Unique profile of academic learning difficulties in Wiedemann-Steiner syndrome.
R Ng, H T Bjornsson, J A Fahrner and 1 others
PMID 36437529WHAT IT FOUND
Parents reported significantly greater difficulties in math and spatial skills than in reading for children with Wiedemann-Steiner syndrome.
Reading problems were variable, but math and spatial deficits were consistent across the group, suggesting a specific learning profile.
Key findings
01Math and spatial composites were significantly more elevated than reading composites, indicating greater difficulties in these areas.
02Relative to a community sample, math and spatial scores were about 2.5 and 3 standard deviations below the mean, while reading scores were about 1.3 standard deviations below.
03The pattern of greater math and spatial difficulties compared to reading persisted regardless of intellectual disability status or mutation type.
STILL TO COME
How it was doneWhat they foundWhat it means for SLPs
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What it does not show
The sample size was small (25 participants) and data were not normally distributed. The study relied entirely on parent report via a screening measure, without objective academic testing or teacher input. The remote survey method may have biased the sample toward families with greater financial resources and access to technology. The wide age range (5 to 33 years) may have affected the relevance of academic questions for the youngest and oldest participants. The comparison was made to a general community sample, not to a matched control group or other specific neurodevelopmental disorders.
Declared interests
Dr. Bjornsson is a consultant for Mahzi Therapeutics. Dr. Harris receives research funding from Oryzon Genomics.
The easy way to misread this
Do not interpret the variability in reading scores as evidence that reading is unaffected. The reading composite was still approximately 1.3 standard deviations below the community mean, indicating a significant risk for learning challenges in this area, even if less severe than the deficits in math and spatial skills.