Download Stress in families of children who are Deaf/HH

Developmental Conditions
Conditions
• Autism spectrum disorders
• Intellectual disability
• Sensory integration continuum
Contributors on Autism Spectrum Disorder
• Amy Szarkowski, PhD
• Susan Wiley
• Christine Yoshinaga-Itano,
PhD
• Deborah Mood, PhD
Potential Topics
• Section 1:Understand the rates and age of
diagnoses of ASD in Deaf/HH > general population
• Section 2: Recognize atypical development in
children with the dual diagnosis
• Section 3: Describe interventions used for children
with ASD which can be applied/adapted for children
who are D/HH
• Section 4: Improve family support and access to
resources
Section 1
• Understand the rates of ASD in Deaf/HH > general
population
• Understand the impact of the late age of identification of
ASD for children who are D/HH
Impact of UNHS/EHDI on language outcomes
• Universal Newborn Hearing Screening has allowed the earlier
identification of children with hearing loss
• This subsequently enhances age of intervention with data to support
language outcomes in the average range for early identified children
• With this improved language trajectory, we should be striving for earlier
identification of children with co-existing autism spectrum disorders,
however diagnosis can be challenging due to a lack recognition and of
validated assessment tools
Why it is important
• As ~ 4% of D/HH children have ASD, this
communication disorder further complicates
communication development
• Limited availability of skilled diagnosticians and of
appropriate interventions for needs for BOTH D/HH and
ASD
• Misdiagnosis can greatly impact outcomes in this group
of children
Rates of ASD
• Increasing over time, possibly related to:
– increased awareness
– broader diagnostic criteria
– study methods for monitoring rates
– changes in educational labels over time
– true increase
Epidemiology
CDC reported
Prevalence
Rates
Annual Survey reported
Prevalence Rates
(D/HH)
(general population)
2004-2005
1:125
1:111
2005-2006
1:110
1:94
2006-2007
-
1:53
2007-2008
1:88
1:81
2009-2010
1:68
1:59
The Disparity
• Later diagnosis of ASD in children who are D/HH is
common
• The average age of identification of ASD in children who
are D/HH often is far outside the goal we have to address
communication needs of young children with ASD
Age of Identification of ASD
• Median age of diagnosis in children without hearing
loss1:
– Autistic disorder: 48 months
– ASD/PDD: 53 months
– Asperger’s: 75 months
• For children with hearing loss, age of diagnosis of an
autism spectrum disorder tends to occur at older ages
(nearly 1 year later)2
1. CDC. MMWR (2012). 61 (SS03);1-19.
2. Mandell et al. Pediatrics (2005).116;1480-86
Meinzen-Derr, J et al “Autism Spectrum Disorders in Children who are Deaf or Hard of
Hearing” International Journal of Pediatric Otorhinolaryngology 2014 Jan;78(1):112-8
Lack of Clarity
• Data on association of severity of HL and ASD
have mixed results
– Impacted by data source, limitations in reporting
• Diagnostic clarity
Prevalence of Autism based on
Severity of Hearing Loss
Data is provided here from the Annual Survey of Deaf and Hard of Hearing Children and Youth Conducted by the Gallaudet Research Institute
Published in Szymanski, Brice, Lam and Hotto, 2012
The diagnosis: A dilemma?
Dilemma of underdiagnosis
• Is it the right one?
• Lack of diagnosis =
lack of early
intervention = lack of
possible gains later in
life
• Lack of appropriate
services
Dilemma of overdiagnosis
• Is it the right one?
• Supports and
intervention, if not
appropriate, may not
help symptoms nor
prepare families
• Schools and
professionals may not
accept the child
Diagnostic Challenges
Lack of
standardized
assessment tools
for Deaf/HH
Providers - trained
in deafness or
ASD, not many
trained in both
Providers trained in
ASD - may not
understand
complex needs of
D/HH children
(Communication,
development,
behaviors, etc.)
Children will act
differently in
different settings
(multiple sources of
information helpful)
Assessment Challenges When Using
Interpreters
May not
know/recognize
or convey atypical
language features
if noted
Potential to
disrupt rapport
necessary for
assessing social
reciprocity
The role of an
interpreter may
be unclear
Why is it important? ASD and HL
When there are two reasons for communication problems
and those problems span varying expertise (teachers of the
deaf, educators with expertise in autism)….
it can be very challenging to implement effective strategies
to develop communication and social interaction
Section 2
• Recognize atypical development in children with the dual
diagnosis
What is ASD: DSM Criteria changes in 2013…
• DSM V revisions
– Autism Spectrum Disorders
• Includes autism, Asperger Syndrome, PDD-NOS, and CDD
– Concentrates required features
• Social/communication deficits
• Restricted, repetitive patterns of behavior, interests, activities
– Addition of sensory criteria
– Increases specificity while maintaining sensitivity
• Important to distinguish spectrum from non-spectrum developmental disabilities
• Improves stability of diagnosis
DSM V Criteria- Social Communication
• Persistent deficits in social communication and social
interaction across contexts, not accounted for by general
developmental delays, manifested by all 3 of the following:
– Deficits in social-emotional reciprocity
– Deficits in nonverbal communicative behaviors
– Deficits in developing and maintaining relationships appropriate
to the developmental level
DSM V Criteria- Restricted/Repetitive Behaviors
• Restricted, repetitive patterns of behavior, interests, or
activities as manifested by at least 2 of the following:
– Stereotyped or repetitive speech, motor movements, or use of
objects
– Excessive adherence to routines
– Highly restricted, fixated interests that are abnormal in intensity or
focus
– Hyper- or hypo-reactivity to sensory input or unusual sensory
interests**
DSM V Criteria
• Symptoms must be present in early childhood
• Symptoms together limit and impair everyday functioning
• Symptoms not explained by intellectual disability or global
developmental delay
• Other diagnoses may be present (ie ADHD)
Severity Level
• Level 1- Requiring Support
– Without supports in place, deficits in social communication cause noticeable impairments
– Inflexibility of behavior causes significant interference with functioning in one or more
contexts
• Level 2- Requiring substantial support
– Marked deficits in verbal/nonverbal social communication
– Inflexibility results in causes significant interference with functioning in one or more
contexts.
