Surgical Management of Submucous Cleft Palate by Radical Muscle Dissection Veloplasty: Speech Outcomes in Patients with 22q11.2 Deletion Syndrome.

OBJECTIVE: To evaluate the benefit of anatomical muscle dissection repair for velopharyngeal insufficiency (VPI) in patients with submucous cleft palate (SMCP) with 22q11.2 deletion syndrome. DESIGN: Retrospective blinded randomised analysis of a surgeon's management over 10 years. SETTING: The study was performed at a specialised Paediatric hospital in the United Kingdom. PATIENTS: Children with SMCP and 22q11.2 deletion syndrome. INTERVENTIONS: All participants underwent radical muscle dissection veloplasty. OUTCOMES MEASURED: Pre- and post- operative measurements included severity of anatomical defect, speech samples and lateral images which were digitised, randomised then externally and blindly analysed using validated techniques. Stata software was used to perform statistical analysis. RESULTS: 57 children with 22q11.2 deletion syndrome were included in this analysis. Intra-operatively, the majority of cases were identified as SMCP Grade I anomalies. Post-operatively, a statistically significant improvement in hypernasality, resting palate length, palate length at maximum closure, palate excursion and gap size at maximum closure was observed. Secondary surgery was performed for 59% of patients by ten years. CONCLUSION: Muscle dissection repair improves hypernasality, palate closure function and the closure gap in patients with 22q11.2 deletion syndrome. Although over 50% of patients may require further surgery, muscle dissection repair should be a first step due to its utility at a younger age, when invasive investigations are impossible, its lower morbidity, speech and language benefits or altering the plans for less obstructive secondary surgery when it lead to reduced velo-pharyngeal gap and improved palate mobility even when adequate velo-pharyngeal closure was not achieved.

Surgical sequence, timing and volume, and variation in dento-facial outcome, speech and secondary surgery in children with unilateral cleft lip and palate: The Cleft Care UK Study.

OBJECTIVES: To estimate both the association of surgical variables in complete unilateral cleft lip and palate (cUCLP) in the UK with outcomes at age 5 years, and the association of secondary speech surgery, volume of surgery, and surgeon with the same outcomes. SETTING AND SAMPLE POPULATION: The Cleft Care UK study, a cross-sectional study of 268 5-year-olds, born from 2005 to 2007, with cUCLP. MATERIALS AND METHODS: Information on surgical variables was extracted from a standardized questionnaire. Dento-facial outcomes were derived from dental study casts of dental arch relationships. Three speech outcomes - intelligibility, structure and articulation - were derived using the Cleft Audit Protocol for Speech-Augmented tool. RESULTS: Surgical and outcome data were available for 211 (79%) children from all cleft centres in the UK. Later soft palate surgery was associated with a 17% increased chance of a poor intelligibility score (P = .02), and high volume surgery with a 249% increased chance of a good articulation score (P = .01). There were no between surgeon effects identified. No association between the surgical variables examined and dento-facial outcome, or secondary speech surgery by the age of 5 years were found. CONCLUSION: This study found associations between surgical variables and speech outcomes at 5 years of age, but not between surgical variables and dento-facial outcome, nor between surgical variables and secondary speech surgery. High surgical volume should be maintained, and any changes towards later surgery monitored for changes in speech outcome.

How differences in anatomy and physiology and other aetiology affect the way we label and describe speech in individuals with cleft lip and palate.

