Red Flags for
Developmental Delays in
Deaf/hoh Children
Susan Wiley, MD
Cincinnati Children’s Hospital Medical Center
Cincinnati, OH.
Mary Pat Moeller, PhD
Boys Town National Research Hospital
Omaha, Nebraska
• In the past 12 months, we have not had a significant
financial interest or other relationship with the
manufacturer(s) of the product(s) or provider(s) of
the service(s) that will be discussed in our
presentation.
• This presentation will not include discussion of
pharmaceuticals or devices that have not been
approved by the FDA or if you will be discussing
unapproved or "off-label" uses of pharmaceuticals or
devices.
Objectives
• To gain knowledge of the risk factors for
developmental delays in children who are
deaf/hoh.
• To be able to identify children with potential
additional developmental disabilities.
• To develop an intervention plan for
confirming and treating an additional
disability.
Developmental Screening
A brief assessment designed to identify
children who need more intensive
diagnosis or evaluation in order to improve
child health and well being.
Developmental Surveillance
• Surveillance = periodic assessments over time
• An on-going process (similar to growth curves).
• Screening tools used to enhance the surveillance
process.
• Brief, objective, validated test with broad
developmental focus.
• Performed at set points in time.
• Differentiate children with no concern from those
needing additional investigation.
Why does it matter?
• Identifying additional concerns early can
allow for more effective intervention
strategies.
• Screening for developmental concerns allows
for a pro-active approach to overall child
development.
• The age of identification of an additional
disability tends to be delayed in children who
are deaf/hoh.
Additional Disabilities in
Children with SNHL
No additional disabilities
MR
Learning disability
ADHD
Blindness and Low Vision
Emotional
Other
From 2003 Gallaudet survey
60.1%
9.8%
10.7%
6.6%
3.9%
1.7%
12.1%
Age of Identification
• Hearing can delay the identification of an
additional disability
– Autism is diagnosed 0.8 years later in children with
HL*
• An additional disability can delay the
identification and intervention for children who
are deaf/hoh.
*Mandell et al Pediatrics 2005:116:1480-1486
Identification to Amplification
Time Between Identification and Amplification
100%
92%
89%
90%
91%
83%
80%
77%
69%
70%
73%
66%
60%
51%
50%
46%
Additional
Disabilities
No Additional
Disabilities
40%
30%
20%
10%
0%
by 1 month
by 2 months
by 3 months
by 5 months
Wiley, S., Meinzen-Derr, J., and Choo, D. in International Congress Series
Volume 1273, (November 2004) Cochlear Implants p 273-276.
Within 1 year
Risk factors for
developmental delays
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Neonatal history (LBW, preemie, asphyxia, IVH)
Congenital infections
Meningitis
Environmental exposures (Pb)
Failure to thrive
Iron Deficiency Anemia
Maternal Substance Abuse
Environmental deprivation
Family history of learning difficulties, attentional
problems
Risk factors for developmental
delay in deaf/hoh
• Neonatal factors (prematurity, intraventricular
hemorrhage, NEC, prolonged ventilation)
• Symptomatic congenital CMV
• Bacterial meningitis
• Some syndromes
• Family history of learning difficulties, attentional
problems
Case Example
• 4 year old referred for lack of speech progress
despite appropriate amplification.
• Just told by audiologist that “his speech issues are
only ¼ due to hearing.”
• ID with conductive HL at 13 months of age due to
aural atresia (canal only), amplified within one month
of identification.
• SAT is in mild-moderate range with amplification in
speech banana.
• Normal pregnancy and neonatal history.
Case Example
• Early on had difficulties with feeding, taking bites
from food, drooling.
• Walked at 18 months of age.
• In a TC preschool setting. Auditory-language
comprehension skills age appropriate.
• Speech is difficult to understand and utterances are
2-3 words in length.
• He and his parents are quite frustrated due to
communication breakdowns.
• Eye contact never very good, but nice pretend play.
What would screening have
done?
• Multiple early warning signs including:
– Feeding difficulties
– Late walking without due cause
– Expressive skills always more significantly behind than
receptive skills.
• Parents now questioning what is wrong at the age of 4.
• Screening at regular intervals would have allowed
identification of concerns at earlier ages,
implementation of interventions, and perhaps less
anxiety at this time.
What did he need?
• Diagnosed with apraxia of speech and fine motor
apraxia, monitoring eye contact following
interventions
• Interventions such as
–
–
–
–
OT
PT
oral-motor stimulation
effective expressive communication system at earlier ages
• May have decreased current frustrations and parent’s
surprise of the problem.
Gross Motor
• Common misconception:
– Children who are deaf walk later because they can’t
hear.
• Children generally walk between 9-15 months of
age.
• Family patterns are common (all children
walking at 14-15 months of age).