• Level 3- Requiring very substantial support
– Severe deficits in verbal/nonverbal social communication
– Severe inflexibility; difficulty coping with change, or other restricted/repetitive behaviors
Pre-Linguistic Communication
Red Flags for Autism in Hearing
Children
Behavior
Barbaro, J. and Dissanayake, C. Prospective
Identification of Autism Spectrum Disorders in
Infancy and Toddlerhood Using Developmental
Surveillance: The Social Attention and
Communication Study. Journal of Developmental
& Behavioral Pediatrics. 31(5):376-385, June
2010.
8 months
12
months
18 months
Eye Contact
X
X
X
Turning to Name
Call
X
X
Imitation
24 months
X
Pointing
X
X
X
Gestures-Waving
X
X
X
X
X
Pretend Play
Showing Behaviors
Fail Criteria
X
Fail 2/2 Fail 3/4
Fail 3/4
Fail 3/5
“Red Flags” for a possible ASD in children
who are Deaf/HH
• Atypical preverbal communication
– poor eye contact, lack of pointing, poor orientation for communication, poor
joint attention
– delays in language acquisition beyond what one could expect based on
hearing loss/etiology/intervention history
• Atypical language features
– echolalia, palm rotation errors, persistent gesture use despite instruction in
formal sign and use of formal sign by others in the child’s environment (distinct
from home signs)
• Social difficulties
– failure to initiate/respond to peers when communication taken into
consideration, failure to recognize Deaf cultural norms, etc
• Repetitive behaviors/restricted interests
Deficits in
Social/Communicati
on
Deficits in
social/emotional
reciprocity
ASD



Atypical social approach
Difficulty with reciprocal
conversations
Reduced sharing of
affect /interests/
enjoyment and
limitations in social
interaction
Typically developing D/HH





Appropriate social smile
Appropriate eye contact
Engages others in their
environment with
integrated eye contact,
give/show behavior,
gestures, vocalizations
Imitate motor/vocal/signs
Appropriate joint attention
Deficits in
Social/Communication
Deficits in
social/emotional
reciprocity
D/HH + ASD






Reduced/absent social smile
Limited or inconsistent eye contact
Limited give/show behavior
Reduced sharing of affect
Difficulties with joint attention
Difficulty engaging in social conversation at
one’s language ability level
 Does not readily respond to name or culturally
appropriate attention getting measures
 Difficulty understanding others’ needs and
feelings or processing facial/signed emotion
cues
Deficits in
Social/Communicati
on
Deficits nonverbal
communicative
behaviors
ASD




Poorly integrated
verbal/nonverbal
behavior
Abnormalities in eye
contact and body
language
Limited facial
expressions/gestures
Difficulties in
understanding nonverbal
cues
Typically developing D/HH


Appropriate eye contact
Well integrated
gestures/eye
contact/vocalizations
 Wide range of facial
expressions; use of ASL
facial grammatical markers
 Will learn incidentally with
visual/auditory access, the
sequence of learning
language will follow typical
developmental norms
May have difficulties with
vocabulary, grammar, word
order, idiomatic expressions
and other aspects of verbal
communication
Deficits in
Social/Communication
and social interaction
Deficits nonverbal
communicative behaviors
D/HH + ASD












Limited gestures
Lack of pointing for shared enjoyment
Difficulty with choice making (e.g. pointing to make
choices)
Using others as objects for communication (e.g. hand as
tool)
Abnormal prosody of speech/sign
May demonstrated poorly integrated sign and spoken
language (if utilizing total communication approach)
Shifting of signing space below typical visual spatial space
Poor understanding/use of integrated ASL facial
grammatical features36
Gaps in acquisition of language and delays beyond
expected for hearing loss/intervention history/accessibility
of language
Limited spontaneous language use of words within child’s
repertoire for social communication (e.g. to comment,
share, request).
