Regulatory And Sensory Flashcards
1
Q
Feeding dysfunction: structural
-examples
-problem
-outcome
A
Examples: anatomical issues (cleft lip/palate, esophageal, craniofacial, tracheomalacia, laryngomalacia), congenital heart disease, GI (esophageal atresia/stricture, pyloric stenosis, short bowel)
Problem: inability to coordinate breath with eating, inability to efficiently chew, coughing/regurgitating/pain with swallowing, aspiration
Outcome: work of eating is hard, uncomfortable or impossible. Selective eating develops and learning does not occur
2
Q
Feeding dysfunction: physiologic
-examples
-problem
-outcome
A
Examples: reflux, EOE, constipation, delayed gastric emptying, BPD, congestive heart failure failure, renal or liver disease, allergy
Problem: aspiration, pain/discomfort, lack of satiety/hunger cues, inability to support need for growth, allergic reaction
Outcome: work of eating is too hard, uncomfortable or impossible. Selective eating develops, learning does not occur
3
Q
Feeding dysfunction: experiences
-examples
-problem
-outcome
A
Examples: lack of schedule and routine with eating, forced feeding, physical abuse, fear during meal time, lack of control, food insecurity, sensory processing differences, missed opportunities due to behavioral or medical complexity
Problem: meals are not predictable with routine and clear roles, negative reinforcement at meal time, lack of experience
Outcome: eating is seen as a negative experience to be avoided, selective eating develops, learning does not occur
4
Q
Growth faltering: nutritional intervention
A
First step is ensure access to foods
Involve nutritionist and take careful history of feeding and feeding behaviors, caregivers knowledge, and cultural beliefs
Multivitamin with iron and zinc. Therapeutic doses of iron and vitamin D if low
Goal is first catch up then maintenance
5
Q
Growth faltering: social determinants
A
Social risks: parental stress, poverty, food insecurity, housing
Inability for parents to provide children with adequate nutrition as result of poverty should not be equated with abuse
When to involve CPS: non accidental trauma, cannot provide or sustain needed outpatient interventions because of parental issue (untreated severe psych or cognitive disability)
Provide mental health support for parents
6
Q
Growth faltering: hospitalization
A
Indicated if severity of malnutrition or associated medical condition warrants.
Hospitalization for purposes of comparing pre admission weight to in hospital gain should not be used as diagnostic of neglect. Most display improved weight gain in hospital regardless of etiology
7
Q
Obesity: role of pediatricians
A
People first language, terms such as weight and BMI instead of obesity
Motivational interviewing (gauge willingness and readiness)
8
Q
Picky eating
-definition
-strategies
A
Children who eat limited amount of food, reject novel foods, or exhibit strong food preferences. Reported by 2/3 of parents during toddlerhood, seen as expression of autonomy in the context of typical development
Use of collaborative rather than directive strategies (modeling, setting positive relational context)
Try one bite rule is counterproductive for strong willed temperaments
Repeated exposure is useful strategy but needs to be 10x
In older children: information sharing (makes your bones stronger), combine preferred and non preferred, compromising techniques that allow more autonomy
Want to rule out medical reasons for food selectivity and review DBP history
9
Q
Anorexia nervosa
-features
A
Restriction of energy intake relative to requirements leading to significantly low body weight
Intense fear of gaining weight or becoming fat
Disturbance in the way an individual’s body weight or shape is experienced (not recognizing seriousness of low body weight)
10
Q
Anorexia nervosa
-medical symptoms
-medical complications
A
Symptoms: bradycardia, hypotension, amenorrhea, hypothermia, pallor, muscle pain, lanugo, heart murmur
Complications: electrolyte abnormalities (hypoK, hypoNa), depression/anxiety, cerebral cortical atrophy, cognitive deficits, seizures, decreased cardiac muscle mass, arrhythmias, conduction delays, mitral valve prolapse, pericardial effusion, CHF, edema, delayed gastric emptying and slow motility, SMA syndrome, pancreatitis, elevated transaminases, hypercholesterolemia, growth retardation, amenorrhea, leukopenia/anemia/thrombocytopenia, elevated ferritin, decreased ESR
11
Q
Bulimia nervosa
-features
A
Recurrent binge eating episodes and compensatory behaviors to prevent weight gain (self induced vomiting or misuse of laxatives)
Binge eating occurs in secret, lack of control over episode
Binge eating episodes happen on average at least 1x/wk for 3 months
Most have other psych disorders (mood/anxiety); fear of social situations and low self esteem
12
Q
Binge eating disorder
-features of binge episodes
A
Recurrent episodes of binge eating on average at least once per week for 3 months
Severity based on frequency: mild is 1-3 times per week, moderate is 4-7 times per week, severe is 8-13 times per week and extreme is 14 or more
Episodes at least 3: eating more rapidly than usual, eating until uncomfortably full, eating large amounts of food when not hungry, eating alone because of embarrassment and feeling depressed/guilty/disgusted after
Marked distress and lack of control during binge eating episodes. May temporarily feel better or feeling of dissociation with episode
Can occur at any weight
13
Q
Binge eating disorder
-treatment
-remission
A
Tx: CBT, IPT, DBT. CBT most established
