Auditory Brainstem Responses in Young Males With Fragile X Syndrome Fragile X syndrome (FXS) is the most common inherited cause of mental retardation resulting in developmental delays in males. Atypical outer ear morphology is characteristic of FXS and may serve as a marker for abnormal auditory function. Despite this abnormality, studies of the hearing of young males with FXS are ... Research Article
Research Article  |   April 01, 2005
Auditory Brainstem Responses in Young Males With Fragile X Syndrome
 
Author Affiliations & Notes
  • Joanne Roberts
    Frank Porter Graham Child Development Institute, University of North Carolina at Chapel Hill
  • Elizabeth A. Hennon
    Frank Porter Graham Child Development Institute, University of North Carolina at Chapel Hill
  • Kathleen Anderson
    Frank Porter Graham Child Development Institute, University of North Carolina at Chapel Hill
  • Jackson Roush
    Frank Porter Graham Child Development Institute, University of North Carolina at Chapel Hill, and Hunter College of the City University of New York
  • Judith Gravel
    Hunter College of the City University of New York
  • Martie Skinner
    Frank Porter Graham Child Development Institute, University of North Carolina at Chapel Hill
  • Jan Misenheimer
    Frank Porter Graham Child Development Institute, University of North Carolina at Chapel Hill
  • Patricia Reitz
    Frank Porter Graham Child Development Institute, University of North Carolina at Chapel Hill
  • Contact author: Joanne E. Roberts, Frank Porter Graham Child Development Institute, University of North Carolina at Chapel Hill, 105 Smith Level Road, CB# 8180, Chapel Hill, NC 27599-8180. E-mail: joanne_roberts@unc.edu
Article Information
Hearing Disorders / Special Populations / Genetic & Congenital Disorders / Hearing / Research Articles
Research Article   |   April 01, 2005
Auditory Brainstem Responses in Young Males With Fragile X Syndrome
Journal of Speech, Language, and Hearing Research, April 2005, Vol. 48, 494-500. doi:10.1044/1092-4388(2005/034)
History: Received March 8, 2004 , Accepted September 28, 2004
 
Journal of Speech, Language, and Hearing Research, April 2005, Vol. 48, 494-500. doi:10.1044/1092-4388(2005/034)
History: Received March 8, 2004; Accepted September 28, 2004
Web of Science® Times Cited: 5

Fragile X syndrome (FXS) is the most common inherited cause of mental retardation resulting in developmental delays in males. Atypical outer ear morphology is characteristic of FXS and may serve as a marker for abnormal auditory function. Despite this abnormality, studies of the hearing of young males with FXS are generally lacking. A few studies have suggested that a significant proportion of individuals with FXS demonstrate prolonged auditory brainstem response (ABR) latencies. The purpose of this study was to determine whether young males with FXS display atypical auditory brainstem function compared to typically developing males when conductive and sensorineural hearing loss are ruled out as possible contributors to atypical findings. Participants were 23 males with FXS, 21 typically developing males who were matched for developmental age, and 17 typically developing males who were matched for chronological age. A battery of tests to assess peripheral hearing, cochlear function, and auditory pathway integrity through the level of the brainstem was completed. Males with FXS were similar to typically developing males who were matched for developmental age level or chronological age level on all measures. They had normal hearing sensitivity and middle ear function and scored similar to the typically developing children on the measures of auditory brainstem pathway integrity. In summary, ABRs in young males with FXS were within normal limits.

Acknowledgments
This research was supported by National Institute of Child Health and Human Development Grants 1 R01 HD38819 and 1 R03 HD40640. We thank the children and families who participated in this study. We greatly appreciate the assistance of Gina Crovato, Anne Edwards, Cheryl Malkin, and Kathleen Neff for assisting with data collection. We also want to thank the staff of the Carolina Fragile X Project directed by Donald Bailey for their assistance with this project. We also thank Sarah Henderson for assistance in manuscript preparation.
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