Kerry Callahan Mandulak, PhD, CCC-SLP Department of Speech and Hearing Sciences Portland State University, Portland, OR

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1 Kerry Callahan Mandulak, PhD, CCC-SLP Department of Speech and Hearing Sciences Portland State University, Portland, OR Adriane Baylis, PhD, CCC-SLP Nationwide Children s Hospital, Columbus, OH Anna Thurmes, PhD, CCC-SLP University of Minnesota, Minneapolis, MN Special Interest Group 5 Short Course November 19, 2011

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3 Orofacial Clefting (cleft lip and palate) are the most PREVALENT birth defects in the United States 1 in 600 Caucasian births Higher in American Indian / Asian populations Lower in African American population Affects approximately 6800 babies / year

4 Percentage requiring speech-language treatment 50%-75%, from various sources Percentage requiring treatment for resonance disorder 25%-38%, from various sources Percentage with middle ear disease/conductive hearing loss in infancy 95%-97%, from various sources

5 Multifactorial Inheritance Combination of genetic and environmental factors Specific genetic regions have been investigated Isolated or associated with no recognized syndrome Over 350 recognized cleft syndromes Only 2 4% of total cases Specific cleft syndromes (Pierre) Robin Sequence Apert Syndrome Crouzon Syndrome Treacher Collins Syndrome Hemifacial Microsomia Stickler Syndrome 22q11.2 deletion syndrome (Velocardiofacial Syndrome)

6 Number of SLPs who are members of ACPA: 387 (2006) Number of SLPs who are members of ASHA Division 5, Speech Science and Orofacial Disorders: 615 (2007) Number of SLPs who are ASHA members: 122,762 (2006) Percentage of SLPs reporting they feel "not competent" to treat children with cleft lip/palate (Bedwinek et. al, 2010): 44.1%

7 Cleft team SLP / Community SLP Evaluation vs. treatment Scope of practice for SLP = Decreased preparation of SLPs to provide services for children with repaired cleft palate

8 Fewer programs offering courses Less courses offered Small number of training programs Less academics trained to teach courses Less preparation for student clinicians Fewer places for doctoral students

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10 Cleft Lip Only (CL) Varies from a unilateral or bilateral notch of the lip (incomplete cleft) to a complete cleft extending back to the incisive foramen Cleft Palate Only (CPO) varies from a unilateral or bilateral cleft of the hard and/or soft palate Cleft Lip & Palate (CLP) May be unilateral or bilateral Complete bilateral clefts of the lip result in a free-floating premaxilla which is not attached to anything except the columella (Bzoch, 1997)

11 Combination of CLP is more common than CL only or CP only CL +/- CP CP only (isolated CP) Clefts of PRIMARY PALATE Anterior to incisive foramen (lip, alveolar ridge) Clefts of SECONDARY PALATE Posterior to incisive foramen (hard palate, soft palate, uvula) Complete vs. Incomplete

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16 Bifid Uvula Zona Pellucida (midline tissue of the soft palate has a thin and/or bluish appearance) Bony notch of the posterior hard palate (need to palpate to feel, cannot be seen) Tenting of palate on production of ah May or may not have accompanying hypernasal speech or nasal air emission (Peterson-Falzone et al., 2001)

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20 Patients with should be their palates repaired evidence of and if there is or (ACPA, 2000)

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30 The VP mechanism does not merely close for oral speech and then fall open for nasal consonants or silence. It closes to different degrees, depending on such variables as vowel height, voicing, and proximity to nasal consonants. Variables: age, gender, phonemic context

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40 Children who have craniofacial anomalies are at for speech-language disorders. Evaluation of speech and language development provides information that is needed by the team in, particularly surgical and dental management. Further, information about the patient s speech and language is important in the.

41 Children with an isolated cleft lip are unlikely to develop abnormal speech patterns related to the cleft Approximately 20% of children with repaired cleft palate will develop speech deficits that require additional intervention (Witt & D Antonio, 1993)

42 Communication development begins at birth. Controversy over time of palate surgery In a child with a cleft beginning with an impaired mechanism 9 months vs. 12 months vs. 18 months Speech vs. growth Heterogeneous population? Errors cleft related? Or not?