Partial Remission if criteria previously met but frequency decreases to less than once per week, full remission is when none of criteria have been met
14
Q
ARFID
-features
A
When disturbed feeding and eating 1) occurs without disturbed perception/experience of body weight or shape
2) results in insufficient oral intake to meet needs
Or 3) is accompanied by dependence on supplementary feeding and/or significant negative impact on weight gain or growth, nutrition, or psychosocial functioning
15
Q
ARFID
-comorbidities
A
Anxiety disorders are common coexisting conditions
-social anxiety may result in avoidance of eating around others
-specific phobia of vomiting or choking can manifest as food avoidance
-rituals involving eating and food seen in OCD
Mental health and ARFID can be diagnosed together when full criteria are met for both and intervention needed for eating disorder
Important to distinguish from medical conditions
16
Q
ARFID
-management
A
Medical monitoring
Psychoeducation
Refeeding
Nutritional modeling and rehabilitation
Psychotherapy (CBT, behavior therapy, family therapy, individual therapy)
Medications: no medication yet established as effective treatment
Olanzapine, fluoxetine, cyrpoheptadine, D cyclosterine have been investigated
17
Q
Obesity vs Binge Eating Disorder
A
Binge eating have binge eating episode features
BED have higher level of functional impairment, more distress, lower QOL, than obesity alone
18
Q
Binge eating vs bulimia
A
In BED do not see the compensatory behaviors
(Though BED may have some attempts at dieting)
19
Q
Other psych vs eating disorder
A
Depression, bipolar can have disordered eating habits, if full criteria also met for eating disorder can diagnose both
20
Q
Anorexia vs buliemia
A
Bulimia has binge eating episodes while anorexia is self starvation (strategies to lose or avoid gaining weight)
Bulimia purge episodes can be purging (vomiting, laxatives) or excessive exercise or starvation
Anorexia can have normal BMI (atypical anorexia)
21
Q
ASD vs ARFID
A
Avoidance of food based on sensory characteristic is common though does not typically result in degree of impairment needed to meet criteria for ARFID
22
Q
ARFID vs anorexia
A
Eating behaviors and consequences can be similar
Anorexia has fear of weight gain/being fat and disturbed body perception
23
Q
Toilet training: age
A
Girls are trained 2-3 mo sooner than boys
For neurotypical recommend to wait until 18 mo and complete 24-36 mo
24
Q
5 Developmental milestones for toilet training
A
- Ability to follow directions
- Awareness of bodily urges
- 2+ hour dry periods
- Motivated to remain dry and copy older family members
- Motor skills to sit on toilet and pull underwear up and down
25
Primary vs secondary enuresis
Primary: nighttime bladder control never obtained
Secondary: reoccurs after at least 6 months of dryness
26
Etiology of nocturnal enuresis
Genetic: Highly correlated within families (75% w first degree relative)
Neurobiology: reduced vasopressin, reduced bladder capacity, detrusor over activity, impaired arousal
Constipation
Parents will say their kids are deep sleepers, though data varied. Nocturnal enuresis can occur at all sleep stages and children with NE may have other sleep problems
27
Most widely studied comorbid condition with enuresis
ADHD
28
First line interventions for enuresis: daytime and nighttime
Day: parent education, lifestyle modifications (reduced fluid intake and bladder irritants), skill building (effective voiding posture and hygiene), timed voiding (every 2-3 hours) with positive reinforcement. ***treat constipation
Night: in addition to above, behavioral interventions (alarm), desmopressin (for short term, can do PRN)
29
Desmopressin
Decreases UOP for 1-7 hours
Do NOT do intranasal form
Give 1 hr before bed then limit fluid intake
Pros easy, minimal SE, can use PRN
Cons 10-30% don’t respond and 60% continue to have wet nights, risk of hyponatremia, expensive
30
Pathophysiology of encopresis
Stool retention —> 1) hardening of stool and 2) stretching of colon (deceased sensory feedback and contractile strength, sphincter function compromised)
Hardening of stool leads to painful defecation
Painful defacation and stretching of colon lead to toilet avoidance
Toilet avoidance leads to stool retention (and the cycle continues)
31
Encopresis age and timing
Chronological age and developmental level of at least 4
At least 1x/mo for at least 3 months
32
Types of encopresis
Over 80% is fecal retentive with overflow incontinence (resolved with treatment of constipation)
Nonretentive: defacation in places not appropriate to social context at least 1x/mo without any other biological process present with normal formed stools
33
Risk factors for encopresis
Physical: constipation, ineffective evacuation
Environmental/behavioral: attention to bodily signals, oppositional behavior, anxiety/OCD, lack of access/disorganized home, trauma, violence, sexual abuse
34
Management of encopresis
Combo of medical and behavioral strategies
1) education, explain pathophysiology
2) remove blame/punishment and use positive approach
3) initial clean out followed by maintenance
35
Approach to toilet training
Regular discussion in story format to introduce and normalize toileting behavior
Can introduce toilet or potty seat (comfortable place to sit or rest feet)
Time to sit either with or without a diaper/pull up and no expectations for voiding
As child becomes comfortable regular sit times. Optimal timing is 20-30 min after a meal
When successful reward with praise or token.
