Red Flags for Developmental Delays in DHH - Red Flags for

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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

• 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
• To gain knowledge of the risk factors for
  developmental delays in children who are

• To be able to identify children with potential
  additional developmental disabilities.

• To develop an intervention plan for
  confirming and treating an additional
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
• 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

• Screening for developmental concerns allows
  for a pro-active approach to overall child

• 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

• 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
  90%                                                                                91%


                                  69%                73%


  40%                                                                           Additional
                                                                                No Additional
  30%                                                                           Disabilities



          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
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
              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
• 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 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

• Children generally walk between 9-15 months of

• 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

• 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

*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
       Motor Patterns in Cerebral
• 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
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
           Fine Motor Skill
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,
             Problem Solving
• Speech perception in children with cochlear implants
  with cognitive delays have shown delays in
  comparison to children with CI and no cognitive
• 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.
• Possible Red Flags (matter of degree)
   • Slow learning rate in spite of strong intervention; gap CA/LA
       • 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
• 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
• Possible Red Flags: Young Child
   – Limited gesture development; motor imitation difficulties
   – Difficulty combining modalities (receptive and/or expressive)…need for
       • 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
• 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
   • 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?
• Possible Red Flags: Preschool
   • Processing based on contextual, extra-linguistic or non-linguistic
     cues for understanding (key words; predictions; global response
   • 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
• 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
• 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

         low threshold

• 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)
• 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
• 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-
  –   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
 • May benefit from “break down-build up” in language
 • On-line analysis and revision is critical (Cycles of
   hypothesize – observe – modify – observe –
 • Help the child/family organize for learning
 • Use meaningful contexts to make concepts explicit

Dr. T. Jones, Gallaudet University