43 Problem Oral and nasal cavities open to each other Lack of place of articulation Fluctuating hearing secondary to chronic otitis media / eustachian tube dysfunction due to faulty insertion of palatal muscles (Hardin- Jones, 2007)

44 Cannot create intra-oral air pressure for sounds like / p b t d k g/ Phonemic repertoire limited to /m/, /n/, ng and vowels Faulty learning for oral air flow sounds Affects manner of articulation

45 Major place of articulation missing 67% of English consonants use alveolar / palatal structures Selective avoidance of consonants that require hard palate contact Research has shown that children with clefts prefer to make sounds in the extremes of the vocal tract (O Gara & Logemann, 1988)

46 VP Dysfunction Palate and pharynx not working together properly for speech production VP Inadequacy VP Insufficiency VP Incompetency Inability of the velum to close off the nasopharynx for the production of oral consonants Structural deficiency: Submucous cleft, short palate/deep pharynx, atrophied adenoids, postadenoidectomy, etc. Neurogenic etiology such as cerebral palsy, TBI, apraxia, etc. (Peterson-Falzone, 2001)

47 Types of resonance disorders Hypernasality Hyponasality Cul-de-sac Resonance Mixed resonance disorder Nasal Air Emission Resonance Disorder Voice Disorder

48 Excessive nasal resonance during speech production Perceptual descriptor (wide range of normal ) Primarily affects vowels and voiced consonants Related to inability to maintain separation of oral and nasal cavities during speech (VPI, fistula, unrepaired cleft) Transpalatal nasalance

49 Reduction in normal nasal resonance Insufficient nasal airflow Primarily affects nasal phonemes (/m/, /n/, ng ) Indicative of obstruction in nasopharynx or nasal cavity

50 Considered disorder Often co-exists w/ hypernasality Can be, not resonance Turbulence / patterns of airflow / size of gap Can be Learned pattern (not due to structural deficit) Normal resonance Treated w/ traditional articulation therapy techniques (Peterson Falzone et al., 2006; Peterson-Falzone & Graham, 1990)

51 Type of hyponasality Caused by an anterior nasal obstruction sound gets trapped in nasopharynx Muffled speech quality

52 Defining resonance is not always clear-cut! The same child can display both hypernasal and hyponasal resonance depending on context Fairly common in cleft population, especially post-pharyngeal flap

53 Hypernasality Hyponasality Mixed Nasality Cul-de-sac Resonance Nasal Air Emission Resonance alteration of vowels and vocalic consonants that occurs when the oral and nasal cavities are abnormally coupled Reduction in nasal resonance that is heard when the nasal airway itself is partially blocked or the entrance to the nasal passages is partially occluded (Peterson Falzone et. al 2001) Elements of both hypo- and hypernasality which cooccur in patients with VPI For example, nasal emission heard on high pressure consonants (s/z), but nasal sounds are hyponasal due to some type of nasal obstruction or septal deviation Muffled characteristic Tight anterior nasal constriction Articulation distortion that affects high pressure consonants (Peterson Falzone et. al 2001)

54 Undiagnosed submucous cleft Palatopharyngeal disproportion (congenitally short palate and/or deep pharynx; ablative surgery to palate and/or pharynx; excessive tissue removal during T & A) Anatomical irregularities involving tonsils, adenoids and/or faucial pillars Limited movement patterns due to neurologic involvement (Peterson-Falzone, 2001)

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56 Glottal Stops Pharyngeal Stops Pharyngeal Fricatives Pharyngeal Affricates Mid-Dorsum Palatal Stops Velar Fricatives (backed oral productions) Posterior Nasal Fricatives Anterior Nasal Fricatives (grimacing) (Riski, 1994)

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58 Also considered obligatory (equipment) Errors Due to Malocclusion Class III ( Underbite or anterior crossbite) May affect tongue placement for /t/, /d/, /s/, /z/ and labiodental placement for /f/, /v/ Class II ( Overbite ) May affect lip closure for bilabials Sibilants may be distorted

59 Crossbite Can contribute to lateralization of sibilants Lowered palatal vault Restricts tongue s articulatory space Dental Deviations Missing teeth may result in lateralization of sibilants Rotated teeth may result in distorted sibilants

