic substrates of WS-associated neurodevelopmental
abnormalities has not been easy, in part because of the
stereotypical nature of the responsible deletion. Study of
atypical deletions as well as mouse models has, however,
implicated several genes in various aspects of the
neurocognitive and craniofacial phenotype, including
/GTF2IRD1/ (a /TFII-I/ transcription factor), /CYLN2/ (cytoplasmic
linker protein 2),/ LIMK1 /(LIM kinase 1), and /FZD9
/(frizzled 9). The fragmented nature of the WS cognitive
phenotype — in particular, the lack of apparent
difficulties with language despite evident severe
impairment in other cognitive domains — has also provided
considerable experimental grist for the mill of
neuropsychological theory.
But WS is something experienced by individuals and those
who care for and about them. To echo neurologist Oliver
Sacks, WS is a “what” that happens to a “who.” And whatever
the genetic substrates, the social environment is critical
to how WS and other developmental disorders are
experienced. These points are illuminated in a compelling
way in /The strangest song/, an account of Gloria Lenhoff
and her parents, written by Sforza with Gloria’s parents.
Born before the syndrome was first described, Gloria had
what was, in retrospect, a typically difficult childhood
for someone with WS. Her likely future was one defined in
large part by limitation of possibility and hope. However,
Gloria’s parents’ recognition of, and delight in, her
musical affinity and gifts, along with their single-minded
pursuit of ways to develop and provide performing outlets
for her musical talents, has led to what clearly seems to
be a remarkably fulfilling life. Despite her varied
intellectual limitations, Gloria turned into an
accomplished lyric soprano (and accordionist) with a vast
repertoire of songs in a profusion of languages who has
enriched a broad public and herself with her gifts.
Of note, Gloria’s father’s activism was itself instrumental
in spurring recognition and study of the WS musical
phenotype. Compared with normal individuals, those
with WS tend to exhibit more engagement with music — more
emotion while listening to music, more time spent on
musical activities, and onset of interest in music at a
younger age. They also have a greater incidence of auditory
anomalies, including hyperacusis, odynacusis, phonophobia,
and auditory fascinations. Using functional MRI imaging,
Levitin et al. have described possible neural correlates of
this unusual auditory and musical sensitivity. Of course,
as in the population at large, the actual musical abilities
of individuals with WS (like their other cognitive
abilities) vary widely. However, music appears to provide a
uniquely important avenue for cognitive development and
emotional fulfillment for many people with WS. The ongoing
struggle by Gloria’s father and others to provide
opportunities for such development and fulfillment drives
much of the narrative of this book.
Sforza, neither professional scientist nor professional
musician, has written an entertaining book for a general
audience. Those looking for a state-of-the-art review of
the science of WS will not find it here. Indeed, scientists
will wince at the casual misinformation (e.g., Watson and
Crick did not /discover/ DNA). Similarly, musicians will
stumble over occasional lapses in tone. But these are minor
quibbles. Sforza beautifully limns the key role played by
the social environment in the individual experience of
genetic developmental disability, careful attention to
which has the potential for helping people transcend
biological and social categories in their own unique ways.
Webmaster's Note: Viewers of this web page are probably
familiar of the activism of Howard Lenhoff on behalf of the
music abilities of people with Williams syndrome, activism
which had a major effect on the starting of the WS Music
Camp at Belvoir Terrace, and in the establishment of the
Berkshire Hills Music Academy. These are described by
author Teri Sforza in the book The Strangest Song.
You also might want to learn where Dr. Lenhoff's activism
started. That story has recently been published in the book
described in the following review.
Gary Rubin, Webmaster
Book Review
BLACK JEWS, JEWS AND OTHER
HEROES
By Howard
M. Lenhoff
Gefen
Publishing House, Jerusalem, New York
Review By: CHRIS LEPPEK
It has been more than a decade since the American
Association for Ethiopian Jews did something virtually
unheard of in the annals of organized American Jewry – it
disbanded itself, declaring its primary goal successfully
reached.
Today, the whole struggle to save Ethiopian Jewry from the
civil war that was racking its homeland tends to be
remembered in the warm and comfortable glow of satisfied
hindsight. And understandably so -- it was a titanic
achievement, a proud milestone in the history of Israel
and, even more so, American Jewry. The dramatic sagas with
Biblical names -- Operations Moses, Solomon and Elijah –
were iconic victories of perseverance over apathy, human
rights over oppression, tolerance over racism.
All undeniably true, but the amber glow of memory also
allows us to forget just how difficult and controversial
those battles really were. Thankfully, for the sake of
historical honesty, the tumultuous dimensions of the story
are provided by Howard M. Lenhoff, one of three people to
serve as president of the AAEJ during its brief but busy
existence. The years of Lenhoff’s tenure, 1978-82, were
among the most eventful in the two-decades-plus effort to
bring tens of thousands of Ethiopian Jews to Israel.
The most obvious drama of the Ethiopian exodus was the
complex process of physically getting the Jews out of
Ethiopia as a fierce civil war raged around them. Indeed,
the largely covert and tautly suspenseful Operation
Solomon, in which some 14,000 Ethiopian Jews were rescued
via airlift in one day, rivals any chapter in Israeli
history for sheer human drama.
But Lenhoff’s book also pays considerable attention to the
abundant drama and trouble that bubbled beneath that
photogenic surface. First was the debate whether Ethiopian
Jews were Jews at all, and thus whether they were entitled
to Israeli citizenship under the Law of Return.
That was followed by an even more intense debate over the
best way to get them to Israel, an argument that largely
mirrored that surrounding another, and somewhat
contemporaneous, immigration issue – that of Soviet Jewry.
Should the task be undertaken with the resources and
methods of the Jewish establishment, i.e., the federation
and UJA system, or through the efforts of grassroots
activist groups which sprung up at the periphery, and
largely independent, of that establishment?
The AAEJ, like the Union of Councils for Soviet Jewry, was
firmly of the peripheral grassroots persuasion, and it
waged a persistent, difficult and often discouraging battle
of strategy and control over the complicated emigration
process. That it ultimately won that battle and found
vindication in the humanitarian results is largely taken
for granted today, but this takes nothing away from the
inherent interest of the story that the author tells.
Characteristically, Lenhoff – who recently completed
another compelling book, one about his daughter’s struggle
with a genetic disorder – writes this story clearly, fairly
and with passion. Although written from an openly partisan
perspective, Lenhoff’s account provides a compelling
history of a fascinating and important chapter in modern
Jewish history.
Importantly, however, Lenhoff is also wise enough to
realize that the story of Ethiopian Jewry is not strictly
historical; that it remains a current, dynamic and, to a
large degree, still troubling phenomenon. Black Jews,
Jews, and Other Heroes doesn’t shy away from discussing
the daunting set of problems Ethiopian immigrants still
face in being fully absorbed into Israeli society.
After explaining why the AAEJ self-dissolved in 1993,
Lenhoff concludes his preface on this ironic note: “Did we
quit too soon?”
|
View
Our Guestbook