ArticleNeurobiology of disease2015
Degraded neural and behavioral processing of speech sounds in a rat model of Rett syndrome.
Article in Neurobiology of disease, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers, 1 of them a synthesis that pooled it.
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Who cites it
22 citing papers in PubMed, 1 synthesis or guideline pooled it, 63 citations in OpenAlex.
- Auditory processing in rodent models of autism: a systematic review.Journal of neurodevelopmental disorders · 2022Pooled it
- Feasibility of event-related potential (ERP) biomarker use to study effects of mother's voice exposure on speech sound differentiation of preterm infants.Developmental neuropsychology · 2018Trial
- Vagus Nerve Stimulation Paired With Tones Alters the Auditory Cortex Proteome in a Rat Model of Rett Syndrome.Developmental neurobiology · 2026Article
- Use of a predictor cue during a speech sound discrimination task in a Cntnap2 knockout rat model of autism.PloS one · 2025Article
- Article
- Degraded inferior colliculus responses to complex sounds in prenatally exposed VPA rats.Journal of neurodevelopmental disorders · 2024Article
- Effect of presentation rate on auditory processing in Rett syndrome: event-related potential study.Molecular autism · 2023Article
- Circuit-level theories for sensory dysfunction in autism: convergence across mouse models.Frontiers in neurology · 2023Review
- Collapse of complexity of brain and body activity due to excessive inhibition and MeCP2 disruption.Proceedings of the National Academy of Sciences of the United States of America · 2021Article
- Advances in Genome Editing and Application to the Generation of Genetically Modified Rat Models.Frontiers in genetics · 2021Review
- Review
- Deficits in skilled motor and auditory learning in a rat model of Rett syndrome.Journal of neurodevelopmental disorders · 2020Article
- Atypical processing of tones and phonemes in Rett Syndrome as biomarkers of disease progression.Translational psychiatry · 2020Article
- Evoked Potentials and EEG Analysis in Rett Syndrome and Related Developmental Encephalopathies: Towards a Biomarker for Translational Research.Frontiers in integrative neuroscience · 2020Article
- Shank3-deficient rats exhibit degraded cortical responses to sound.Autism research : official journal of the International Society for Autism Research · 2018Article
- Individual Differences in Human Auditory Processing: Insights From Single-Trial Auditory Midbrain Activity in an Animal Model.Cerebral cortex (New York, N.Y. : 1991) · 2017Article
- Lessons learned from studying syndromic autism spectrum disorders.Nature neuroscience · 2016Review
- Loss of MeCP2 in the rat models regression, impaired sociability and transcriptional deficits of Rett syndrome.Human molecular genetics · 2016Article
- Knockdown of Dyslexia-Gene Dcdc2 Interferes with Speech Sound Discrimination in Continuous Streams.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2016Article
- Characterization of Rett Syndrome-like phenotypes in Mecp2-knockout rats.Journal of neurodevelopmental disorders · 2016Article
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Authors and funding
9 authors at 1 institution in 1 country.
Funding
Abstract
Individuals with Rett syndrome have greatly impaired speech and language abilities. Auditory brainstem responses to sounds are normal, but cortical responses are highly abnormal. In this study, we used the novel rat Mecp2 knockout model of Rett syndrome to document the neural and behavioral processing of speech sounds. We hypothesized that both speech discrimination ability and the neural response to speech sounds would be impaired in Mecp2 rats. We expected that extensive speech training would improve speech discrimination ability and the cortical response to speech sounds. Our results reveal that speech responses across all four auditory cortex fields of Mecp2 rats were hyperexcitable, responded slower, and were less able to follow rapidly presented sounds. While Mecp2 rats could accurately perform consonant and vowel discrimination tasks in quiet, they were significantly impaired at speech sound discrimination in background noise. Extensive speech training improved discrimination ability. Training shifted cortical responses in both Mecp2 and control rats to favor the onset of speech sounds. While training increased the response to low frequency sounds in control rats, the opposite occurred in Mecp2 rats. Although neural coding and plasticity are abnormal in the rat model of Rett syndrome, extensive therapy appears to be effective. These findings may help to explain some aspects of communication deficits in Rett syndrome and suggest that extensive rehabilitation therapy might prove beneficial.
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Registered trials
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