BACKGROUND: Speech in individuals with cleft lip and/or palate (CLP) is a complex myriad of presenting symptoms. It is uniquely associated with the structural difference of velopharyngeal insufficiency (VPI), together with a wide and heterogeneous range of other aetiologies which often co-occur. The nature of the speech sound disorder (SSD) including VPI may also change over the course of an individual's care pathway. Differences in terminology and approaches to analysis are currently used, resulting in confusion internationally. Additionally, current diagnostic labels and classification systems in SSD do not capture the complexity and full nature of speech characteristics in CLP. AIMS: This paper aims to explore the different aetiologies of cleft palate/VPI speech and to relate aetiology with speech characteristic(s). In so doing, it attempts to unravel the different terminology used in the field, describing commonalities and differences, and identifying overlaps with the speech summary patterns used in the United Kingdom and elsewhere. The paper also aims to explore the applicability of current diagnostic labels and classification systems in the non-cleft SSD literature and illustrate certain implications for speech intervention in CLP. METHODS AND PROCEDURES: The different aetiologies were identified from the literature and mapped onto cleft palate/VPI speech characteristics. Different terminology and approaches to analysis are defined and overlaps described. The applicability of current classification systems in SSD is discussed including additional diagnostic labels proposed in the field. OUTCOMES AND RESULTS: Aetiologies of cleft palate/VPI speech identified include developmental (cognitive-linguistic), middle ear disease and fluctuating hearing loss, altered oral structure, abnormal facial growth, VPI-structural (abnormal palate muscle) and VPI-iatrogenic (maxillary advancement surgery). There are four main terminologies used to describe cleft palate/VPI speech: active/passive and compensatory/obligatory, which overlap with the four categories used in the UK speech summary patterns: anterior oral cleft speech characteristics (CSCs), posterior oral CSCs, non-oral CSCs and passive CSCs, although not directly comparable. Current classification systems in non-cleft SSD do not sufficiently capture the full nature and complexity of cleft palate/VPI speech. CONCLUSIONS AND IMPLICATIONS: Our attempt at identifying the heterogeneous range of aetiologies provides clinicians with a better understanding of cleft palate/VPI speech to inform the management pathway and the nature and type of speech intervention required. We hope that the unravelling of the different terminology in relation to the UK speech summary patterns, and those used elsewhere, reduces confusion and provides more clarity for clinicians in the field. Diagnostic labels and classification require international agreement. WHAT THIS PAPER ADDS: What is already known on the subject Speech associated with cleft palate/velopharyngeal insufficiency (VPI) is a complex myriad of speech characteristics with a wide and heterogeneous range of aetiologies. Different terminology and speech summary patterns are used to describe the speech characteristics. The traditional classification of cleft palate/VPI speech is Articulation Disorder, although evidence is building for Phonological Disorder and contrastive approaches in cleft speech intervention. What this paper adds to existing knowledge This paper explores the range of aetiologies of cleft palate/VPI speech (e.g., altered oral structure, abnormal facial growth, abnormal palate muscle and iatrogenic aetiologies) and attempts to relate aetiology with speech characteristic(s). An attempt is made at unravelling the different terminology used in relation to a well-known and validated approach to analysis, used in the United Kingdom and elsewhere. Complexities of current diagnostic labels and classifications in Speech Sound Disorder to describe cleft palate/VPI speech are discussed. What are the potential or actual clinical implications of this work? There needs to be a common language for describing and summarising cleft palate/VPI speech. Speech summary patterns based on narrow phonetic transcription and correct identification of aetiology are essential for the accurate classification of the speech disorder and identification of speech intervention approaches. There is an urgent need for research to identify the most appropriate type of contrastive (phonological) approach in cleft lip and/or palate.

Talking with children and young people with 22q11DS about their mental health, behaviour, learning and communication.

BACKGROUND: 22q11DS11.2 deletion syndrome (22q11DS) is a complex multisystem syndrome characterized by physical abnormalities, psychiatric comorbidities and cognitive deficits. The views of children and young people (CYP) about the challenges associated with their mental health, behaviour, learning and communication difficulties have not been reported. The aim of this study was to address this gap and to understand whether they had help and support with these and their views of this. METHODS: A three-phase mixed-methods study was undertaken, involving interviews with CYP with 22q11DS, a follow-up survey for those aged 11-25 years and a stakeholder workshop at which CYP presented their views of living with 22q11DS to health professionals and parents. Interview transcripts were thematically analysed, and non-parametric statistics were used to analyse survey data. RESULTS: The interviews (n = 13) and survey (n = 32) indicated a mixed picture, with some CYP not reporting ongoing problems; others who had problems received help but a sizeable proportion had unmet needs and wanted to receive help. Two-thirds reported often experiencing negative feelings, and almost half had difficulties with social interactions. Family members were the main sources of support, with teaching assistants identified as an important support at school. CONCLUSIONS: The emotional impact of 22q11DS on CYP can be significant: They often do not understand the consequences of having 22q11DS and are frequently not given strategies to understand and manage their feelings, behaviour or problems. This leads to a range of emotions that manifest in different ways at home and at school. CYP are able to talk about the impact of different aspects of 22q11DS on them, facilitated by the use of creative methods, but they differ in how the condition affects them and their perceptions about that. It is imperative that CYP themselves are asked about their experiences, feelings and needs to ensure tailoring of interventions to their individual requirements.

Submucous Cleft Palate (SMCP): Indications and Outcomes of Radical Muscle Dissection Palatoplasty in Children Under 4 Years of Age.

