Health-related quality of life in patients with cleft palate: Reproducibility, responsiveness and construct validity of the Dutch version of the VELO questionnaire.

OBJECTIVES: Recently, the Velopharyngeal Insufficiency (VPI) Effects on Life Outcomes (VELO) questionnaire, which evaluates the impact of speech and swallowing difficulties on health-related quality of life (HRQoL) in patients with VPI (Skirko et al., 2012), was translated to Dutch (Bruneel et al., 2017). The purpose of this study was to evaluate the reproducibility, responsiveness and construct validity of this Dutch version of the questionnaire. METHODS: To evaluate the reproducibility, 50 parents and 14 children with cleft palate re-completed the questionnaire after two weeks. Thirty-five parents and 8 children with cleft palate completed the VELO questionnaire after one year for the evaluation of the responsiveness. The correlation between age and the VELO questionnaire (construct validity), and the internal consistency (Cronbach's α) were re-determined based on the responses of 73 parents and 24 children. RESULTS: Based on descriptive statistics, results of the Wilcoxon signed rank-test, and the absolute (SEM) and relative (ICC) consistency, the questionnaire showed good reproducibility. VELO scores did not significantly differ after one year, neither when performing separate analyses for the intervention (speech therapy) and the non-intervention group. Correlations indicated higher HRQoL, as perceived by the parents, with increasing age. The reverse was the case for the youth report. Cronbach's α showed excellent internal consistency for both reports. CONCLUSIONS: The VELO questionnaire showed good reproducibility and internal consistency. Moreover, results reconfirmed the age effect on VELO scores. To understand the implications of the results regarding the questionnaire's responsiveness, future research should focus on the identification of factors influencing the patient's evolution in HRQoL.

Validation of the Chinese Velopharyngeal Insufficiency Effects on Life Outcomes Instrument.

OBJECTIVES/HYPOTHESIS: To translate the Velopharyngeal Insufficiency Effects on Life Outcomes (VELO) instrument into Chinese and test its psychometric properties. STUDY DESIGN: Quality of life instrument translation and validation. METHODS: The original English version of the VELO instrument was translated into Mandarin, back-translated, and adapted among the Chinese population, based on the standardized guidelines for the cross-culture adaption process. Velopharyngeal insufficiency (VPI) patients were identified by a professional speech and language pathologist. Internal reliability of the VELO instrument was assessed by the Cronbach's α coefficient. Discriminant validity was tested by the Mann-Whitney U test. Construct validity was assessed by factor analysis. RESULTS: A total of 113 patients with VPI and 72 parents of the patients were enrolled. The mean age of the VPI patients was 14.8 years. Internal reliability was excellent; Cronbach's α coefficients were 0.92 and 0.94 for VPI patients and their parents, respectively. The Chinese VELO discriminated well between the VPI group and the controls, with a mean (standard deviation) score that was significantly lower for the VPI group (74.8 [25.7]) than the control group (98.0 [15.9]) (P < .001). The total scores and scores in the emotional domain or perception domain showed differences between VPI patients and their parents. Similar to the original study, the factor loading after rotation followed hypothesized domains largely, in spite of items from several domains loaded on the same factor. CONCLUSIONS: The translated Chinese version of the VELO instrument demonstrated an acceptable reliability, discriminant validity, and construct validity. These psychometric properties suggested theoretical evidence for the further use of the VELO instrument among Chinese patients with VPI. LEVEL OF EVIDENCE: 3b Laryngoscope, 129:E395-E401, 2019.

Speech and language characteristics in individuals with nonsyndromic submucous cleft palate-A systematic review.

