Abstract
Objective: To investigate working memory (WM), phonological skills, lexical skills, and reading comprehension in adults with Usher syndrome type 2 (USH2).Design: The participants performed tests of phonological processing, lexical access, WM, and reading comprehension. The design of the test situation and tests was specifically considered for use with persons with low vision in combination with hearing impairment. The performance of the group with USH2 on the different cognitive measures was compared to that of a matched control group with normal hearing and vision (NVH).Study Sample: Thirteen participants with USH2 aged 21–60 years and a control group of 10 individuals with NVH, matched on age and level of education.Results: The group with USH2 displayed significantly lower performance on tests of phonological processing, and on measures requiring both fast visual judgment and phonological processing. There was a larger variation in performance among the individuals with USH2 than in the matched control group.Conclusion: The performance of the group with USH2 indicated similar problems with phonological processing skills and phonological WM as in individuals with long-term hearing loss. The group with USH2 also had significantly longer reaction times, indicating that processing of visual stimuli is difficult due to the visual impairment. These findings point toward the difficulties in accessing information that persons with USH2 experience, and could be part of the explanation of why individuals with USH2 report high levels of fatigue and feelings of stress (Wahlqvist et al., 2013).
Highlights
Impairment in both hearing and vision, deafblindness, causes major reduction in intake of sensory information from the environment
While there was no significant difference between groups in performance on the test of reading comprehension in this study, three individuals with Usher syndrome type 2 (USH2) performed at or below more than 1 SD of both groups’ means
On tests of phonological processing and phonological working memory (WM) performance level was significantly lower in the group with USH2 than in the control group with normal hearing and vision (NHV)
Summary
Impairment in both hearing and vision, deafblindness, causes major reduction in intake of sensory information from the environment. There can be several etiologies behind deafblindness, but Usher syndrome is one of the most common causes (Pennings, 2004; Sadeghi, 2005). In Sweden the clinically defined types 1 and 2 of the syndrome are most common, and type 3 is unusual (Sadeghi et al, 2004b). Individuals with USH2 have a severely limited, central visual field and suffer visual deficts such as poor photo- and contrast sensitivity due to the retinal disorder retinitis pigmentosa (RP; Sadeghi et al, 2004b; van Wijk et al, 2004). The first symptoms (poor contrast sensitivity and night blindness) of RP, are typically evident during ages 5–10 years but is commonly diagnosed in the late teens in individuals with USH2 (Sadeghi et al, 2004b). The degenerative process in the retina typically stabilizes at about 40–50 years of age (Sadeghi et al, 2004b)
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