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, MDCincinnati Children’s Hospital Medical CenterCincinnati, OH.Mary Pat Moeller, PhDBoys Town National Research HospitalOmaha, 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 ObjectivesTo 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 identifychildren who need more intensivediagnosis or evaluation in order to improvechild health and well being.
5 Developmental Surveillance Surveillance = periodic assessments over timeAn 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 surveyApproximately 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 IdentificationHearing can delay the identification of an additional disabilityAutism 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 infectionsMeningitisEnvironmental exposures (Pb)Failure to thriveIron Deficiency AnemiaMaternal Substance AbuseEnvironmental deprivationFamily 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 CMVBacterial meningitisSome syndromesFamily history of learning difficulties, attentional problems
12 Case Example4 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 ExampleEarly 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 difficultiesLate walking without due causeExpressive 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 interventionsInterventions such asOTPToral-motor stimulationeffective expressive communication system at earlier agesMay 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 Motor93% 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 MotorIf 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 ageCross midline to pick up a toyPersistent fisting after 4 months of ageLog roll rather than segmental rollLeg scissoring when picked upPersistent primitive reflexes
20 Gross Motor Skill Development SKILL Median age RangeSits alone 6 months 5-8Rolls from prone 6.4 months 4-10Stands alone 11 months 9-16Walks alone months 9-17Walks up stairs (rail) 16.1 months
21 Fine MotorFine 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
22 Fine Motor Skill Development SKILL Median age RangeObject transfer 5.5 months 4-8Neat pincer grasp 8.9 months 7-12Holds crayon well 11.2 months 8-15
23 Fine Motor: Grasp Patterns 4 mths: finger & palm5 mths: thumb active7 mths: raking grasp7-8 mths: inferior pincer9-10 mths: refined pincerBy 2 years: holds item in hand with wrist supination
24 Problem SolvingAlthough 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 SolvingSpeech 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 SolvingChildren 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 widensCan 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 ChildNeed for extended processing timeQualitative differences in comprehensionOver-reliance on comprehension strategiesExtensive gaps between receptive & expressive language (in either direction)May acquire basic vocabulary, but especially slow in acquiring:Relational concepts (perceptual vs. conceptual)Diverse semantic classesQuestion understandingBasic grammatical relations
29 Communication/Language Possible Red Flags: Young ChildLimited gesture development; motor imitation difficultiesDifficulty combining modalities (receptive and/or expressive)…need for chainingMay have shifting modality preferencesProblems with retention and generalization of learned informationWord 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 productionsAtypical play developmentRestricted range of pragmatic functions
30 Communication/Language Possible Red Flags: Preschool“Expectation” of non-understanding; weak meta-cognitive skillsDifficulty attending to and integrating multiple pieces of informationAtypical semantic errors (Daddy is holeing the ground with that big fork!)Difficulties processing sequentially & planning common routinesFormulation challenges in expressive language (word storage and retrieval difficulties; sequential planning)Social difficultiesIn responding to cognitive-linguistic demands of classroomChild temperament: mismatch?