Limited range of facial expression or poorly coordinated
Difficulty grasping Deaf cultural norms (e.g. use of
attention getting strategies, entering/exiting conversations)
Language features of ASD in ASL
Features similar to oral language
but may present differently in
visual language
•
•
•
•
Palm reversals (Shield, 2014)
Pronoun avoidance vs. pronoun reversal (Shield, 2014)
Echolalia
Persistent use of individual’s own gestures rather than formally
instructed/used sign vs. neologisms (e.g. “red” vs. “ketchup”)
• Failure to use appropriate sign space
• Mixed results regarding use of facial aspects of sign language
and impact of ASD (Denmark, 2011,2014)
Deficits in
Social/Communicati
on
Deficits in developing
and maintaining
appropriate
relationships to the
developmental level
ASD




Difficulties building

relationships appropriate
to developmental level
Difficulty adjusting
behavior to context
Difficulty with imaginative 
play
Difficulty making friends
or limited interest in
people


Typically developing D/HH
Interested in people and
able to develop ageappropriate relationships
when communication is
accessible
Imaginative play follows
typical developmental
course (commensurate with
language and nonverbal IQ)
Flexible play
May prefer to control
conversation or play if
having troubles following
changes in conversation
based on language level or
in challenging listening
environments (when using
an auditory/oral approach)
Deficits in
Social/Communication
Deficits in developing and
maintaining appropriate
relationships appropriate to
the developmental level
D/HH + ASD
 Reduced shared enjoyment
 Delayed acquisition of symbolic play skills
inconsistent with nonverbal IQ
 Difficulty making and sustaining friendships
even when communication is accessible
 Unusual social overtures toward others (e.g.
backing into parents, grunting at peers, hitting
peers to initiate contact)
 Play is rigid and unimaginative
Restricted/Repetitive
Patterns of Behavior
Stereotyped or
repetitive speech,
motor movements, or
use of objects
ASD
Typically developing D/HH
 Stereotyped repetitive  Usually not
speech (i.e., echolalia,
demonstrated,
repetitive language
particularly in children
use, idiosyncratic
with well-established
phrases)
communication system
 Repetitive motor
and average nonverbal
movements
IQ
 Repetitive use of
 Echolalia can occur as a
objects
typical developmental
 Difficulties with
pattern, but should be for
transitions
a brief period of time
 You/I pronoun reversals
can occur as part of
typical development for
children with cooccurring visual
impairments
Restricted/Repetitive
Patterns of Behavior
Stereotyped or repetitive
speech, motor movements,
or use of objects
D/HH + ASD
 Echolalia in sign or spoken language
 Idiosyncratic gestures (e.g. persistent use of
made up gesture, distinct from home sign,
when formal sign taught/used)
 Palm rotation errors
 Difficulty with pronoun use (not using point
gesture to indicate others, fingerspelling name
instead of using pronoun/point, “you”/”I”
confusion in auditory/verbal children)7
 Rocking, twirling, flapping, spinning
 Highly repetitive play with objects (e.g.
persistence in lining up toys with significant
upset if disrupted)
Restricted/Repetitive
Patterns of Behavior
Excessive
adherence to
routines
ASD
 Verbal rituals
 Excessive resistance
to change
Typically developing D/HH
 Given an understanding/
communication, child will
change routines,
activities
 The resistance seen is
typical for all children or
due to comprehension
issues
 May struggle with
transitions if language
level doesn’t yet support
understanding first-then
concept
Restricted/Repetitive
Patterns of Behavior
Excessive adherence to
routines
D/HH + ASD
 May require parents/caretakers to say things in
exactly the same way
 Resistant to change, transitions are difficult
(these difficulties are beyond that anticipated
by language level)
 Significant upset when routines are disrupted
Restricted/Repetitive
Patterns of Behavior
Highly restricted,

fixated interests that
are abnormal in

intensity or focus
ASD
Typically developing D/HH
Preoccupation with a

particular object or topic
Highly unusual interest
for child’s developmental
age (i.e., ceiling fans)
Hyper-or hypo Unusual sensory
reactivity to sensory
interests (visual
input or unusual
inspection, smelling
interest in sensory
objects), fascination
aspects of
with lights/spinning
environment
objects
 Indifference or
oversensitivity to
pain/heat/cold
Usually not demonstrated or
very brief; able to move to
new toys, objects
 May have some atypical
sensory responses-or
hyper/hypo sensitivities,
these are more typically
differences with
vestibular processing;
less likely visual
inspection or persistent
tactile/olfactory
exploration of objects
Restricted/Repetitive
Patterns of Behavior
Highly restricted,
fixated interests that
are abnormal in
intensity or focus
Hyper-or hyporeactivity to sensory
input or unusual
interest in sensory
aspects of
environment
D/HH + ASD
 Repeated play with toy or object (often rather
than playing with a wide variety of toys)
 Play with toy for other than intended purpose
 Unusual interests of unusual intensity or for
child’s developmental age (e.g., perseveration on
street signs, ceiling fans, researching all
presidents of the US at age 3)
 With some DHH children, may see limited
response to amplification (seem to be more deaf
than you would expect based on their audiogram
or amplified responses)
 May show sensitivity to wearing amplification
 Hypo and hyper-sensitivities
 Sensory seeking behaviors (pushing head on
floor in inverted “V” position, repeatedly watching
blinds opening and closing, sniffing non-food
objects before use)
 Unusual reactions to environment unlikely related
to hearing loss (e.g., avoidance of
Other Diagnostic Considerations
Learning/
Communication:
• Intellectual
Disability
• Communication
Disorders
Behavioral
Conditions
• ADHD
• Anxiety disorder
• Obsessive
compulsive
disorder
• Sensory
