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Background <p>The Observer-Reported Communication Ability (ORCA) measure was developed to assess communication in individuals with Angelman syndrome (AS), a rare neurodevelopmental disorder characterized by severely impaired communication, global developmental delay, sleep disturbances, seizures, and motor impairments. Our goal was to evaluate the construct validity of the ORCA measure in AS using longitudinal data and comparisons with other communication measures.</p> Methods <p>Caregivers of individuals with AS enrolled in a multisite natural history study completed the ORCA measure alongside at least one of three other instruments: the Vineland Adaptive Behavior Scales, 3rd Edition (Vineland-3), Bayley Scales of Infant and Toddler Development, 4th Edition (Bayley-4), or Communication Matrix (CM). ORCA Emerging &amp; Mastery (E&amp;M) T-scores for expressive, receptive, and pragmatic communication were compared with other measures. ORCA change scores over time were examined by age group and genotype.</p> Results <p>Individuals with AS (<i>n</i> = 192) had a mean age of 12.1 years (range 0.5 to 50.6 years), with 54.7% male. Genotypes included deletions (53.6%), <i>UBE3A</i> mutations (25.5%), and uniparental disomy/imprinting defects (16.1%). ORCA Overall, Expressive, and Receptive T-scores had strong to very strong correlations with Vineland-3, Bayley-4, and CM domains (<i>r</i> = 0.64–0.71). Correlations of the ORCA Pragmatic T-scores showed strong correlations with all measures. ORCA T-score changes were most pronounced in children under 2.</p> Discussion and Conclusion <p>The evidence for the construct validity of the ORCA measure supports its use as a research tool for individuals with AS.</p>

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Association of the Observer-Reported Communication Ability (ORCA) Measure with Established Communication Measures: Insights From the Angelman Syndrome Natural History Study

  • 期刊论文

  • 发表日期：

    2026年1月23日

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  • Abigail Rader,
  • Li Lin,
  • Harrison N. Jones,
  • Wen-Hann Tan,
  • Anjali Sadhwani,
  • Allyson Berent,
  • Batsheva Friedman,
  • Nicole Lucas,
  • Kelly Gordon,
  • Bryce B. Reeve

摘要

Background

The Observer-Reported Communication Ability (ORCA) measure was developed to assess communication in individuals with Angelman syndrome (AS), a rare neurodevelopmental disorder characterized by severely impaired communication, global developmental delay, sleep disturbances, seizures, and motor impairments. Our goal was to evaluate the construct validity of the ORCA measure in AS using longitudinal data and comparisons with other communication measures.

Methods

Caregivers of individuals with AS enrolled in a multisite natural history study completed the ORCA measure alongside at least one of three other instruments: the Vineland Adaptive Behavior Scales, 3rd Edition (Vineland-3), Bayley Scales of Infant and Toddler Development, 4th Edition (Bayley-4), or Communication Matrix (CM). ORCA Emerging & Mastery (E&M) T-scores for expressive, receptive, and pragmatic communication were compared with other measures. ORCA change scores over time were examined by age group and genotype.

Results

Individuals with AS (n = 192) had a mean age of 12.1 years (range 0.5 to 50.6 years), with 54.7% male. Genotypes included deletions (53.6%), UBE3A mutations (25.5%), and uniparental disomy/imprinting defects (16.1%). ORCA Overall, Expressive, and Receptive T-scores had strong to very strong correlations with Vineland-3, Bayley-4, and CM domains (r = 0.64–0.71). Correlations of the ORCA Pragmatic T-scores showed strong correlations with all measures. ORCA T-score changes were most pronounced in children under 2.

Discussion and Conclusion

The evidence for the construct validity of the ORCA measure supports its use as a research tool for individuals with AS.