The Implications of Williams Syndrome
April 7, 2009 at 5:04 pm | In Extra Stuff, Language Disability | 1 CommentTags: annette karmiloff-smith, language, SLI, specific language impairment, steven pinker, williams syndrome
Williams Syndrome is a rare genetic disorder, first identified in 1961, that is characterized by, among other things, outgoing personalities and deficits in processing and adaptive behavior skils. These individuals frequently have comparatively low IQs with comparatively high language skills. While initially the facts of the preceding sentence were eagerly seized by proponents of the separation of intelligence and language, the accumulation of research has (as it so often does) muddied the picture. Williams Syndrome and Specific Language Impairment (SLI) have frequently been used as converse examples of evidence to support the dissociation between cognition and language. The cognitive scientist, Steven Pinker, has made this argument in several popular books. In 1999 he wrote,
Overall, the genetic double dissociation is striking, suggesting that language is both a specialisation of the brain and that it depends on generative rules that are visible in the ability to compute regular forms. The genes of one group of children [SLI] impair their grammar while sparing their intelligence; the genes of another group of children [WS] impair their intelligence while sparing their grammar.
Subsequent assertions by Pinker leaning more toward an inextricable relationship between genes and environment seem not to have been as widely read as his earlier work.
Comparisons of SLI and Williams Syndrome hinge on the notion that SLI is inherited. While the research does suggest that at least a predisposition toward SLI is inherited, the complexity of its causes makes any comparisons like that of apples and oranges. The exact cause of Williams Syndrome is known: it is the result of missing genetic material on chromosome seven. SLI is likely the result of a stew of ingredients, with varied recipes, and varied results. Individuals with Williams Syndrome are gregarious. They enjoy talking. Is it any wonder that they may become relatively proficient at something they enjoy? As with other human behaviors and skills, language acquisition will likely never be reduced to one cause. Similarly, the fact that these individuals are poor puzzle solvers is more likely related to visual-spacial deficits than an impairment in some “puzzle solving” gene.
People with Williams Syndrome are often outgoing...and smiling.
Anette Karmiloff-Smith has done a lot of great work on Williams Syndrome, much with a focus on accurately describing its characteristic language skills and deficits. Many of her publications are available for download on her personal web site. Language log has published an interesting post on the science and state of language research in Williams Syndrome, found here: Language Log link.
Language Deficits of Mental Retardation and TBI
November 30, 2008 at 4:49 pm | In Language Disability | Leave a CommentTags: language deficits, M.R., mental retardation, TBI, traumatic brain injury
Mental Retardation
- distractibility and a short attention span
- semantic difficulties, with small, more concrete vocabularies
- comprehension superior to expression
- poor morphology
- telegraphic speech
- passive interaction, or physically aggressive interaction
- delays across multiple domains
Traumatic Brain Injury (TBI)
- comprehension problems, especially of sentences
- word-retrieval problems leading to reduced fluency
- syntactic problems, including limited MLU, fewer utterances, and difficulty expressing and understanding long, complex sentences
- reading and writing problems; poor academic performance
- pragmatic problems such as difficulty with turn taking and topic maintenance (often related to poor inhibition and lack of self-monitoring)
- difficulty with attention and focus
- memory problems
- inability to recognize one’s own difficulties
- reduced speed of information processing
- difficulties with reasoning and organization
(from An Advanced Review of Speech-Language Pathology, Celeste Roseberry-McKibben and M.N. Hedge; ProEd; 2000.)
