title: Cognitive skills and reading in adults with Usher syndrome type 2.
abstract: OBJECTIVE
  To investigate working memory (WM), phonological skills, lexical skills, and reading comprehension in adults with Usher syndrome type 2 (USH2).
  
  
  METHODS
  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).
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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).
authors: Swedish Institute for Disability Research (SIDR) Linköping, Sweden ; Linnaeus Centre for Research on Hearing and Deafness (HEAD) Linköping, Sweden ; Department of Behavioral Sciences and Learning, Linköping University Linköping, Sweden.
authors: Cecilia
authors: C
authors: Henricson
authors: Linnaeus Centre for Research on Hearing and Deafness (HEAD) Linköping, Sweden ; Department of Behavioral Sciences and Learning, Linköping University Linköping, Sweden.
authors: Björn
authors: B
authors: Lidestam
authors: Swedish Institute for Disability Research (SIDR) Linköping, Sweden ; Linnaeus Centre for Research on Hearing and Deafness (HEAD) Linköping, Sweden ; Department of Behavioral Sciences and Learning, Linköping University Linköping, Sweden.
authors: Björn
authors: B
authors: Lyxell
authors: Swedish Institute for Disability Research (SIDR) Linköping, Sweden ; Audiological Research Centre, Örebro University Hospital Örebro, Sweden ; School of Medicine and Health, Örebro University Örebro, Sweden.
authors: Claes
authors: C
authors: Möller
keywords: Usher syndrome; deafblindness; lexical skill; phonological skill; reading; working memory