Ph D CCCSLP LambGSHA2020 1 Financial and nonfinancial disclosure statement Relevant Financial Relationships I am a salaried employee of Valdosta State University University System of Georgia ID: 910985
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Cleft Lip and Palate
Katherine Lamb, Ph.,D. CCC/SLP
Lamb_GSHA_2020
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Slide2Financial and non-financial disclosure statement
• Relevant Financial Relationship(s): I am a salaried employee of Valdosta State University; University System of Georgia.
• Relevant Nonfinancial Relationship(s): I have no relevant non-financial relationship(s) to disclose related to this presentation at GSHA.Lamb_GSHA_2020
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Slide3ANATOMY REVIEW
Three cavitiesMuscles of velopharynxLevator veli
palatini Tensor veli palatiniMusculus
uvulae Superior constrictor Palatopharyngeus
Palaoglossus
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Slide4Cranial Nerves
Motor GlossopharyngealVagus Accessory
Trigeminal Facial Sensory Vagusglossopharyngeal
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Slide5Physiology subsystems
RespirationPhonationBernoulli effectResonance
ArticulationStress and intonationcoordination
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Slide6Physiology of velar mechanism
Velar movement SpeechVelar dimpleInferior side or velum
Superior sideElevates and elongates
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Slide7Classification of clefts
Primary palateAnterior to incisive foramenLips and alveolusSecondary palate
Posterior to incisive foramenHard and soft palate
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Slide8Causes
Disruption or delay:Embryological developmentChromosome/genetic disordersTeratogens or mechanical factors
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Slide9Causes
VirusesMaternal nutritional deficiency and obesityLip w & w/o palate 2X malesPalate 2X females
Timing differences in developmentMechanical interferenceMultifactorial inheritance
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Slide10Impact
cleft of primary palateNose separation of orbicularis oris
muscles misalignedColumellashortened(appear) attached to noseNasal cavitiesdeformed and airway reduced in size
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Slide11Impact
cleft of secondary palateComplete thru velum velar/palatine
Muscle insertions are abnormalLevator veli palatini-midlinepalatophayngeous
insert into posterior borderTensor veli palatini
not functional
cannot regulate eustachian tube
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Slide12Submucous cleft palate
OvertBifid uvula, zona pellucida
and notch in posterior HPOccultNo overt signs Nasal regurgitation, chronic ear infection
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Slide13Prevalence and treatment of submucous cleft
.02 and 0.08% in general population25-50% will have velopharyngeal dysfunctionSx if leads to velopharyngeal dysfunction
Palatoplastyyoung childpharyngeal flap for older
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Slide14Facial clefts
Oblique cleftMouthMidline cleftnotch in midline of vermillion
or slight upper lipor with brain involvementEncephalocele-gap in skullAbsence of corpus callosumHoloprosencephaly-failure of forebrain to divide
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Slide15Embryological development of lip and palate
Development of lip and alveolus begin at 6-7 weeks Development of palate begins at 8-9 weeks lingual was highdrop at 7-8 weeks
palatal shelves move from vertical to horizontal and fusepremaxilla at incisive foramenVelum Uvula 12 weeks
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Slide16Embryological Development
Variations during:
(1) embryonic period (2) very early fetal period
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Slide17Normal Embryological Development
1st
3 weeks: single cell to multicellular Flat disc of 1 layer 7th day
Beginning to 2nd week:
2 layers
3 layers
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Slide18Development
3 layers = all tissues and organs
Disc thickens as cells differentiate greater thickening at cranial end
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Slide1919
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Slide20Genetics evaluation
Dx Hx
Counseling and family supportDysmorphologyMalformationDeformation
TeratogensAmniotic bands
Maternal illness
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Slide21Syndromes, sequences, associations
SyndromeSequence
AssociationLamb_GSHA_2020
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Slide22Dentition
Normal dentitionAnomaliesIncisor relationship
OverjetOverbiteUnderbite
Missing teethRotated teethSupernumary or ectopic
Crossbite
Protruding
premaxilla
Open bite
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Slide23Dental development
Infant stagePrimary stageEarly mixedLate mixed
AdolescentLamb_GSHA_2020
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Slide24Psychosocial Issues
Family Shock and adjustmentSupportEmotional
Feeding School EducateMost: average cognitionSocial interaction
Fewer friendsLess socially competentTeasing
Self-perception
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Slide25Societal issues
Physical appearanceSpeech qualityHearing impairment
StigmaLamb_GSHA_2020
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Slide26Impact
Cleft of primary palateNose separation of orbicularis oris
muscles misalignedcurveColumella: shortened Appears attached to noseNasal cavities deformed
reduced in size
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Slide27Impact
Cleft of secondary palateVelumMuscle insertions are abnormal
Levator veli palatini palatophayngeous Tensor veli palatini
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Slide28Presurgical management
Lip Alignment neededThree optionsTape lip along with dental elastics for 4-6 weeks
Latham appliance uses 2-piece acrylic device gradually brings palate together and lip as well; used with nasal alveolar molding deviceLip adhesion at 6 weeks of age with full repair 3-4 mos. laterLamb_GSHA_2020
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Slide29Techniques for unilateral lip repair
Millard
rotation advancement flap technique; aka cut as you goRandall-Tennisontriangular flap technique; fixed technique that is precise and measured
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Slide30Cleft lip repair
Cheilopasty is usually completed @10 lbs
10 weeks oldHemoglobin of 10gmMost repair lip between 4 and 12 weeks
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Slide31Techniques for bilateral lip repair
Millard
Broadbent-ManchesterBoth done between 9 months and 5 yearsOnly difference is how white roll of philtrum is created
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Slide32Palatoplasty
Two groups for repair
Early = 6-15 mos. Late = 15-24 mos.LOTS of debate on when and how but overall, but generally accepted that delayed closure of HP has negative effect on speech.
