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					     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   60.1%
 MR                            9.8%
 Learning disability           10.7%
 ADHD                          6.6%
 Blindness and Low Vision      3.9%
 Emotional                     1.7%
 Other                         12.1%

 From 2003 Gallaudet survey
          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%                73%
  70%
                                   66%
  60%

                 51%
  50%
                 46%

  40%                                                                           Additional
                                                                                Disabilities
                                                                                No Additional
  30%                                                                           Disabilities

  20%


  10%


   0%
          by 1 month       by 2 months       by 3 months       by 5 months   Within 1 year

Wiley, S., Meinzen-Derr, J., and Choo, D. in International Congress Series
Volume 1273, (November 2004) Cochlear Implants p 273-276.
            Risk factors for
         developmental delays
•   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.
   –   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         Range

Sits alone                6 months          5-8
Rolls from prone          6.4 months        4-10
Stands alone              11 months         9-16
Walks alone               11.7 months       9-17
Walks up stairs (rail)    16.1 months       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     5.5 months    4-8
Neat pincer grasp   8.9 months    7-12
Holds crayon well   11.2 months   8-15
Fine Motor: Grasp Patterns

 4 mths:       finger & palm
 5 mths:       thumb active
 7 mths:       raking grasp
 7-8 mths:     inferior pincer
 9-10 mths:    refined pincer
 By 2 years:   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:
       •   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
        • 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)
•   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 (praxis-
  related)
  –   4 rules explained all errors
  –   Reversal of sign path
  –   Unable to cross midline
  –   Non dominant hand inaccurate
             Case Example 2
• Occupational therapist observed:
  –   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)
      Lack   of   appropriate eye gaze
      Lack   of   warm, joyful expressions
      Lack   of   shared interest or enjoyment
      Lack   of   response to contextual cues
      Lack   of   response to name
      Lack   of   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
•   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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