Research:
Communication
Study
Communication Studies of
Preschool Males with FXS: Profiles, Environmental Influences, and Intervention Strategies
Principal Investigator: Joanne Roberts
Project Director: Penny Mirrett
Funded by Grant No. H133G960186,
National Instiute on Disability and Rehabilitation Research, 1996-1999

This project was funded in 1996 to support an in-depth study of the communication of young boys with fragile X syndrome. It was the third largest grant awarded to investigators at FPG to fund research on young males with FXS.

Dr. Joanne Roberts, a senior FPG researcher in speech language pathology, has studied the effects of otitis media (middle ear infections) upon speech and language. She became interested in the communication skills of boys with fragile X when her colleagues from the FXS Preschool Boys Longitudinal Study began to share their early findings. Preliminary findings indicated that communication was the most severely affected developmental domain and that many of the boys had a history of chronic otitis media. This study, following up on previous ones, provided a closer examination of early language development in boys with FXS-information critical to develop and evaluate appropriate intervention strategies, and to understand other aspects of their behavior and development.
Research Goals

  • Describe the communication skills of boys with fragile X syndrome during the preschool years.
  • Identify which factors (e.g., child's cognitive level, autistic characteristics) contribute to the
    development of communication skills in boys with fragile X syndrome.
  • Identify early intervention strategies that should promote optimal communication development in
    males with fragile X syndrome.

Methods

    Participants

    • Forty-three males with full mutation fragile X syndrome were recruited before the age of seven years. Most of the children were recruited from the Carolina Fragile X Project. A speech-language pathologist and research assistant traveled to the home and spent 3 to 4 hours assessing each child annually. Children's communication skills were assessed using standardized speech and language tests and less formal measures such as language samples. Parents completed questionnaires about their children's language development.

    Staff

    • Professionals with a background in speech, language, and hearing as well as experience serving young children with special needs were recruited to conduct the research assessments.

    Data Collection

    • Staff members traveled to the home of each participant annually and spent 2-3 hours with the child and family. Assessments involved evaluation of subjects' speech and language skills using several standardized measures, structured tasks that tested memory and narrative skills, a pre-literacy test, a tympanometry test (showing integrity of the middle ear), an interview with the parent, and questionnaires filled out by the parent. Each assessment was video- and audio-taped. Six months after each annual assessment, parents filled out an update survey to document changes in communication needs services and filled out a communication development questionnaire. At the conclusion of 3 years of data collection, 43 children had been tested once, 37 had been tested two times, and 19 children had been tested 3 times.

    Measures

    • Speech Measures
      • Goldman-Fristoe Test of Articulation
      • Oral Structure and Function Exam (Robbins and Klee, 1987)
    • Language and Memory Measures
      • Reynell Language Scales (receptive and expressive)
      • Test of Early Reading Ability (pre-literacy)
      • Peabody Picture Vocabulary Test
      • Mother-Child Interaction Tasks
    • Parent Questionnaires
      • MacArthur Communicative Development Questionnaire
      • Early Literacy Acquisition Questionnaire/Interview
      • Early Language Questionnaire/Interview
    • Middle Ear
      • Tympanometry test--measures mobility of ear drum and middle ear and is used to screen for middle ear disease such as otitis media
    • Other
      • HOME--measure of home environment, completed by staff after the assessment

Results

  • We studied the development of receptive and expressive communication skills for 39 boys with fragile X syndrome between the ages of 2 and 7 years of age who were seen from one to three times. Eight of the children showed features characteristic of autism. We found that the children had delayed language development, but there was substantial individual variability. Children acquired expressive language skills more slowly than receptive language skills over time. The boys with fragile X syndrome gained receptive language at about half the rate expected for typically developing children and they developed expressive language at about one-third of the rate expected. Both cognitive development and autistic characteristics were related to receptive and expressive language development. Boys who showed less autistic characteristics and had higher cognitive levels had higher receptive and expressive language skills. Neither cognitive development or autistic characteristics predicted the discrepancies between the children's expressive and receptive language acquisition over time.
  • We examined joint attention skills of 38 young males with fragile X syndrome (with and without autism) using the Communication and Symbolic Behavior Scales (CSBS). Joint attention can be defined as nonverbal and verbal acts used to direct another's attention to an object, event, or topic (e.g., looking, pointing, labeling). We found that the boys who demonstrated higher scores on joint attention on the CSBS showed higher expressive language and faster acquisition of receptive language over time. In addition, these findings remained the same after partialing out the characteristics of autism.
  • We studied the communication and symbolic behavior profiles of 22 males with fragile X syndrome developmentally younger than 28 months and the relationship of these profiles to the children's communication skills one year later. The males, ranging in age from 21 to 77 months, were tested using the Communication and Symbolic Behavior Scales and the Reynell Developmental Language Scales. The children showed significant delays and substantial individual variability in their profiles. Overall, they showed relative strengths in verbal (e.g., use of words) and vocal (e.g., use of sounds) communication and relative weaknesses in gestures (e.g., pointing), reciprocity (e.g., responding) and symbolic play (e.g. doing familiar routines such as brushing hair). Children who scored higher in communicative functions (e.g., commenting), vocalizations, verbalizations, and reciprocity scored higher in verbal comprehension one year later. Children with higher scores in verbal communication also scored higher in expressive language development when tested one year later.
  • We compared assessments of the communication skills of 34 preschool males with fragile X syndrome done by families and professionals. Parents and professionals rated the boys' receptive and expressive communication. Parents also reported on the vocabulary skills of 16 of the boys, whereas professionals assessed their vocabulary diversity using a communication sample. Moderate agreement was found between parents and professionals for expressive communication ratings, whereas agreement for receptive communication ratings was low to moderate. Parents rated their children significantly higher than professionals for receptive communication but not for expressive communication.
  • We interviewed speech-language pathologists concerning the goals for intervention and intervention strategies they used when working with young males with fragile X syndrome. Two staff speech-language pathologists interviewed 51 speech-language pathologists (SLPs) providing intervention to 41 males with full mutation fragile X syndrome ranging in age from 2 years 6 months to 9 years 8 months (mean age 6 years 3 months). The majority of SLPs reported that males with fragile X syndrome benefited from a visually based, experiential or holistic learning style. They emphasized the need to make specific environmental accommodations for attention, transitioning, sensory deficits and anxiety. The goals for speech intervention included slowing the rate and increasing articulation accuracy and use of technology for nonverbal or minimally verbal children. Language goals focused on improving listening and auditory comprehension skills, and conversational skills such as the topic of conversation.

References

Jackson, S.C., & Roberts, J.E. (1999). Family and professional congruence in communication assessments of preschool boys with fragile X syndrome. Journal of Early Intervention; 22, 137-151.

Mirrett, P., Roberts, J. E., & Price, J. (in press). Early Intervention Practices and Communication Intervention Strategies for Young Males with Fragile X syndrome.

Roberts, J. E., Mirrett, P., & Burchinal, M. (2001). Receptive and expressive communication development of young males with fragile X syndrome. American Journal on Mental Retardation, 106, 216-230.

Roberts, J. E., Mirrett, P., Anderson, K., Burchinal, M., & Neebe, E. (2002). Early communication profiles of young males with fragile X syndrome. American Journal of Speech-Language Pathology, 11, 295-304.