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Slide33Techniques for Palatoplasty
Von
Langenbeck – oldest and most successfulWardill-Kilner V to Y – higher incidence of anterior fistulae
Intravelar veloplasty (IVVP) – to repair
levator
sling
Two-flap palatoplasty
Furlow
palatoplasty
Early repair is better
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Slide34Fistula repair techniques
Most cases, fistula is deliberately left in alveolus
Usually closed during time of mixed dentition with bone graft from rib or iliac crestUnintentional fistulas can be fixed but carry >37% recurrence risk
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Slide35Surgery for VPI
PharyngoplastyCannot dx until able to produce speechEarliest: 3.5 to 4 y/oVPI is a surgical disorder
Needs of patientLamb_GSHA_2020
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Slide36Techniques
Redo palatoplastyLengthens the palatePharyngeal wall augmentationVP opening is small
Implant deep in superior pharyngeal constrictorsRolled flap –pharyngeal wall to produce bulgeLamb_GSHA_2020
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Slide37Techniques
PharyngoplastyPharyngeal flapBest for midline gaps
Long and high as possibleSphincter pharyngoplastyMyomucosal flaps
Rotate posteriorlyInset into nasopharynx
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Slide38Complications
EdemaApneaLess common with pharyngeal flap
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Slide39Facial structures
MaxillaRetrusion: small maxilla relative to mandibleFacial nerveImpacts what phonemes?
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Slide40Oral cavity
LipsShorten upper lipPhonemes affected?
MouthMacrostomiaMicrostomia
lingualMacroglossiaMicroglossia
Lobulated: multiple lobes with fissures
Ankyloglossia
Tonsils and adenoids
Faucial
Lingual
Pharyngeal
Palate
High arched vs. low flatPalatal or oronasal fistula40
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Slide41Upper airway obstruction
Adenotonsillar hypertrophyTonsillar hypertrophy
Lingual tonsil hypertrophyAdenoid hypertrophyImpact nasal breathingObstruct Eustachian tube functioningSpecific facial presentation
Mouth breathingAnterior lingual carriageLowered mandible
Puffy eyes
Pinched nostrils
Stertorous
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Slide42Treatment of UAO
TonsillectomyAdenoidectomyTracheostomyUvulopalatopharyngoplasty (UPPP)
CPAPLamb_GSHA_2020
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Slide43Nasometry
Nasometer: measures nasal acoustic energymeasuring acoustic energy in nasal cavity and oral cavity
calculate ratio Score higher = hypernasality; lower = hyponasalityClinical useMeasure Equipment
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Slide44Nasometric procedures
Sensitivityabnormal resonance
Specificitynormal resonanceInterpreting numbers
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Slide45Sensitivity
ID individuals with abnormal resonance
SpecificityCorrectly excludes those with normal resonanceVariability of scores and lack of agreement PERCEPTION not just numbers
Interpreting numbers
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Slide46Interpreting
nasogram
Normal oral resonance is 15-20% pointsExpected difference between /a/ (ah) and /i/ (
ee) is about 10 points with oral consonants and 20% with nasal consonants
Higher contour on screen, more hypernasality to expect
If most data points are normal, but have occasional peaks, suggests normal resonance with nasal emission
Gradual rise indicates muscle fatigue
No data points on /s/ or /
sh
/, but if do get data points on these without others then consider phoneme specific nasal emission
Lingua-
alveolars and bilabials higher, consider fistulaHigh on vowels but prolonged /s/ is zero, consider thin velum, high tongue placement or vowel-specific nasalityLow data points overall, hyponasality and maybe upper airway obstructionLamb_GSHA_2020
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Slide47Pressure –Flow technique
Small bore cathetersoral cavitynostril
Other nostrildetermining presence and extent of VPIEquipment and calibrationLamb_GSHA_2020
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Slide48Assessment of nasal airway
Obstruction via nasal resistancePosterior & anterior rhinomanometryClinical procedures
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Slide49Low or No technology assessment
Counseling and support1st yearAnnual screenings & assessments
Language screeningSpeechArticStimulabilityNasal air emission
Weak consonantsUtterance lengthOral-motor dysfunction
Resonance
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Slide50Basic Assessment
Visual detectionMirror test figureAir paddle-figure Tactile detection
Auditory detection Nose pinch Stethoscope Listening tube
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Slide51Differential dx of cause
VPIOrofacial fistulaArtic disorder
