![]() ![]() The authors emphasized that, if a validated questionnaire were used, the prevalence of hyperacusis in WS would tend to decrease. To date, few studies have made a formal evaluation to find objective results that could measure hyperacusis in WS.īaguley and McFerran stressed that hyperacusis is often used indiscriminately to refer to hearing sensitivity in general, including phonophobia and misophonia (which is a strong aversion to sounds). In most of the studies that described the presence of hyperacusis in WS, this information was only collected through interviews with parents or guardians or with the individuals with WS themselves, ,, ,, ] or by observing behavioral reactions in a playful game. Notably, there are few standardized protocols to measure hyperacusis in this syndrome, and studies differ as to classification of auditory hypersensitivity. Thus, hyperacusis in this population may be associated with non-auditory factors, such as fear and anxiety. Odynacusis, which was described as a reduction in the pain threshold, was found in 90.6% of individuals with WS. The authors classified hyperacusis as a reduction in the sound detection threshold, and it was found in 4.7% of the sample. One study identified phonophobia in 79.8% of cases and fascination with sounds in 9.3% of individuals. The presence of specific phobias is also a behavioral characteristic of individuals with WS, with loud noises being most commonly reported, namely sirens, thunder, engine sounds, fireworks, cries and screams. Dysfunction of acoustics reflexes and auditory hypersensitivity can be associated, considering that one of the functions of the stapedius reflex is to protect the auditory system from intense sounds. Some studies have hypothesized that the absence of acoustic reflexes, which is very common in these individuals, may be due to auditory hypersensitivity. It was more pronounced in childhood and tended towards attenuation during adolescence. ![]() Likewise, mothers answered a questionnaire, in which hyperacusis was reported in 84% of cases. They presented lower levels of discomfort than individuals with typical development. Ī study that evaluated hyperacusis through a standardized protocol, detected moderate to severe hyperacusis in 84% of individuals with WS. It is more prevalent among females than males, but it tends to decrease throughout adolescence. Previous studies have reported a history or presence of hyperacusis in 77%–100% of individuals with WS, ,, ]. Auditory fascinations are a substantial attraction to or fascination with certain sounds hyperacusis is abnormal sensitivity to sounds, in which low- and medium-intensity sounds can cause discomfort or even pain and phonophobia refers to a fear reaction to certain sounds, , ]. ![]() However, mild sensorineural hearing loss, mainly with impairment of high frequencies, has been commonly described in the literature, ,, ,, ].Ī characteristic also widely reported in the WS individuals is auditory hypersensitivity, which can be observed by means of three behavioral phenomena: fascination or strong attraction to sounds, phonophobia and hyperacusis, , ]. Previous studies have shown that individuals with WS have a strong musical interest, , ] and have absolute pitch. Such losses are due to a microdeletion in the region of the long arm of chromosome 7 (7q11.23), compromising approximately 28 genes, , ]. Williams Syndrome (WS) is a genetic neurodevelopmental disease that manifests itself in several physical, neurological and behavioral changes, and has a rare prevalence, estimated at 1:7500 live births.
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |