1. Theory-of-mind In Individuals
With Acquired Deafblindness
Is Affected By Social Relations
Wednesday, 14 August 2019, ID 69
Dr. Hans-Erik Frölander
Lic. Psychologist, PhD
The National Agency for Special needs
Education and Schools (SPSM)
Audiological Research Centre, Örebro
University Hospital
School of Health Sciences, Faculty of
Medicine and Health, Örebro University

2. Homo Sapiens
• Social primates
• Standing on legs, using hands to grasp
• Tactile sense of importance to understand.
• Storytelling
• Attached, empatical and sympatical – feeling sorry for others
• Learning by observing and imitating
• Habits – culture, murder and vendettas

3. Consequences of Acquired
Deafblindness
• Young adults are bonding
• Seeking significant others, settle down, establish a family, get
employed
• Daily life and self-reliance affected
• Participation depends on help from others and the society

4. Usher syndrome type 2 (USH2)
A congenital non-progressive moderate to severe hearingloss
Retinitis Pigmentosa (RP), onset in early teenage, a remaining
visual field of 10 degrees
No additional dysfunctions
Health problems

5. Developmental delays in USH2
• Speech-development in a proportion of individuals, due to the moderate
hearingloss
• The slow progression of visual aquity and visual field to a minor degre
affects psychological development
• Variability in vocabulary related to visual status, level of hearingloss, and
to Phonological Working memory (PhoWM)

6. Alström syndrome (AS)
Multisystemic ciliopathy, endocrine and metabolic diseases;
organ failure
Affecting prognosis and survival
Progressing sensorineural hearing impairment, moderate to
severe loss in 2nd decade
Retinitis pigmentosa (RP), Juvenile blindness

7. Developmental delays in AS
Early receptive language
Motor milestones
Learning

8. Atypical behavior in AS
Behavioral issues prevalent, e.g. Obsessive compulsive disorder
traits (OCD)
Difficulties to inhibit behavior
Increased frequency of Autism Spectrum disorders (ASD)

9. Theory-of-Mind (ToM) - the capacity to
impute mental states to self and to others
Deviations in ASD
Basic innate biological ability
Developed by experience of interplay, attention to faces, motions
and gestures, attributing thoughts and feelings to expressions
Delay related to deafness and blindness - to deafblindness

10. ToM is developed through social relations
and communication
• Depth and range of social relations
• Communicative skills, e.g. to initiate and sustain interplay
• Verbal ability … to reason about mental states is required in
advanced ToM

11. The use of Executive functions (Efs)
• Sensory loss is challenging speech perception
• Focused attention required to perceive and process input:
- to suppress irrelevant information or noise and prepotent
responses (inhibition)
- to replace old information (updating)
EFs-capacity related to ToM performance in individuals with AS

12. General aims
• To explore and examine ToM in adults with acquired
deafblindness, and how ToM was related to sensory loss,
cognitive skills and characteristics of the social network

13. Participants with AS and USH
females
/tot. part.
6/12 4/6 2/6 4/13
Mean age 26.7 (6.5) 29.5 (6.9) 23.8 (5.1) 38.8 (12.7)
AS AS better AS poorer USH
VF Mean
1-5
4.70 4.67 4.83 3.54
VA Mean
1.0 – 0.0
0.04 0.04 0.02 0.45
Onset HL
(months)
8.9 (6.7) 5.8 (3.7) 12.4 (8.8)
Onset VL
(months)
8.1 (13.4) 7.7 (4.3) 0.6 (11.3)

14. Testbattery
• Vocabulary test
• Phonological Working memory test
• Cognitive spare capacity test
• Questionnaire covering communication and behaviour
• Social network – inventory
• Test of Advanced ToM

15. Happe´s Strange stories – test of
advanced ToM
• Persuation, social blunders & white lies
• ”Peter thinks his aunt looks silly in her new hat, but when she asks if he
likes it he answers that it is very nice “ Why does he say that?“
2 = reference to white lie or wanting to spare his aunt’s feelings
1 = more general reference to trait (he’s a nice boy), or relationship (he
likes his aunt)
0 = ref to irrelevant or incorrect facts/feelings (He likes the hat. He wants
to trick her)

16. Results
•The USH-group was outperformed by the nondisabled group, in
ToM
•The AS group was outperformed by both the USH-group and the
nondisabled group, in ToM
• Later onset of visual loss connected to a better ToM in AS
• Verbal ability and Efs predicted ToM performance in AS
• ToM was predicted by PhoWM-capacity in USH (in turn
negatively related to the duration of loss), and related to verbal
ability
• Pretendplay related to ToM development in AS