Language Deficits of ADHD and Hearing Impairment
November 14, 2008 at 11:53 am | In Language Disability | 1 CommentTags: adhd, attention deficit disorder, hearing impairment, language deficits, language disabilities
Attention Deficit Hyperactivity Disorder
- often blurting out answers to questions before the questions have been completed
- difficulty following through on instructions; often do not seem to be listening
- talking excessively
- interrupting or intruding on others and poor turn-taking skills
- frequent false starts because they change their minds while structuring a response
- excessive number of fillers and pauses because verbal expression occurs with minimal preplanning
- difficulty describing things in an organized, coherent manner – general difficulty with expressive language organization
- do not tell stories or use narrative skills effectively
- difficulty with social entry into conversations
- use inappropriate register; for example, use the same interactive style with adults and peers
- do not perceive or act appropriately upon interlocutors’ nonverbal cues
- do not use comprehension monitoring strategies
Hearing Impairment
- use of a limited variety of sentence types
- use of sentences of reduced length and complexity
- difficulty comprehending and producing compound, complex, and embedded sentences
- occasional irrelevance of speech, including non sequiturs
- limited oral communication, including lack of elaborated speech and reluctance to speak
- difficulty understanding proverbs, metaphors, and other abstract utterances
- slower acquisition of gramamtic morphemes
- omission or inconsistent use of many morphemes including past tense and plural inflections, third-person singular -s, indefinite pronouns, present progressive -ing, articles, prepositions, and conjunctions
- poor reading comprehension
- writing that reflects oral language problems (e.g., deviant syntax, limited variety of sentence types, omission of grammatic morphemes)
(from An Advanced Review of Speech-Language Pathology, Celeste Roseberry-McKibben and M.N. Hedge; ProEd; 2000.)
Recent Research
November 12, 2008 at 11:15 pm | In Language Disability, Recent Research | Leave a CommentTags: diabetes, Ginette Dionne, language impairment, research
Pregnancy diabetes doubles the risk of language impairment in study
This research, gleaned from this link from COMD news was led by Professor Ginette Dionne of Canada’s Universite Laval. Details have been published in the journal Pediatrics. Their results showed that children born to mothers with gestational diabetes achieved lower scores on tests of grammar and vocabulary than individuals in control groups. This difference is not inevitable, however, as children from more educated mothers are much less affected. Risk factors of gestational diabetes include the mother’s age and weight.
What Happens When We Learn – And When We Don’t
September 17, 2008 at 11:13 am | In Language Acquisition, Language Disability | Leave a CommentTags: Language Acquisition, Language Development, language difficulty, learning language
A child with a deficit in a skill typically has not discovered the power of that skill. Thus remains the initial opening for novelty. I believe that children are often more open to suggestion than we often give them credit for. In other words, initially discussing the benefits of a skill can be an extremely effective introduction to the teaching of a skill. However, because complex language is not yet a favored method of input for children in language therapy, these explanations can be brief. Why are working on verbs? Because every sentence has them, and with them you can talk about what anything does. Why practice comparatives and superlatives? Because with them we can greatly increase our powers to describe. And it always helps to relate these introductions in personal ways. Statements such as, “With superlatives you can tell me that you are a faster runner than your brother.” tend to work well.
Continue reading What Happens When We Learn – And When We Don’t…
A Few Facts About… SLI (Specific Language Impairment)
August 11, 2008 at 11:47 am | In A Few Facts About..., Language Disability | Leave a CommentTags: SLI Specific Language Impairment research few facts
SLI, the common abbreviation of Specific Language Impairment, is usually defined as a language impairment of unknown etiology in the presence of normal cognition. In layman’s terms, these are kids with a language problem and no one knows why. Some (IMO) interesting tidbits are:
- SLI occurs in about 7% of the general population (Tomblin et al, 1997)
- It is more prevalent in males than in females (Flax et al, 2003)
- It is widely acknowledged that individuals with SLI commonly experience learning difficulties of a comparable magnitude across all domains, including mathematics (Arvedson, 2002; Donlan and Gourlay, 1999; Fazio, 1996)
- “ “… and literacy (Bishop and Adams, 1990; Catts, Fey, Tomblin, and Zhang; Flax et al 2003)
SLI seems to be a term more prevalant in the speech pathology community than elsewhere. Because I like to interject my opinion occasionally, I’ll do that here, at the end of this post. There are many possible causes of SLI, including environmental, motivational, and perhaps, genetic.
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