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Slide52Follow up
RecommendationsFamily counselingEvaluation report
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Slide53Videofluoroscopy
Multiple viewsLateralFrontalBase
Oblique Barium:orally noseSpeech sample
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Slide54Advantages and limitations
AdvantagesMovementVelumlateral/posterior
Length of velumDisadvantagesRadiation Not as good as direct viewNot 3D
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Slide55Nasopharyngoscopy
Flexible fiberoptic nasopharyngoscopyCameraHigh res monitorMicrophone
RecordingLamb_GSHA_2020
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Slide56Nasopharyngoscopy
Advantages/disadvantagesNo radiationDetailBiofeedback
Minimal risk CooperationLamb_GSHA_2020
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Slide57Resonance disorders and VP dysfunction
VPD Not = VP insufficiencyNot = VP incompetence
Not = VP mislearningLamb_GSHA_2020
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Slide58Nasality
HypernasalityMore noticeable on vowelsNasal twang Hyponasality and denasality
Hypo Denasal Cul-de-sac resonanceMixed resonance
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Slide59More effects of VPD
Nasal emission: build up oral pressure Leak
Pressure-sensitive phonemes Sibilant soundsNasal grimaceeffort to close VPWeak or omitted consonants
Short utterance lengthAltered rate and speech segment durations
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Slide60Compensatory and obligatory articulation
Passive speech characteristics structural abnormalityActive speech characteristics
response to VPDMiddorsum palatal stop: sub: /t,d,n,l,k,g/; sometimes: /s,z,,zh,
sh,ch,j/Generalized backingVelar fricative
Nasalization of v/c
Nasal
snort
sniff
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Slide61Compensatory and obligatory articulation
Pharyngeal fricativeGlottal stop/h/ for voiceless plosives
BreathinessDysphoniabreathy, hoarse, low intensity, glottal fryIncreased muscular effort and respirationNodules
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Slide62Factors impacting speech and severity
Size of VP openingInconsistency of VP closureAbnormal artic and phonation
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Slide63Causes of VPI dysfunction
VPI (insuff)Hx of cleft palate
Submucous cleft palateShort velum/deep pharynxAdenoid atrophyIrregular adenoidsHypertrophic tonsils
VPI post treatmentAdenoidectomyTonsillectomyMaxillary advancement
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Slide64Causes of VPI dysfunction
VPI (incomp)Abnormal muscle insertion
Hypotonia/poor pharyngeal wall movementDysarthriaApraxiaCranial nerve defectVelar fatigue or stress incompetence
VP mislearningFaulty articHabituated speech patterns
Lack of auditory feedback
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Slide65Anatomy of the ear
External Middle Inner
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Slide66Malformation of ear
ExternalMicrotia: malformed pinnaAural atresiaMiddle
Eustachian tube dysfunction and middle ear diseaseInnerRare (syndromes)Sensorineural HL
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Slide67Infant anatomy for feeding
Smaller / closer Oral cavity is ideal for sucklingRestricted ability to open oral cavity Extension/retraction of lingual
Larynx Epiglottis protrudes into nasopharynx Lamb_GSHA_2020
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Slide68Oral phase of swallowing
Rhythmic suckling create pressurePalate must be intact; compression of nippleOral cavity must be closed posteriorly
Any opening Lamb_GSHA_2020
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Slide69Pharyngeal phase of swallow
Bolus channeledCoordination of breathing and swallow and sucklelingual, velum and pharyngeal wall Velum elevates and closes VP
lingual base movesBolus diverts around epiglottisBreathing thru noseAbduction of VFArytenoids
EpiglottisLamb_GSHA_2020
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Slide70Esophageal phase of swallowing
Pharynx to esophagusUESLES After swallow
suckling and breathing resumeUES and LES closeSuck-swallow-breathe ratio is 1:1:1 to 2:1:1Lamb_GSHA_2020
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Slide71Characteristics of feeding problems with clefts
Poor oral suctionInadequate volume Lengthy feeding Nasal regurgitation
Excessive air intakeCoughingChokingVaries
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Slide72Characteristics of feeding problems with clefts
Pharyngeal phase normalProblems maintaining airwaypharyngeal, esophageal or CNS abnormalities airway protection
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Slide73Characteristics of feeding problems with clefts
Cleft of lip usually not as problematicMinimal cleft of velum may be able to feedMore anterior cleft Nasal regurgitation (common)
StressfulLamb_GSHA_2020
