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Red Flags for Developmental Delays in Deaf/hoh Children

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Presentation on theme: "Red Flags for Developmental Delays in Deaf/hoh Children"— Presentation transcript:

1 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

2 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.

3 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.

4 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.

5 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.

6 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.

7 Additional Disabilities in Children with SNHL
No additional disabilities % MR % Learning disability 10.7% ADHD % Blindness and Low Vision 3.9% Emotional 1.7% Other % From 2003 Gallaudet survey Approximately 30-40% of individuals with hearing impairments have an additional disability. This is found in the literature and in a recent survey from Gallaudet.

8 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:

9 Identification to Amplification
Wiley, S., Meinzen-Derr, J., and Choo, D. in International Congress Series Volume 1273, (November 2004) Cochlear Implants p

10 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

11 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

12 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.

13 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.

14 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.

15 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.

16 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 months of age).

17 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 :

18 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).

19 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

20 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 months 9-17 Walks up stairs (rail) 16.1 months

21 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

22 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

23 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

24 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).

25 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 :

26 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.

27 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

28 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

29 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

30 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?

31 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

32 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

33 Syllable Complexity (MBL)
Vowels and glides = 1 True cv syllables /ba/ = 2 Mix of cv patterns /mida/ = 3 Average 50 utterances = MBL

34 Slow Transitions in Syllable Complexity
Another cluster of children – early identified but outcomes not as optimal -consistently low complexity over time -I call them “slow transitions” Slow to acquire first words; for a lengthy period continued producing low level vocalizations Little boy in earlier movie contrast…went on to dx of speech motor apraxia…he differences seen early and over time; supported use of signing approach

35 Sensory Integration Dysfunction
Definition Sensory Integration is the organization of sensation from the body and the environment for use.

36 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

37 Sensory Threshold Point at which the summed sensory input activates the CNS high threshold (hyporeactivity) low threshold (hyperreactivity)

38 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)

39 Treatment Helping parents/professionals understand the child’s responses Modify the environment for better “fit” Sensory diet Child-directed Make activities purposeful

40 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 The first child was identified with profound deafness at 11 months of age by Auditory Brainstem Response testing. He was immediately referred to an early intervention team; deaf educator began working with the family at home This child had a medical history significant for hypotonia, tactile defensiveness, motor overflow, limited eye contact, slow learning, limited peer interaction (in preschool) Family support was optimal; parents became fluent signers; child still “plodder” When the child was 3.5 and attending preschool, observant teacher referred him for further assessment due to problems forming signs (she reported that he made lots of errors in production that made his signs hard for others to read…she thought this was interfering with social interaction in the classroom setting)….most preschoolers at this age are fairly accurate in signed productions – at least to the point that most messages are clear. This child was distinct from the others in this regard.

41 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 Our team requested that an OT evaluation be done due to some concerns for sensory processing and hx of hypotonia. School district did a screening and found him to be “within normal limits” Developmental pediatrician on the team assisted educational team in linking with an OT from a nearby University who had experience with motor aspects of sign language We joined together in diagnostics and diagnostic teaching Analysis of sign motor control revealed: Numerous production errors could be explained by 4 rules (give examples of rule governed error behaviors) Sign path reversed – could not process multiple features (handshape, path, direction) Signs that required crossing at midline were produced without crossing (making them confusable with other signs) Nondominant hand often mirrored the dominant handshape or was imprecise

42 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 The Occupational therapist provided motor explanations for a number of these behaviors and noted additional concerns: Reduced proprioception made it difficult for him to perceive how his hand was positioned (he would have to look at his hands and sometimes physically manipulate them to put them in correct position Weakness in bilateral coordination & motor planning (praxis) contributed to problems with signs requiring different handshapes; nondominant hand usage Reduced proximal trunk stability interfered with strength, accuracy Primitive reflexes still present Motor overflow observed along with associated reactions (e.g., uninvolved hand producing same movement as signing hand) Avoided crossing midline

