Meta-analyses of cognitive functions in early-treated adults with phenylketonuria

Our study estimated size of impairment for different cognitive functions in early-treated adults with PKU (AwPKU) by combining literature results in a meta-analytic way. We analysed a large set of functions (N=19), each probed by different measures (average=12). Data were extracted from 26 PKU groups and matched controls, with 757 AwPKU contributing 220 measures. Effect sizes (ESs) were computed using Glass' ∆ where differences in performance between clinical/PKU and control groups are standardized using the mean and standard deviation of the control groups. Significance was assessed using 'measures nested within independent PKU groups' as a random factor. The weighted Glass' ∆ was -.44 for all functions taken together, and -.60 for IQ, both highly significant. Separate, significant impairments were found for most functions, but with great variability (ESs from -1.02 to -.18). The most severe impairments were in reasoning, visual-spatial attention speed, sustained attention, visuo-motor control, and flexibility. Effect sizes were larger with speed than accuracy measures, and with visuo-spatial than verbal stimuli. Results show a specific PKU profile that needs consideration when monitoring the disease

Reading skills in adolescents with cochlear implants

Reading outcomes following cochlear implantation are extremely variable. Understanding the factors underlying this variability is of clinical importance, and research has concentrated on how children with cochlear implants encode and process information. Existing research has focussed on younger children; the present study investigated the relationship between short-term and working memory and reading outcomes in adolescents with cochlear implants. A cross-sectional comparative design was selected. 18 adolescents with cochlear implants were recruited from a cochlear implant programme in London. All participants completed neuropsycho- logical measures of reading, phonological and visual working memory and non-verbal IQ, and scores were compared with normative data for hearing children: Significantly more adolescents with cochlear implants had below average reading outcomes than would be expected. Reading Composite scores were positively correlated with performance on a phonological working memory task (WISe-IV Digit Span). This correlation was not significantly different from manual norms for normal-hearing children. These findings show that the difference in reading attainment between children with cochlear implants and their hearing peers continues into adolescence. In keeping with previous research, outcomes were highly variable. Reading scores in adolescents are significantly correlated with Digit Span scores, consistent with findings in younger children. The size of this correlation did not differ significantly from normative data for hearing children. The results of this study stress the importance of careful, individualized assessments of reading for children with cochlear implants, and indicate that monitoring should continue into adolescence. The effect of interventions designed to improve or compensate for limited working memory capacity on reading outcomes is an important area for future research.EThOS - Electronic Theses Online ServiceGBUnited Kingdo