integration
difficulties
Medical
Conditions
• Tourette’s
Syndrome
• Epilepsy
• Landau-Kleffner
and other
epileptiform
language
disorders(rare)
• Peripheral vision
cuts
• Benign
stereotypies
Section 3
• Interventions used for children with ASD which can be
applied/adapted for children who are D/HH
Interventions for Dual Diagnosis
• Evidence of effectiveness of interventions is lacking
(mostly case studies)
• It is reasonable to take interventions which have been
successful for hearing children to modify/adapt for
children who are deaf/HH
Implications of Communication on Interventions
for Dual Diagnosis
• Communication needed:
– joint attention, turn-taking, imitation, choice-making, play
• Communication modality can be complex
– Picture Exchange Communication System (PECS)
– Technology/Augmentative Communication
– Signs, gestures, spoken
Communication considerations
Multifaceted
approach to
language is
warranted
Language must
be accessible to
children who are
D/HH
Child’s means of
accessing
language
(receptive
language) may
differ from most
reliable means of
using language
(expressive
language)
Targeting core
symptoms of ASD
(responsiveness to
CI  may be ASD,
not failure of CI;
problems with ASL
 poor motor in
ASD)
Communication Considerations
• Features of ASD may impact language acquisition and should be
targeted as symptoms of ASD
– Joint attention deficits
– Reduced eye contact
– Motor imitation
• Not responding to sound may be a symptom of ASD rather than a failure
of technology or the child’s hearing – ASD treatment helpful
• Sensory sensitivities may lead to device resistance
– Treat with OT and desensitization protocol with consultation from behavioral
specialist
• Use of Assistive/Augmentative Communication (AAC)
Communication Strategies/Modalities
Interventions for Dual Diagnosis: Social
Communication
• Parent Training
– Fostering social communication skills, teaching parents about
importance of communication & language access in general
• Social Skills Groups
• Social Stories
• Who is the peer group?
– Learning cultural norms for both hearing and Deaf worlds
Review of Interventions for ASD
(in general population)
Warren et al, 2011, Pediatrics
• 4120 studies; 34 met inclusion criteria – 1 rated good – 10
fair – 23 poor
• Interventions thought to show improved outcomes in
cognition, adaptive functioning & early educational
attainment
Categories of Effective Intervention
(Warren et al., 2011, Pediatrics)
• Lovaas-based & Early Intensive Behavioral Intervention (EIBI)
– Discrete trial teaching (DTT)
– Widely known in the public as Applied Behavioral Analysis (ABA)
– Uses praise & reinforcers  transfer to naturalized settings
• Comprehensive Approaches - Children < 2 yr
– Early Start Denver Model  ABA techniques in a functional
developmental framework, sensitive to developmental sequence,
positive, affect-based relationship
• 2 yrs enrolled – significant cognitive & language gains
• Must be “implemented with fidelity” and supervision
• Parent Training
– Best at promoting social communication & language; less impact
on child’s IQ
Implementation of Interventions
Children with ASD who are D/HH
• Lovaas/Early Intensive Behavioral Intervention
– Direct teaching (breaking down a task and building the skill).
– Generalization of skills learned
– Finding appropriate motivators, rewards
• Comprehensive, developmental approaches
– “What is ASD, what is hearing loss?”
– Promoting interactions with typical peers – more challenges?
– Begins early (12-18 mo.) – Delayed diagnosis of ASD in D/hh
population may make this challenging?
Section 4
• Improve family support and access to resources
Focus Group: Parents
Objective: To gain an understanding of the
experience of families with children who have PHL
with a co-existing ASD.
• Recruited families from around the region based on children who
had received a dual diagnosis through CCHMC.
• Of the 16 families invited, five RSVP’d, but only three families
attended (2 of 4 parents were fathers)
Wiley, S, Gustafson, S, Rozniak, J. “Needs of Parents of Children who are
Deaf/Hard of Hearing with Autism Spectrum Disorder" The Journal of Deaf Studies
and Deaf Education 2014 Jan;19(1):40-9.
Children of Participants
Children varied in degree of hearing loss
and in ranges of severity of ASD
Characteristic
Child 1
Child 2
Child 3
Type and Degree
of HL
Unilateral mild to
moderate
Profound bilateral
Profound bilateral
Age of HL
identification
3 years
2 months by
UNHS
Birth due to
Syndrome
Type of
Amplification
Hearing Aid
Bilateral CI
Unilateral CI, no
longer using
Autism spectrum
label
PDD NOS
Autism
PDD NOS
Age of
Identification of
ASD
6 years
4 years
6 years
Children of Participants
Characteristic
Reminder
Current
Educational
Placement
Child 1
Child 2
Child 3
Unilateral,
PDD-NOS
Mainstream
setting with
IEP supports
Profound bilateral,
Autism
Social
Communication
Classroom
Profound bilateral,
PDD-NOS
Autism Classroom
Home-based EI
Home-based EI
Early Educational
Placements
Special Needs
Preschool
Communication
Mode
Aural/Oral
Autism specific
strategies used
Visual Schedules
Social Stories
School for the Deaf
with manual
approach in
apraxia program
Total
Communication:
writing, signing,
spoken language
Visual Schedules
PECS
Classroom for children with
motor disabilities
Special Needs Preschool
Developmental Disabilities
Classroom
Signing
Visual Schedules, PECS,
Dynavox
Topics of Interest
•
•
•
•
Experiences during diagnosis
Impact of dual-diagnosis on family
Useful resources
Wish-list
Discussion Themes
•
•
•
•
Diagnostic Experiences
Family Impact
Functional Skill Development
Academic considerations
Functional Skill Development
• Functional skills are a priority
– Struggle to balance development of social skills and academic
performance
– reported more interest in the development of their child’s social and
functional skills
“At some point, I’m going to look at him and say, it doesn’t
matter to me you know your multiplication table, I can’t take
you anywhere.
I can’t take you to the grocery store.”
• Access to social groups is a challenge
– Accessible social groups based on geographic location and
scheduling convenience are difficult to find within already busy
schedules
Academic Considerations
• Parents were happy with services that were provided in
school
– Concerned about potential of cuts in funding
• Limitations of Extended School Year
– Need continuation of the academic school year’s structure on a
year-round basis, particularly related to their child’s difficulties
with changing schedules and reliance on structured teaching
• Requested better training of professionals
– Ensure that professionals working with their children are
proficient in both PHL and ASD
Useful resources
• All families noted that schools were primary
resources for information and support.
– Important to have strong communication between school
and home
• Allows consistent follow-through for supporting their child’s
development at home
“It all depends on your teacher. We used to get
materials to bring home that we could work on with her
on vacations and breaks.
Now we aren’t getting any of that.”
Useful Resources
• Visual schedules
• Internet was a consistent resource used by all families to:
– Learn more about strategies to help their children
– Network with other families
– Connect with professionals to provide guidance with problems
“The internet has been a godsend, I couldn’t do
anything without it.”
Wish-list
• Ability to observe their child’s out-patient clinical therapy.
– E.g. video monitors
– Thought to be helpful in allowing them to observe interventions
to try at home with their child
• Someone that they can call/email with questions.
– Contact should have a knowledge base of both PHL and ASD
Strategies to support families
•
•
•
•
Do not delay raising concerns with families
Families may not understand what is/is not attributable to hearing loss
Start with strengths and discuss concerns in a kind, clear manner
Help families recognize symptoms of ASD are broad: filter what does/does
not apply to their child
• Be able to help families identify resources for seeking diagnostic clarification
and support
• Connect with other families with children with similar strengths/needs
Thoughts
• Collaboration
– Professionals with experience in ASD, specific subpopulation
– OT, Behavior specialists
• Creativity
– Think from the perspective of the child
– Adapt known strategies to meet all of a child’s needs
• Peer exposure
– Define peer group, may need a variety of peer groups
for different skills
How to families experience compare to those
families with ASD or those families with children
who are D/HH?
• Having a child with ASD has been linked with elevated levels of
parental stress
• Families of children with the dual diagnosis have described
challenges in accessing appropriate services for their child’s unique
needs
• Stress in families with children who are Deaf/HH with an ASD has
not been reported in the literature
Wiley S, Meinzen-Derr J, Hunter L, Hudock R, Murphy D, Bentley K, Williams T.
Understanding the Needs of Families of Children who are Deaf/Hard of Hearing with
an Autism Spectrum Disorder. Journal of the American Academy of Audiology 2017 (in
press).
Background: Stress in families of children
who are Deaf/HH
• In general, families felt positive about identifying a hearing
loss at a young age
• Pressures related to achieving based on a “time-table”
• Families desire to avoid abnormal development and had
greater dissatisfaction when it wasn’t clear how to best
intervene for their child’s needs
• Families desire family-to-family support as well as receiving
family-centered practice
Young, A and Tattersall, H. (2007) Journal of Deaf Studies and Deaf Education 12:2 209-220.
McCracken, W, Young, A, Tattersall, H. (2008) Ear and Hearing 29;54–64.
Background: Stress in families of children
who are Deaf/HH
• Using the Pediatric hearing impairment caregiver experience
(PHICE), indicated parental stress changes over time
• At young ages/soon after identification, higher levels of stress
around healthcare
• Older ages, higher stress around educational needs
• Communication needs was a common stress across
ages/duration since identification
Meinzen-Derr, J, et al. International Journal of Pediatric Otorhinolaryngology 2008 72:1693-1703.
Background: Stress in families of children
with ASD
• Parents of children with ASD experience large amounts of
stress that may persist throughout a lifetime of caring for
their child
• Parents of children with ASD experience levels of stress that
are higher than levels experienced by parents of other
children
• Stress may result from child characteristics such as behavior
problems or indirect sources and outcomes related to this
stress such as marital strain, or increased anxiety,
depression, or social isolation
Baker-Ericzen, MJ, et al (2005). Research & Practice for Persons with Severe Disabilities 30(4), 194-204.
Dumas, J.E et al (1991). Exceptionality 2, 97-110.
Konstantareas, MM, & Homatidis, S. (1989). Journal of Child Psychology and Psychiatry, 30(3), 459-470.
Background
• The prevalence rate of autism spectrum disorders (ASD)
in children who are Deaf/HH is approximately 4-7%, a
much higher rate than the general population
• There has been minimal research on this group of
children and families
Szymanski, CA, et al (2012). Journal of Autism Developmental Disorder, 42, 2027-2037.
Jure, R et al (1991). Developmental Medicine and Child Neurology, 33, 1062–72.
Background Deaf/HH and ASD
• Beals’ (one family) experience with EI
– feeling pulled between communication modalities (oral vs
sign) with deaf professionals
– lack of focus and concrete guidance from ASD
professionals
• In a targeted interview of 4 families,
– families felt shuffled between providers (deaf/HH and ASD),
– needed to focus more on behavioral needs
– experienced a lack of sign language environment within
ASD programming
Beals, K. (2004) Infants and Young Children, 17, 284-290.