BACKGROUND: Up to 80% of individuals with unrepaired submucous cleft palate (SMCP) experience speech difficulties secondary to velopharyngeal insufficiency. Language delays are reported in the broader cleft lip and/or palate population, suggesting that individuals with SMCP may also be at risk. However, contemporary understanding of this population remains limited as there has been no systematic examination of the literature. This review aims to systematically review and document the speech and language characteristics of individuals with nonsyndromic SMCP and, in addition, to identify factors reported to impact speech and language outcomes. METHOD: This review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Five databases were comprehensively searched using keywords and indexed headings. Included studies had to report speech or language outcomes of individuals with nonsyndromic SMCP. Risk of bias and methodological design quality were examined using tools from the Scottish Intercollegiate Guidelines Network. Relevant data were extracted for analysis. RESULTS: Eighteen studies met inclusion criteria, yielding 598 participants. Study results showed that individuals with unrepaired nonsyndromic SMCP may have speech difficulties secondary to velopharyngeal insufficiency including increased nasal resonance and palatalized or glottal articulation. Lower age at primary surgical repair led to better postsurgical speech outcomes. There is a paucity of literature outlining motor or phonological aspects of speech and receptive or expressive language abilities of this population. CONCLUSION: Individuals with nonsyndromic SMCP present with speech difficulties similar to those experienced by individuals with overt cleft palate. Health care professionals should be aware of possible presenting symptoms and consider early SMCP diagnoses where appropriate. Further research is needed to specify the broader communication profile in this population.

Association of Velopharyngeal Insufficiency With Quality of Life and Patient-Reported Outcomes After Speech Surgery.

IMPORTANCE: Patients with cleft palate and other causes of velopharyngeal insufficiency (VPI) suffer adverse effects on social interactions and communication. Measurement of these patient-reported outcomes is needed to help guide surgical and nonsurgical care. OBJECTIVES: To further validate the VPI Effects on Life Outcomes (VELO) instrument, measure the change in quality of life (QOL) after speech surgery, and test the association of change in speech with change in QOL. DESIGN, SETTING, AND PARTICIPANTS: Prospective descriptive cohort including children and young adults undergoing speech surgery for VPI in a tertiary academic center. Participants completed the validated VELO instrument before and after surgical treatment. MAIN OUTCOMES AND MEASURES: The main outcome measures were preoperative and postoperative VELO scores and the perceptual speech assessment of speech intelligibility. The VELO scores are divided into subscale domains. Changes in VELO after surgery were analyzed using linear regression models. VELO scores were analyzed as a function of speech intelligibility adjusting for age and cleft type. The correlation between speech intelligibility rating and VELO scores was estimated using the polyserial correlation. RESULTS: Twenty-nine patients (13 males and 16 females) were included. Mean (SD) age was 7.9 (4.1) years (range, 4-20 years). Pharyngeal flap was used in 14 (48%) cases, Furlow palatoplasty in 12 (41%), and sphincter pharyngoplasty in 1 (3%). The mean (SD) preoperative speech intelligibility rating was 1.71 (1.08), which decreased postoperatively to 0.79 (0.93) in 24 patients who completed protocol (P < .01). The VELO scores improved after surgery (P<.001) as did most subscale scores. Caregiver impact did not change after surgery (P = .36). Speech Intelligibility was correlated with preoperative and postoperative total VELO score (P < .01) and to preoperative subscale domains (situational difficulty [VELO-SiD, P = .005] and perception by others [VELO-PO, P = .05]) and postoperative subscale domains (VELO-SiD [P = .03], VELO-PO [P = .003]). Neither the VELO total nor subscale score change after surgery was correlated with change in speech intelligibility. CONCLUSIONS AND RELEVANCE: Speech surgery improves VPI-specific quality of life. We confirmed validation in a population of untreated patients with VPI and included pharyngeal flap surgery, which had not previously been included in validation studies. The VELO instrument provides patient-specific outcomes, which allows a broader understanding of the social, emotional, and physical effects of VPI. LEVEL OF EVIDENCE: 2.

Health-related quality of life in patients with cleft palate: Validity and reliability of the VPI Effects on Life Outcomes (VELO) questionnaire translated to Dutch.