To delineate the indications, referral sources and outcomes of radical muscle dissection palate repair in the first 4 years of life for patients with submucous cleft palate (SMCP).This study presents a retrospective analysis of a single surgeon's management.All children were treated at Great Ormond Street Hospital, United Kingdom.Twenty-three children with nonsyndromic SMCP were included in this study.All participants underwent radical muscle dissection repair before their fourth birthday.Parameters analyzed included: referral sources, indications for referral, extent of anatomical abnormality, and postoperative outcomes.Paediatricians provided the largest proportion of referrals to the cleft lip and palate clinic (39%) due to the presence of cleft lip/palate on prenatal scans or during neonatal examination. Over half (n = 12, 52%) of patients had severe anatomical anomalies being in grade IV (score 8-9), with the classical triad present to some extent in all but 2 of the patients. The main indication for surgery was nasal regurgitation of food and liquid (n = 9, 35%) followed by hypernasality (n = 6, 21%), difficulty feeding (n = 3, 8%), and severe anatomical defect (n = 2; 4%). Postoperatively, the presenting complaint improved in the majority of cases.Nonsyndromic SMCP is often missed, though may present with nasal regurgitation, feeding problems, and/or hypernasality, though may be missed in nonsyndromic children. Early radical muscle dissection repair in the first 4 years of life is safe and effective, facilitating normal speech development.

Adapting Elements of Cleft Care Protocols in Low- and Middle-income Countries During and After COVID-19: A Process-driven Review With Recommendations.

A consortium of global cleft professionals, predominantly from low- and middle-income countries, identified adaptations to cleft care protocols during and after COVID-19 as a priority learning area of need.A multidisciplinary international working group met on a videoconferencing platform in a multi-staged process to make consensus recommendations for adaptations to cleft protocols within resource-constrained settings. Feedback was sought from a roundtable discussion forum and global organizations involved in comprehensive cleft care.Foundational principles were agreed to enable recommendations to be globally relevant and two areas of focus within the specified topic were identified. First the safety aspects of cleft surgery protocols were scrutinized and COVID-19 adaptations, specifically in the pre- and perioperative periods, were highlighted. Second, surgical procedures and cleft care services were prioritized according to their relationship to functional outcomes and time-sensitivity. The surgical procedures assigned the highest priority were emergent interventions for breathing and nutritional requirements and primary palatoplasty. The cleft care services assigned the highest priority were new-born assessments, pediatric support for children with syndromes, management of acute dental or auditory infections and speech pathology intervention.A collaborative, interdisciplinary and international working group delivered consensus recommendations to assist with the provision of cleft care in low- and middle-income countries. At a time of global cleft care delays due to COVID-19, a united approach amongst global cleft care providers will be advantageous to advocate for children born with cleft lip and palate in resource-constrained settings.

The First Hybrid International Educational Comprehensive Cleft Care Workshop.

OBJECTIVE: Describe the first hybrid global simulation-based comprehensive cleft care workshop, evaluate impact on participants, and compare experiences based on in-person versus virtual attendance. DESIGN: Cross-sectional survey-based evaluation. SETTING: International comprehensive cleft care workshop. PARTICIPANTS: Total of 489 participants. INTERVENTIONS: Three-day simulation-based hybrid comprehensive cleft care workshop. MAIN OUTCOME MEASURES: Participant demographic data, perceived barriers and interventions needed for global comprehensive cleft care delivery, participant workshop satisfaction, and perceived short-term impact on practice stratified by in-person versus virtual attendance. RESULTS: The workshop included 489 participants from 5 continents. The response rate was 39.9%. Participants perceived financial factors (30.3%) the most significant barrier and improvement in training (39.8%) as the most important intervention to overcome barriers facing cleft care delivery in low to middle-income countries. All participants reported a high level of satisfaction with the workshop and a strong positive perceived short-term impact on their practice. Importantly, while this was true for both in-person and virtual attendees, in-person attendees reported a significantly higher satisfaction with the workshop (28.63 ± 3.08 vs 27.63 ± 3.93; P = .04) and perceived impact on their clinical practice (22.37 ± 3.42 vs 21.02 ± 3.45 P = .01). CONCLUSION: Hybrid simulation-based educational comprehensive cleft care workshops are overall well received by participants and have a positive perceived impact on their clinical practices. In-person attendance is associated with significantly higher satisfaction and perceived impact on practice. Considering that financial and health constraints may limit live meeting attendance, future efforts will focus on making in-person and virtual attendance more comparable.

Culture, cognisance, capacity and capability: The interrelationship of individual and organisational factors in developing a research hospital.

