| Hyperacusis |
| Bedeschi et al. 2011 | To investigate medical problems in WS | 45 WS (23 m, 22 f)
Age range 17–44 years old (M = 23.6 years) | Audiograms | Audiologic problem were recorded in 13/45 subject. Five of them (38.4%) had been diagnosed with hyperacusia in infancy, and 8 (61.6%) in adulthood | The onset of hyperacusis in majority of WS patients was in adulthood |
| Blomberg et al. 2006 | To investigate the prevalence of fear and hyperacusis and to explore the possible connections between fear, hyperacusis and musicality in a Swedish sample of individuals with WS | 38 WS (25 m, 13 f)
M = 21.00, SD = 8.13 years | Fear Survey Schedule for Children-Revised (FSSC-R), hyperacusis questionnaire (HQ), Musicality Interest Scale (MIS) | Mean score on the HQ was 19.55 (SD = 7.58) with 13% of the participants scoring above suggested cut-off for hyperacusis. There were many significant correlations between the HQ (total score and the attention, social aspects, emotional aspects subscales) and the FSSC-R (total score and the danger and death, failure and criticism, the unknown, animals, agoraphobic situations subscales), but very few between the HQ and the MIS | A high reported prevalence of fear and hyperacusis was reported among the WS participants and correlations between reported fears and hyperacusis were found. Female individuals with WS (particularly adult women) had higher reported fears and hyperacusis compared to male individuals with WS |
| Don et al. 1999 | To examine the music and language skills in children with WS | 19 WS (10 m, 9 f)
Age range 8–13 years old (M = 10 years and 6 months, SD = 1 year and 10 months)
19 TD (11 m, 8 f)
Age range 5–12 years old (M = 7 years and 11 months, SD = 2 years and 5 months) | Parent Music Questionnaire | History of hyperacusis evident for the entire WS group, but only for 10% TD group. All the WS children had unusual fearfulness toward sounds (comparing to 47% of TD children) and 75% exhibited unusual liking for specific sounds (comparing to 1 TD child) | The overwhelming prevalence of hyperacusis and unusual emotional responses to specific sounds were characteristic of the children with WS and distinguished them from the TD children |
| Einfeld et al. 1997 | To assess psychopathology in WS | 70 WS
M = 9.2 years old
454 TD
M = 12 years old | Developmental Behavior Checklist | 80% of the WS participants covered ears to avoid particular sounds comparing to 35% of the TD participants (t = 7.15, p < .001) | Children with WS reported hyperacusis more often than TD children |
| Elsabbagh et al. 2011 | To assess the relationship between speech perception in noise in WS and their subjective rating of the severity of hyperacusis in everyday situations | 32 WS
Age range 7.5–56.7 years old
32 TD
Age range 7.4–11.8 years old
24 TD
Age range 19.1–58.1 years old | Hyperacusis questionnaire, word-pairs discrimination/speech perception task | Higher severity of hyperacusis was associated with worse discrimination performance on the speech perception task | Hyperacusis influences speech perception, which may thus contribute to a qualitatively different process of language acquisition in WS |
| Gallo et al. 2008 | To broaden the understanding of atypical behavioural reactivity to everyday sounds in the WS population using observational methods | 21 WS (14 m, 7 f)
Age range 30–65 months old (M = 44.48, SD = 10.84 months)
20 mixed ethology (ME; 12 m, 8 f)
Age range 30–78 months old (M = 44.8, SD = 12.55 months) | Autism diagnostic observation schedule-module 1 | Approximately 90% of the young children in the WS showed discomfort, fear, and/or anxiety in response to (or in anticipation of) everyday sounds, compared to 20% of mixed ethology controls. Over half the children with WS exhibited two or more different behaviours reflective of sound reactivity during the brief play interaction, compared to 15% in the ME group | Adverse reactions to sound were very common. A large proportion of the behaviours may be interpreted as ‘anticipations’, rather than ‘direct responses’ to aversive sound stimuli, highlighting the presence of anxiety that is pervasive among children with WS |
| Gothelf et al. 2006 | To describe the clinical characteristics of hyperacusis and phonophobia in WS and to investigate the audiologic and neurologic abnormalities in subjects with WS and hyperacusis | 49 WS (20 m, 29 f)