Over time child will go outside of this window.
When child starting to use toilet regularly can remove diaper or pull up
36
Toilet refusal and withholding approach
May occur because of: physical discomfort (constipation,pain), perceived negative experience in the bathroom, fears, etc
Assess for constipation and then behavioral approach. start with whatever part the child is willing to perform and use reward system
37
Sleep problems in ASD
Delayed sleep onset
Night waking
38
Genetic disorders associated with sleep disordered breathing
Trisomy 21
Prader Willi
39
Sleep problems in Smith Magenis Syndrome
Night waking
Early morning waking
40
Sleep recs by age: 4 to 12 mo
12-16 hours
41
Sleep recs: 1-2 year olds
11-14 hours
42
Sleep milestones in infants
Sleep consolidation: sleep for continuous period concentrated in nocturnal hours
Sleep regulation: ability to control internal states of arousal to both fall asleep at bedtime without parent intervention or assistance and fall back to sleep after normal arousals
43
Sleep issues in toddlers
Separation anxiety (bedtime resistance, problematic night waking)
Nighttime fears (development of imagination)
Symbolic meaning of objects (transitional objects)
Bedtime resistance (increased drive for autonomy)
44
Sleep recs: 3-5 year olds
10-13 hours, most give up napping by 5 years
45
Sleep issues in preschoolers
Expanding language and cognitive skills: increased bedtime resistance and limit testing
Developing capacity to delay gratification and anticipate consequences: respond to positive reinforcement
46
Sleep recs: 6-12 years
9-12 hours
47
Sleep recs: 13-18 years
8-10 hours
At risk for delayed sleep phase syndrome
48
OSA
-pathophys
-risk factors
Pathophys: sensation or restriction of airflow through nose and mouth during sleep, just wraps, normal ventilation, leading to hypoxemic or hypo ventilation and sleep pattern characterized by frequent arousal
Risk factors: reduced upper airway patency (adenotonsillar hypertrophy, allergies, RAD, reduced airway size, obesity, craniofacial syndromes), reduced upper airway tone (neuromuscular disorders, down syndrome), family history
49
OSA
-manifestations
-management
Loud nightly snoring, choking, gasping, increased work of breathing.
May be present with less traumatic findings and primary neurobehavioral symptoms (in attention, hyperactivity, poor academic functioning)
First line treatment: T&A, nutrition, and exercise counseling, positive airway pressure
50
Causes of insomnia
Behavioral (sleep phase problem, late napping, irregular schedule, sleep association, limit setting), stress, chemical (caffeine, medication), medical condition (allergies, reflux, OSA), developmental or psychiatric (ASD, ADHD, depression, anxiety), environmental (light noise temperature)
51
Most important factor in nighttime waking
Sleep associations: child falls asleep with certain condition and cannot fall back to sleep on their own (rocking, parents presence, bottle, TV)
52
Sleep terrors vs nightmares: stage of sleep
Sleep terrors: partial awakening for slow wave sleep
Nightmares: any phase, more common during REM
53
Sleep terrors vs. nightmares: age
Sleep terrors: 2-12 years (most common 2-6)
Nightmares: begin in toddler, occur at any age
54
Sleep terrors vs nightmares: appearance
Sleep terrrors: terrified, panicked, sweating, rapid heart rate and breathing.