AIM: To share our experience of implementing a programme of interventions aimed at building research capacity and capability of nurses and allied health professionals in a specialist children's hospital. BACKGROUND: Clinicians at the forefront of care are well positioned to lead on research to improve outcomes and experiences of patients but some professional groups continue to be underrepresented. Inequities persist alongside robust national infrastructures to support Clinical Academic Careers for non-medical health professionals, further highlighting the need to address local infrastructure and leadership to successfully build research capacity. DESIGN: An evolving programme of inquiry and analysis was established in one organisation, this included targeted interventions to mitigate barriers and enable research capacity and capability. METHODS: An all-staff survey was conducted in 2015 to understand the existing research culture. Interventions were put in place, evaluated through a second survey (2018), and focus group interviews with staff who had accessed interventions. RESULTS: Respondents demonstrated high levels of interest and commitment to research at the individual level which were not always harnessed at the organisational level. Inequities between professional groups existed in terms of training, time to undertake research and opportunities and outputs. Follow-up revealed continuing structural barriers at an organisational level, however at an individual level, interventions were reflected in >30 fellowship awards; major concerns were reported about sustaining these research ambitions. CONCLUSIONS: Success in building a research-active clinical workforce is multifactorial and all professional groups report increasing challenges to undertake research alongside clinical responsibilities. Individuals report concerns about the depth and pace of cultural change to sustain Clinical Academic Careers and build a truly organisation-wide research hospital ethos to benefit patients. RELEVANCE TO CLINICAL PRACTICE: The achievements of individual nurses and allied health professionals indicate that with supportive infrastructure, capacity, cognisance and capability are not insurmountable barriers for determined clinicians. We use the standards for reporting organisational case studies to report our findings (Rodgers et al., 2016 Health Services and Delivery Research, 4 and 1).

Core Outcome Set for Reporting Outcomes of Interventions for Velopharyngeal Dysfunction: Final Results of the COS-VPD Initiative.

OBJECTIVE: To date, the recording of outcomes of interventions for velopharyngeal dysfunction (VPD) has not been standardized. This makes a comparison of results between studies challenging. The aim of this study was to develop a core outcome set (COS) for reporting outcomes in studies examining the management of VPD. DESIGN: A two-round Delphi consensus process was used to develop the COS. PATIENTS, PARTICIPANTS: The expert Delphi panel comprised patients and caregivers of patients with VPD, surgeons and speech and language therapists specializing in cleft palate, and researchers with expertise in VPD. INTERVENTIONS: A long list of outcomes was derived from the published literature. In each round of a Delphi survey, participants were asked to score outcomes using the Grading of Recommendations, Assessment, Development, and Evaluations scale of 1 to 9, with 1 to 3 labeled "not important," 4 to 6 labeled "important but not critical," and 7 to 9 labeled "critical." MAIN OUTCOME MEASURE: Consensus criteria were specified a priori. Outcomes with a rating of 75% or more of the panel rating 7 to 9 and 25% or fewer rating 1 to 3 were included in the COS. RESULTS: A total of 31 core outcomes were identified from the Delphi process. This list was condensed to combine topic areas to produce a final COS of 10 outcomes, including both processes of care and patient-reported outcomes that should be considered for reporting in future studies of VPD. CONCLUSIONS: Implementation of the COS-VPD will facilitate consistency of outcomes data collection and comparison of results across studies.

The Impact of Maxillary Osteotomy on Fricatives in Cleft Lip and Palate: A Perceptual Speech and Acoustic Study.

INTRODUCTION: Abnormal facial growth is a recognized outcome in cleft lip and palate (CLP), resulting in a concave profile and a class III occlusal status. Maxillary osteotomy (MO) is undertaken to correct this facial deformity, and the surgery can impact speech articulation, although the evidence remains limited and ill-defined for the CLP population. AIMS: The aim of the study was to investigate the impact of MO on the production of the fricatives /f/ and /s/, using perceptual and acoustic analyses, and to explore the nature of speech changes. METHODS: Twenty participants with CLP were seen 0-3 months pre-operatively (T1) and 3 months (T2) and 12 months (T3) after MO. A normal group (N = 20) was similarly recruited. Perceptual speech data was collected according to a validated framework and ratings made on audio and audio-video recordings (VIDRat). Spectral moments were centre of gravity (CG), standard deviation (SD), skewness (SK) and kurtosis (KU). Reliability studies were carried out for all speech analyses. RESULTS: For the CLP group, VIDRat identified dentalization/interdentalization as the main type of pre-operative error for /s/ with a statistically significant improvement over time, χ2(2) = 6.889, p = 0.032. Effect sizes were medium between T1 and T3 (d = 0.631) and small between T2 and T3 (d = 0.194). For the acoustic data, effect sizes were similarly medium between T1 and T2 (e.g., SK, /f/ d = 0.579, /s/ d = 0.642) and small between T1 and T3 across all acoustic parameters. Independent t tests showed mainly statistically significant differences between both groups at all time points with large effect sizes (e.g., T2 CG, t = -4.571, p < 0.001, d =1.581), indicating that /s/ was not normalized post-operatively. For /f/, differences tended to be at T1 with large effect sizes (e.g., CG, t = -2.307, p = 0.028, d = 0.797), reflecting normalization. CONCLUSIONS AND IMPLICATIONS: This is the first speech acoustic study on /f/ for individuals with CLP undergoing MO. The surgery has a positive impact on /f/ and /s/, which appear to stabilize 3 months post-operatively. Speech changes are an automatic and a direct consequence of the physical changes brought about by MO, effecting articulatory re-organization. The results of the study have direct clinical implications for the clinical care pathway for patients with CLP undergoing MO.