60 Kummer, 2001)

61 Kummer, 2001)

62 Kummer, 2001)

63 Hypernasality, nasal air emission, weakened oral pressure consonants and/or structural errors Obligatory or unavoidable errors (probably require physical management) Secondary surgery / Orthodontic / Orthognathic management Compensatory articulation errors Learned errors (can remain after physical management; require speech therapy)

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65 Classification and description of communication disorders (Metz, Schiavetti, & Sacco, 1990; Southwood & Weismer, 1993; Eadie and Doyle, 2002) Gold standard for clinical speech assessment of persons with cleft palate (Kuehn and Moller, 2000) Provide standards against which instrumental measures are evaluated (Kent, 1996) Treatment should only be indicated when a problem is perceived by a listener (Moller, 1991; Conley et al., 1997)

66 Types of Rating Tasks Categorical Mild-moderate-severe Equal-Appearing Interval Scaling 0-6 rating scale Direct Magnitude Estimation Rate the magnitude or ratio of difference between stimuli Other: Visual Analog Scaling, Binary, etc.

67 Intelligibility Articulation Nasal air emission Resonance Voice Acceptability

68 Methods of assessment Categorical Rating scales Percentages Comparison to peers Listener: familiar vs unfamiliar Context: conversation vs predictable tasks

69 Listen for: compensatory articulation errors audible nasal emission weak oral pressure consonants other articulation and phonological errors

70 Listen and watch for: Anterior Crossbite (Class III malocclusion) Distortions: fricatives, affricates, sibilants Labiodentals: reversed labiodental placement Overjet (Class II malocclusion) Bilabials: labiodental placement Missing / malopositioned teeth Distortions of sibilants

71 Listen for: Reduction or elimination the plosive quality of oral stop consonants or fricatives (high pressure consonants p, b, t, d, k, g, f, s, sh, z, ch, dg, th) Pressure improves when you plug the nose Often co-occur with audible nasal emission When severe, oral stop consonants can be perceived as nasal consonants (b m)

72 Listen for: (includes nasal turbulence, nasal snort, and nasal rustle) Obligatory: consistent nasal emission on virtually all pressure consonants usually accompanied by hypernasality Usually present on all pressure consonants except VELARS if due to a fistula only VS. Learned: only heard on specific sounds such as /s/, /z/, /ʃ/, /ʧ/, and/ or /f/ Watch for nasal/facial grimacing

73 Typically used as substitutions for /s/, /z/, /ʃ/, /ʧ/, /f/ Phoneme-specific nasal emission Diagnostic approaches to differentiate nasal fricatives vs. obligatory audible nasal emission Nasal occlusion

74 VPD usually associated with PERCEPTION of excessive nasality (too much nasal resonance) during production of vowels (esp. /i/, /u/), glides (/w/, /j/), and liquids (/l/, /r/) Often co-occurs with audible nasal emission (although inverse relationship with gap size and turbulence of nasal emission) Often co-occurs with weak pressure consonants and/or nasalized oral consonants

75 usually associated with nasal obstruction, obstructive pharyngeal flap, midface hypoplasia, septal deviation, choanal atresia, adenoid hypertrophy PERCEPTION of denasality/too little nasal resonance during production of vowels and nasal consonants /n, m/ and ng Denasalized nasal consonants, makes them perceptually similar to their oral counterpart (/m/ /b/, /n/ /d/)

76 Resonance distortions can vary in or Hypernasality may be inconsistent in presence or severity Hyponasality tends to be consistent in both respects Mixed distortions tend to be the most variable If articulation skills are severely impaired, may make it very difficult to judge resonance E.g., child with pervasive glottal stops

77 Listen for: often associated with VPD or pervasive glottal stop errors Characteristics: rough, breathy, strained, decreased loudness Possible vocal nodules Volume/intensity Is reduced loudness or dysphonia masking the severity of hypernasality? VPD may result in the perception of reduced loudness Is increased loudness/effort being utilized to increase pressure to compensate for VPD?