Gross Motor
• 93% of Deaf/hoh children without vestibular
abnormalities have normal or above average motor
development*
• Deaf/HOH children walking later than 15 months
warrant an evaluation of why they are delayed.
• If children have significant vestibular abnormalities
(cochlear malformations: mondini deformities,
cochlear hypoplasia), this can impact balance for
walking.
*Lieberman et al American Annals of the Deaf 2005 149:281-289
Gross Motor
• If children have significant vision issues, or
Usher Type I, age of walking can be delayed.
• Children with CHARGE Syndrome almost
uniformly walk late and should receive PT
early on (vision and balance and tone
affected).
Motor Patterns in Cerebral
Palsy
• Children with cerebral palsy tend to have atypical motor
patterns, not just delayed milestones.
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Acquire handedness before a year of age
Cross midline to pick up a toy
Persistent fisting after 4 months of age
Log roll rather than segmental roll
Leg scissoring when picked up
Persistent primitive reflexes
Gross Motor Skill
Development
SKILL
Median age
Sits alone
Rolls from prone
Stands alone
Walks alone
Walks up stairs (rail)
6 months
6.4 months
11 months
11.7 months
16.1 months
Range
5-8
4-10
9-16
9-17
12-23
Fine Motor
• Fine motor development can mirror language
development, however there are no good physiologic
reasons why fine motor skills should be delayed in
children who are deaf/hoh.
• Abstract on children with cochlear implants noted gross
motor skills at chronological age, but fine motor skills
more consistent with language age equivalents.
Triological Society Abstract 708
www.triological.com/admin2/views.cfm?is=708
Fine Motor Skill
Development
SKILL
Median age
Range
Object transfer
Neat pincer grasp
Holds crayon well
5.5 months
8.9 months
11.2 months
4-8
7-12
8-15
Fine Motor: Grasp Patterns
4 mths:
5 mths:
7 mths:
7-8 mths:
9-10 mths:
By 2 years:
finger & palm
thumb active
raking grasp
inferior pincer
refined pincer
holds item in hand
with wrist supination
Problem Solving
• Although verbal problem solving can be
delayed in children who are deaf/hoh related
to language development, non-verbal
problem solving is typically preserved.
• In children under 3, non-verbal problem
solving typically relies on fine motor skill
development (stacking blocks, puzzles,
matching).
Problem Solving
• Speech perception in children with cochlear implants
with cognitive delays have shown delays in
comparison to children with CI and no cognitive
delays.
• 1 year post implant, the group of children with MR
(Mean IQ of 65) were performing at 65% of the
group with normal intelligence (Mean IQ of 100).
• At 2 years post implant, the group of children with
MR were performing within 70% of the group with
normal intelligence.
Yang et al IJPO 2004 68:1185-1188
Problem Solving
• Children with delays in non-verbal problem solving
may be at risk for on-going cognitive issues and learn
all skills at a slower rate.
• They often require more hands-on approach to
learning and repetition and rote strategies.
• Some children are perceived as having “memory”
problems as they seem to learn something and need
it re-taught.
Communication/Language
• Possible Red Flags (matter of degree)
• Slow learning rate in spite of strong intervention; gap CA/LA
widens
• Can be hard to differentiate from “limited opportunity” (device use,
parent involvement, personal resources, second language use,
quality of program, program access, response to Rx)
• Learning rate does not match “expectations” (i.e., in relation to
residual hearing or communication access)
• Lack of synchrony of auditory, speech, language development
Communication/Language
• Possible Red Flags: Young Child
– Need for extended processing time
– Qualitative differences in comprehension
• Over-reliance on comprehension strategies
– Extensive gaps between receptive & expressive language (in
either direction)
– May acquire basic vocabulary, but especially slow in acquiring:
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•
•
•
Relational concepts (perceptual vs. conceptual)
Diverse semantic classes
Question understanding
Basic grammatical relations
Communication/Language
• Possible Red Flags: Young Child
– Limited gesture development; motor imitation difficulties
– Difficulty combining modalities (receptive and/or expressive)…need for
chaining
• May have shifting modality preferences
– Problems with retention and generalization of learned information
– Word learning differences (cannot assume same associations,
classification skills)
– Auditory learners may focus on “gestalt” (giant words)
– Perseveration; Persistent echolalia in speech and/or sign; slow changes
from imitation to spontaneous productions
– Atypical play development
– Restricted range of pragmatic functions
Communication/Language
• Possible Red Flags: Preschool
• “Expectation” of non-understanding; weak meta-cognitive skills
• Difficulty attending to and integrating multiple pieces of information
• Atypical semantic errors (Daddy is holeing the ground with that big
fork!)
• Difficulties processing sequentially & planning common routines
• Formulation challenges in expressive language (word storage and
retrieval difficulties; sequential planning)
• Social difficulties
• In responding to cognitive-linguistic demands of classroom
• Child temperament: mismatch?
