Identification of Hearing Impairment: A Multi-Centre Study

"Hyperacusis" is used to describe an intolerance or aversion to sounds that would be tolerated by most normal listeners. Although the term "hyperacusis" can be applied to peripheral and central causes of over-sensitivity to sound, this presentation will focus on the central auditory processing disorders. Hyperacusis is one of many physical and behavioural features of the Williams syndrome phenotype. The author's initial research work in this area examined auditory over-sensitivity in subjects with Williams Syndrome (WS). These Ws hyperacusics represent a clinical group with a shared genetic lesion. However, over the course of the project it became apparent that there are a number of other clinical groups for whom oversensitive hearing is a major problem. A synopsis of the work on hyperacusis in Williams Syndrome will be presented, followed by some data from hyperacusics in the general population. Thirdly, the role of sensory sensitivity in children with communication disorders and other learning disabilities will be discussed. Strategies for A collaborative nine-centre study was designed to follow the routes to identification of all chil;dren up to the age of seven years, newly diagnosed with permanent hearing impairment (>50 dBHL) during the period 1993-94. Ages of identification were compared with the standards set by the National Deaf Children's Society (NDCS), ascertaining whether these targets could be achieved with current service provision. Of the 126 children identified, 104 had congenital sensori-neural hearing loss: 19% were identified by the age of 6 months and 39% by their first year. These results compare unfavourably with the NDCS targets of 40% and 80% respectively and point to the need for modifications of current practice.