Nightmares: fearful
55
Sleep terrors vs nightmares: time since sleep onset
Sleep terrors: usually 1-3 hours
Nightmares: usually >4 hours
56
Sleep terrors vs nightmares: response to parent entering room
Sleep terrors: continue to cry, scream, thrash and do not seem to be awake or know parent is there
Nightmares: consoled by parent’s presence
57
Sleep terrors vs nightmares: recall next morning
Sleep terrors: no memory of event unless awakened
Nightmare: vivid recall of dream
58
Sleep terrors vs nightmares: predisposing factors
Sleep terrors: illness, stress, sleep deprivation
Nightmares: stress, anxiety
59
Management of parasomnias (sleep terrors, nightmares, sleep walking, sleep talking)
Reassurance and education about benign and self limited nature, safety measures (gates, locking doors/windows, reducing clutter, alarm systems), avoidance of triggers (sleep deprivation, changes in sleep environment)
If frequent (nightly) events, parent can wake 15-30 min before typical time when occurs
Benzos or TCAs restricted to severe or chronic cases
60
Rhythmic movement disorders in sleep
Body rocking, head rolling, head banging,
Occur largely in sleep-wake transition
Much more common in first year of life and generally disappear by 4 years.
Most occur in typical children and do not result in physical injury
61
Bruxism
Nocturnal teeth grinding. Consider possibility of low iron. Symptomatic treatment may be useful for older children
62
Insomnia:
-definition
-timing
Dissatisfaction with sleep quantity or quality associated with difficulty initiating sleep, maintaining sleep, or returning to sleep after awakening in the early morning
At least 3 nights per week for 3 months in the setting of adequate opportunity for sleep
Categorized according to timing of sleep difficulties into 1) initial/sleep onset insomnia, 2) middle or maintenance insomnia, 3) late insomnia
More than 20 min to fall asleep and more than 20 min of wakefulness after sleep starts are considered clinically significant
63
Presentations of insomnia
Sleep onset association insomnia: in children who need specific set of conditions in order to go to sleep (rocking, feeding, caregiver presence) difficulty self soothing
Limit setting insomnia (preschool age and up): child resists bedtime, protests and demands. Often from inconsistent bedtime rules but important to look at other factors
Psychopathologic insomnia: older children and up, thoughts negatively impact ability to fall asleep
Delayed sleep phase disorder: in adolescents, sleep patterns delayed by 2 hours or more than expected. Normal sleep quantity and quality of able to sleep at will
64
Management of insomnia
First step is behavioral interventions/ address sleep hygiene
Second step is melatonin (0.5-3mg, up to 6, given 30-60 min before bed)
Other meds you may see, not well studied and not FDA approved, not frequently needed:
-alpha 2 agonists: decreased sleep latency, suppress REM. Often given if impulsive or hyperactive nighttime behavior. SE hypotension, bradycardia with high doses, if given too early or dose too low can get irritable activated
-antihistamines: decreased sleep latency no effect on night waking, anticholinergic effects (daytime sedation, dryness)
Sleep meds that likely won’t be the answer:
-risperidone: may promote sleep as secondary effect but not primarily for sleep
-zolpidem: failed to reduce sleep latency and frequent adverse events
-trazodone: reserved for associated mood disorder, suicidal ideation and may have significant side effects
65
Restless leg syndrome: presentation
Urge to move legs associated with or due to uncomfortable feeling in legs. Typically worse in evening and exacerbated by inactivity, children more likely to have symptoms during the day than adults.
Medications can precipitate or exacerbate (SSRI, TCA, neuroleptic, sedative antihistamines)
66
Restless leg syndrome: management
***sleep hygiene (avoid caffeine, electronics, etc)
Physical activity, stretching, massage, cooling/heating pads
Iron supplementation for those with ferritin <35 to 50 (goal is >50)
If on medication that causes (eg SSRI, adjust it)
Sleep study confirms diagnosis, but do iron supplementation before
67
DDx: restless leg syndrome (3 other conditions)
Periodic limb movement: brief extremity jerks >5 per hour on polysomnography without urge to move legs (though can co occur with RLS). More common in ADHD increasingly recognized in ASD
Growing pains: involve both legs, intermittent, peak at 5 years. More common than RLS and don’t involve urge to move
Sleep related leg cramps: brief, painful contractions, typically unilateral, calf or foot muscles, improve with stretching, not common in children
68
Narcolepsy: presentation and onset
Adolescence (rare in prepubertal)
Repetitive episodes of profound sleepiness that occur both at rest and during periods of activity. May be very brief (3 seconds) resulting in lapses in attention. May initially present as being tired and prone to napping though not always in uncontrolled fashion
69
Features of narcolepsy
1) excessive daytime sleepiness
2) cataplexy: sudden loss of total body or partial muscle tone, usually in response to emotional stimulus
3) hypnagogic (at sleep onset) or hypnopompic (at waking) hallucinations (tactile, visual, auditory)
4) sleep paralysis: temporary loss of voluntary muscle control at sleep onset or offset
70
Pathophysiology of narcolepsy and diagnosis
Alterations in sleep neuro regulatory system and may have autoimmune basis
Gold standard is polysomnography followed by multiple sleep latency test
71
Treatment of narcolepsy
Lifestyle (scheduled naps)
meds to combat daytime sleepiness (stimulants)
REM sleep suppressants (SSRIs)
72
REM sleep
-time of night
-function
-features
-changes by age
Occurs in each sleep cycle but becomes longer and more intense throughout the sleep period and thus predominates in second half of the night
Important for emotional processing of memories and emotion regulation abilities
Most dreaming occurs during REM.
