Articulation in Beckwith-Wiedemann Syndrome: Two Case Studies Beckwith-Wiedemann syndrome (BWS) is a genetic disorder with abdominal wall defects, gigantism, and macroglossia as its main characteristics. A number of investigators have reported the presence of articulation errors in individuals with BWS due to macroglossia. However, few data are available on the exact nature of the articulation problems of ... Research Article
Research Article  |   August 01, 2000
Articulation in Beckwith-Wiedemann Syndrome: Two Case Studies
 
Author Affiliations & Notes
  • John Van Borsel
    UZ Gent, Centrum voor Gehoor- en Spraakrevalidatie, Gent, Belgium
  • Bieke Morlion
    UZ Gent, Centrum voor Gehoor- en Spraakrevalidatie, Gent, Belgium
  • Kathleen Van Snick
    UZ Gent, Centrum voor Gehoor- en Spraakrevalidatie, Gent, Belgium
  • Jules S. Leroy
    UZ Gent, Pediatrie, Gent, Belgium
  • Contact author: John Van Borsel, PhD, UZ Gent, 2P1, Centrum voor Gehoor- en Spraakrevalidatie, De Pintelaan 185, B-9000 Gent, Belgium. Email: john.vanborsel@rug.ac.be
Article Information
Research Articles
Research Article   |   August 01, 2000
Articulation in Beckwith-Wiedemann Syndrome: Two Case Studies
American Journal of Speech-Language Pathology, August 2000, Vol. 9, 202-213. doi:10.1044/1058-0360.0903.202
History: Received November 8, 1999 , Accepted March 21, 2000
 
American Journal of Speech-Language Pathology, August 2000, Vol. 9, 202-213. doi:10.1044/1058-0360.0903.202
History: Received November 8, 1999; Accepted March 21, 2000

Beckwith-Wiedemann syndrome (BWS) is a genetic disorder with abdominal wall defects, gigantism, and macroglossia as its main characteristics. A number of investigators have reported the presence of articulation errors in individuals with BWS due to macroglossia. However, few data are available on the exact nature of the articulation problems of subjects with BWS. This paper presents two case studies that highlight the articulatory characteristics associated with BWS. Subjects were a boy aged 5 years 9 months and a girl aged 3 years 6 months. A phonetic analysis was conducted in which it was found that primarily consonants with an anterior place of articulation were affected. The error patterns appeared to be related to inappropriate tongue and lip postures. An observer experiment in which naive and expert observers rated speech samples from three modes of presentation (auditory-only, visual-only, and audiovisual) showed that the subjects’ speech was more disturbed visually than auditorily.

Author Note
We wish to thank three anonymous reviewers for their thoughtful comments and suggestions during the revision of the manuscript, and we also greatly appreciate their most generous efforts in identifying errors in grammar and usage.
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