78 To the patient? Family? Clinician? Surgeon? Based on input of one or all? Impact on future vocational choices?

79 Obtain an adequate speech sample Perceptually analyze each speech parameter Correlate perceptual speech data with any orofacial examination findings Interpret your observations Make impressions/diagnosis Recommend instrumental assessment and imaging if concerns with VPD

80 Target your listening to different portions of the speech sample and RECORD the speech sample so you can listen again later Intelligibility: conversational sample (include some low-predictability questions) Articulation: use standard sentences and standardized testing (e.g., GFTA-2) Voice, resonance, nasal emission: use standard sentences, reading passage, conversational speech

81 Depends on age of patient, cognitive ability, reading ability, attention span Conversational sample Reading passages Picture description task, counting, recite words to a nursery rhyme, etc.

82 Repetition of a standard list of phrases and sentences (Henningsson et al., 2008; Trost Cardamone et al., 2011) Oral-only (no nasal phonemes, usually loaded with high pressure phonemes) Buy baby a bib. Pet the puppy. Take it to Ted. Daddy did it. Go get a bigger egg. I like cookies. I have six sisters. Shelly wears shoes. Zebras live at the zoo. Chuck goes to church. Judges like to jump. Fifty five fish. Nasal-loaded Mama made lemonade. Ten men came in when Jane rang. Mixed (oral and nasal phonemes)

83 Listen during: Oral-only stimuli (test for VPD symptoms) Nasal-loaded stimuli (test for hyponasality) Vowels or sustained SSS, FFF If a patient has listen for increase in pressure, improved resonance, and elimination of nasal emission when nose is closed. If patient is : listen for lack of change in nasal consonants when nose is closed.

84 Nasal emission may or may not be audible Listen for presence, consistency, and quality (turbulence) of nasal emission Determine if phoneme-specific or not Determine if only related to a fistula or not Confirm what you hear look for fogging during oral consonants; Fogging immediately prior to and after sentence production is NORMAL Can also use See-Scape, listening tubes, straw

85 Various standardized articulation tests are acceptable for use with patients with cleft palate/vpd (e.g., GFTA-2) Compare to norms Older Cleft Palate Tests are not widely used anymore Iowa Pressure Articulation Test Bzoch Test Fisher Logemann

86 What CAN the speaker do? Stimulability testing with the nose plugged for oral consonants Consider dental-occlusal hazards too

87 Are any related to the articulation errors? Dentition: missing/malposed teeth Occlusion Orthodontic appliances present (e.g., expander, W-arch wire) Maxillary arch width

88 Lips: closure at rest/breathing and speech Open mouth posture? Difficulty with lip closure due to overjet? Don t overanalyze tension of upper lip after cleft lip repair Fistula in hard or soft palate Soft palate: Length: short? Can you see the adenoid pad? Elevation during /a/ phonation: hints Symmetrical? Degree of elevation? Uvula (for noncleft patients): single and at midline? bifid? Other signs of SMCP? Tonsils: size

89 Consider: Is the child s speech appropriate for their age? Is intelligibility and/or acceptability significantly impacted by the child s speech disorder? Is the speech profile suggestive of underlying VPD? Are there articulation distortions due to dental-occlusal hazards? Is the child stimulable for improved articulation? What other factors are contributing to the speech disorder (e.g., hearing loss, fistula, nasal congestion, learning difficulties, etc.)?

90 Is additional diagnostic information necessary? of VP closure for speech (e.g., imaging)? Acoustic assessment of nasality (e.g., Nasometry)? Other medical testing (e.g., audiometry, genetics)? Is a necessary BEFORE performing imaging studies or making recommendations for physical management of VP closure for speech?

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92 Does the instrumental assessment support the need for behavioral treatment (therapy) or physical management (surgery or a prosthesis)? What can the instrumental assessment tell us about the type of intervention that should be recommended?

