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Board Review 4/30/2013

Board Review 4/30/2013. Disorders of Cognition, Language, and Learning. Test Question. Which topic would you prefer for our final board review? Emergency Care Disorders of the Eye. Speech and language disorders. Normal Language: Pearls. Intelligibility of speech by a stranger: Age 2: 50%

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Board Review 4/30/2013

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  1. Board Review 4/30/2013 Disorders of Cognition, Language, and Learning

  2. Test Question Which topic would you prefer for our final board review? • Emergency Care • Disorders of the Eye

  3. Speech and language disorders

  4. Normal Language: Pearls • Intelligibility of speech by a stranger: • Age 2: 50% • Age 3: 75% • Age 4: 100% • Dysfluency of speech is normal during the rapid attainment of speech in preschool children (around age 2-3) • Typically resolves by age 4 • Articulation of all consonant sounds is not complete until 6-8 years of age

  5. Factors that influence language development • Exposure to language • Economically disadvantaged homes have less exposure to language and varied verbal interactions • The more parents talk to their children the more rapid their vocabulary growth • Exposure to reading • Amount of time being read to influences reading proficiency

  6. Question #1 You are seeing a 3yo boy for a health supervision visit. He has a vocabulary of 50 words and does not combine words. Past medical history is negative. He has no history of recurrent ear infections and achieved gross motor milestones at appropriate ages. He resides in a bilingual household. His father had delayed speech, his older brother received speech therapy in elementary school, and his older sister had difficulty with reading comprehension. Of the following, the MOST likely contributing factor for this boy's language delay is • A bilingual household • Being third born • Genetic predisposition • Hearing loss • His gender

  7. Language Delay and Disorders • Language development in infancy and early childhood is a better predictor of cognitive function than motor development • ‘Language Disorder’ refers to a deficit in the comprehension (receptive) or production (expressive) of language • ‘Disorder’ = causes impairment in functioning • Delayed language may or may not be clinically significant  need speech-language clinician to help • 13-18% of toddlers have “late-talking” • 50% of these will have persistent language deficit • Factors that are associated with delays: family history**, low socioeconomic status, richness of language environment

  8. Bilingual Families • Bilingual family is NOT a risk factor for language delay • First words may emerge slightly later but within normal limits • Mixing of words or grammar can be seen until age 3 or 4

  9. Causes of Language Delay/Disorder • Specific Language Impairment (SLI) • Biologically based neuodevelopmental disorder of oral language acquisition • No other cause identified (autism, brain damage, hearing loss, etc) • Problems with syntax, grammar, tenses, plurals, possessives, open-ended questions, reading comprehension • Presents in preschool years or kindergarten • Phonologic Disorder • Impaired ability to articulate speech sounds • Childhood Apraxia of Speech • Severe and persistent speech intelligibility disorder • Impaired ability to imitate and produce speech sounds • Due to CNS-based problems planning, sequencing, and coordinating oral-motor movements • Likely genetic in origin

  10. Question #2 During a health supervision visit for a 5-year-old girl, her father reports that she has developed a stutter over the past 6 months. He explains that at times she seems a little frustrated when she speaks but otherwise is happy and well-adjusted. You notice the father also has a mild stutter. Of the following, the risk factor that MOST strongly suggests the need for speech therapy for the girl is: • Age of onset of the stutter • Her reaction to stuttering • Her gender • The father’s stutter • Time since onset of stutter

  11. Causes of Language Delay/Disorder • Stuttering • M > F (3:1), strong genetic component • Onset typically around 4-5 years • **Normal dyfluency of speech until age 4, after this age a referral is warranted for the stutter** • Dysarthria • Disorder of motor control of muscles required for speech production • Dyslexia • Deficit in the ability to recognize words in print and phonemes within words • Genetic influence • Hearing Impairment • Auditory input is critical for organizing the neural pathways associated with speech • Concern for ANY speech/language delays should involve an audiologic assessment • Autism/Genetic Syndromes

  12. Identification of Language Disorder • Surveillance for child’s speech development at all health supervision visits

  13. Identification of Language Disorder • Surveillance for child’s speech development at all health supervision visits • Formal screening at 9, 18, and 24 or 30 months • No “gold standard” screening tool • Ages and Stages is an example of one • Risk factors: positive family history, prematurity, male sex • Obvious risk factors: hearing impairment, craniofacial abnormalities, syndromes associated with language impairment • Immediate referral for evaluation and treatment

  14. Question #3 You are seeing an 20-month old boy who does not say any words at all. His parents are concerned that he is not developing like his peers and that he is not yet talking. Other than two episodes of otitis media in the past year he has been in good health. His physical exam is normal. Of the following options, the most appropriate next step is to: • Order EEG • Order brain MRI • Reassure the parents and follow up in 6 months • Refer the child for a neurologic evaluation • Refer the child to audiology

  15. Referral and Treatment • Suspected speech/language problem  • Refer to audiology and speech specialist • Age <3: refer to local early intervention program • Age > 3: refer to public school early childhood program • Concern about global delay or autism  further diagnosis and evaluation by development specialist • Educate parents • Language-rich environment, child-directed conversation, early reading, vocabulary building

  16. Evidence-based Treatment for Language Delay • Speech Therapy!! • Interventions range from clinician-directed to child-centered • If speech therapy has limited success: they will consider an augmented communication device • Range from picture communication boards to computers that have synthesized speech output. • Once speech therapy is initiated, psychoeducational evaluation may be considered to determine if additional specialized educational services are required

  17. Autism spectrum disorders (ASD)

  18. ASDs • Diagnosis is difficult prior to age 3 • However, early intervention initiated prior to age 3 has research proven positive benefits for these children • There are signs of ASDs that are present as early as 14-18 months: • Lack of spontaneous seeking to share enjoyment • Lack of social and emotional reciprocity • Impairment in the use of non-verbal behaviors • Delay or lack of spoken language

  19. ASD: Early Social Skill Deficits • Development of social skills and language is delayed or “out of sync” with motor, adaptive, and cognitive function • Lack of social relatedness • Often content being alone • Deficits in “joint attention” • Normal: • Follow a point (10-12mos) • ‘requesting’ (12-14mos) • ‘commenting’ (14-16mos) • Video: http://www.autismspeaks.org/what-autism/video-glossary/glossary-terms [2,3,2]

  20. ASD: Early Social Skill Deficits • Deficits in social orienting • Normal: Turn head when name called (8-10mos) • Autism: Do not respond when name called • May create concern about hearing • Lack of or delayed symbolic play • Normal: • Simple pretend play (16-18mos) • Complex pretend play (18-20mos) • Autism • Remain in sensory-motor stage • Enjoy trial and error tasks; “rough house” play • Little interest in toys; or use them in unusual ways • Video [4,1,2]

  21. Question #4 During a health supervision visit for a 24 mo boy you notice that he does not respond to his name and he repeatedly echoes words and phrases. He recites a fairly complex car advertisement that plays on television. An audiology evaluation is normal. On a general developmental screening questionnaire, he is below the “cut-off” for both communication and personal-social development. He also scored in the risk range on the parent-completed autism-specific screening questionnaire. You refer him to a developmental-behavioral specialist, whose next available appointment is in 6 months. Of the following, the MOST appropriate additional step for this boy is to • Order baseline EEG • Encourage his mom to provide language stimulation activities and re-evaluate in 6 months • Start an amphetamine medication • Refer him for early intervention services • Refer him for evaluation for an augmented communication device

  22. ASD: Early Language Skill Deficits • Delays in language or speech development have been the most common presenting signs in children diagnosed with ASD • Preverbal red flags: • Unusually quiet or irritable • Atypical vocalizations • Fewer gestures • Lack of to and fro babbling • Lack of inflection (jargoning) at 10-12 mos

  23. ASD: Early Language Skill Deficits • Absent or delayed speech • Usually sensed around 18mos by parents • Sometimes rationalized by the child being “shy” or an only child • Parents often overestimate the child’s receptive language ability • Referral for audiology and speech is appropriate first step • If hearing and receptive language are normal, can monitor and try to stimulate speech • If hearing is normal but receptive language is delayed  refer to developmental specialist or early intervention program • Confirmed diagnosis is not necessary to make referral • Language regression • Seen in 25-30% of children with autism

  24. ASD: Early Language Skill Deficits • Atypical Language • Echolalic (immediate or delayed), ritualistic, not functional (ie. pop-up words) • Exceptional verbal memory or labeling skills

  25. ASD: Restrictive Interests, Stereotypies, Repetitive Behaviors • May form strong attachment to specific unusual item • Piece of string, pen, etc • Sterotypies • Hand flapping, twirling, finger movements, rocking, head nodding, toe walking, licking, sniffing • Repetitive behaviors • Lining objects up • Hypo or Hypersensitivities to stimuli

  26. Question #5 Of the following, which characteristic would make you more concerned about an Autism Spectrum Disorder over an isolated profound hearing loss? • Child is hypervisual and makes eye contact to communicate • Delay in onset of language • Child uses expressive hand gestures to communicate his/her needs • Child doesn’t respond to his mother’s voice or to any other environmental noise • Child engages in self-injurious behavior when he is directed to a new activity

  27. ASD are distinctive from other disorders • Child with profound hearing loss • Child is hypervisual; uses eye-contact and gestures to communicate • No reaction to human voice OR environmental sound • Autism: will respond to environmental noise • Cerebral Palsy • Eye contact, sounds, facial expressions, conversations • Isolated Intellectual Disability • Don’t typically see language delays, odd interests/activities • Autism can be co-morbid with other conditions with intellectual disability (ie Fragile X) • Isolated Speech/Language Delay • Child uses eye-contact, gestures, social interaction

  28. Diagnosis of Autism Disorder

  29. Asperger’s Disorder No Language Delay!

  30. Red Flags for Autism • No babbling by 9 months • No pointing or other gesture by 12months • No single words by 16 months • No 2-word spontaneous (not echolalic) phrases by 24 months • Loss of language or social skills at any age

  31. Question #6 The AAP published surveillance and screening guidelines for autism spectrum disorders as part of a clinical report on ASD. They recommend 3 referrals for children with greater than 2 risk factors for ASD. Which of the following is NOT one of those mandated referrals? • Early intervention or school-based program based on the child’s age • An ASD specialist or team of specialists for a comprehensive evaluation • Neurology • Audiology

  32. ASD: Screening and Evaluation • Surveillance at every health supervision visit • Standardized ASD-screening tool at the 18 month and 24 or 30 month visit or whenever a concern is raised • Risk factors: • Older sibling with ASD • Parental concern • Other caregiver concern • Physician concern • If ≥ 2 risk factors OR concerning results on ASD screening tool  refer!! • Early intervention (<3yo) or school program (>3yo) • ASD specialist for comprehensive evaluation • Audiology

  33. Question #7 A 9yo boy with Autistic Disorder is displaying severely aggressive behavior to his parents and teachers. He has frequent tantrums when frustrated or upset which include self-injurious behavior and throwing objects. This behavior has persisted even despite intensive behavioral and educational interventions. His parents are interested in medication to help handle him more safely at home. Of the following, the MOST appropriate medication with which to begin a trial is: • Lithium • Atypical antipsychotic • Melatonin • Serotonin reuptake inhibitor • Stimulant medication

  34. ASD: Management • Comprehensive evaluation by ASD specialist • Coordinated care between variety of practioners • Developmental/behavioral specialists, therapists, teachers, social workers, subspecialists, vocational staff • Treat co-existing medical, psychiatric, behavioral problems • Medications • Risperidone • Based on symptoms: • Hyperactivity/inattention  ADHD medications

  35. Hearing impairment

  36. Hearing Loss in Children • Can be a debilitating condition • Deafness is associated with an increased risk of learning disabilities and a resultant low reading level. • The first 36 months after birth represent a critical period in cognitive and linguistic development • Early identification and intervention are CRITICAL • Allows deaf and hearing-impaired children to approach their peers in language skills and academics • Those identified late often won’t reach the same level

  37. Question #8 You are seeing a newborn with sensorineural hearing loss on her newborn hearing screen. Mom’s reports prenatal history as unremarkable. On exam, the baby has microcephaly and hepatomegaly with NO other obvious physical abnormalities. The MOST likely cause of the hearing loss is • Congenital cytomegalovirus infection • Alport syndrome • Middle ear effusion • Prenatal rubella exposure • Usher syndrome

  38. congenital hearing loss 50%

  39. Question #9 You are seeing a 4 month old new patient in clinic today. The family recently moved to your area. You review the records sent from her previous PCP and note that she failed her ABR in the hospital as a newborn. The parents say they never made it to the follow-up test because of the move. Their reports on developmental history seem appropriate. The baby smiles, coos, and laughs out load during your exam. What do you want to do next? • Ignore the test results from the newborn period. Obviously the baby is developing fine • Perform an audiometry test in the office to test her hearing • Refer her for formal hearing evaluation with an audiologist • Reassure the parent but schedule a follow-up appointment in 1 month so that you can continue to monitor her language development

  40. Infants • Congenital hearing impairment cannot be detected through simple observation • Hearing-impaired infants achieve early language milestones • Smiling • Cooing • Babbling • Gesturing • There may be NO initial presenting complaints…or the problems may be very SUBTLE! • This is why mandatory newborn hearing tests are SO important!

  41. Newborn Hearing Screens • Should be performed in the newborn period…within 1 month after birth!! • Otoacoustic Emissions (OAE) • Detects sounds emitted from the cochlea in response to clicks or tones • Does not assess auditory neuropathy or cortical processing of sound • Significantly affected by fluid in the ear canal • Auditory brainstem response (ABR) • Measures the EEG response from the vestibulocochlear nerve • Can detect auditory neuropathy…a condition prevalent in the NICU population • Does not assess cortical processing of sound

  42. Newborn Hearing Screens • IF abnormal screening results are obtained • Formal hearing evaluation NO LATER than 3 months! • Referral to audiologist for formal testing with diagnostic ABR and other appropriate tests • Implementation of services BY 6 months! • False positives are fairly common, so reassure the parents but also emphasize importance of repeat test! • Unfortunately, all congenital hearing loss is NOT detected by newborn hearing screens • Pediatricians should remain aware of risk factors and signs of hearing loss in infants • Refer for formal evaluation if present

  43. Older Children • We must be able to recognize presenting complaints in pre-school children with hearing impairment • Speech delay and difficulty with articulation • Asking people to repeat themselves • Not hearing instructions • Listening to a loud television or music • Behavioral problems • Other areas that require interaction for learning • Ability to understand and regulate emotions • Accomplish complex motor skills • We must ALSO recognize risk factors

  44. Risk factors • Because only 50% of children who have hearing loss are identified by the use of risk indicators , all children should have periodic objective assessment of their hearing. • Conventional audiometry not reliable until at least 4y

  45. Question #10 What is the MOST common infectious cause of acquired SENSORINEURAL hearing loss? • Matoiditis • Bacterial meningitis • Pneumonia • Otitis media • Retropharyngeal abscess

  46. Causes of Acquired hearing loss The most common cause of acquired CHL is otitis media with effusion ***Among acquired infections, bacterial meningitis is the MOST COMMON cause of childhood SNHL in (infants and children), ranging from mild to profound depending on the severity of illness. ALL children who have meningitis should have a hearing screen as soon as possible!

  47. Formal Tests

  48. Formal Tests

  49. Impact on Development • The severity of the hearing impairment impacts the level of language development in individual patients. • Mild-to-moderate impairment (hearing loss less than 90 dB) • Often improved with external amplification devices (hearing aids) • With early therapy services can reach full academic potential • Severe-to-profound impairment • If SNHL will benefit from cochlear implants • Will require more extensive educational activities/therapies • The onset of hearing loss after age 5 years has a smaller, but still significant, impact on language development.

  50. Approaches to Education • Educating deaf patients can be accomplished with appropriately trained personnel and involvement of the parents/family. • “Family involvement, including verbal and nonverbal (gestures) communication, has a more significant positive effect on language development than any other specific type of intervention.” (PREP) • The pediatrician should help facilitate this process. • Other approaches: • Hand-cued speech • Sign language • Manually coded English • Oral aural

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