BACKGROUND: Disease-specific health-related quality of life (HRQOL) questionnaires provide the clinician with important information regarding the impact of the disease on functioning and well-being. For patients with velopharyngeal insufficiency (VPI), the VPI Effects on Life Outcomes (VELO) questionnaire was developed and validated in English by Skirko et al. (2012). However, a valid and reliable Dutch translation of this questionnaire is not available yet. METHODS: The English questionnaire was translated to Dutch following a forward-backward translation procedure. A linguistic validation and the evaluation of the internal consistency (Cronbach's α) of this Dutch version were performed based on the responses of 39 parents of patients with cleft (lip and) palate (mean age: 6.8 years) (parent report) and the responses of 14 patients older than 8 years (mean age: 9.5 years) (child report). Additionally, the concurrent validity was assessed by comparing the scores on the parent report to those on the pediatric voice handicap index. Furthermore, the validity of the parent proxy assessment and the relationship between age and responses on the VELO questionnaire were investigated. Based on the responses of an age and gender matched control group without cleft palate, the discriminant validity was evaluated. RESULTS: The parent report was easy to complete for all parents. Nine of the fourteen (64%) patients were able to complete the child report independently. The median scores on the parent report and the child report were 82.7 and 95.1 respectively. The patient group had a significantly worse perception of HRQOL compared to the control group (p < 0.001; p = 0.029). There were no significant differences between the responses of the parent and their child's (p = 0.345). A significant positive correlation was found between the score on the parent report and the age of the patients (p = 0.001). Furthermore, a significant negative correlation was found between the parent report and the P-VHI (p < 0.001). Cronbach's α was 0.955 and 0.817 for the parent report and the child report respectively. CONCLUSION: The Dutch VELO questionnaire is a valid, reliable and user-friendly tool that provides important information about HRQOL in patients with cleft (lip and) palate.

Change in Quality of Life with Velopharyngeal Insufficiency Surgery.

OBJECTIVES: (1) To define the minimal clinically important difference (MCID) of the Velopharyngeal Insufficiency (VPI) Effects on Life Outcomes (VELO) instrument, and (2) to test for the change in quality of life (QOL) after VPI surgery. STUDY DESIGN: Prospective observational cohort. SETTING: VPI clinic at a tertiary pediatric medical center. SUBJECTS AND METHODS: Children with VPI and their parents completed the VELO instrument (higher score is better QOL) at enrollment and then underwent VPI surgery (Furlow palatoplasty or sphincter pharyngoplasty, n = 32), other treatments (obturator or oronasal fistula repair, n = 7), or no treatment (n = 18). They completed the VELO instrument again and an instrument of global rating of change in QOL at 1 year. The MCID was anchored to the global change instrument scores corresponding to "a little" or "somewhat" better. Within-group (paired t test) and between-group (Student t test) changes in VELO scores were tested for the VPI surgery and no treatment groups. The association between treatment group and change in VELO scores was tested with multivariate linear regression, adjusting for confounders. RESULTS: Follow-up was obtained for 37 of 57 (65%) patients. The mean (±standard deviation) change in VELO scores corresponding to the MCID anchor was 15 ± 13. The VELO score improved significantly more in the VPI surgery group (change, 22 ± 15; P < .001) than in the no treatment group (change, 9 ± 12; P = .04), after adjusting for confounders (P = .007 between groups). CONCLUSION: VPI surgery using the Furlow palatoplasty or sphincter pharyngoplasty improves VPI-specific QOL, and the improvement is clinically important.

A comprehensive description of functioning and disability in children with velopharyngeal insufficiency.

UNLABELLED: Children with velopharyngeal insufficiency (VPI) experience functional impairments in a variety of areas that extend beyond the primary physical impairment associated with this disorder. At present, the physical deficits associated with VPI have been studied extensively; however, a comprehensive description of social and communicative participation in this population is needed. Therefore, a biopsychosocial framework such as the International Classification of Functioning, Disability and Health for Children and Youth (ICF-CY), may offer an enhanced understanding of the daily experiences of children with VPI. Specifically, the ICF-CY framework is intended to model complex nonlinear systems, and as such, to describe functioning as the interaction of multiple components from which a limitation in communicative participation may emerge. This paper describes how the ICF-CY framework can be utilized to comprehensively describe functioning and disability in children with VPI by describing the interaction of components of this framework. LEARNING OUTCOMES: As a result of this activity, the reader will be able to: (1) discuss the utility of the ICF-CY in describing the multi-dimensional nature of velopharyngeal insufficiency (VPI); (2) describe interrelationships between functioning and disability in children with VPI; and (3) identify how limitations in communicative participation may emerge from the interaction of components of the ICF-CY in children with VPI.

Validity and responsiveness of VELO: a velopharyngeal insufficiency quality of life measure.

