Review of Williams Syndrome across Languages
Date: Sat, 18 Jun 2005 20:31:46 -0700 (PDT)
From: Alona Soschen
Subject: Williams Syndrome across Languages
EDITORS: Bartke, Susanne; Siegmüller, Julia
TITLE: Williams Syndrome across Languages
SERIES: Language Acquisition and Language Disorders 36
PUBLISHER: John Benjamins
Alona Soschen, Departments of Linguistics and Philosophy, and Brain
and Cognitive Science, Massachusetts Institute of Technology
This book focuses on the cross-linguistic aspects of language
development and language competence in Williams syndrome (WS).
Emphasis is given to the relationship between language and cognitive
abilities. Due to a deletion in chromosome band 7q11.23, subjects
with WS have neuro-developmental deficits, including deficits in global
processing. People with WS are usually characterized by hyper-
sociability and heightened affect. Language skills are relatively
sparse, along with some non-verbal skills such as facial recognition.
The book consists of a series of 14 articles, collected and
concentrated on the current interdisciplinary research on WS. It
begins with introduction of the phenotype of WS individuals.
Siegmüller and Bartke (Williams syndrome from a clinical perspective)
describe in detail the physiological, social, cognitive, and neuro-
physiological characteristics of WS. An essential part of the discussion
of the link between phenotype and genotype, and review of the
current research on brain characteristics in WS is discussed. This
chapter is an excellent introduction and summary of what is known
about WS to date. It reviews the clinical aspects of WS as described in
the 60's, up to current methods of diagnosis.
Grzeschik (Genetics of Williams-Beuren syndrome) provides insights
into the molecular genetic basis of the syndrome. Grzeschik
concentrates on the deletion of particular mega bases from
chromosome 7q11.23 as a cause for individual WS-symptoms, and
supplies information concerning the effects of this deletion on genes.
Grzeschik reviews the recent research on the WS-homologous
genomic region in the mouse, and concludes that further studies with
animal models for WS addressing other candidate genes are required
to help in human gene mapping.
Mervis, Robinson, Rowe, Becerra, and Klein-Tasman (Relations
between language and cognition in Williams syndrome) address the
relationship of general control processes to the intellectual abilities of
people with WS. Correlational analyses are used to consider the
relations between language and cognition in school children and
adults. The discussion of the initial stage of language acquisition leads
to the conclusion that in general, it proceeds in the same manner as in
TD (typically developing) individuals. However, WS individuals rely
more on verbal short-term memory than their TD peers.
The idea that language and cognition cannot be regarded separately
is supported in the Schaner-Wolles' article Spared domain-specific
cognitive capacities, syntax and morphology in Williams and Down
syndromes. This article compares an unimpaired development to WS
and to DS language acquisition, and offers the answer to a question
whether this development is guided by domain-specific or domain-
general cognitive capacities. The study is based on one child with WS
and 82 children with DS. It is concluded that in both, certain interactive
subsystems involved in language processing are spared - an
argument in favor of their modular character. The link between
language and cognition is established by showing that the linguistic
system can be deprived of the necessary support for language
acquisition because of syndrome-specific cognitive limitations.
Phonological processing in Williams syndrome by Steve Majerus
concentrates on the effect of complex phonological representations in
French-speaking children with WS. He argues that although basic
phonological processes such as identification, segmentation, short-
term storage and articulation of phonological information appear to be
at a regular level, this performance is achieved atypically, in the sense
that phonological representations in WS are more specific than in
typically developing children.
Böhning, Starke and Weissenborn in their article Fast mapping in
Williams syndrome examine the data of vocabulary acquisition, in
German, by one child with WS, and four groups of ND controls
matched for chronological, lexical, and syntactic age. They argue that
the acoustic capacities are different in WS due to over-detailed
phonological memory representations, while the mechanisms of
linguistic performance are the same as in TD controls.
Volterra, Capirci, and Vicari (Language in preschool Italian children
with Williams and Down syndromes) present the studies of
communicative and linguistic abilities in Italian speaking children with
DS and with WS, and compare the results of more recent
developments in the investigation of early language acquisition for
these two syndromes. The authors discuss the development of
morphology, syntax, and vocabulary. They confirm that although a
spared ability of children with WS to hear and store speech sounds
accounts for a relatively intact language capacity - in contrast with
what is found in DS - children with WS do not speak like their TD
peers. The differences between WS and DS language acquisition and
TD language development are viewed as related to the general
cognitive impairment rather than to a specific syndrome.