Most similar to wakefulness and easiest to awaken from
Characterized by active EEG, irregular heart rate and breathing, rapid eye movements and body twitches
Infants spend 50% of the time in REM at 3 mo, by 12 mo 30% and by adulthood 25% or less
73
Non REM sleep
-stages
-features
-timing
Three stages: drowsy (N1), light (N2), deep or slow wave sleep (N3)
N3 predominates in first third to half of the night
N3 is most important stage for memory consolidation
74
Vision anatomy: posterior visual pathway
Areas involved in processing signals AFTER the signals are relayed through the lateral genticulate nucleus in the thalamus including optic radiations, occipital cortex, visual associative areas in the temporal and parietal lobes
75
Vision anatomy: anterior visual pathway
Includes the retina, optic nerves, optic chiasm, and optic tracts
76
Vision anatomy: ventral stream (what it processes)
Inferior temporal visual association cortex
Processes the WHAT of a stimulus (face and shape recognition, orientation)
Getting lost in familiar environments
77
Vision anatomy: dorsal stream (and what it processes)
Parietal association cortex
The WHERE of a stimulus (visual guidance, visual search, visual attention)
Difficulty finding someone in a crowded room or something in a cluttered toy box
78
Vision anatomy: middle temporal lobe
Motion perception
Difficulty seeing moving objects
Increasingly recognized in children with PVL
79
Cortical visual impairment
-causes
-associated conditions
-natural history and features
-Dx
One of the most common causes of visual impairment in developed countries
Caused by injury to or abnormal development of visual areas in the brain (HIE, PVL, hydrocephalus, CNS infections, TBI, congenital brain malformations)
Associated with: CP, GDD, epilepsy,PVL
Improves over time especially between ages 1 and 3 because of visual neuroplasticity
Respond to light and dark, do not follow faces, vision fluctuates throughout the day, respond well to color stimulation
Eye exam may be normal, normal pupillary responses
80
Craniosynostosis syndromes
Premature fusion of one or more cranial sutures
Eg: crouzon, pfeiffer, apert
Papilledema and secondary optic atrophy are major concerns
81
Pierre Robin Sequence triad
(And syndrome correlations)
Micrognathia
Glossoptosis
Upper airway obstruction
Strong correlation with syndromes associated with ocular abnormalities (stickler, 22q11, treacher collins)
82
Aniridia
Iris located between cornea and lens
Those born with hypoplastic iris presenting with seemingly dilated or irregular pupil
Associated syndrome is WAGR (wilms tumor, aniridia, GU abnormalities, retardation/ID)
83
Cataract
Clouding of normally clear lens
May be congenital or acquired
84
Glaucoma (and one of the most common associated conditions)
Progressive damage to child’s eye and optic nerve cause by elevated intraocular pressure
May be primary condition or secondary to systemic disease
In children most commonly maldevelopment of drainage pathway of the aqueous humor in the anterior segment of the eye
Sturge Weber= most common systemic condition associated with glaucoma
85
Optic nerve hypoplasia
Abnormal development of the optic nerve, can be unilateral or bilateral
Impaired pupillary response to light. Nystagmus often present. Variable degree of vision impairment
Etiologies: chromosomal abnormalities, maternal diabetes, fetal alcohol exposure, OI, apert syndrome
Associated with underdevelopment of other midline structures including the pituitary
86
Nystagmus
Rhythmic eye movements that can be caused by sensory or motor pathway
Severe visual deprivation causes nystagmus
Severe congenital nystagmus can be treated with Botox or surgery
87
Spasmus nutans
Triad of nystagmus, torticollis, and head bobbing
Benign but need brain MRI because can be associated with optic chiasm tumors or neuroblastoma
88
Strabismus
Condition where eyes do not align properly when focusing on an object
Can be associated with conditions like CP
89
Eye deviation direction terms
Esotropia: one eye turns in
Exotropia: one eye turns out
Hypertropia: one eye turns up
Hypotropia: one eye turns down
90
Photoscreeners
Tool for vision screening involving a special camera that can automatically detect refractive errors
Can be useful with very young children and older whilst with disabilities who are nonverbal
91
Legal blindness and low vision
For the pediatric age group and especially nonverbal children there is no universally accepted definition of blindness