Myck-Wayne, J, et al (2011) Am Ann Deaf, 156(4), 379-390.
Wiley, S et al The Journal of Deaf Studies and Deaf Education 2014 Jan;19(1):40-9.
Study Objective
• To compare the experiences of stress of 3 groups of
families:
– Families of children who are Deaf/HH
– Families of children with an ASD
– Families of children who are Deaf/HH with an ASD
Methods
• Participants were identified through clinical and research
registries
• 24 families within each category were mailed packets of
questionnaires focused on demographic characteristics
and parenting stress measures
• The study was approved by the Institutional Review Board
Questionnaires
• Qualitative questionnaire about supports were designed
based on prior questions from a focus group
Stress Questionnaires specific to Deaf/HH:
• The Pediatric Hearing Impairment; Caregiver Experience
(PHICE) was developed and validated by a pediatric
otolaryngology fellow at CCHMC
• Includes domains of communication, education, emotional
well being, equipment, financial, healthcare, social, and
support
Meinzen-Derr, J et al. International Journal of Pediatric Otorhinolaryngology 2008 72:1693-1703.
Questionnaires
Broad-based parental stress questionnaire
• The Parenting Stress Index-4th Edition (PSI)
• Includes Domains of Parent Characteristics and Child
Characteristics.
• Within the Parent Domain there are seven subscales:
Depression, Attachment, Restriction of Role, Sense of
Competence, Social Isolation, Relationship with Spouse, and
Parent Health.
• Within the Child Domain, there are six subscales: Adaptability,
Acceptability, Demandingness, Mood,
Distractibility/Hyperactivity, and Reinforces Parent
Abidin, R. R. (1995). Parenting Stress Index: Professional manual. Odessa: Psychological
Assessment Resources, Inc.
Child Age at Study (range)
Number of Children in
Family (median/range)
Level of Parent Education
High School
Some College
Completed College
Graduate School
Dual
Diagnosis
(n=6)
Deaf/HH
Alone
(n=4)
ASD
Alone
(n=3)
7-20 yrs
11-17 yrs
9-12 yrs
2
(1-4)
3
(2-4)
1
(1-2)
0
1
3
2
1
1
0
2
0
0
2
1
1
1
0
1
2
1
0
1
0
0
2
1
0
0
2
0
0
1
Household Income
<20,000
20,000-29,000
30,000-39,000
50,000-59,000
70-79,000
>80,000
Child Age at Study (range)
Degree of Hearing Loss
Unilateral/Mild
Moderate-Severe
Profound
Dual
Diagnosis
(n=6)
Deaf/HH
Alone
(n=4)
ASD
Alone
(n=3)
7-20 yrs
11-17 yrs
9-12 yrs
N/A
2
0
4
1
1
2
Oral
Oral/Signing
Oral/Behavior
Signing/Behavior
2
1
0
2
4
0
0
0
2
0
1
0
Educational Setting
Fully Mainstreamed
Partially Mainstreamed
Self-Contained
1
4
1
4
0
0
1
2
0
Communication Mode
Results: PSI
• Compared to the Deaf/HH only group, the dual diagnosis
group had higher median Total Stress (41.5 vs 58.5, p=0.02)
and higher Child Domain scores (43 vs 60, p=0.02).
• The dual diagnosis group was similar to the ASD only group
in reporting stress
• Total scores were above the 85th percentile in 38% of the
families (All were dual diagnosis or ASD respondents only)
• The deaf/HH only group did not have clinically elevated
stress scores in any domains
PSI
Child Domain
Parent Domain
Results: PHICE
• Families of children with the dual diagnosis had higher
median scores on the PHICE than families of children who
were solely Deaf/HH (total score 150.5 vs 179.5, p=0.08)
• Only two families reported high levels of stress in the
communication domain, 1 dual diagnosis group and 1 in
deaf/HH
In the Dual Diagnosis Group:
• 50% noted high levels of stress about their child’s future
• 33% worried about not doing enough for their child and not
attending to other family needs
• 33% indicated high levels of stress related to childcare
Deaf/HH Only Qualitative Questionnaire
How has the diagnosis in your child impacted your family?
• The diagnosis was heartbreaking
• Major reorganization of the family, needed to understand
navigating therapy, billing, sign language
• Diagnosis was difficult for some members of our family
• We needed a safety plan, including changing where our child sat in
the car
Where do you find support
• Family (x2)
• School (x2)
• Friends
• Internet
• Books
Deaf/HH Only Qualitative Questionnaire
How has the diagnosis impacted communication decisions
and strategies?
• We sent our child to an oral school
• The hearing loss turns a non-decision situation into a
situation. We chose a cochlear implant route.
• Our child has troubles in loud settings (i.e. cafeteria), making
friends
What resources/supports have you found helpful?
• Early Intervention supports (x2)
• FM system
• Bureau of Children of Medical Handicaps (State Title V
program)
Deaf/HH Only Qualitative Questionnaire
What is on your wishlist?
• I have gotten my wish, for him to talk
• Transition successfully to high school and increase his
circle of friends
• Increased ability to socialize, make friends
• Hear sounds for spelling words
• Waterproof Hearing Aid
• Better understanding of school system/professionals
related to HL
ASD Only Qualitative Questionnaire
How has the diagnosis in your child impacted your family?
