Theory-of-mind in adolescents and young adults with Alström syndrome.
OBJECTIVE
The study focuses on theory-of-mind in adolescents and young adults with Alström syndrome (ALMS). ALMS, an autosomal recessive syndrome causes juvenile blindness, sensorineural hearing loss, cardiomyopathy, endocrinological disorders and metabolic dysfunction. Theory-of-mind (ToM) refers to the ability to impute mental states to one self and to others. Clinical observations have revealed an increased occurrence of deviances in mental state understanding in ALMS. In the present study ToM will be examined and related to working memory (WM), verbal ability and sensory loss.


METHODS
Twelve young individuals (16-37 years) with ALMS and 24 nondisabled individuals matched on age, gender and educational level participated. ToM was assessed by means of a multiple task that taxes the ability to understand thoughts and feelings of story characters'. WM was examined by means of a reading span task and verbal ability by means of a vocabulary test.


RESULTS
The ALMS group performed at significantly lower levels in ToM tasks and displayed a higher variability in performance than the control group. Individuals with ALMS and a relatively poor level performance provided fewer correct mental state inferences in ToM tasks than ALMS individuals with relatively higher performance levels. ALMS individuals with relatively high performance levels made as many correct inferences in ToM tasks as the control group, but their inferences were more often incomplete. Vocabulary skills and educational level, but not WM-capacity predicted ToM performance. Degree of deafblindness did not have an impact on ToM. Age of onset of visual loss but not hearing loss related to ToM.


CONCLUSIONS
The individuals with ALMS display a high degree of heterogeneity in terms of ToM, where some individuals reached performance levels comparable to nondisabled individuals. The results are discussed with respect to how cognitive and verbal abilities and factors related to the disability affect ToM.
Health Academy, Örebro University, Örebro SE 701 82, Sweden; Audiological Research Centre, Örebro University Hospital, Örebro SE 701 85, Sweden; The Swedish Institute for Disability Research, Linköping, Sweden; Research on Hearing and Deafness (HEAD) Graduate School, Linköping, Sweden. Electronic address: hans-erik.frolander@spsm.se.
Hans Erik
HE
Frölander
Health Academy, Örebro University, Örebro SE 701 82, Sweden; Audiological Research Centre, Örebro University Hospital, Örebro SE 701 85, Sweden; Department of Audiology, Örebro University Hospital, Örebro SE 701 85, Sweden; The Swedish Institute for Disability Research, Linköping, Sweden; The Linnaeus Centre HEAD, Linköping, Sweden.
Claes
C
Möller
The Jackson laboratory, Bar Harbor, ME, USA.
Jan D
JD
Marshall
Department of Behavioral Science and Learning, Linköping University, Linköping SE 581 83, Sweden; The Swedish Institute for Disability Research, Linköping, Sweden; The Linnaeus Centre HEAD, Linköping, Sweden.
Annette
A
Sundqvist
Health Academy, Örebro University, Örebro SE 701 82, Sweden; Audiological Research Centre, Örebro University Hospital, Örebro SE 701 85, Sweden; The Swedish Institute for Disability Research, Linköping, Sweden; Research on Hearing and Deafness (HEAD) Graduate School, Linköping, Sweden.
Berit
B
Rönnåsen
The Swedish National Expert Team for the Diagnoses of Deafblindness, National Resource Centre, Lund, SE 223 50, Sweden.
Lil
L
Falkensson
Department of Behavioral Science and Learning, Linköping University, Linköping SE 581 83, Sweden; The Swedish Institute for Disability Research, Linköping, Sweden; The Linnaeus Centre HEAD, Linköping, Sweden.
Björn
B
Lyxell
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D056769:Alstrom Syndrome / Q000175:diagnosis / Q000523:psychology*; D016022:Case-Control Studies; D003072:Cognition Disorders / Q000503:physiopathology; D004185:Disability Evaluation; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis* / Q000628:therapy; D006320:Hearing Tests / Q000379:methods; D006801:Humans; D008297:Male; D008570:Memory, Short-Term; D015999:Multivariate Analysis; D009483:Neuropsychological Tests; D012016:Reference Values; D012720:Severity of Illness Index; D013548:Sweden; D056345:Theory of Mind; D014825:Vocabulary; D055815:Young Adult
Alström syndrome (ALMS); Deafblindness; Dual sensory loss; Theory-of-mind; Verbal ability; Working memory