Age range 1–35 years old (M = 11.1, SD = 7.4 years) | Hyperacusis Screening Questionnaire | 83.7% of WS participants were reported to be frightened or bothered by normal environmental sounds, the hyperacusis was most severe at age 5.7 ± 3.8 years and tended to decline thereafter; children sensitive most frequently to electric machines, thunder, bursting balloons, and fireworks | Hyperacusis occurred in 84% of the participants; aversive responses to noise were present as early as infancy |
| Honjo et al. 2015 | To investigate medical problems in WS | 55 WS (34 m, 21 f)
Age range 2–30 years old | Clinical and Laboratory Assessments | Hyperacusis prevalent in 94.5% of the individuals | High prevalence of hyperacusis in WS |
| Klein et al. 1990 | To obtain more data on the characteristic of hyperacusis | 65 WS (36 m, 29 f)
Age range 1–28 years old
65 TD (32 m, 33 f)
Age range 2–17 years old | Questionnaire | Hyperacusis found in 95% of WS participants (and 12% of TD participants), with a slight decrease in severity with age | High prevalence of hyperacusis in WS; many of the adverse reactions noted prior to 1 year of age, suggesting that hyperacusis may be an innate condition |
| Lense and Dykens 2013 | To identify correlates of musical instrument learning | 46 WS (47.8% m)
Age range 7–49 years old (M = 23.13, SD = 9.55) | Sensitivity to Sounds Questionnaire | The mean of the sound sensitivity score was 18.27, with SD = 6.61 (range 5–30) | The achievement of learning a new instrument was not associated with sound sensitivity |
| Lense et al. 2013 | To examine how the auditory sensitivities and love of music that characterise WS relate to their variable musical perception and production abilities | 73 WS (49.3% m)
Age range 10–51 years old (M = 26.2, SD = 9.4 years) | Sensitivity to Sounds Questionnaire, Musical Questionnaires And Behavioural Assessments | The mean of the sensitivity to specific (non-musical) sounds was 50.1 (SD = 19.1), and for the sensitivity to sound characteristics was 18.2 (SD = 6.4). Musical interest was not associated with sensitivity to sound characteristics or sensitivity to specific (non-musical) sounds. | Musical perception in WS is not related to general auditory sensitivities |
| Levitin et al. 2005 | To clarify and document the incidence of auditory abnormalities in and among people with WS | 118 WS (61 m, 57 f)
M = 20.4 (SD = 10.4) years old
30 autism (24 m, 6 f)
M = 18.2 (SD = 7.7) years old
40 Down syndrome (20 m, 20 f)
M = 17.2 (SD = 9.2) years old
118 TD (28 m, 90 f)
M = 20.9 (SD = 7.4) years old | Open-ended Questionnaire | 4.7% of the WS sample reported true hyperacusis. 79.8% of the WS sample reported odynacusis (compared to 33% of the people with autism and Down syndrome, and 4% of the TD sample). Auditory aversions were reported by 90.6% of the WS sample (and 27% of the autism, 7% of the Down syndrome and 2% of the TD groups). There was a significant difference in the age of onset of auditory aversions with onset in WS occurring significantly earlier than in other groups. Auditory fascinations were found in 9% of the WS sample (and only 1 TD participant (0.8%) reported auditory fascinations. | Unusual auditory behaviours were more common in the WS sample than in any other comparison group. The concepts of aversion, awareness and attraction seem to characterise the auditory abnormalities observed in WS |
| O’Reilly et al. 2000 | To examine how hyperacusis influenced operant responding under functional analysis assessment conditions for an individual with WS who exhibited problem behaviour | Case study (f)
5 years and 2 months | Williams Syndrome Questionnaire and functional analysis | Little problem behaviour was observed during the functional assessment under the no-noise condition; during the noise condition, high level of problems were observed under the demand assessment condition, but little problem behaviour occurred during the attention and play conditions | Background noise seemed to influence responding under functional analysis conditions by increasing the aversiveness of task demands |
| Udwin 1990 | To collect more information on adults with WS | 119 WS (51 m, 68 f)
Age range 16 years and 2 months–38 years and 10 months
M = 22y10m | Survey/questionnaires | 110 adults (92%) were hypersensitive to sounds as children, and 93 of these (78% of the total group) remained hypersensitive as adults | The high rate of hyperacusis was found in the sample |
| Sensory processing |
| Janes et al. 2014 | To capture information about sensory processing experiences and repetitive behaviours | 21 WS (12 m, 9 f)