• We don’t take things for granted
• Increased reliance on routines, impact of therapy appointments
• Change in relationship with spouse
Where do you find support
• Family (x2)
• Therapists (x2)
• Friends
• Reading
ASD Only Qualitative Questionnaire
What resources/supports have you found helpful?
• Speech and OT
• Using picture schedules
• Accessing biomedical/DAN supports
What is on your wishlist?
• For people not to judge and be more understanding
• For my child to become more social, social skills,
independence
• For my child to become independent and happy
Deaf/HH + ASD: Qualitative Questionnaire
How has the diagnosis in your child impacted your family?
• Physical and emotional toll
• Challenges in getting school to recognize both issues
• Parental feeling of social isolation from friends
• Frustration with lack of progress
• The family moved to be closer to services for child
Where do you find support
• Family (x4)
• Friends (x3)
• School
• Challenger league
Deaf/HH + ASD Qualitative Questionnaire
How has the diagnosis impacted communication
decisions and strategies?
• Signs (child with cochlear implant)
• Always looking for communication strategy (i.e. I-pad)
• My child doesn’t remember much that has been taught
• The dual diagnosis complicates sign language, we try all
tools and follow his lead
What resources/supports have you found helpful?
• School (x2)
• Trial and Error
• I don’t know anyone else with a child with a dual diagnosis
• ARC/Developmental Disability Services
Deaf/HH + ASD Qualitative Questionnaire
What is on your wishlist?
• Acceptance, tolerance by others
• Independence, healthy, happy, have a job
• To be able to communicate and function in a
regular class
• Finding an educational setting which can meet all
of his needs
• Finding a cause of the issue and fixing it
Results: Qualitative Questionnaire
Common Themes:
• Families from all groups described accessing supports from
families, friends, therapists, and schools
• Families from all groups also wanted their child to achieve
independence and happiness
Unique Themes:
• Families of children with the dual diagnosis tended to
describe more challenges with feelings of isolation, broader
ramifications, and communication needs
• Families of children with the dual diagnosis and ASD only
described a desire for broader acceptance and understanding
of their child’s needs by others
Conclusions
• Families of children with the dual diagnosis of Deaf/HH
and ASD report higher levels of overall stress than
families of children with Deaf/HH alone
• A common concern among all families related to
worries about their child’s future
• Although limited by sample size (response rate of 18%),
this exploratory study suggests a need to assess stress
and more effectively support families with children with
a dual diagnosis
Family Resources
• Seminars in Speech/Language special edition devoted to ASD among children who are
D/HH (November, 2014, vol 4)
– https://www.thieme-connect.com/products/ejournals/issue/10.1055/s-004-27930
• Gallaudet Odyssey special editions re: deafness/autism
– www.gallaudet.edu/documents/clerc/odyssey-2008-v9i1.pdf and
www.gallaudet.edu/Images/Clerc/.../Odyssey_SPR_2012_Szymanski.pdf
• Deafness and Family Communication Center of the Department of Child and Adolescent
Psychiatry- Children’s Hospital of Philadelphia http://www.raisingdeafkids.org/special/autism/
• Colorado Hands and Voices- Deaf Plus
http://www.cohandsandvoices.org/plus/index.html
- Autism Society http://www.autism-society.org/
Helpful Readings
• Odyssey: New Directions in Deaf Education: Autism
Issue
• Spring/Summer 2008; Volume 9: Issue 1
• http://www.gallaudet.edu/documents/clerc/odyssey-2008v9i1.pdf
Literature Review: General Articles
• Mandell et al Factors associated with age of diagnosis among children with autism
spectrum disorders. Pediatrics 2005:116:1480-1486.
• Jure, R, Rapin, I and Tuchman, R Hearing-impaired autistic children. Dev Med
Child Neurol 1991 33(12):1062-72.
• Rosenhall et al Autism and Hearing Loss. J Autism Dev Disord 1999 29(5):349-57.
• Worley, J, Matson, J, Kozlowski, A. “The effects of hearing impairment on
symptoms of autism in toddlers” Developmental Neurorehabilitation, 2011 (14)
171–176.
• Szymanski, C, Brice, P, Lam, K, Gotto, S. Deaf Children with Autism Spectrum
Disorders J Autism Dev Disord 2012 e-pub ahead of print Jan
Literature Review
• Gense and Gense: Autism and Deafness Workshop OCALLI
2002, 2005, 2007
• Guarinello et al Deafness and Attention in Deaf Children.
American Annals of the Deaf 2007: 151:5:499-507.
• Peterson, C, Wellman, H, Liu, D. “Steps in Theory-of-Mind
Development for Children With Deafness or Autism” Child
Development 2005 (76) 502-517.
Literature Review: Assessment
• Johnson, K, DesJardin, J, Barker, D, Quittner, A, Winter, M. “Assessing Joint Attention and
Symbolic Play in Children With Cochlear Implants and Multiple Disabilities: Two Case
Studies” Otology and Neurotology 2008 (29) 246-250.
• Roper, et. al Co-occurrence of autism and deafness: diagnostic considerations. Autism 2003
7(3): 245-253.