OBJECTIVE: Test the Velopharyngeal Insufficiency (VPI) Effects on Life Outcomes (VELO) instrument for validity, reliability, and responsiveness. STUDY DESIGN: Observational cohort. SETTING: Academic tertiary medical center. SUBJECTS: Children with VPI (n = 59) and their parents (n = 84) were prospectively enrolled from a pediatric VPI clinic. METHODS: Pediatric speech language pathologists diagnosed VPI using perceptual speech analysis and rated VPI severity and speech intelligibility deficit (each as minimal, mild, moderate, or severe). All parents and youth 8+ years old (n = 24) completed the VELO instrument and other quality-of-life questionnaires at baseline; the first 40 subjects completed the VELO instrument again 2 weeks later. Treatments included Furlow palatoplasty (n = 20), sphincter pharyngoplasty (n = 14), or an obturator (n = 2), and 29 of 36 (81%) subjects completed the questionnaires 3 months posttreatment. VELO was tested with correlations for criterion validity against VPI severity, construct validity against speech intelligibility and velopharyngeal gap size, and concurrent validity against other quality-of-life measures (r > .40 demonstrating validity); for test-retest reliability using intraclass correlation (>.6 demonstrating reliability); and for responsiveness with the 3-month posttreatment measure using the paired t test. RESULTS: Parental responses are reported; youth responses showed similar results. The VELO instrument did not meet criterion validity (r = -.18, P = .10), or functional construct validity (r = -.37, P = .001), but did meet anatomic construct and concurrent validity (each r > .50, P < .01). VELO scores demonstrated excellent test-retest reliability (r = .85, P < .001) and responsiveness (baseline 54 ± 14 to posttreatment 70 ± 18, P < .001). CONCLUSION: VELO provides a VPI-specific quality-of-life instrument that demonstrates concurrent validity, test-retest reliability, and responsiveness to change in quality of life with treatment.

An evaluation of communication apprehension in adolescents with velopharyngeal inadequacy.

OBJECTIVE: This investigation evaluated the construct of communication apprehension (CA) in adolescents with velopharyngeal inadequacy (VPI). DESIGN: Prospective comparative study. SETTINGS: The investigation was conducted in a hospital-based clinic, voice laboratory, and the homes of participants. PARTICIPANTS: Two groups of children participated in this study. The first group was composed of 14 children between the ages of 8 and 14 years who attended the velopharyngeal inadequacy (VPI) clinic at our institution, and the second group was composed of 14 age-matched children from the community with no history of speech or voice disorders. INTERVENTIONS: The study involved completion of the Measure of Elementary Communication Apprehension (MECA) by all 28 participants (14 experimental and 14 control subjects). Reliability of the subjects' responses to the MECA was also evaluated. OUTCOME MEASURES: Communication apprehension scores obtained from the MECA were the main focus of the study. RESULTS: Children with VPI experienced significantly higher levels of CA than did those in the control group. Moderate-to-good reliability of the MECA was found. CONCLUSIONS: Based on these data, children with VPI may be experiencing increased levels of CA. Consequently, the experience of high CA has the potential to affect the communication performance and social functioning of children with VPI.

Effectiveness of nasopharyngoscopic biofeedback in clients with cleft palate speech: a systematic review.

OBJECTIVE: To conduct a systematic review analyzing the effectiveness of nasopharyngoscopic biofeedback in clients with cleft lip and palate and velopharyngeal dysfunction. METHOD: Extensive electronic search and analysis of the databases of Cochrane Library, MEDLINE, EMBASE, ERIC, PsycInfo, CINAHL, AMED, Journals@Ovid, and German Databases, including all papers published since 1970 plus a manual search of the Cleft Palate-Craniofacial Journal (1970-3/2010). RESULTS: Six studies met the inclusion criteria. Their analysis reflects a low level of evidence and a broad heterogeneity concerning age range, intervention methods, and outcome measurement. CONCLUSION: The analyzed studies show that nasopharyngoscopy may be effective only in combination with traditional speech therapy in helping patients with cleft palate speech optimize their velopharyngeal closure in articulation, but the quantity and quality of studies are limited.

Impact of delayed prosthetic treatment of velopharyngeal insufficiency on quality of life.

