Abstract No. 1.1

Tuesday 5th September 2017 at 12:00-12:45

Title: Deafblindness – Theory-of-mind, cognitive functioning and social network in
Alström syndrome

Room: W2/Radiosalen

Presenter: Hans-Erik Frölander, The National Agency for Special Needs Education and Schools, Sweden

Co-presenters: Claes Möller1,2,3, Björn Lyxell 3.

1 School of Health and Medical Sciences, Örebro University, the Swedish Institute for Disability Research, Linköping, Sweden
2 Audiological Research Centre, Örebro University Hospital
3 Department of Behavioural Science and Learning, Linköping
Main focus: Both research and practice, Acquired deafblindness
Abstract: Purpose: The aim of this presented PhD-project has been to explore the
emergence and expression of Theory-of-mind (ToM) in challenging conditions. ToM refers
to the ability to understand thoughts and feelings of others. The challenge referred to is
acquired deafblindness.
The presentation deals with young adults with Alström syndrome (AS). AS causes a
severe progressive combined auditory and visual impairment, in addition to a multisystemic
pathology. To feature general consequences of dual sensory-loss and the
impact of health, individuals with Usher syndrome type 2 (USH2) were included in one
study. This deafblind-related group exhibits a slower rate of progression of visual loss
than the AS group and the syndrome is not multi-systemic, enabling conclusions also
about the impact of health. The main focus in this presentation is on the development of
ToM, and how this relates to communicative prerequisites, development of some
cognitive skills and social circumstances.
Methods: The design was quasi-experimental, with an exploratory focus. 12 individuals
with AS, and 13 individuals with USH2 were matched to 24 nondisabled individuals on
background variables as age, gender and educational level. Sensory functions were
measured. Information about communicative skills and social behaviour was obtained
from responses to a questionnaire. Advanced ToM was measured by a multiple task that
taxes the ability to understand thoughts and feelings of story characters´. Verbal ability
was assessed with a test of vocabulary. Executive functions (EFs) were measured by a
test of inhibition and updating. Working memory (WM) was measured by a test of serial
recall of non-words. Structured interviews were conducted with a social network inventory,
to measure the size of the social network
Results; The group of individuals with AS was outperformed by both the nondisabled
individuals and the individuals with USH2, in ToM-tasks. Individuals with AS further
displayed a significantly higher degree of heterogeneity in performance in this respect.
Some individuals with AS performed on equal level with nondisabled individuals. ToM
performance was predicted by verbal ability and EFs, whereas WM proved to be an
indirect predictor. A later onset of visual loss further characterized AS individuals with
better ToM The size of the social network by individuals with AS was smaller relative to
that of nondisabled individuals, and many of the acquaintances were professionals
working with individuals with AS (mainly health professionals). Amount of friends
correlated with ToM performance
Conclusions: Variability in ToM in the AS group, seem to be highly dependent upon
social prerequisites and communicative skills, in which EFs have a mediating role,
Training of EFs could support ToM development in children with AS, by improving the
prerequisites for social interaction. Implementation of assistive technology for optimal
development of verbal ability and to enhance participation in childhood is further required.
Assistive technology within different social environments is in addition required to
maintain reciprocal social relationships in adulthood.