31 Communication/Language Possible Red Flags: PreschoolProcessing based on contextual, extra-linguistic or non-linguistic cues for understanding (key words; predictions; global response strategy)Unusual focus of attentionBehavioral responses increase when language is challengingDifficulty 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 exposureDifficulties sequencing and coordinating movementsDifferent or limited oral motor movement (open lip posture; difficulty with automated lip closure; non precise tongue tip, lingual mobility)Difficulty coordinating voice and signLimited trunk stability; secondary reactionsLow intelligibility of word combinationsDrooling; asymmetry or one side weaknessFeeding/drinking issues; texture intoleranceProtracted jargonSyllable complexity remains low
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 vocalizationsLittle 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 DefinitionSensory 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 actionHyporeactionmanage input by withdrawing, easily over-lookedSensory Defensivenesshyper-vigilant to avoid sensory overload
37 Sensory ThresholdPoint at which the summed sensory input activates the CNS highthreshold(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 TreatmentHelping parents/professionals understand the child’s responsesModify the environment for better “fit”Sensory dietChild-directedMake 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 peersStrong family support; optimal stimulation through sign languageReferred by preschool teacher due to concerns for low intelligibility of sign productionsThe 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 homeThis 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 2Diagnostic teaching with language specialist and occupational therapistAnalysis revealed rule based sign errors (praxis-related)4 rules explained all errorsReversal of sign pathUnable to cross midlineNon dominant hand inaccurateOur 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 languageWe joined together in diagnostics and diagnostic teachingAnalysis 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 perceptionWeak bilateral coordination and motor planningReduced proximal trunk stabilityATNR presentMotor overflow and associated reactionsAvoidance of crossing midlineThe 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 positionWeakness in bilateral coordination & motor planning (praxis) contributed to problems with signs requiring different handshapes; nondominant hand usageReduced proximal trunk stability interfered with strength, accuracyPrimitive reflexes still presentMotor overflow observed along with associated reactions (e.g., uninvolved hand producing same movement as signing hand)Avoided crossing midline
43 Case Example 2Successive approximation based on motor complexity (break down-build up)Increase visual and perceptual salienceModel matching side by sideTarget contrastive patternsMassed motor practice in functional contextsPresentation to facilitate midline crossingGuidance and support of motor planDeveloped 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 puppetTo simplify path processing, produced signs side by side instead of mirroringProvided contrastive patterns and responded literally (6 vs. 9; a vs y)Numerous opportunities for motor practice in meaningful activitiesPositioned items to encourage midline crossingProvided guidance for motor plan – sign symbols in printed string; carrier phrases; selecting phrases with less complex motor demandsLets look at some examples on videotape…first, an example of the first evaluation; then some examples from the intervention program
44 Case Example 2Motor based sign errors resolved in response to sensory integration approachPersistent difficulties in socialization, attention and compulsive behaviorsLearned language in practiced contexts; did not generalize to social useStrength in episodic memory used to promote social interaction, symbolic playTeam approach needed throughout school yearsOutcomes 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 wayOT needs to consider the motor demands of signMaybe 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 pictureDuring 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 RxReduced spontaneous communication in preschool – had to accept all forms of communicationWorked 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 DisorderReciprocal Social Interaction (RSI)Lack of appropriate eye gazeLack of warm, joyful expressionsLack of shared interest or enjoymentLack of response to contextual cuesLack of response to nameLack of coordination of nonverbal communicationWetherby, Woods, Allen, Cleary, Dickinson & Lord, 2004
46 Systematic Observation of Red Flags (SORF) Communication (COM)Unusual prosodyLack of showingLack of pointingLack of communicative vocalizations with consonantsWetherby, Woods, Allen, Cleary, Dickinson & Lord, 2004
47 Systematic Observation of Red Flags (SORF) Repetitive Behaviors & Restricted Interests (RBRI)Repetitive movements with objectsRepetitive movements or posturing of bodyLack of playing with a variety of toysWetherby, Woods, Allen, Cleary, Dickinson & Lord, 2004
48 Visual ImpairmentsDeaf 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 children39.1% in group of deaf childrenUsher Syndrome (3 types)Should have a full ophthalmologic evaluationNeed regular vision evaluations
49 Characteristics of Students with Multiple Disabilities HeterogeneityHistory of strugglesBehavioral challengesHigh need for adult attentionHigh need for task variationDifficulty with generalizationLanguage and communication differencesSynergistic effects of combined challengesMPM –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 lossThese children may progress slowly, encounter failure often and have low self esteem; disabilities are synergistic, not additiveBecause communication deficits are usually present, behavioral issues can be exacerbated; sensory integration issues may influence this as wellMay have a high need for adult attention and task variationNeed to learn in multiple settings, as may be at risk for difficulties with generalizationAtypical 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 holeBuild communication one link at a time (task analysis); Carefully address comprehensionCelebrate successes great and smallSpecialists 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 progressThis 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 objectiveMay benefit from “break down-build up” in language learningOn-line analysis and revision is critical (Cycles of hypothesize – observe – modify – observe – hypothesize…)Help the child/family organize for learningUse meaningful contexts to make concepts explicitThis 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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