Abstract

Abstract 1 Dana Boatman, 1,2 William Trescher, 3 Cynthia Smith, 1 Jenna Los, 1 Qian Gao, 2 Joshua Ewen, and 1 Eileen Vining ( 1 Department of Neurology, Johns Hopkins School of Medicine, Baltimore, MD ; 2 Kennedy Krieger Institute, Baltimore, MD ; and 3 Mt. Washington Pediatric Hosptial, Baltimore, MD ) Rationale: The purpose of this study was to investigate speech recognition abilities in children with Benign Epilepsy with Central-Temporal Spikes (BECTS). Accurate decoding of spoken speech under different listening conditions is critical for normal language development and verbal communication. Although cognitive and language functions are generally considered normal in BECTS, recent studies have revealed subtle abnormalities. Based on potential involvement of temporal lobe auditory areas, we hypothesized that speech recognition under adverse listening conditions (e.g., background noise) is impaired in BECTS. To test this hypothesis, we combined behavioral and objective measures of speech recognition in a within-subject, repeated-measures design. Methods: Five children, ages 7–11, were tested. All had normal hearing and intelligence (FSIQ ≥ 97), and no speech-language delays. Criteria for the diagnosis of BECTS included EEG sharp activity greater than 100 microvolts with a central-temporal distribution and no focal slowing. Standardized behavioral tests were administered, under headphones, to assess word recognition under good and poor listening conditions. Continuous 26-channel EEG recordings were obtained during behavioral testing. Cortical auditory evoked potentials were recorded separately using speech and tones in a passive oddball paradigm. Subjects underwent repeat testing at 1-month intervals for a total of 2–3 sessions, with no medication changes between sessions. Matched normal controls provided comparative behavioral test-retest data. Results: All BECTS subjects demonstrated excellent word recognition in quiet (≥ 92%) with no changes between sessions (p ≥ 0.12, Fisher exact test). Conversely, 4/5 subjects demonstrated impaired word recognition under adverse listening conditions (background noise, filtered speech) in one or more sessions based on age norms (scaled scores 10 ± 3). Unilateral or bilateral central-temporal sharp waves were present during all but one session when word recognition was impaired. One subject had normal behavioral performance across 3 sessions and no EEG abnormalities. Cortical auditory response latencies for speech showed borderline significant increases when word recognition was impaired (p ≤ 0.057, t-test). Normal controls demonstrated stable word recognition across sessions, regardless of listening conditions (p ≥ 0.49, Fisher exact tests). Conclusions: Results indicate that children with BECTS experience transient speech recognition impairments under everyday listening conditions and associated central-temporal sharp waves. Cortical auditory evoked responses ruled out attention or test-retest familiarity effects. The prevalence of adverse listening conditions in classrooms underscores the potential impact of speech recognition impairments in children with BECTS.

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