A perceptual outcome measure of velopharyngeal function based on the Cleft Audit Protocol for Speech-Augmented (CAPS-A VPC-Sum): Validation through a speech osteotomy study.

BACKGROUND: The status of the velopharyngeal mechanism can be inferred from perceptual ratings of specified speech parameters. Several studies have proposed the measure of an overall velopharyngeal composite score based on these perceptual ratings and have reported good validity. The Cleft Audit Protocol for Speech-Augmented (CAPS-A) is a validated and reliable perceptual framework for the assessment of cleft speech and velopharyngeal function used by all Regional Cleft Services in the UK and Ireland. An overall velopharyngeal composite summary score based on the CAPS-A would serve as an important surgical outcome measure of speech. AIMS: To develop and validate a velopharyngeal composite summary score based on perceptual ratings made on the CAPS-A (CAPS-A VPC-Sum) using data from a maxillary osteotomy (MO) study. METHODS & PROCEDURES: There were two surgical groups: a cleft lip and palate (CLP) (N = 20) group and a non-CLP group (N = 10), and a normal control group (N = 20). Participants in groups 1 and 2 were seen for perceptual and instrumental assessments of speech and velopharyngeal function preoperatively (T1), 3 months (T2) and 12 months (T3) postoperatively. Perceptual speech data were collected and rated by independent listeners using CAPS-A. OUTCOMES & RESULTS: Moderate to strong interrater reliability for perceptual data (rs = 0.503-1.000, all p < 0.01) and strong to very strong reliability for videofluoroscopic measurements (rs = 0.746-0.947) were found. Construct validity of the CAPS-A VPC-Sum was shown by an increase in postoperative scores for the CLP group only Ï°2 (2) = 9.769, p = 0.008 and significant differences between the CLP and the other two groups at T2 and T3 using independent t-tests. Convergent and divergent validity was indicated by a positive moderate correlation with related parameters (e.g., hypernasality rs = 0.869, p < 0.01) and a weak correlation with unrelated parameters (e.g., amount of forward advancement rs = 0.160, p = 0.526). Criterion validity was found by a moderate correlation between closure ratio rs = -0.541, p = 0.020 and CAPS-A VPC-Sum. CONCLUSIONS & IMPLICATIONS: A velopharyngeal composite score based on perceptually rated parameters serves as an important surgical speech outcome measure. The CAPS-A VPC-Sum is a useful, reliable and valid outcome measure of velopharyngeal function. There are added positive implications for other clinicians using geographically and language-specific adapted versions of the CAPS-A internationally. WHAT THIS PAPER ADDS: What is already known on this subject Velopharyngeal composite scores based on perceptually rated speech parameters have been shown to have both clinical and research utility, serving as a useful surgical outcome measure. However, such a composite score must be specifically validated on the perceptual speech framework upon which it is based, as there are differences in measurement methods and terminology across cleft speech perceptual frameworks internationally. What this paper adds to existing knowledge The CAPS-A is a nationally used tool in the UK and Ireland for audit and research purposes with validated and adapted international versions. This paper reports on the validation of the derived velopharyngeal composite score measure based on the CAPS-A and an English-speaking sample, providing evidence of its validity through a speech osteotomy study. What are the potential or actual clinical implications of this work? This work provides CLP teams who use CAPS-A with a validated surgical speech outcome measure of velopharyngeal function. It has positive implications also for adapted versions of the CAPS-A internationally.

Are Vowels Normalized After Maxillary Osteotomy? An Acoustic Study in Cleft Lip and Palate.

BACKGROUND: Maxillary hypoplasia is a common skeletal condition in cleft lip and palate (CLP). Maxillary osteotomy is typically used to reposition the maxilla in CLP with maxillary hypoplasia. Previous studies have suggested that vowel articulations are adjusted postsurgically due to altered vocal tract configuration and articulatory reorganization. This acoustic study aims to investigate whether vowels are normalized postoperatively and to explore the nature of articulatory reorganization. METHODS AND PROCEDURES: A prospective study was conducted to examine the vowel production of a group of individuals with CLP (N = 17) undergoing maxillary osteotomy and a group of normal controls (N = 20), using speech acoustic data. The data were collected at 0 to 3 months presurgery (T1), 3-months (T2), and 12-months (T3) postsurgery. General linear model repeated measures and independent t-tests were undertaken on F1, F2, and vowel space area. RESULTS: General linear model repeated measures revealed no main effects of time for F1 (F [2, 22] = 1.094, P = 0.352), F2 (F [2, 22] = 1.269, P = 0.301), and vowel space area (F [2, 28] = 0.059, P = 0.943). Independent t-tests showed statistically significant differences (P < 0.05) for all acoustic parameters and all vowels between the CLP and the normal groups at all time points. CONCLUSIONS: Vowels were not normalized after maxillary osteotomy despite positive anatomical changes within the oral cavity. Individuals with CLP tended to adjust their vowel articulatory gestures to match presurgical patterns. The nature of articulatory reorganization appears to be prompt, sensory-driven, complete, and permanent.