Palatopharyngeal dysfunction may take place when palatopharyngeal valve is unable to perform its own closing due to a lack of tissue (palatopharyngeal insufficiency) or lack of proper movement (palatopharyngeal incompetence). Palatopharyngeal insufficiency induces nasal regurgitation of liquids, hypernasal speech, nasal escape, disarticulations and impaired speech intelligibility. Prosthetic management of palatopharyngeal insufficiency requires a close co-operation between an otolaryngologist and a speech pathologist. As a result, the patient can be socially and physically rehabilitated with the improved speech quality as well as prevention of leakage of liquids.

Quality of life in children with velopharyngeal insufficiency.

OBJECTIVE: To determine if quality of life (QOL) is affected in children with velopharyngeal insufficiency (VPI). DESIGN: Interview and survey. SETTING: Two university pediatric ambulatory centers. PARTICIPANTS: This population-based sample included 58 children, aged 5 to 17 years, and their parents; 29 of the children were diagnosed as having VPI and 29 were age-matched normal controls. INTERVENTION: Each participant (child and parent) completed 2 questionnaires: the Velopharyngeal Insufficiency Quality of Life (VPIQL), an instrument specifically designed for children with VPI, and the Pediatric Quality of Life Inventory, version 4.0 (PedsQL(4.0)), a standardized generic assessment instrument that systematically assesses the perception of health-related QOL in pediatric patients with chronic health conditions. MAIN OUTCOME MEASURES: The VPIQL questionnaire assessed patients' and parents' perceptions of speech, swallowing, situational and emotional difficulty, activity limitations, and perception of the patient by others. Caregiver impact was also assessed in the parent version of the VPIQL. RESULTS: The patients with VPI and their parents perceived a more statistically significant negative QOL compared with the normal controls and their parents in all domains of the VPIQL and the PedsQL(4.0). The parents of the children with VPI perceived a more negative emotional impact (P = .02), greater speech limitations (P = .05), and fewer swallowing problems (P<.001) compared with their children. CONCLUSIONS: Children with VPI and their parents perceive negative QOL greater than that of normal controls and their parents. Parental proxy may be an adequate substitute in this population.

Parenting, family contexts, and personality characteristics in youngsters with VCFS.

Parenting, family contexts, and personality characteristics in youngsters with VCFS: The personality profiles for 48 youths with Velo-Cardio-Facial syndrome (VCFS) were described using the California Child Q-Set (CCQ). Associations between personality characteristics and parenting (i.e., Control and Warmth vs. Anger) and family contexts (i.e., Experienced Family Stress, Marital Conflict and Parental Consistency) were investigated. Personality characteristics were found to be related to parenting (in particular, Parental Warmth vs. Anger) but not to family contexts. Parents who reported more Parental Warmth (and less Anger) in interactions had children with higher Agreeableness, Conscientiousness and Emotional Stability and with lower Irritability and Dependency. Parental Control was positively related to children's Dependency and negatively to children's Conscientiousness. Compared to fathers, mothers exerted more Control. Differences in parenting and family contexts were related to the mode of inheritance but not to IQ, age, gender, and cardiac defects. Families in which a familial deletion occurred reported higher levels of Marital Conflict and lower Warmth in the parent-child interactions.

Obsessive-compulsive disorder in patients with velocardiofacial (22q11 deletion) syndrome.

The study of neurogenetic microdeletion syndromes provides an insight into the developmental psychopathology of psychiatric disorders. The aim of the study was to evaluate the prevalence of psychiatric disorders, especially obsessive-compulsive disorder (OCD), in patients with velocardiofacial syndrome (VCFS), a 22q11 microdeletion syndrome. Forty-three subjects with VCFS of mean age 18.3 +/- 10.6 years were comprehensively assessed using semi-structured psychiatric interview and the Yale-Brown obsessive compulsive scale (Y-BOCS). Best estimate diagnoses were made on the basis of information gathered from subjects, parents, teachers, and social workers. Fourteen VCFS subjects (32.6%) met the DSM-IV criteria for OCD. OCD had an early age of onset and generally responded to fluoxetine treatment. It was not related to mental retardation. The most common obsessive-compulsive symptoms were contamination, aggression, somatic worries, hoarding, repetitive questions, and cleaning. Sixteen of the 43 patients (37.2%) had attention-deficit/hyperactivity disorder (ADHD), and 7 (16.2%) had psychotic disorder. The results of our study suggest that there is a strong association between VCFS and early-onset OCD. This finding may be significant in the understanding of the underlying genetic basis of OCD.