93 Perceptual speech assessment Acoustic (e.g., Nasometry) Aerodynamic (e.g., Pressure-flow) Visualization of the VP mechanism Nasopharyngoscopy Multiview Videofluoroscopy

94 Acoustic correlate of nasality Nasalance = nasal sound energy / nasal + oral sound energy Higher nasalance correlates with perception of hypernasality Not a 1:1 relationship to listener perceptual judgments of resonance, but should confirm what you hear Good tool for comparison pre/post treatment Different products on the market can measure nasalance Nasometer II (KayPentax), Glottal Enterprises

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96 Expressed as a percentage (or ratio), ranges from 1-100% (you can t have 0% nasalance) Norms available for a variety of stimuli, languages, dialects

97 Various stimuli options Standard reading passages Zoo Passage Rainbow Passage Nasal Sentences SNAP test-r (Simplified Nasometric Assessment Protocol, McKay-Kummer) Patient repeats syllables and prolonged sounds or produces sentences using read or picturecued stimuli

98 Treatment applications Biofeedback therapy Different treatment targets Ideal candidate: age 8 years+, good attention span, cooperative, can read sentences/passages, stimulable to modify nasal emission or resonance, good articulation, motivated

99 The Nasometer does not measure hypernasality The Nasometer does not measure nasal airflow Multiple score confounds lead to risk for underestimation or overestimation of nasalance score: Articulation errors, nasal congestion, nasal turbulence, voice disorders, mixed resonance, dialect/accent Surgical decisions should not be based only on nasalance scores

100 Often called multiview videofluoroscopy Lateral view, frontal view, base view, Towne s view Exposure to radiation Usually involves administration of barium via the nasal cavity Assess VP closure for speech, palatal length and elevation, size of adenoids and tonsils Patient repeats words and phrases

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102 Provides direct view of VP (and laryngeal) structures and the VP port during speech Flexible fiberoptic or CHIP TIP technology combined with a nasopharyngoscope (adult or pediatric sizes) Topical anesthetic and nasal decongestant typically used Higher cooperation required Patient repeats standard words and phrases (primarily oral-only sample used to assess VP closure)

103 Determine presence and extent of VPD during speech Size of gap, shape Consistency of VP closure Movement of individual structures (velum, LPW, PPW) Size of adenoids, tonsils, and potential role in VPC Signs of occult SMCP

104 Limitations of Nasopharyngoscopy Subjective cannot obtain absolute measurements of gap size Differences in viewing perspectives, position of scope Possible tongue backing contribution to VPC Minimally invasive Discomfort Cooperation best if age 4 yrs+

105 Biofeedback therapy with Nasopharyngoscopy Option for treatment of compensatory errors (e.g., glottal stops, nasal fricatives) Provide visual cues for closure on specific sounds Compare and contrast open/closed port Requires highly cooperative patient, typically older schoolage, teen, or adult

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107 Readily intelligible speech Socially acceptable speech Age-appropriate articulation skills Age-appropriate language skills

108 Improve velopharyngeal closure as soon as it is clear that the potential for adequacy is not present Most surgery for VPD is between 3-6 years

109 1. Improve VPC (physical management) but no speech therapy needed 2. Improve VPC and speech therapy needed 3. Speech therapy only- defer decision on VP management until additional therapy completed 4. Speech therapy needed and VPC is adequate 5. No treatment needed (continue to monitor)

110 Start therapy as soon as possible- for surgery or the cleft team to tell you to start therapy Need to establish at least some correct articulation placement prior to VP imaging or surgical decision Utilize a hierarchical- traditional articulation approach that (minimum 2x/week) on an especially when compensatory articulation errors are present

111 Only one sound is targeted and stabilized at a time in a hierarchical progression of speech contexts Start with isolation, syllable level, words, multisyllabic words, phrases, sentences, then connected speech Make sure that the child can produce the sound in spontaneous/connected speech before moving to the next goal. Give the child sufficient practice to solidify the new sound aim for high accuracy (90%+)

112 Where do I start? Options: Start with the sounds causing the most negative impact on intelligibility or VPC ( the compensatory errors) Target sounds for which the child is stimulable Keep developmental progression in mind, but don t be afraid to deviate from it Start with the sounds that are most visible (anterior) Usually, it is easier to start with voiceless sounds before voiced ones (especially if glottal stops are present)

113 Articulation errors compensatory articulation errors placement errors (substitutions, distortions) omissions backing of phonemes and other phonological errors In carefully selected cases, inconsistent mild hypernasality or inconsistent audible nasal emission MAY be improved with behavioral therapy Usually with biofeedback refer to a cleft SLP expert for this treatment

