An investigation of voice quality in individuals with inherited elastin gene abnormalities.

The human elastin gene (ELN) is responsible for the generation of elastic fibres in the extracellular matrix of connective tissue throughout the body, including the vocal folds. Individuals with Supravalvular aortic stenosis (SVAS) and Williams syndrome (WS) lack one normal ELN allele due to heteroz...

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Publicado en:Clinical Linguistics & Phonetics Vol. 22; no. 3; pp. 199 - 214
Autores principales: Watts CR, Awan SN, Marler JA
Formato: research tables/charts Journal Article
Publicado: Taylor & Francis Ltd Mar2008
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Mar2008
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      pub: Taylor & Francis Ltd
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        atl: An investigation of voice quality in individuals with inherited elastin gene abnormalities.
      aug:
        au:
          Watts CR
          Awan SN
          Marler JA
        affil: Department of Communication Sciences & Disorders, James Madison University, VA.
      sug:
        subj:
          Aortic Valve Stenosis Familial and Genetic
          Voice Quality Evaluation
          Williams Syndrome Familial and Genetic
          Adolescence
          Adult
          Analysis of Variance
          Biomechanics
          Descriptive Statistics
          Experimental Studies
          Female
          Interrater Reliability
          Intraclass Correlation Coefficient
          Male
          Mann-Whitney U Test
          Mutation
          Phonation
          Speech Acoustics
          Speech Production Measurement
          Vocal Cords Abnormalities
          Human
          Adolescent: 13-18 years
          Adult: 19-44 years
          Female
          Male
      ab: The human elastin gene (ELN) is responsible for the generation of elastic fibres in the extracellular matrix of connective tissue throughout the body, including the vocal folds. Individuals with Supravalvular aortic stenosis (SVAS) and Williams syndrome (WS) lack one normal ELN allele due to heterozygous ELN abnormalities, resulting in a haploinsufficiency. We measured perceptual and acoustic characteristics of voice quality in individuals with SVAS and WS to investigate the consequences to vocal function secondary to ELN haploinsufficiency. Results indicated that the voice quality of individuals with SVAS/WS was rated as significantly more abnormal, rough, and hoarse compared to normal controls, and that adults with SVAS/WS were rated as significantly lower in pitch. Acoustic measures indicated that individuals with SVAS/WS produced greater instability of fundamental frequency during phonation (as reflected via increased pitch sigma and increased jitter). These findings support the possibility that heterozygous ELN abnormalities negatively influence vocal fold biomechanics and the resulting sound produced by the vibrating glottis.
      pubtype: Academic Journal
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    language: English
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