Abstract

Background: The cognitive profile of Turner syndrome, a genetic disorder resulting from partial or complete X-chromosome deletion, presents characteristic deficits. Despite this, studies have yet to evaluate how deficits translate into and are compensated for in academic settings. This study seeks to explore cognitive functioning, as well as the accessibility and development of academic accommodations in females with Turner syndrome from adolescence to adulthood.Materials and Methods: This cross-sectional study took place at the National Institutes of Health. Females with Turner syndrome (age range: 10–68; n = 142) were evaluated on need for and procurement of academic accommodations. Cognitive functioning was evaluated in participants aged 20 years and older (n = 101), as per the age validation of the Repeatable Battery for the Assessment of Neuropsychological Status. Data were analyzed using descriptive statistics, one-sample comparisons, and analyses of variance.Results: Females with Turner syndrome scored significantly lower than the normative population on visuospatial (p < 0.001), delayed memory (p < 0.001), and overall (p < 0.001) functioning. About 25.9% of participants reported that accommodations were not needed, despite displaying one or more cognitive deficits. Approximately 12.7% reported needing but not receiving accommodations, however, this is only reported by females 30 years and older; no females aged 10–29 years indicated this discrepancy.Conclusions: Findings suggest that procurement of academic accommodations has increased within recent decades. Still, there is a discrepancy between those displaying cognitive deficits and those receiving academic accommodations. We highlight frequently received accommodations so that students and professionals can target deficits with appropriate accommodations.

Highlights

  • IntroductionTurner syndrome is a common genetic disorder, occurring in *1 in 2000–2500 live female births.1,2

  • Turner syndrome is a common genetic disorder, occurring in *1 in 2000–2500 live female births.1,2 It is characterized by partial or complete deletion of one X-chromosome, and is often associated with short stature, ovarian failure, dysmorphia, hearing loss, cardiac complications, and cognitive deficits.3Studies on this disorder have advanced understanding of how X-chromosome deletion affects cognitive development

  • This study explores cognitive deficits and provision of academic accommodations in females with Turner syndrome

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Summary

Introduction

Turner syndrome is a common genetic disorder, occurring in *1 in 2000–2500 live female births.1,2 It is characterized by partial or complete deletion of one X-chromosome, and is often associated with short stature, ovarian failure, dysmorphia, hearing loss, cardiac complications, and cognitive deficits.. It is characterized by partial or complete deletion of one X-chromosome, and is often associated with short stature, ovarian failure, dysmorphia, hearing loss, cardiac complications, and cognitive deficits.3 Studies on this disorder have advanced understanding of how X-chromosome deletion affects cognitive development. The cognitive profile of Turner syndrome, a genetic disorder resulting from partial or complete X-chromosome deletion, presents characteristic deficits. This study seeks to explore cognitive functioning, as well as the accessibility and development of academic accommodations in females with Turner syndrome from adolescence to adulthood. Females with Turner syndrome (age range: 10–68; n = 142) were evaluated on need for and procurement of academic accommodations. We highlight frequently received accommodations so that students and professionals can target deficits with appropriate accommodations

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