114 Traditional Articulation Therapy is the preferred method of treatment In most cases, this is NOT phonological therapy, at least not initially NOT oral-motor therapy This is therapy focused on phonetic-based approaches, with perceptual training using auditory, tactile, and visual cueing, and self-monitoring techniques Also utilizes motor-learning principles Drill practice as soon as child is mature enough

115 Teach identity, location, and actions of oral structures Teeth, lips, tongue Use a picture, mirror, Mr. Potato, Mighty-Mouth Teach sounds and their corresponding structures /p/: lip sound, popping sound, poof sound /t/: tongue sound or teeth sound /s/: snake sound /ʃ/: quiet/windy sound

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119 Get the target sounds into the inventory Use easier sounds to elicit new sounds (shaping) /w/ or /m/ /p/, /b/ /l/ or /n/ /d/ /j/ /ʃ/ Provide auditory, visual, and tactile cues Use nasal occlusion as needed Teach correct oral target vs error sound contrasts auditory discrimination & negative practice: old sound vs new sound Establish reliable self-monitoring

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121 Teach them to discriminate and identify throat sounds vs mouth sounds (or nose vs mouth ) Start teaching most visible or anterior sounds (e.g., /w/, /h/, /p/, /t/) and shape into target sounds Try voiceless sounds first (e.g., /p/, /t/ before /b/, /d/) Plug nose as needed to provide sensation of oral pressure Try /h/ words with the final consonants as targets (e.g., hoop, hop) Use overaspiration of air and whispering as needed Avoid words with nasal sounds or vowel initial words

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123 horn-therapy program straw blowing/lip strengthening program palate massage/stimulation program yawning/sighing exercises whistle blowing exercises tongue movement exercises cookbooks to improve resonance

124 See-Scape Sometimes useful for treating learned nasal emission (nasal fricative substitutions) only if Phoneme Specific Auditory Biofeedback: listening tube, straw, oral-nasal listener Nasopharyngoscopy Nasometry? CPaP (Kuehn 1991, Kuehn et al., 2002)

125 Surgical Palatal lengthening and muscle repair (Furlow Z-plasty) Superiorly-based pharyngeal flap Sphincter pharyngoplasty Posterior wall augmentation / Fat injections Prosthetic Palatal lift Speech bulb

126 Indicated for short soft palate (or minimal elevation) when at least some degree of LPW movement is present

127 Pharyngeal Flap

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129 Indicated when soft palate length and elevation are adequate but there is minimal LPW movement

130 Furlow z-plasty Muscle reorientation/repair and lengthening Often used for SMCP repair Option for very small VP gaps, nonsyndromic cases Pharyngeal wall augmentation (rarely used) Fat injection: results may only be temporary

131 When most commonly considered: Multiple surgical failures Surgical risk Airway concerns Neuromuscular etiology of VPD Borderline cases Diagnostic treatment

132 Palatal Lift Speech Bulb Obturator KNC/AT/LG/KM '10

133 Palatal Lift Palatal Bulb KNC/AT/LG/KM '10

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135 5 year old female, adopted from China Repaired bilateral cleft lip and palate (lip-china, palate-age 2 years in US) Chronic OME, unilateral conductive hearing loss at last visit with right ear perforation In therapy at a local private practice, 1x per week, targets include /p/, /t/, and blowing exercises Unable to enroll in school-based therapy Held back from kindergarten this year due to concerns with intelligibility

136 Perceptual Speech Evaluation Standardized Articulation Testing (Language Testing) Oral Exam

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139 Articulation errors: Pharyngeal fricatives for S, F, SH, Z, CH, TH, J Glottal stops for T, P, K, SH, Z, G, and inconsistent glottal coarticulation Nasal substitutions for K, G, Z, F, TH, CH, B, V inconsistently W/R substitutions D/G substitutions inconsistently B/V substitutions H substitutions for S, Z inconsistently Consistent weak pressure consonants Standard Score: <40, Percentile Rank <1st

140 Intelligibility : Child's connected speech is usually intelligible to familiar listeners but only occasionally intelligible to unfamiliar listeners Hypernasality: severe Hyponasality: none Audible Nasal Emission: present Voice Quality: rough, breathy Articulation: compensatory errors present

141 Dentition: Anterior crossbite (underbite) Fistula: Anterior hard palate fistula Palate: Soft palate is judged to be short in length Passavant's ridge activity is visible intraorally

142 Is there a role for continued or increased speech therapy? What suggestions would you make to her current therapy programming? Would instrumental assessment of nasality be helpful (i.e., Nasometry)? Is VP imaging indicated now? If yes, what stimuli would you use? What is your tentative hypothesis for the etiology of her speech disorder?