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Slide74Breast or Bottle feeding
Breast feedingNot challenge (lip)Modified nippleBottle feeding
PliabilityShapeSizeHole type and size
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Slide75Oral-motor facilitation techniques
Positioningupright at 60 degreeschin-tuckarms forward
trunk in midlinehips flexedNipple under shelf of hard palatePacing intakeOral facilitation
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Slide76Oral-motor facilitation techniques
Manage nasal regurgitationConsistency of method Feeding obturatorOral hygiene
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Slide77Feeding older infant
Soft cup feeder to aid transitionSolid foods-thickened
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Slide78Prosthetic devices
Dental appliancesFixed bridgeDenturesOverlay dentures
Facial prosthesesFeeding obturatorsCovers unrepaired cleftlingual out of cleftProvides surface
Does not help with velum
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Slide79Speech Appliances
Palatal liftRemovable deviceelevates velumVP incompetence
Takes timeInterferes with nasals and resonancePalatal ObturatorCover palatal fistulaSx
not in near future
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Slide80Speech Appliances
Speech bulb obturatorRemovableVP insufficiency
Stops nasal regurgitationSwallowingRarely used with childrenFabricationFittingEasier to fit palatal obturator for fistula
Lift or bulb function
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Slide81Indicators and contraindicators
ProsPersistent VPI Sx
not workNeurological compromisedGood teeth ConsRemoveNight & eating
ExpensiveEasy to lose and damageUncomfortable
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Slide82Speech therapy
Eliminate compensatory artic productions can help eliminate nasal rustleReduction therapyLength gradually shortened or…
Time wearing it reducedLamb_GSHA_2020
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Slide83Timeline for Intervention
Infants and toddlersFeeding is number one Language development next
Quantity of speechSLP & language stimulation activitiesEnroll if feeding or language issuesFacilitate appropriate placement
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Slide84Intervention
Preschool childrenAssess language and speech development and VP function (3y/o)Sx best between ages 3-5
Include parents and siblingsEasier to fix errors earlyMay alleviate teasing later
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Slide85Intervention
School age childrenVPI should be corrected Hypernasality or nasal emission Focus is on any artic errors
Only time we target obligatory errorsTarget compensatory errors < developmental Rarely provide treatment for teen or adult
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Slide86Interventions
Basic PrinciplesEarly involvement ParentsPlan aimed at problems identified
Simple explanations for the childStart with an area of likely successFocus on motivation and increase insight
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Slide87Interventions
Attention to volume, rate, and prosody Insufficient or incompetent SP: exercises will not helpBlowing and sucking exercises are not effective
Follow normal phonetic and phonological developmentAddress both language and speech developmentIncrease sensory awareness and movement
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Slide88Interventions: Lips
Gentle stroking from top in midline encourages lip pursingStimulation with soft brush
Rub lips with Vaseline: sensation of contact Soft toysMassage with a facial spongeFunny facesPull small object to extract object; lip pressure
Paint face; pay attention to lipsLip movement--encourage vocalization/babblingMouth opening
Wide-open
Yawning
Encourage vocalization with wide-open mouth
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Slide89Interventions:
lingual tip/Alveolar ridge
Sensory awareness with toothbrushPressure on alveolar ridgePlace lingual-tip on pressed spot
Identify spots with cotton swabs or toothettes“Sticky” to identify points
Food around the mouth and lick
Oral air-stream
Feel the air-stream on back of their hand
Blowing activities to demonstrate result of air-stream
cotton balls
bubbles
feathers
Blow into cupped hands or down a tube89
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Slide90Interventions:
AirstreamBlow up cheeks Introduce consonants /f/ or /s/
Feeling the vocalization (parent/clinician)Drumming fingers on lipsAssist in producing bilabials, labio-dentals
Imitation of oral vs. nasalImitated vegetative noises & reinforce
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Slide91Interventions: Babbling and Sounds
CV combinations: includevoiced/voiceless variations of loudness and intonation
Repeat /wa/wa/wa
/Encourage blowingInterrupt: stop air-stream by opening/closing lips or moving lingual up and down
Feel larynx of others
Sing /la/la/la