Legal blindness refers to best corrected distance visual acuity in the better eye of 20/200 or less or a visual field in the better eye of less than 20 degrees
Low vision refers to best corrected visual acuity 20/70 to 20/200 although not considered legally blind to meet eligibility requirements for special education services in a public setting
75% of legally blind children have some remaining vision that is functional
92
Amblyopia
Reduced visual acuity and depth perception due to abnormal visual development
Usually unilateral
Caused by lack of visual stimulation during critical periods for development of visual pathways (birth to age 7 or 8)
Can occur due to uncorrected anisometropia or strabismus. brain not able to sue both eyes together so the eye with the blurrier or deviating vision is ignored. Another cause is deprecation (cataract or corneal opacity obstructs visual axis)
Treatment is remove the cause (fix strabismus, corrective lens) and force use of affected eye (patch the stronger eye). Most effective early (before age 8)
93
Anisometropia
When eyes develop different refractive powers
94
Educational supports for blindness
Important to refer to EI early
Teacher of the visually impaired: provides information and training about possible developmental and academic challenges, specialized instruction such as teaching braille or training on equipment or low vision devices
Orientation and mobility specialist: determined need for adaptive equipment or mobility device and teaches about concepts of space and directionality and strategies for safe and effective travel within familiar and unfamiliar environments
For school age kids, educational eval to assess impact and determine recommendations
95
Threshold dB for normal hearing in adults and infants/young children
Adults = 20
Infants and young children = 15 (require better sensitivity)
96
Conductive hearing loss
Most common
Results from interference with mechanical transmission of sound through the external and inner ear. May resolve with time or be correctable
97
Sensorineural hearing loss
Permanent hearing loss resulting from dysfunction of the inner ear. Failure to transfuse vibrations to neural impulses in cochlea or transmit those impulses in the vestibulocochlear nerve
98
Mixed hearing loss
CHL and SNHL in same ear
99
Auditory neuropathy spectrum disorder
Neural hearing loss likely due to dysfunction in the ability to encode the signal in the transmission process between the auditory nerve and auditory cortex
Typically bilateral and explains 10% of new cases
Auditory, speech, and language outcomes are highly variable
100
Cortical deafness
Rare. Can occur in context of diffuse brain damage and is associated with central level difficulties perceiving or discriminating sounds
101
Level of hearing loss: profound
>90 dB
102
Level of hearing loss: moderate
41-55 dB
103
Level of hearing loss: mild
26 to 40 dB
104
Level of hearing loss: moderately severe
56-70 dB
105
Level of hearing loss: severe
71-90 dB
106
Genetics of hearing loss
> 400 genetic causes explain 30-50% of SNHL
>70% of those are non syndromic and commonly autosomal recessive
107
Genetic conditions commonly associated with hearing loss
Down syndrome (most common CHL due to middle ear effusion and craniofacial characteristics)
Turner (also most frequently CHL)
22q11 (mostly CHL 68% but also SNHL 14% and 18% mixed)
108
Neurodevelopmental conditions and hearing loss
Articulation delay
Speech delay
Developmental delay
ID
ASD
CP
For children with developmental disabilities hearing loss may be overshadowed by complex medical conditions or developmental needs
109
Risk factors for hearing loss: perinatal
Family history
NICU > 5 days
Low birth weight
Low apgars
Hyperbilirubinemia w ET
Aminoglycoside > 5 days
Asphyxia or HIE
In utero infections
Birth conditions or findings (craniofacial, microcephaly, hydrocephalus)
Genetic syndrome associated with hearing loss
110
Risk factors for hearing loss postnatal
Infection (meningitis, encephalitis)
Head trauma
Chemotherapy
Caregiver concern
Persistent otitis media with effusion
111
Hearing evaluations: tympanometry
-method
-ages
-results or pros
-conditions or cons
Ear canal pressure varied while sound reflection by TM measured
All ages except newborn
Diagnose middle ear disorders and normal in SNHL
Does not test hearing and assess inner ear
112
Hearing evaluations: ABR = auditory brain stem response
-method
-ages
-results or pros
-conditions or cons
Earphones, EEG wave forms in auditory nerve and brain stem are recorded. Measures activity of neural fibers along the auditory pathway.
