Hiperacusia: Sección Etiologia: Sindrome de Williams

Fears, hyperacusis and musicality in Williams syndrome

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Stefan Blomberg^{a, ,} , Michael Rosander^a and Gerhard Andersson^{a, b}

^aDepartment of Behavioural Sciences, Linköping University, Sweden
^bDepartment of Ear, Nose, Throat, Linköping University Hospital, Sweden

Received 26 May 2005; 

revised 6 September 2005; 

accepted 9 September 2005. 

Available online 2 November 2005.

Abstract

The study investigated the prevalence of fear and hyperacusis and the possible connections between fear, hyperacusis and musicality in a Swedish sample of individuals with Williams syndrome (WS).

The study included 38 individuals and a cross-sectional design, with no matched control group.

Two persons, who knew the participant well, completed a questionnaire.

On reported fears, 58% of the participants scored higher than +2S.D., compared to a psychometric study. Thirteen percent scored above the suggested cut-off for hyperacusis, compared to 2.5% in a psychometric study.

Female participants generally had higher reported fears and hyperacusis compared to male participants.

There were also startling findings of correlations between reported fears and hyperacusis.

This preliminary report supports a hypothesis that fears and anxiety could be associated with hyperacusis in the WS population.

A hypothesis that musicality could serve as a protective factor and prevent anxiety, received no or very limited support.

A hypothesis that hyperacusis could be connected to a general, readily arousal, tendency in the sympathetic nervous system and could be seen as vulnerability for psychopathology is discussed.

Keywords: Williams syndrome; Fear; Anxiety; Hyperacusis; Musicality; Gender differences; Vulnerability; Psychopathology

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Fig. 2. Participants’ result on the Hyperacusis Questionnaire compared with standard deviations (S.D.) based on a psychometric study of Khalfa et al. (2002) and cut-off for hyperacusis.

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Table 1. Means, standard deviations and internal consistencies (Cronbach's alphas) for the Fear Survey Schedule for Children—Revised (FSSC-R), the Hyperacusis Questionnaire (HQ), the Musicality Interest Scale (MIS) and their subscales

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Table 2. The ten most frequent reported fears on the Fear Survey Schedule for Children—Revised (FSSC-R), based on percentage of participants where two informants have rated an item as a 3 (a lot of fear), and the mean of the different items
N = 33.
^a Item reported on top ten fears in Svensson's and Öst (1999), self report.
^b Item reported on top ten fears in Dykens’ study (2003a), parent report.
^c Item reported on top ten fears in Dykens’ study (2003a), self report.

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Table 3. Means, standard deviations and t-values for men and women on total score and subscales of the Fear Survey Schedule for Children—Revised (FSSC-R), the Hyperacusis Questionnaire (HQ) and the Musicality Interest Scale (MIS)
^* p < .01.
^** p < .001.

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Table 4. Means of the total score of the Fear Survey Schedule for Children—Revised (FSSC-R), and the Hyperacusis Questionnaire (HQ) for men and women in the age group 10–17 years and 18 years or older
A significant difference was found between values with superscript letters a and b, and c and d.

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Table 5. Correlations between total score and subscales on the Fear Survey Schedule for Children—Revised (FSSC-R), the Hyperacusis Questionnaire (HQ) and the Musicality Interest Scale (MIS)
N = 38.
^* p < .05.
^** p < .01.
^*** p < .001.

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Correspondence at: Medical Centre for Refugees, Vrinnevi Hospital, SE-601 82 Norrköping, Sweden. Fax: +46 11 22 27 67.

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Fuente: Research in Developmental Disabilities
Volume 27, Issue 6, November-December 2006, Pages 668-680