43 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 Developed a therapy program to address these concerns: Some of the strategies used took into account the unique motor problems: Successive approximation to the goal – broke a sign into component parts and gradually built up to the whole (example…spider) Increase visual and proprioceptive salience – sticker on thumb and forefinger to get W or 6-7-8; fingers became legs for puppet To simplify path processing, produced signs side by side instead of mirroring Provided contrastive patterns and responded literally (6 vs. 9; a vs y) Numerous opportunities for motor practice in meaningful activities Positioned items to encourage midline crossing Provided guidance for motor plan – sign symbols in printed string; carrier phrases; selecting phrases with less complex motor demands Lets look at some examples on videotape…first, an example of the first evaluation; then some examples from the intervention program

44 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 Outcomes of team approach (Show post intervention videotape – Susan - the three tape samples are about 3:25 in length total): After less than six months of intervention, the majority of his motor based sign errors resolved…. We do NOT typically need to intervene in this way OT needs to consider the motor demands of sign Maybe these concerns would have dissipated with time, but because he was so unintelligible, we were not willing to let it go…need to look at the whole child picture During process of dx teaching, other observations were of greater concern: Socialization, attention, compulsive behaviors (pdd) Good episodic memory; at risk to memorize words and not generalize them outside of Rx Reduced spontaneous communication in preschool – had to accept all forms of communication Worked through his strengths in episodic memory – built off scripts to help him socialize (I’m in charge of cookies) Has required a team approach to management throughout the school years

45 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

46 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

47 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

48 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

49 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 MPM – When students with hearing loss have additional disabilities, there are several characteristics we can expect: Individual profiles of performance is the rule, not the exception – unique and difficult to predict based on others with hearing loss These children may progress slowly, encounter failure often and have low self esteem; disabilities are synergistic, not additive Because communication deficits are usually present, behavioral issues can be exacerbated; sensory integration issues may influence this as well May have a high need for adult attention and task variation Need to learn in multiple settings, as may be at risk for difficulties with generalization Atypical language learning patterns may be seen (not just simple delays) – i.e., problems with the cognitive bases for language can lead to difficulties making associations among words & concepts during vocabulary learning; Language organizational difficulties lead to extreme formulation problems; etc.

50 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 Specialists in deaf/hard of hearing children with multiple disabilities offer some general guidelines for working with these children in educational and therapeutic settings… Teams need to consider that THIS child will not be likely to learn the same material at the same rate using the same strategies as “typical” deaf and HH children. This cannot become an “all purpose excuse” for reducing expectations…rather, it needs to engage tools like diagnostic teaching and constant task analysis (not why isn’t he learning, but rather…what do I need to change so he can learn?) Parents need support to adopt an “individual child metric” for measuring progress This recommendation from Dr. Tom Jones at Gallaudet U is an easy to recall guideline…With these children, focus on the donut (the substance of what the child CAN do), not the hole (the disabilities) With these kids, we also need to build the structure one brick at a time…lay a solid foundation and build. This takes task analysis to ensure that appropriate layers of information are put in place in solid ways. Imagine placement in a regular classroom…instructional level surpassing language levels by a lot; child expected to sit through lengthy discussion…lots of off task behaviors/difficulty attending – cycle of negatives – These kids need to live by the 4:1 rule – 4 positives to one negative – this requires engineering the environment for success. Dr. T. Jones, Gallaudet University

51 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 This is my favorite of Dr. Jones’ rules – If a dead man can do it, it is probably not an appropriate educational/therapy objective for the child….Example: Johnny will sit still in his chair…Johnny will be quiet during story time. These kids need multi-disciplinary perspectives…a developmental pediatrician can be the team member to help teachers remember what accommodations are needed to ensure progress. Post hoc evaluation – discerning how well this approach worked and what modifications are needed – is often a better guide than a priori assessments…Why? Because children with multiple disabilities do not demonstrate their knowledge best in de-contextualized test settings with strangers…at least part of the team evaluation should be observation in real settings (or videotape of that) – ecologically valid evaluation is KEY. Teams can also support instructors by guiding them to provide instructive feedback when there are behavior/social/language issues; Instead of “stop kicking the table leg” feedback can be, let’s try putting your feet on this stool so they can be still; or Instead of “no pushing in line,” John, use your words. Tell Joey, excuse me, my turn to be first.” Visualization strategies are immensely helpful with these children…Use of a low tech schedule of events can help a student transition well from one event to the next during a school day. Teams can demonstrate the use of such a tool in the assessment setting. (concrete representation of events with check off boxes) Dr. T. Jones, Gallaudet University

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