143 Increase frequency/intensity of current speechlanguage services to at least 2-3x per week with primary focus on articulation placement skills Therapy should target remediation of glottal stops, nasal substitutions, and pharyngeal fricatives first, before moving to other goals. Provide extensive placement cues for target sounds. Discontinue use of blowing exercises.

144 Carryover of accuracy at the word, phrase, sentence, and spontaneous speech levels at 90% accuracy for each target, prior to adding new goals to ensure mastery of skills. Nasal occlusion may be helpful (nose plugging) if tolerated intermittently during therapy. Avoid excessive loudness/effort during therapy sessions to minimize vocal hyperfunction.

145 Nasometry was not completed due to frequency and type of articulation errors and dysphonia, as well as some cooperation challenges

146 Cooperation was poor Level of speech sample: Single syllables and words only (sample focused on accurate phonemes only) Consistent velopharyngeal gap present during an oral-only speech sample. VP Gap Size: 4-Large Location of VP gap: Central VP Closure Pattern: Coronal Degree of Palatal Elevation: Minimal Medial Movement of the Lateral Pharyngeal Walls: Minimal

147 Would you proceed with recommending physical management? If yes, surgical or prosthetic?

148 Pharyngeal flap Increased speech therapy Future fistula closure/obturation Management of hearing loss Reassess speech at Team visit 6 months post-surgery

149 12 year old male 22q11.2 deletion syndrome (velocardiofacial syndrome) Congenital velopharyngeal dysfunction S/P pharyngeal flap, flap revision, previous tonsillectomy and adenoidectomy Seasonal allergies, learning disabilities, social skills deficits Home schooled, receiving teletherapy for articulation and language Parent voices concerns with resonance and intelligibility

150 Perceptual Speech Evaluation Standardized Articulation Testing (Language Testing) Oral Exam Nasopharyngoscopy and Nasometry

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152 Intelligibility: intelligible to familiar listeners consistently, occasionally not understood by unfamiliar listeners Hypernasality: moderate Hyponasality: none Audible Nasal Emission: Consistently inaudible, inconsistently audible Voice Quality: rough Articulation: compensatory errors present, weak pressure for consonants? Dysarthric features

153 Standard score: 40, Percentile Rank: <1 st Errors: glottal stop substitutions for K and G, nasal fricatives for F and V, N for NG, N for L inconsistently, nasal fricative inconsistently for "J", nasal fricatives for TH, H/K substitutions only in blends. W/R substitutions distortions of CH S/SH substitutions Articulation errors were noted to increase with an increase in utterance length and complexity. This includes a significant increase in frequency of glottal stop errors in conversation as compared to structured tasks.

154 Facial and lip asymmetry at rest and in motion The left side of his face/lips has reduced movement Facial/lip motion for smiling is reduced. Significant overjet which interferes with lip closure Soft palate demonstrates asymmetrical elevation toward the right side Suggests left palatal weakness. Sensitive gag reflex observed Unlikely to tolerate palatal prosthesis

155 Zoo passage: 46% (mean 15%, +/- 5%) SNAP stimuli (picture-cued subtest): Bilabials: 20% (mean 11%, S.D. +/- 5%) Alveolars 27% (mean 11%, S.D., +/- 5%)

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157 Very cooperative for the procedure. Slightly narrow and asymmetrical pharyngeal flap is present. The left port is larger than the right at rest. The right port demonstrates virtually complete (.90) closure during most speech tasks, with some mucous/bubbling. The left port demonstrates an inconsistent degree of closure, based on the speech stimuli and accuracy.