17. • The size of the social network correlated with ToM performance
in AS
• Many acquaintances to individuals with AS were professionals
or semiprofessionals
• The social networks of individuals with USH2 were smaller than
the networks of nondisabled individuals
• Familymembers and friends within organizations for individuals
with deafblindness common in networks of USH2-individuals
• Guidedogs regularly included in the social network in both the
AS and the USH2-group

18. Deafblindness – onset and degree off
loss and ToM
• General differences in visual capacity could explain differences
in ToM between individuals with AS and USH2
• Poorer visual field and acuity constituted individuals with poorer
ToM, mainly including individuals with AS.
• Early loss in AS is related to poorer ToM, by affecting interaction
negatively in sensitive periods of ToM development

19. The compensatory role of attention in challenged
communication, and consequences for ToM development
• That ToM delay related to verbal ability and EF capacity reflects the importance of
attention to compensate for loss, perceive and process social input
• Good updating capacity enables inferences of meaning from incompletely received
signals, contributing to verbal ability - of importance for ToM development
• Good inhibitory capacity enables sustained social interaction despite challenged
communication, ecually important for ToM development,
• Poor inhibition may cause manners, observed in some individuals with AS

20. The social network in AS
• Individuals with AS mainly established unequal contacts with
professionals and semiprofessionals, whereas the informal
network of friends on an ecqual level was more restricted
• AS individuals with fewer friends displayed poorer ToM
• Differences in ToM capacity seem to depend on prerequisites
for social interaction in childhood and youth

21. The social network in USH2
• Adults with USH2 mainly established friendshiprelations within
organizations for people with deafblindness, as did adults with
AS
• In contrast to individuals with AS, individuals with USH2 did not
address professionals or semiprofessionalas as close friends

22. • Individuals with USH mentioned family and siblings aside of
people within organizations, when asked about
friendshiprelations
• Anecdotal data revealed that the social network is diminishing
over time, familymembers, siblings and other once close
persons dissapear from the network.
• Guidedogs regerded as close friends

23. Comments about the social network
”I need support, but have no intention to develop relations to
professionals”
”It is strenuous to relate socially…”
”The jiggsaw of life is not easy to put togehter…”
”I have a few good friends, most of them within
organizations for individuals with deafblindness and their
relatives …”
”My family is my life…”
”I have no superficial social relations…”

24. Wolfs became dogs – mens best friend
• A mapping of the social network in AS and USH2 verify the
importance of the dogs in everyday-situations
• Social wolfes began following groups of man, 20 000 years ago
• Hunted together
• Through breed, dogs of different kind developed, e.g. the
guidedog

25. Summary of findings
• Early loss of vision in combination with a slower loss of hearing as in AS affect ToM development negatively
• Visual loss in AS affect early interaction negatively
• Loss of hearing in AS is associated to a delay in vocabulary development, negatively influencing ToM
development
• Variability in ToM was highly dependent on Working memory capacity and communicative skills.
• Efs have a mediating role in both AS and USH, compensating for loss
• Friendship-relations in childhood and adolescense are of importance to establish advanced ToM
• Professionals contribute to ontological security, but poor health is connected to unequal relations delaying ToM

26. In the eye of the observer –
a loopingeffect
• Social recognition is connected to primary relations but also to
participation in social activities
• Support is vital, but may contribute to development of an
identity of lacking ability and a perceived constant need of
support and care
• Respectful treatment turns to selfrespect, experience of
capability and a better selfesteem

27. Interventions
• Modify Efs with cognitive interventions
• Training my decrease behavioral issues and enhance capacity to direct attention and
compensate for sensory loss
• Address the specific role of EFs in efforts to improve the social participation for individuals
with deafblindness
• Support social interaction by implementing assistive technology
• Adapt social environments, use adequate hearingaids and tactile cues
• Expand the use of internet to support development of reciprocal social relations
• Encourage cooperation between users, semiprofessionals, professionals and scientists.

28. Future research
Theory-of-mind in adolecents with CHARGE and Ushers
syndrome in relation to communicative prerequisites, cognitive
abilities and social relationships

29. Hans-erik.frolander@spsm.se
• To the colleagues at the Resourcecenter for deafblind children, within the Swedish
Agency for Special Needs Education and Schools; Thanks for rewarding discussions and
cooperation
• To the colleagues at the Audiological Research Center at Örebro University Hospital,
thanks for support, feedback and valuable discussions.
• To ”Mogårds forskningsfond (MOFO)” thanks for the researchgrant from the foundation.
Without it the ongoing project would not be possible to conduct