Assessing speech at three years of age in the cleft palate population: a scoping review of assessment practices.

BACKGROUND: There is no consensus in the UK regarding the types of speech samples or parameters of speech that should be assessed at 3 years of age in children with cleft palate ± cleft lip (CP±L), despite cleft units routinely assessing speech at this age. The standardization of assessment practices would facilitate comparisons of outcomes across UK cleft units; earlier identification of speech impairments-which could support more timely treatments; and more reliable recording of therapy impacts and surgical interventions. AIMS: To explore assessment practices used to assess speech in 3-year-old children with CP±L, including speech parameters, methods of assessment and the nature of the speech sample used. METHODS & PROCEDURES: A broad examination of the literature was undertaken through the use of a scoping review conducted in accordance with Joanna Briggs Institute guidelines. Search terms were generated from a preliminary search and then used in the main search (Medline, CINAHL, Embase, AMED and PsycINFO). MAIN CONTRIBUTION: A combination of approaches (medical, linguistic, developmental and functional) is required to assess CP±L speech at age 3. A developmental approach is recommended at this age, considering the complexity of speech profiles at age 3, in which typically developing speech processes may occur alongside cleft speech characteristics. A combined measure for both nasal emission and turbulence, and an overall measure for velopharyngeal function for speech, show potential for assessment at this age. Categorical ordinal scales are frequently used; the use of continuous scales has yet to be fully explored at age 3. Although single-word assessments, including a subset of words developed for cross-linguistic comparisons, are frequently used, more than one type of speech sample may be needed to assess speech at this age validly. The lack of consensus regarding speech samples highlights a need for further research into the types of speech samples 3-year-olds can complete; the impact of incomplete speech samples on outcome measures (particularly relevant at this age when children may be less able to complete a full sample); the impact of different speech samples on the validity of assessments; and the reliability of listener judgements. CONCLUSIONS & IMPLICATIONS: Whilst a medical model and linguistic approaches are often central in assessments of age-3 cleft speech, this review highlights the importance of developmental and functional approaches to assessment. Cross-linguistic single-word assessments show potential, and would facilitate the comparison of UK speech outcomes with other countries. Further research should explore the impact of different speech samples and rating scales on assessment validity and listener reliability.

Randomized controlled trial comparing Parent Led Therapist Supervised Articulation Therapy (PLAT) with routine intervention for children with speech disorders associated with cleft palate.

BACKGROUND: A total of 68% of pre-school children with cleft palate have speech problems requiring speech therapy. There is a lack of access to regular targeted therapy. Parent training leads to positive outcomes in early communication skills in cleft palate and non-cleft speech disorders. Connected health has been used to address inadequate access to therapy, providing intervention to those who would not otherwise receive therapy. AIMS: To evaluate the speech, activity and participation outcomes of Parent Led, Therapist Supervised, Articulation Therapy (PLAT) compared with routine speech therapy intervention in parent-child dyads. METHODS & PROCEDURES: A total of 44 children, aged 2.9-7.5 years, were included in a two-centre, two-phase randomized controlled trial. Informed consent and assent were obtained. Participants and speech and language therapists (SLTs) were unblinded to the groups. Parents, in the parent-trained group (n = 23), attended 2 days' training, received a detailed speech therapy programme, and undertook intervention over 12 weeks supported by the cleft specialist SLT using FaceTime and one face-to-face session. In the control arm (n = 21), parent-child dyads received six therapy sessions over 12 weeks with a research SLT, comparable with usual care. Speech recordings were undertaken pre- and post-intervention. Percent consonant correct (PCC) was analysed by external SLTs blinded to the time and group. Activity and participation were measured using the Intelligibility in Context Scale (ICS) and Focus on Outcomes for Children Under Six (FOCUS) questionnaire. OUTCOMES & RESULTS: There was no evidence of an interaction between Time and Group or an overall statistical difference between groups for PCC scores. There was a statistically significant difference over time for both groups (words: p < 0.002; confidence interval (CI) = 9.38-16.27; d = 0.57; sentences: p < 0.002; CI = 16.04-25.97; d = 0.23). Effect sizes were medium for words and small for sentences. For intelligibility and participation, there was no evidence of an interaction between Time and Group or an overall statistical difference between groups. A statistically significant difference over time was found for intelligibility (F = 29.97, d.f. = 1, 42, p < 0.001, 95 % CI = 1.45-3.15 d = 0.46) and for participation (F = 14.19, d.f. = 1, 41, p < 0.001 95% CI = 7.63-25.03; d = 0.36) with FOCUS results indicating clinically meaningful (parent-led group) and significant (control group) change in participation. CONCLUSIONS & IMPLICATIONS: PLAT can be as effective as routine care in changing speech, activity and participation outcomes for children with cleft palate, when supported by a specialist cleft SLT using connected health. What this paper adds What is already known on this subject Over 50% of children with cleft palate require speech therapy. However, there is a lack of timely, accessible speech therapy services in the UK and Ireland. Previous studies have shown that parents can deliver therapy effectively, and that connected health can support the delivery of speech therapy. This study aims to provide evidence that parent-led therapy with the supervision of a specialist cleft therapist using FaceTime is effective. What this paper adds to existing knowledge This randomized controlled trial indicates that parents can be trained to deliver therapy for children with cleft palate speech disorders, under the supervision of an SLT. This approach results in improved speech, activity and participation outcomes similar to routine care. What are the potential or actual clinical implications of this work? This study indicates that both parent-led articulation therapy and routine care showed meaningful gains in speech, activity and participation, and that parent-led articulation therapy when supported by a cleft SLT using connected health could be an additional service delivery model for children with cleft palate speech disorders.

