Electrophysiological differences and similarities in audiovisual speech processing in CI users with unilateral and bilateral hearing loss.

Hearing with a cochlear implant (CI) is limited compared to natural hearing. Although CI users may develop compensatory strategies, it is currently unknown whether these extend from auditory to visual functions, and whether compensatory strategies vary between different CI user groups. To better understand the experience-dependent contributions to multisensory plasticity in audiovisual speech perception, the current event-related potential (ERP) study presented syllables in auditory, visual, and audiovisual conditions to CI users with unilateral or bilateral hearing loss, as well as to normal-hearing (NH) controls. Behavioural results revealed shorter audiovisual response times compared to unisensory conditions for all groups. Multisensory integration was confirmed by electrical neuroimaging, including topographic and ERP source analysis, showing a visual modulation of the auditory-cortex response at N1 and P2 latency. However, CI users with bilateral hearing loss showed a distinct pattern of N1 topography, indicating a stronger visual impact on auditory speech processing compared to CI users with unilateral hearing loss and NH listeners. Furthermore, both CI user groups showed a delayed auditory-cortex activation and an additional recruitment of the visual cortex, and a better lip-reading ability compared to NH listeners. In sum, these results extend previous findings by showing distinct multisensory processes not only between NH listeners and CI users in general, but even between CI users with unilateral and bilateral hearing loss. However, the comparably enhanced lip-reading ability and visual-cortex activation in both CI user groups suggest that these visual improvements are evident regardless of the hearing status of the contralateral ear

The timecourse of multisensory speech processing in unilaterally stimulated cochlear implant users revealed by ERPs.

A cochlear implant (CI) is an auditory prosthesis which can partially restore the auditory function in patients with severe to profound hearing loss. However, this bionic device provides only limited auditory information, and CI patients may compensate for this limitation by means of a stronger interaction between the auditory and visual system. To better understand the electrophysiological correlates of audiovisual speech perception, the present study used electroencephalography (EEG) and a redundant target paradigm. Postlingually deafened CI users and normal-hearing (NH) listeners were compared in auditory, visual and audiovisual speech conditions. The behavioural results revealed multisensory integration for both groups, as indicated by shortened response times for the audiovisual as compared to the two unisensory conditions. The analysis of the N1 and P2 event-related potentials (ERPs), including topographic and source analyses, confirmed a multisensory effect for both groups and showed a cortical auditory response which was modulated by the simultaneous processing of the visual stimulus. Nevertheless, the CI users in particular revealed a distinct pattern of N1 topography, pointing to a strong visual impact on auditory speech processing. Apart from these condition effects, the results revealed ERP differences between CI users and NH listeners, not only in N1/P2 ERP topographies, but also in the cortical source configuration. When compared to the NH listeners, the CI users showed an additional activation in the visual cortex at N1 latency, which was positively correlated with CI experience, and a delayed auditory-cortex activation with a reversed, rightward functional lateralisation. In sum, our behavioural and ERP findings demonstrate a clear audiovisual benefit for both groups, and a CI-specific alteration in cortical activation at N1 latency when auditory and visual input is combined. These cortical alterations may reflect a compensatory strategy to overcome the limited CI input, which allows the CI users to improve the lip-reading skills and to approximate the behavioural performance of NH listeners in audiovisual speech conditions. Our results are clinically relevant, as they highlight the importance of assessing the CI outcome not only in auditory-only, but also in audiovisual speech conditions

Adult cognitive outcomes in phenylketonuria:explaining causes of variability beyond average Phe levels

OBJECTIVE: The objective was to deepen the understanding of the causes of individual variability in phenylketonuria (PKU) by investigating which metabolic variables are most important for predicting cognitive outcomes (Phe average vs Phe variation) and by assessing the risk of cognitive impairment associated with adopting a more relaxed approach to the diet than is currently recommended. METHOD: We analysed associations between metabolic and cognitive measures in a mixed sample of English and Italian early-treated adults with PKU (N = 56). Metabolic measures were collected through childhood, adolescence and adulthood; cognitive measures were collected in adulthood. Metabolic measures included average Phe levels (average of median values for each year in a given period) and average Phe variations (average yearly standard deviations). Cognition was measured with IQ and a battery of cognitive tasks. RESULTS: Phe variation was as important, if not more important, than Phe average in predicting adult outcomes and contributed independently. Phe variation was particularly detrimental in childhood. Together, childhood Phe variation and adult Phe average predicted around 40% of the variation in cognitive scores. Poor cognitive scores (> 1 SD from controls) occurred almost exclusively in individuals with poor metabolic control and the risk of poor scores was about 30% higher in individuals with Phe values exceeding recommended thresholds. CONCLUSIONS: Our results provide support for current European guidelines (average Phe value = < 360 μmol/l in childhood; = < 600 μmo/l from 12 years onwards), but they suggest an additional recommendation to maintain stable levels (possibly Phe SD = < 180 μmol/l throughout life). PUBLIC SIGNIFICANCE STATEMENTS: We investigated the relationship between how well people with phenylketonuria control blood Phe throughout their life and their ability to carry out cognitive tasks in adulthood. We found that avoiding blood Phe peaks was as important if not more important that maintaining average low Phe levels. This was particularly essential in childhood. We also found that blood Phe levels above recommended European guidelines was associated with around 30% increase in the risk of poor cognitive outcomes

Pre-attentive processing in children with early and continuously-treated PKU. Effects of concurrent Phe level and lifetime dietary control

Sixty-four children, aged 7 to 14 years, with early-treated PKU, were compared with control children on visual evoked potential (VEP) amplitudes and latencies and auditory mismatch negativity (MMN) amplitudes. It was further investigated whether indices of dietary control would be associated with these evoked potentials parameters. There were no significant differences between controls and children with PKU in VEP- and MMN-indices. However, higher lifetime Phe levels were, in varying degree and stronger than concurrent Phe level, related to increased N75 amplitudes, suggesting abnormalities in attention, and longer P110 latencies, indicating a reduction in speed of neural processing, possibly due to deficits in myelination or reduced dopamine levels in brain and retina. Similarly, higher lifetime Phe levels and Index of Dietary Control (IDC) were associated with decreased MMN amplitudes, suggesting a reduced ability to respond to stimulus change and poorer triggering of the frontally mediated attention switch. In summary, the present study in children with PKU investigated bottom-up information processing, i.e., triggered by external events, a fundamental prerequisite for the individual’s responsiveness to the outside world. Results provide evidence that quality of dietary control may affect the optimal development of these pre-attentive processes, and suggest the existence of windows of vulnerability to Phe exposure