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Updated: Feb 2, 2026

Author Spotlight: A Battery of Highly Reproducible Behavioral Tests to Validate an Angelman Syndrome Murine Model
Published on: October 20, 2023
Feasibility of using auditory event-related potentials to investigate learning and memory in nonverbal individuals
Alexandra P Key1, Dorita Jones2, Sarika Peters3
1Vanderbilt Kennedy Center for Research on Human Development, Vanderbilt University Medical Center, United States; Department of Hearing and Speech Sciences, Vanderbilt University Medical Center, United States; Department of Psychiatry and Behavioral Sciences, Vanderbilt University Medical Center, United States.
Abstract:
The combination of intellectual, communicative, and motor deficits limit the use of standardized behavioral assessments of cognition in individuals with Angelman syndrome (AS). The current study is the first to objectively evaluate learning and memory in AS using auditory event-related potentials (ERP) during passive exposure to spoken stimuli. Fifteen nonverbal individuals with the deletion subtype of AS (age 4-45 years) completed the auditory incidental memory paradigm. Auditory ERPs were recorded in response to a sequence of unfamiliar nonwords, in which one randomly selected stimulus was repeated multiple times and the rest were presented once. Larger parietal responses within 200-500 ms for the repeated nonword compared to novel distracters were associated with caregiver reports of more adaptive communication skills. These findings demonstrate good tolerability of ERP procedures (94% success rate) and indicate that persons with AS can acquire new information following repeated auditory exposure, even in the absence of explicit memorization instructions. Strong associations between the caregiver reports of adaptive functioning and neural indices of auditory learning and memory support the utility of brain-based measures for objectively evaluating higher-order information processing in nonverbal persons with neurodevelopmental disorders.
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