Identifying Predictors of Acquired Velopharyngeal Insufficiency in Cleft Lip and Palate Following Maxillary Osteotomy Using Multiple Regression Analyses.

BACKGROUND: Maxillary osteotomy is typically undertaken to correct abnormal facial growth in cleft lip and palate. The surgery can cause velopharyngeal insufficiency resulting in hypernasality. This study aims to identify valid predictors of acquired velopharyngeal insufficiency following maxillary osteotomy by using a range of perceptual and instrumental speech investigations and multiple regression. METHODS: A prospective study was undertaken consisting of a consecutive series of patients with cleft lip and palate (N = 20) undergoing maxillary osteotomy by a single surgeon. Participants were seen at: 0 to 3 months pre-surgery (T1), 3-months (T2), and 12-months (T3) post-surgery. Hypernasality was rated using the cleft audit protocol for speech-augmented (CAPS-A) and visual analog scales, and nasalance was measured on the Nasometer II 6400. For lateral videofluorosopic and nasendoscopic images, visual perceptual ratings and quantitative ratiometric measurements were undertaken. Multiple regression analyses were undertaken to identify predictors. RESULTS: T3 models with hypernasality as the dependent variable were found to be a good fit and significant (eg, CAPS-A: R2 = 0.920, F(11,7) = 7.303, P = 0.007). Closure ratio (a quantitative ratiometric measurement) and proportion of palate contacting the posterior pharyngeal wall (a visual perceptual rating) were identified as significant predictors for the CAPS-A model (P = 0.030, P = 0.002).

Benchmarking Speech, Velopharyngeal Function Outcomes and Surgical Characteristics Following the Sommerlad Protocol and Palate Repair Technique.

OBJECTIVE: To report speech and velopharyngeal function (VPF) outcomes, and surgical characteristics, at age 5 following early complete palate closure using the Sommerlad protocol. DESIGN: A retrospective, descriptive, cross-sectional consecutive series. SETTING: A regional twin site center; a district general hospital and tertiary children's hospital. PARTICIPANTS: Between 1993 and 2006, 877 participants underwent surgery; 712 (81%) were eligible for inclusion; 391 (55%) were included, 321 (45%) excluded. Thirteen percent had bilateral cleft lip and palate (CLP), 40% unilateral CLP, and 47% isolated cleft palate. INTERVENTION: Lip and vomerine flap repair at 3 months of age (BCLP, UCLP) with soft palate closure using radical muscle dissection and retropositioning at a mean age of 6.6 months, range 4 to 23 months, as described and undertaken by Sommerlad. OUTCOME MEASURES: Velopharyngeal Composite (VPC) CAPS-A and articulation summary scores derived from analysis using the Cleft Audit Protocol for Speech-Augmented. RESULTS: A VPC-SUM CAPS-A score of "0" was found in 97% reflecting adequate VPF; 73% had no cleft articulation difficulties. The BCLP group had the poorest articulation. Secondary speech surgery rate was 2.6% and 10.7% had fistula repair. Levator muscle quality and degree of retropositioning was associated with speech outcomes related to VPF. 2.6% had current features of VPI. CONCLUSIONS: Complete palate closure by around 6 months of age by the surgeon who described the technique led to excellent perceived VPF and very good speech outcomes at age 5, with less speech therapy and secondary speech surgery than commonly reported. These findings serve as a benchmark for the Sommerlad protocol and technique.

