The Speech and Language Characteristics of Children with Prader-Willi Syndrome

Abstract

Prader-Willi syndrome was initially identified in 1956. Since then, a majority of the literature pertaining to Prader-Willi has focused on the medical and genetic aspects of the syndrome. There has been limited information available regarding the speech and language abilities of children with Prader-Willi. This study investigated the communicative development of 18 children with the syndrome, ranging in age from 8:8 to 17:1. A number of evaluative procedures were used to evaluate the subjects' spontaneous speech, articulation, and receptive and expressive language abilities, as well as their voice, fluency, oral mechanisms, hearing, and their developmental histories. A variety of communicative deficiencies were found in the children's speech, language, voice, and fluency.

Fluency disorders in genetic syndromes

“…Sound repetitions and prolongations, some of the hallmarks of stuttering, did not occur. Kleppe et al (1990) concluded that many of their subjects were disfluent, but as a group the Prader-Willi children did not present the clinical picture of stuttering. Only 1 child was considered by them to have the symptoms of stuttering.…”

“…There is evidence that speech and language development is frequently problematic in PraderWilli syndrome (Akefeldt, Akefeldt, & Gilberg, 1997;Branson, 1981;Downey & Knutson, 1995;Dyson & Lombardino, 1989;Edmonston, 1982;Hall & Smith, 1972;Kleppe, Katayama, Shipley, & Foushee, 1990;Munson-Davis, 1988;Zellweger, 1988) and a number of authors make note of fluency disorders. Kleppe et al (1990) investigated fluency in 18 Prader-Willi children and found percentages of nonfluency ranging from 1 to 34%.…”

“…Kleppe et al (1990) investigated fluency in 18 Prader-Willi children and found percentages of nonfluency ranging from 1 to 34%. Interjections and revisions were frequently observed, accounting for 36 and 26% of all nonfluencies observed.…”

Clinical management of behavioral characteristics of Prader–Willi syndrome

“…43 Speech and language development is often delayed and problematic in PWS. 46,60,61 The severity differs greatly among individuals with PWS. There are those who are nonverbal and there are those who acquire normal speech and language by adulthood.…”

“…With regard to language development, individuals with PWS frequently demonstrate poor receptive and expressive Dovepress language skills, with expressive language showing greater deficits than receptive skills. 5,61,62 Individuals with PWS also have poor narrative skills which negatively affect how they can communicate with others in both a social and work environment. 5 These poor language skills may be the result of a deficit in auditory short-term memory, 52 poor temporal sequencing skills, 46 and poor auditory processing skills.…”