Pre-academic and early academic achievement in children with velocardiofacial syndrome (del22q11.2) of borderline or normal intelligence.

School-aged children with del22q11.2 tend to show a typical learning and neuropsychological profile, which is characterised by a VIQ-PIQ discrepancy (in favour of the VIQ) and significantly better scores for reading (decoding) and spelling compared to mathematics. To the best of our knowledge, there exists no systematic research on the pre-academic and early academic skills that might underpin these learning difficulties. The purpose of the current study was to investigate more systematically these pre-academic and early academic skills in borderline to normal intelligent (FSIQ > 70) children with del22q11.2 in the last year of kindergarten and first grade of primary school in Flanders. In the kindergarten group, meta-linguistic awareness and counting skills were examined. In the group of first graders, children were tested on reading, spelling and mathematics. Thirteen children (mean age: 6 years 4 months (SD = 0.84); 9 boys, 4 girls) participated in this study. In the present study, there were no differences in intelligence and academic outcomes between boys and girls, and no differences in IQ and academic achievement between children with cardiac defects or severe velopharyngeal insufficiency (VPI) and children without these deficits. With regard to pre-academic achievement in general, a characteristic profile with clearly better results for meta-linguistic awareness in comparison to counting skills was found, but this difference is not statistically significant. Concerning early academic achievement, children with del22q11.2, as a group, perform (despite their somewhat lower general intelligence) on average compared with their age-related peers. However, at an individual level--especially within the domain of counting skills and mathematics--there is a wide variability, with some children showing remarkable learning difficulties already at an early age.

Neuropsychological, learning and psychosocial profile of primary school aged children with the velo-cardio-facial syndrome (22q11 deletion): evidence for a nonverbal learning disability?

In this exploratory study, the neuropsychological and learning profile of nine primary school age children with velo-cardio-facial syndrome (VCFS) was studied by systematic neuropsychological testing. In five out of nine children, the following profile was found: a VIQ-PIQ discrepancy (in favor of the VIQ), significantly better scores (.05 level) for reading (decoding) and spelling compared to arithmetic, deficient tactile-perceptual skills (difficulties mainly on the left side of the body), weak but not deficient visual-perceptual abilities, deficient visual-spatial skills, extremely poor psychomotor skills (gross motor skills more deficient than fine motor skills), problems with processing of new and complex material, poor visual attention, good auditory memory and relatively good language skills. These findings correspond to the pattern of neuropsychological assets and deficits that has been described for the syndrome of nonverbal learning disabilities (NLD) (Rourke, 1987, 1988, 1989, 1995). The psychosocial profile of all nine children with VCFS also correspond to that of children with NLD. Further studies on the relationship between cognitive function, behavior, psychiatric disorder and abnormalities in brain anatomy in young people with VCFS will be needed. In clinical practice, it is worthwhile exploring in greater depth the neuropsychological functions of children with VCFS to rule out NLD, since they may benefit from specific remediation following the learning principles of the NLD-treatment.

Restoration of intelligible speech 13 years post-head injury.

This case study demonstrates the efficacy of treatment of a patient with severe dysarthria long after the accepted period of 'neurological recovery'. A physiological approach to treatment was utilized and resulted in a change from non-verbal communication to functional verbal communication.

Evaluation of velopharyngeal insufficiency.

To summarize briefly, in our opinion, the combined use of end-viewing flexible fiber optic nasopharyngoscopy and multiview videofluoroscopy (which must include lateral and frontal views) and a behavioral assessment of the patient's speech provides the examiner with all of the information necessary to select the appropriate treatment for VPI. Indirect assessment procedures, such as airflow studies, do not enhance the decision making process even though they may provide what would appear to be objective data. Treatment decisions depend on the ability to confirm the type of speech abnormality present (by behavioral assessment) followed by a direct view of the defect (by endoscopy and fluoroscopy).