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Slide92Interventions: Young Children
Perceptual skills
Discriminatory work (phonological contrast) Auditory input tied to attempts at production Explain correctedCorrect articulation
presence of persisting nasal emission place is prime
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Slide93Interventions
Introduce new sounds:developmental sequence
difficultydemonstrationUse any procedures=results
air-stream on handfeeling larynx
movement of air-stream
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Slide94Interventions Hypernasality
Adequate breath support
Ear training to recognize desired target Encourage:low lingual carriagemouth openingAdequate loudness
Optimum pitchDo not precipitate laryngeal elevation to reduce hypernasality Practice CV sequences/words for desired resonance (easy)
Practice sequences low in nasals
Eliciting Plosives
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Slide95Principles
Auditory firstAnd on production
Imitation firstMirrorPlace and manner
Diagrams
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Slide96Plosives /p/b
Achieving manner and voicingshort sounds, bursts, puffs
voiceless, puffy; voiced, buzzy Begin with voicelessInterrupt gentle air-streamShow visible resultStrengthen lips for
plosionmaintaining seal against resistance
Puff out cheeks before release
Feel larynx for /b/
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Slide97Achieving place
Awareness of articulators by increasing sensory awarenessGood lip movement /w/
Vaseline Imitate silent open/closing of lipsgradually introduce breathHum with pressed lips, followed by a /p/ or /b/
Test stimulability in all phonotactic positions
Imitate silently, words which include bilabial plosives
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Slide98Alveolar plosives t/d
Frequently replaced by glottalLack precise lingual-tip contactNasal emission
Achieving manner and voicingNature of soundsSound concepts
Voiceless first
Symbols for voiced
Diagrams
Feel larynx for /d/
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Slide99Achieving place
Children: stories Adolescents/adults: diagrams
Head forward Clinician press on alveolar ridgeChild presses Lift lingual to contact
Dental elastic between lingual-tip and contactHold
Out of mouth on lingual-tip
Return, holding elastic
/n/ for start
Increase sensory awareness in lingual-tip
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Slide100Velar plosives /k/g/
May be replaced by glottal plosive or fricativeachieving place is difficult
Achieving manner and voicingConcepts: tapping, knockingBe careful introducing /g/ may trigger glottal
Diagrams of vocal tract and functionslingual and velum tap for plosives
Vocal cords buzz
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Slide101Achieving place
Mirror and diagramsLingual at posterior
clinician touch with toolchild with fingerBig open mouth
Tap under chin at base of lingualId placeEncourage movement
Introduce /k/ and /g/ next to high vowel
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Slide102Fricatives
TermsPlace lacks frication
nasal emissionabsentglottal or pharyngeal fricativeLabio-dental easiest fricative
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Slide103Achieving manner and voicing
Gentle air-stream without strictureFeel air-stream from clinician
Feel voice/no voiceProduction of VC first Use mirror
Manually assist to achieve labio-dental contact
Increase awareness of contact point on lower lip:
Prolong frication initially to increase sensory and auditory awareness
If incisors are missing
upper gum ridge to lower lip
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Slide104Alveolar fricatives /s/z/
Usually most difficult to achieveIncreased intra-oral pressure and precision
Achieving manner and voiceFeel air-stream from clinician to handConcepts of fine air-stream Press spot-to identify
Lift lingual-tip up to spot stimulated by pressure on alveolar ridge
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Slide105Exercises to strengthen lingual tip
Is it easier to produce with lowered or raised lingual-tip
Lingual tip with elastic exerciseEstablish lip spread with teeth close together, Facilitate with /s/z/ adjacent to /t/d/
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Slide106Palato-alveolar
Achieving manner and voiceThick stream vs. thin stream for /s/z/
Achieving placeBroad palatal contactlingual (lateral) pressing teeth
Forward square lips106
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Slide107Affricates
Last Until /t/d/ can be produced
Achieving manner and voiceConceptsCompare fricatives/affricates
Contrastive pairAvoid words that trigger old patterns
Use nonsense words for older children and adults
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Slide108Achieving place
Elicit from place of plosive elementPlace for/t/d/ with slow release
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