Abstract
Language delay and communication deficits are a core characteristic of the fragile X syndrome (FXS) phenotype. To date, the literature examining early language development in FXS is limited potentially due to barriers in language assessment in very young children. The present study is one of the first to examine early language development through vocal production and the language learning environment in infants and toddlers with FXS utilizing an automated vocal analysis system. Child vocalizations, conversational turns, and adult word counts in the home environment were collected and analyzed in a group of nine infants and toddlers with FXS and compared to a typically developing (TD) normative sample. Results suggest infants and toddlers with FXS are exhibiting deficits in their early language skills when compared to their chronological expectations. Despite this, when accounting for overall developmental level, their early language skills appear to be on track. Additionally, FXS caregivers utilize less vocalizations around infants and toddlers with FXS; however, additional research is needed to understand the true gap between FXS caregivers and TD caregivers. These findings provide preliminary information about the early language learning environment and support for the feasibility of utilizing an automated vocal analysis system within the FXS population that could ease data collection and further our understanding of the emergence of language development.
Highlights
Fragile X Syndrome (FXS) is the leading inherited cause of intellectual disability (ID) associated with a mutation on an unstable trinucleotide (CCG) repeat expansion on the fragile X mental retardation1 (FMR1) gene [1]
Data were extracted from the LENA Foundation Natural Language Study [29] to derive a typically developing (TD) normative dataset to compare to the performance of the children with FXS
The present study aimed to build on our current understanding of early language development in FXS utilizing new methodology
Summary
Fragile X Syndrome (FXS) is the leading inherited cause of intellectual disability (ID) associated with a mutation on an unstable trinucleotide (CCG) repeat expansion on the fragile X mental retardation1 (FMR1) gene [1]. FXS is characterized by mild to severe ID with a series of other features including: anxiety, social deficits, communication deficits, gaze aversion, inattention, impulsivity, aggression and hyperactivity [2] Within communication deficits, it is evident in the current literature that FXS is associated with significant language delay, above that expected by given cognitive deficits, with relevant strengths in receptive communication and relative weaknesses in expressive communication [3,4]. Brain Sci. 2019, 9, 27 language acquisition in infants and young children due to the natural development of language. This can be difficult in clinical populations with known speech delays (e.g., FXS, autism spectrum disorder, Down syndrome) potentially impacting early diagnostic and treatment efforts
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