Birth to 9 mo
Objective, ear specific, screens for auditory neuropathy. Detect SNHL due to cochlear and auditory nerve abnormalities
Infant must be sleeping, does not assess cortical processing, automated only, pass/fail. More difficult to do and more invasive than OAE
113
Hearing evaluations: OAE (otoacoustic emissions)
-method
-ages
-results or pros
-conditions or cons
Click and tone bursts presented, sound generated by hair cell response (acoustic emissions) is recorded by a microphone
Measures function of auditory pathway from outer ear to external cochlear hair cells
All ages
Objective, ear specific, rapid, inexpensive
Infant must be quiet, not assess cortical processing. If infant passes hearing is presumed normal but only to level of cochlear hair cells
affected by middle ear debris (effusion, cerumen) that can interfere and high rate of false positives
114
Hearing evaluations: behavioral audiometry
-method
-ages
-results or pros
-conditions or cons
Soundproof room presented with toned, speech, noise responses are recorded (startle, head turn)
Birth to 6 mo
Assess auditory function and perception
High intensity stimulus required, skilled examiner, does not assess ears individually
115
Hearing evaluation: visual reinforcement
-method
-ages
-results or pros
-conditions or cons
Conditioned to look at speaker by animated toy which is lit, centering toy to turn back. Earphone can be used
6-9 mo to 2.5 years
Assess auditory function and perception. Accurate frequency specific thresholds can be gained
Requires skilled examiner
116
Hearing evaluations: play audiometry
-method
-ages
-results or pros
-conditions or cons
Drop block or place peg in response to tone. Varying frequencies delivered via headphones or bone vibrator
2 to 5 years (and up, can change the activity to hand raise)
Assess auditory function and perception, ear specific
Attention span is limiting factor
117
Newborn hearing screen
Performed prior to 1 month
Most widely used in full term nurseries is OAE but can use ABR.
Typically if don’t pass OAE will have ABR
118
Hearing screening: when to screen
Even if newborn passes hearing screen, if risk factors, eval recommended at 9 mo
Regardless of previous screening outcomes or risk factors should provide ongoing surveillance.
Under 4 years: monitoring and parental report, can use OAE
4 and up: behavioral screening
If caregiver concern or do not pass screener, refer for eval
AAP screening audiometry at 4,5,6,8 and 10 years. High frequency screen between 12 and 14, 15 and 17, and 18 and 21
119
Impact of hearing impairment on speech and how to mitigate
A child’s hearing status in and of itself does not necessarily have an impact on development when hearing loss is identified early and consistent access to communication is established
Appropriate access to language is critically important (ASL or spoken)
Deaf children at significant risk of language delays compared to their peers
Children with severe to profound may not follow typical patterns and progress may plateau
Gains made during EI may not be maintained through childhood
Even with slight, at risk for academic and behavioral problems
Mild hearing impairment at risk for problems with various aspects of language (morphosyntax, word sentence formation, grammar). Not at increased risk of difficulty with other skills (processing speed, spatial relationships, working memory)
25-50% of speech may be missed with mild impairment and most missed with moderate impairment
Speech quality may be typical with mild impairment but more likely to be poor in moderate impairment
Consistent use of hearing aids can mitigate negative outcomes
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Percent of deaf children with an additional developmental disability
40%
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When to cochlear implant
Early intervention is key
FDA approved for 9 mo and up.