• Hoevenaars-van den Boom, MAA, Antonissen, ACFM, Knoors, H, Vervloed, MPJ
“Differentiating characteristics of deafblindness and autism in people with congenital
deafblindness and profound intellectual disability” Journal of Intellectual Disabilities
Research 2009 (53) 548-558.
Literature Review: Intervention Strategies
• Garcia, R and Turk J “The Applicability of Webster-Stratton Parenting Programmes to Deaf
Children with Emotional and Behavioural Problems, and Autism, and Their Families:
Annotation and Case Report of a Child with Autistic Spectrum Disorder” Clinical Child
Psychology and Psychiatry 2007 (12) 125-136.
• Malandraki, G, Okalidou, A “The Application of PECS in a Deaf Child With Autism : A Case
Study” Focus Autism Other Dev Disabl 2007 22: 23.
Literature Review:
Outcomes Studies
• Donaldson, et. al. Measuring progress in children
with autism spectrum disorder who have cochlear
implants. Archives of Otolaryngology Head and
Neck Surgery 2004:130:666-671.
Literature Review
• Gal, E, Dyck, M, Passmore, A. “Relationships Between Stereotyped Movements and
Sensory Processing Disorders in Children With and Without Developmental or Sensory
Disorders” American Journal of Occupational Therapy 2010 (64) 453-461.
• Maljaars, JPW, Noens, ILJ, Scholte, EM, Verpoorten, RAW, vanBerckelaer-Onnes, IA.
“Visual local and global processing in low-functioning deaf individuals with and without
autism spectrum disorder” Journal of Intellectual Disability Research 2011 (55) 95-105.
Literature Review:
Family Perspectives
• Beals, K “Early Intervention in Deafness and Autism: One Family’s
Experiences, Reflections, Recommendations Infants and Young Children
2004 (17) 284-290.
• Myck-Wayne, J, Robingon, S, Henson, E. Serving and Supporting Young
Children with a Dual Diagnosis of Hearing Loss and Autism: The Stories of 4
Families. American Annals of the Deaf 2011 156:4, 379-390.
Intellectual Disability
• Delays in problem-solving AND adaptive functioning
Definition
• Limitation in cognitive AND adaptive functioning.
• At or below 2 standard deviations from the mean.
• Classifications
– Mild
50-70
– Moderate 35-49
– Severe
20-34
– Profound <20
Cognitive Functioning
• Assessed via IQ testing.
– Stanford Binet (not suggested for children who are D/HH)
– Weschler Intelligence Scale for Children
• Performance usually stabilizes around 6 years of age.
• IQ tests were initially developed to predict who would do well in
school.
• IQ tests have performance (visual-perceptual) tasks and verbal
(language based) tasks.
Etiology
• Known in 43-70% of children with severe intellectual disability
• Known in 20-24% of children with mild intellectual disability
Etiology
Mild ID
Severe ID
Prenatal
7-23%
25-55%
Perinatal
4-18%
10-15%
Postnatal
2-4%
7-10%
Chromosomal
4-8%
30%
Co-existing Conditions: General population
data
Condition
Mild ID
Severe ID
Epilepsy
4-7%
20-32%
Cerebral Palsy
6-8%
30%
Sensory deficits
2%
11%
Autism Spectrum
Disorder
9-20% in all ID
categories
ID in 40-70% of
children with ASD
Considerations for children who are D/HH
• Non-Verbal IQ in general more appropriate
– Leiter International Performance Scale
– Test of Nonverbal Intelligence
– Performance domains on the Wechsler Intelligence Scales
• Verbal IQ is important
• Patterns can vary (high VIQ, low NVIQ)
Sensory Integration Dysfunction
Definition
• Sensory Integration is the organization of
sensation from the body and the
environment for use.
Types of Sensory Issues
• Sensory Overload (hyper-reactive)
– high arousal, inability to focus attention, negative affect,
impulsive or defensive action
• Hyporeaction
– manage input by withdrawing, easily over-looked
• Sensory Defensiveness
– hyper-vigilant to avoid sensory overload
Sensory Threshold
Point at which the summed sensory input
activates the CNS
High threshold
(hyporeactivity)
low threshold
(hyperreactivity)
Sensory Integration Disorder
• A way of conceptualizing certain behaviors.
• Body takes in all of the sensations (vision, hearing, tactile,
smell, taste), sends them up to the brain, integrates those
sensations and sends out a signal to respond to the input.
• Tactile sensitivities
• Auditory sensitivities
• Vestibular processing
Diagnosis
• Sensory profile questionnaire
• Look at patterns of sensory issues (movement,
vestibular, touch, auditory stimuli, visual stimuli,
taste/texture)
• Important to focus treatment on the pattern of
issues (one treatment protocol will not help every
child, must individualize programming)
Treatment
• Helping parents/professionals understand the
child’s responses
• Modify the environment for better “fit”
• Sensory diet
• Child-directed
• Make activities purposeful
SI and CI
• MAPping considerations
– CI 2005 poster on children with sensory integration issues (not
necessarily with autism) seemed to respond more appropriately to
lower thresholds.
– Re-MAPping improved their auditory outcomes.
• Therapy accommodations
– Co-treat with OT
– Sensory diet during therapy
Sensory vs Behavior?
• Sensory integration has many overlapping features with
– ADHD
– Anxiety/OCD
– Autism Spectrum Disorder
• Where does sensory end and behavior begin?
• (behavior slides in communication section)