Age range 6–15 years old (M = 9.3 years) | Short Sensory Profile (SSP); Assessment of Sensory Processing, Repetitive Behaviour, Anxiety, Fears in WS - Semi-structured Interview (SRAF-SSI) | The majority of the sample experienced sensory processing difficulties. The areas of sensory processing most frequently endorsed by parents were vestibular, auditory, gustatory, and proprioceptive hypersensitivities. | The sensory profile of children with WS is characterised by hypersensitivities. Visual and tactile processing was not frequently reported as problematic. |
| John and Mervis 2010 | It was hypothesised that children with WS would demonstrate symptoms of poor sensory modulation and that these sensory modulation abnormalities contribute to the phenotype | 78 WS (34 m, 44 f)
Age range 4–10.95 years old (M = 6.63, SD = 2.14) | Short Sensory Profile (SSP), Behavior Rating Inventory of Executive Functioning (BRIEF), Children’s Behavior Questionnaire (CBQ), Scales of independent Behavior – Revised (SIB-R), Conner’s Parent Rating Scale – Revised (CPRS-R(L)) | Only 7 children (9.9%) were classified as “typical performance” based on their SSP total score. 56.3% was classified as definitely having overall sensory modulation issues and 33.8% as probably having overall sensory modulation issues. Definite abnormalities on the auditory filtering were found in 59% of the children, on the low energy/weak in 64.1% and on the under-responsive/seeks Sensations in 62.8%. Executive functioning, temperament, adaptive functioning and problem behaviours were associated with sensory modulation difficulties | Most children with WS demonstrated abnormalities in sensory modulation. The ability to use muscles to move, noticing everyday sensory events and hypo- and hyper-responsiveness to sounds were the most problematic areas of sensory modulation. Children with high impairments in sensory modulation had significantly poorer executive functioning and adaptive functioning, had also more problem behaviours and more difficult temperament |
| Riby et al. 2013 | To explore sensory processing abnormalities and repetitive behaviours in children with WS. It was hypothesised that children with WS who demonstrated more sensory processing abnormalities would exhibit more repetitive behaviours | 21 WS (12 m, 9 f)
Age range 6–15 years old (M = 9.3 years) | Short sensory profile (SSP), repetitive behaviour questionnaire (RBQ) | A significant negative correlation between the total score of the RBQ and the total score of the SSP (r = − .60, p = .01)
Significant correlations existed between RBQ Repetitive Movement and Tactile Sensitivity (r = − .48, p = .03), Taste/Smell Sensitivity (r = − .52, p = .02) and Under-responsive/Seeks Sensation (r = − .58, p = .01). RBQ Repetitive Language was significantly correlated with Under-responsive/Seeks Sensation subscale (r = − .54, p = .01). RBQ Sameness of Behaviours was significantly correlated with the taste/smell sensitivity subscale (r = − .58, p = .01) | Children with WS who experienced more sensory processing abnormalities demonstrated more repetitive behaviours. Engagement in some of the behaviours reported in the RBQ repetitive movement subscale occur as a consequence of tactile sensitivity. |
| Wuang and Tsai 2017 | To examine the developmental profiles of school-aged children with WS | 38 WS (23 m, 15 f)
Age range 6–12 years old (M = 90.66 months old, SD = 4.47) | Sensory profile (SP)
Wechsler intelligence scale for children-third edition (WISC-III)
test of visual perceptual skill, third edition (TVPS-3)
Bruininks-Oseretsky test of motor proficiency-second edition (BOT-2)
vineland adaptive behaviour scale-Chinese version (VABS-C)
school function assessment-Chinese version (SFA-C) | Definitive differences in sensory processing (mean = 124.4, typical performance range 256–325) and modulation (mean = 72.1, typical performance range 134–170); and probable difference in behavioural and emotional responses (mean = 89.9, typical performance range 97–130).
The correlations between SP domains: and cognitive level were small (r, 0.15–0.22), medium to large for the BOT-2 (r, 0.33–0.73), VABS-C (r, 0.32–0.79), SFA-C (r, 0.32–0.62), and large for the TVPS-3 (r, 0.57–0.63). | Children with WS showed significant challenges in sensory processing and modulation and these multisensory processing difficulties were associated with lower participation in school activities and poor adaptive behaviours. |