Degraded neural and behavioral processing of speech sounds in a rat model of Rett syndrome

Neurobiology of Disease
C T EngineerMichael P Kilgard

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 codi...Continue Reading

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Citations

Dec 5, 2015·Frontiers in Systems Neuroscience·Renata Figueiredo AnomalRodrigo N Romcy-Pereira
Jun 18, 2016·Journal of Neurodevelopmental Disorders·Yang WuChun Jiang
Oct 28, 2016·Nature Neuroscience·Yehezkel Sztainberg, Huda Y Zoghbi
Sep 8, 2020·Journal of Pediatric Rehabilitation Medicine·David YangVithya Gnanakumar
Oct 21, 2017·Autism Research : Official Journal of the International Society for Autism Research·Crystal T EngineerMichael P Kilgard
Jun 12, 2020·Translational Psychiatry·Olga V SysoevaJohn J Foxe
Sep 30, 2020·Journal of Neurodevelopmental Disorders·Katherine S AdcockSeth A Hays
Aug 18, 2020·Brain Stimulation·Katherine S AdcockCrystal T Engineer
Jul 25, 2018·Brain Stimulation·E P BuellM P Kilgard
Jan 31, 2017·Brain Stimulation·Crystal T EngineerMichael P Kilgard
May 8, 2021·Frontiers in Genetics·Vanessa ChenouardIgnacio Anegon

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