9-24 mo for bilateral profound
2 years + bilateral severe to profound
Unilateral and asymmetric can qualify if one ear is profound in children over 5
Benefit most from implantation before 3 years
As a general rule children with developmental disabilities are candidates
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What is the 1,3,6 guidelines for hearing loss (rule of thirds)
Hearing screen done by 1 month of age
Diagnosis of hearing loss by 3 months
Start EI by 6 months
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If fail hearing screen, follow up diagnostic testing by:
3 months
124
Top three causes of blindness in the US
1. Cortical vision impairment
2. Retinopathy of prematurity
3. Optic nerve hypoplasia
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Percent of those with vision impairment with co occurring diagnosis
50-70%
126
Visual impairment impact on cognitive development
Delayed object permanence
Delayed understanding of concepts about their environment
Exploratory play typically develops, functional and symbolic play are delayed
More concrete descriptions of people and objects
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Visual impairment impact on social emotional development
Different eye contact
Appear withdrawn and more passive
Delayed understanding of self as separate
Reduced response to cues
Less responsive smiling
Increased risk for attachment disorders
Initiate fewer social interactions
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Visual impairment impact on adaptive functioning
Feeding problems, slower progression to solids
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Visual impairment impact on communication development
Prolonged echolalia
Delay in production of phrases
Pronoun reversals
Vocabulary develops typically
More descriptive and less action words
Qualitative difference noted by 2 years of age
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Visual impairment impact on motor development
More sedentary without visual motivators
May lead to hypotonia or low arousal or sleepy state
Sitting and standing occur at typical time
Delays in walking, climbing
Broad based gait with balance challenges
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Visual impairment impact on behavior
May appear to have stereotypic behaviors such as rocking, head weaving, preventative movements, to gain sensory input in other ways that aren’t necessarily RRBs
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Functional vision assessment
Provides understanding of a child’s degree of vision loss (acuity) and the aspects of visual function that are impacted (things like color perception, convergence, depth perception, light sensitivity etc). These assessments are used to determine the types of tools, technology, and accommodations needed
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ASL and spoken language
Lack of research to support use of ASL or signs interfering with spoken language. Access to natural sign language may enable neurological structures for learning language in deaf children, ultimately facilitating spoken language
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Hearing impairment impact on cognitive development
Deaf children have equal cognitive potential as hearing children
As a group deaf children outperform on particular visual spatial tasks
Where cognitive deficits have been implied, differences associated with reduced language access or language proficiency
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Hearing impairment impact on social emotional development
On average deaf children have increased social, emotional, and behavioral challenges regardless of the severity or onset point for their hearing loss
Variability in social emotional function attributed in part to reduced language access
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Hearing impairment impact on academics
Deaf children’s academic skills have lagged behind, need additional supports at school to mitigate effects
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Hearing loss management: services and education
Refer to EI ASAP
Language intervention (ASL instruction, teacher of the deaf)
Determining accessible education depended on how child accesses communication, how child communicates most effectively and opportunities for interaction with deaf peers. Can be: gen ed, center based resource program, day program, residential school
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Hearing loss management: assistive technology
FM system (remote microphone systems, improve signal to noise ratio)
Closed captioning and telecommunication
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Hearing loss management: audiology and medical
After diagnosis hearing reevaluated q6-12 mo
Listening devices options:
Traditional air condition hearing aids
Bone conducting hearing devices (CHL)
Cochlear implant: SNHL or ANSD who show little benefit with rational hearing aids
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Sexuality and children with disabilities, as compared to typical
-desire for sexual behavior
-engagement in sexual behavior
-use of protection
-coercion and abuse
-sexual education
-family comfort with discussion
-disclosure requirements
Same
Increased
Decreased
Increased
Decreased
Decreased
Most with ID not required to disclose and if guardianship not established often capable of addressing their medical, mental health, and sexuality needs
High interest with low knowledge can lead to risky behavior
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Puberty and those with disabilities
-puberty onset and progression as compared with typical
-HPV vaccination
-menstruation
Mostly the same
Encourage
Start anticipatory guidance at stage 2 or earlier; watchful waiting at stage 3 with washable underwear, plan for dysmenorrhea
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Spina bifida and fertility
Male altered
Women normal
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ASD and gender/sexual identity
Greater diversity than typical peers
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Sexual behaviors in children that are rare and cause for concern
Trying to have sexual intercourse
Mouth to genital contact
Asking others to do sex acts
Pretending toys are having sex
Inserting object or finger into rectum or vagina
Behaviors invoking children of disparate ages or abilities
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Development of gender identity
Children identify their sex by 2 years and express gender specific behaviors by 2-4 years
Distress with gender in congruence increases with age
Anatomy dysphoria more common in adolescence
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Gender dysphoria
Significant distress from having a gender identity that does not match one’s assigned gender
Strong desire to be or insistence on being a different gender than the assigned gender. For children 5 of the following 7
Preference for clothing
Preference for roles in imaginative play
Preference for toys, games, activities
Preference for playmates
Preference for sex characteristics of experienced/expressed gender
Strong rejection or dislike of toys, games, activities of assigned gender
Strong rejection for sexual anatomy
Present for 6 months
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Four phases of swallowing
Oral preparatory:
Oral propulsive
Pharyngeal
Esophageal
During pharyngeal phase bolus is prevented from entering nasal cavity and airway
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Causes of dysphagia
1) Cranial nerve palsy, dental issues, and reduced saliva production can impact bolus preparation
2)Cleft palate can result in nasal aspiration
3) webs or strictures can cause narrowing in the pharynx, esophagus or sphincter is leading to obstruction of bolus movement
4) low muscle tone, cerebral palsy, and other neuromuscular disorders can impact muscle function and coordination that can result in poor oral preparation and propulsion of bolus through the pharynx and esophagus