Communication/Language
• Possible Red Flags: Preschool
• Processing based on contextual, extra-linguistic or non-linguistic
cues for understanding (key words; predictions; global response
strategy)
• Unusual focus of attention
• Behavioral responses increase when language is challenging
• Difficulty responding to questions at varied levels of abstraction
&/or supports; tracking topics in discourse
Speech &/or Sign
Production
• Possible Red Flags:
• Limited repertoire of sound types or hand shapes which does not
expand with time and exposure
• Difficulties sequencing and coordinating movements
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•
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• Different or limited oral motor movement (open lip posture; difficulty with
automated lip closure; non precise tongue tip, lingual mobility)
• Difficulty coordinating voice and sign
Limited trunk stability; secondary reactions
Low intelligibility of word combinations
Drooling; asymmetry or one side weakness
Feeding/drinking issues; texture intolerance
Protracted jargon
Syllable complexity remains low
Syllable Complexity (MBL)
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•
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Vowels and glides = 1
True cv syllables /ba/ = 2
Mix of cv patterns /mida/ = 3
Average 50 utterances = MBL
Slow Transitions in Syllable Complexity
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)
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
Case Example 2
• Profoundly deaf, identified at 11 months
• Developmental history of hypotonia, tactile
defensiveness, motor overflow, poor eye contact, slow
learning rate, limited social interaction with peers
• Strong family support; optimal stimulation through sign
language
• Referred by preschool teacher due to concerns for low
intelligibility of sign productions
Case Example 2
• Diagnostic teaching with language specialist and
occupational therapist
• Analysis revealed rule based sign errors (praxisrelated)
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4 rules explained all errors
Reversal of sign path
Unable to cross midline
Non dominant hand inaccurate
Case Example 2
• Occupational therapist observed:
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Reduced proprioceptive perception
Weak bilateral coordination and motor planning
Reduced proximal trunk stability
ATNR present
Motor overflow and associated reactions
Avoidance of crossing midline
Case Example 2
• Successive approximation based on motor complexity
(break down-build up)
• Increase visual and perceptual salience
• Model matching side by side
• Target contrastive patterns
• Massed motor practice in functional contexts
• Presentation to facilitate midline crossing
• Guidance and support of motor plan
Case Example 2
• Motor based sign errors resolved in response to sensory
integration approach
• Persistent difficulties in socialization, attention and
compulsive behaviors
• Learned language in practiced contexts; did not
generalize to social use
• Strength in episodic memory used to promote social
interaction, symbolic play
• Team approach needed throughout school years
Systematic Observation of Red
Flags (SORF)
• 13 Red Flags for Autism Spectrum Disorder
Reciprocal Social Interaction (RSI)
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Lack
Lack
Lack
Lack
Lack
Lack
of
of
of
of
of
of
appropriate eye gaze
warm, joyful expressions
shared interest or enjoyment
response to contextual cues
response to name
coordination of nonverbal communication
Wetherby, Woods, Allen, Cleary, Dickinson & Lord, 2004
Systematic Observation of Red
Flags (SORF)
Communication (COM)
 Unusual prosody
 Lack of showing
 Lack of pointing
 Lack of communicative vocalizations with consonants
Wetherby, Woods, Allen, Cleary, Dickinson & Lord, 2004
Systematic Observation of Red
Flags (SORF)
Repetitive Behaviors & Restricted Interests (RBRI)
 Repetitive movements with objects
 Repetitive movements or posturing of body
 Lack of playing with a variety of toys
Wetherby, Woods, Allen, Cleary, Dickinson & Lord, 2004
Visual Impairments
• Deaf children are 2-3 times more likely to develop vision
problems than hearing peers (Guy et al, 2003)
– 15.3% incidence of refractive errors hearing children
– 39.1% in group of deaf children
• Usher Syndrome (3 types)
• Should have a full ophthalmologic evaluation
• Need regular vision evaluations
Characteristics of Students
with Multiple Disabilities
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Heterogeneity
History of struggles
Behavioral challenges
High need for adult attention
High need for task variation
Difficulty with generalization
Language and communication differences
Synergistic effects of combined challenges
Rules to Guide Instruction:
• Need for differentiated instruction and
expectations (will not learn the same material
in same time with same methods)
• Focus on the donut, not the hole
• Build communication one link at a time (task
analysis); Carefully address comprehension
• Celebrate successes great and small
Dr. T. Jones, Gallaudet University
Rules to Guide Instruction:
• If a dead man can do it, it is not an appropriate
objective
• May benefit from “break down-build up” in language
learning
• On-line analysis and revision is critical (Cycles of
hypothesize – observe – modify – observe –
hypothesize…)
• Help the child/family organize for learning
• Use meaningful contexts to make concepts explicit
Dr. T. Jones, Gallaudet University
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Red Flags for Developmental Delays in Deaf/hoh Children