Lucács, Pléh, and Racsmány's article Language in Hungarian children
with Williams syndrome addresses the issue of the WS mental lexicon
and grammar. The authors present four studies that show in WS
patients language lags behind verbal age, while organization does
not. However, the results are not taken as evidence that language in
WS corresponds to that in TD; there is a need for more fine-grained
studies of representation, processing, and language acquisition.
Clahsen, Ring, and Temple (Lexical and morphological skills in
English-speaking children with Williams syndrome) confirm that in WS
the rules-based system for language is selectively spared, while the
access to lexical representations is impaired. This modular approach
is supported by the results of the study of morphology (particularly
past-tense formation) and comparative adjective formation, as well as
lexical skills, in WS children and closely matched typically developing
Regular and irregular inflectional morphology in German Williams
syndrome by Penke and Krause presents data from German to
provide an explanation for the selective deficit with irregular inflected
forms. According to Penke and Krause, WS subjects have difficulty
accessing the sub nodes where irregular inflected forms are stored.
This accounts for both the selective deficit with irregular forms and the
over regularization with regular affixes to irregular verbs and nouns.
The data also suggests that the language system is affected by other
deficits as well.
In her study of Hebrew speaking toddlers (Emergent linguistic
competence in children with Williams syndrome), Yonata Levy
explores the development of grammar in WS children. She suggests
that because the error rates in acquisition of complex clauses as well
as percent of usage of noun complement construction were within the
range of TD controls, the developmental pattern in WS should not be
interpreted as atypical.
Stavrakaki (Wh-questions in Greek children with Williams syndrome)
investigates the performance of Greek children with WS in the
production of referential and non-referential wh-questions. The study
compares the results to the performance of SLI (specifically language
impaired) and typically developing children. The results show that both
the WS and TD subjects have a high level of performance, while the
SLI subjects have severe deficits with wh-question formation.
Siegmüller and Weissenborn (The comprehension of complex wh-
questions in German -speaking individuals with WS) tackle the
question whether WS-individuals can achieve adult-like grammatical
competence. Siegmüller and Weissenborn found that WS competence
corresponds to the stages of unimpaired linguistic development. They
also state that German-speaking individuals with WS have difficulties
with adjunct-questions as compared to the controls. They conclude
that the WS-individuals' learning mechanisms are more sensitive to
predictable patterns in the input, such as formed by the verb and its
Bartke (Passives in German children with WS) reports findings in
passive acquisition, which show that WS children are able to analyze
syntactic regularities and use linguistic rules. She concludes that
language development in WS may be interpreted in terms of a delay
rather than a deviation from the norm.
In this review I have provided a brief summary of each article of the
volume. Most of the papers are well organized and very readable,
ending with a short summary convincingly illustrating the main
argument of the article. The experiments are described in a very
accessible way. There are almost no unnecessary technical terms
and/or tables. However, Grzeschik's article abounds in narrowly
specialized terminology relevant to the studies of genetics and
requires a glossary.
As an all-embracing review of work on language acquisition in Williams
syndrome, this book brings together the results of experiments in
seven different languages with participation of various age groups.
This represents two of the major strengths of this anthology. It also
compares language acquisition in WS and Down syndrome, and
draws important conclusions.
A valuable contribution of this book to the field of science is its
unbiased theoretical approach that allowing the authors to express a
variety of viewpoints. This collection supports the idea that adherence
to modularity hypothesis should not prevent researchers from
investigating language as a part of a more general cognitive system
which can be deprived of the necessary support for language
acquisition in WS. This work is an important step forward in the
development of interdisciplinary approaches to the study of language
development and of the human mind in general.
| ABOUT THE REVIEWER:
ABOUT THE REVIEWER
Alona Soschen is a postdoctoral researcher at the Department of
Linguistics and Philosophy and the Department of Cognitive Science,
MIT, a leading center for research on formal models of human
language and the interrelations between linguistics, psychology,
philosophy, and mathematics. Her interests include language
acquisition, studies of concept formation, syntactic theories, and
comparative syntax (Slavic, Germanic, Romance, Semitic). Her
present work focuses on the Minimalist program, and combines a
cognitively based approach to minimal syntactic representations and
acquisition of syntax with the analyses implemented by means of