158 For accurate oral phonemes, closure is estimated at , but during inaccurate speech, closure is approximately.20 on the left Demonstrated the ability to modify and increase the closure of the left port during speech with endoscopic biofeedback L lateral pharyngeal wall demonstrates reduced degree of closure and slower motion than R side Consistent with suspected left pharyngeal weakness Atypical posterior pharyngeal wall motion emanating near midline with extension toward the left side of the pharynx is noted during speech Likely a compensatory behavior to assist with VP closure.

159 COMBINATION of traditional and biofeedback 2x per week TREATMENT GOALS: Increased VP closure for speech (left VP port) Accurate articulation placement Additional formal language testing. Candidate for flap revision in future Depends on results of therapy Re-eval with Cleft/Cranio team in 6 months Orthodontic treatment to correct his overjet to facilitate lip closure.

160 TRADITIONAL: Placement for velars Placement / oral airflow for /ʃ/, /ʧ/, /ɵ/, /f/, /j/ NASOPHARYNGOSCOPY: 100% closure in 80% of words and phrases targeted. NASOMETRY: Nasalance scores within normal limits at phrase level for SNAP stimuli 80% accuracy (below target reference line) for specific target words and phrases Consistent home practice

161 Achieved stimulability for velars with slight distortion, with higher accuracy for /k/ than /g/ CURRENT STATUS: /k/ in all positions of words (with slight distortion) with a model and intermittent auditory cues in 90% of opportunities /g/ in isolation with 75% accuracy (inconsistent uvularized placement or glottals) /ʃ/, /ʧ/, /f/ and /ɵ/ in words and phrases reviewed and 75% accuracy noted with frequent placement and auditory cues needed

162 Can produce all vowels except /i/ at or below a 40% nasalance reference line with and without a model; CV syllables for /p/, /t/, /k/, /s/, /ʃ/ with a model for all vowels except /i/ with 90% accuracy Can produce these CV syllables in oral-nasal contrasts with 90% accuracy Can produce CVC words for this set of sounds below the 40% reference line for all vowels except /i/ with 75% accuracy

163 Articulation placement for /k/ at the word and short phrase level. Achieved 90% accuracy in words and 80% in phrases (in all word positions) with a model, with slight distortion persisting inconsistently. /g/ is produced with uvular placement or as a /k/ substitution Fricatives and affricates: 90% accurate for oral airflow (with S/SH placement issues) at the phrase level Decreased accuracy noted in spontaneous speech--but good self correction with minimal cues

164 No change in resonance in spontaneous speech. Ccontinued difficulties with /i/ and /u/ Anticipatory nasal emission noted for /t/ and /d/ but generally oral and accurate /p/, /b/, /k/ and fricatives accurate at the syllable and selected word level Goals sustained at 30% reference line in 80% of cases at the syllable level only for constrained stimuli (without /i/, /g/, /u/ and diphthongs)

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167 5:10 year old male Adopted from China Lip repair: China (age 7 months) Palate Repair: Age 15 months Fistula Repair: Age 2 years Secondary Surgery: Palatal lengthening at 4:9 Highly compliant for nasopharyngoscopy

168 Received speech therapy through EI and privately (outpatient) Had attended speech therapy at PSU, 2x weekly, in Spring 2011 term Enrolled in 2011 Summer Clinic program at PSU CONCERN: speech intelligibility

169 Receptive / Expressive Language: Not formally assessed, but believed to be well aboveaverage Reads at 2 nd grade level (at age 5) Speech: Distorted /s/ and /ʃ/ Gliding errors on /r/ and /l/ Final consonant deletion of plosives and /s/ Speech intelligibility improved with slower rate and focus on precision Nasal air emission on labial and alveolar sounds Resonance: WNL

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173 Large anterior oral-nasal fistula Advanced cognitive skills Maintenance of speech skills Financial / Insurance constraints Adequate velopharyngeal closure

174 Accurate placement of /s/, /ʃ/, /l/ ISSUES: Loss of airflow, especially at the end of a sentence is, was, his Equipment issues! PROGRESS: Accurate production of /l/ as singleton (not yet in blends) Implementation of self-monitoring skills for rate Slower rate / over articulation Visual and verbal cues Reading aloud

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178 Work within constraints Promote effective / efficient communication Preserve self-image / self-esteem Communicate with parents regarding expectations

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