Familial Absent Uvula With Velopharyngeal Incompetence-A New Syndrome?

We present a family with a previously undescribed abnormality of the palate and oropharynx which involved the absence of the uvula and the anterior pillar of the fauces, rudimentary posterior pillar of the fauces, and hypernasality. Eight family members over 4 generations are affected in a pattern consistent with autosomal dominant inheritance. A causal role for the FOXF2 gene has been identified and previously reported. We describe the management of the proband, which involved attempting to lengthen the palate and to retroposition the abnormally anteriorly directed velar musculature, along with speech therapy.

Effect of Maxillary Osteotomy on Speech in Cleft Lip and Palate: Instrumental Outcomes of Velopharyngeal Function.

OBJECTIVE: To investigate the effect of maxillary osteotomy on velopharyngeal function in cleft lip and palate (CLP) using instrumental measures. DESIGN: A prospective study. PARTICIPANTS: A consecutive series of 20 patients with CLP undergoing maxillary osteotomy by a single surgeon were seen at 0 to 3 months presurgery (T1), 3 months (T2), and 12 months (T3) post-surgery. INTERVENTIONS: Nasalance was measured on the Nasometer II 6400. For videofluoroscopy and nasendoscopy data, visual perceptual ratings, for example, palatal lift angle (PLAn), and quantitative ratiometric measurements, for example, closure ratio (CRa), were made using a validated methodology and computer software. Reliability studies were undertaken for all instrumental measures. MAIN OUTCOME MEASURES: Repeated measures analysis of variance (with time at 3 levels) for nasalance and each velar parameter. Planned comparisons across pairs of time points (T1-T2, T1-T3, and T2-T3) including effect sizes. RESULTS: A significant difference over time was found for nasalance (P = .001) and planned comparisons across pairs of time points were significant between T1 and T2 (P = .008), T1 and T3 (P = .002), but not between T2 and T3 (P = .459) providing evidence that maxillary osteotomy can impact on nasalance adversely and that the changes seen are permanent and stable. There were also significant differences over time for PLAn (P = .012) and CRa (P = -.059) and planned comparisons for both velar parameters reflected similar findings to those of nasalance. CONCLUSIONS: Maxillary osteotomy can adversely affect velopharyngeal function in patients with CLP. The study provides evidence for a much earlier post-surgery review even as early as 3 months after surgery.

Percent Consonant Correct as an Outcome Measure for Cleft Speech in an Intervention Study.

BACKGROUND/AIMS: Percent consonant correct (PCC) was originally described by Shriberg and Kwiatkowski [J Speech Hear Disord. 1982 Aug;47(3):256-70] as a severity metric for phonological speech disorders, and has been adapted and used in many studies on speech sound disorders. It is well-recognized that cleft speech is complex, consisting of several interacting parameters assessed simultaneously, with error sounds not in the listener's own language. In speech outcome studies, narrow phonetic transcription and the reporting of intra- and inter-rater reliability are acknowledged as the gold standard. However, cleft speech brings special challenges to this task, as complex speech disorders are known to be associated with low transcriber agreement. Recent studies informed the decision to use PCC as the primary outcome measure in a cleft speech intervention study, given its common usage and familiarity. The aim was to specifically evaluate the intra- and inter-rater reliability of PCC in an intervention study, in contrast to other types of speech outcome studies. METHODS: Two trained and experienced listeners analyzed 119 recordings, randomly selected from five data points before, during, and following intervention. The PCC score was separately calculated for words and sentences/phrases. RESULTS: Using intraclass correlations (ICCs), Phase 1 results showed poor reliability for targets elicited for words (ICC = 0.07) and sentences/phrases (ICC = 0.42). Differences in classification of errors as glottal stops and consonant deletion accounted for this. Following further training, a second reliability study was undertaken showing improvement in the number of targets elicited in words (ICC = 0.85) and sentences/phrases (ICC = 0.94). There was very good inter-rater reliability for the PCC score on the word dataset (ICC = 0.9) and the sentence dataset (ICC = 0.88). Very good intra-rater reliability (ICC = 1.0) was found for the PCC score in both words and sentences/phrases for each listener. One listener consistently gave higher modified PCC scores. CONCLUSIONS: In cleft speech intervention studies, reliability of the number of targets elicited should be reported. Listeners need to distinguish between glottal articulation and consonant deletion, in order that the PCC score is meaningful. Attention should be paid to where listeners are reliable, but their pattern of scores consistently differs but in a consistent way. More research is needed on measuring the resolution of articulation difficulties in cleft intervention studies.

Hospitalized children with intellectual disability: Parents as partners in their care.

BACKGROUND: Limited evidence exists of what hospital care is like for parents of children and young people (CYP) with intellectual disability (ID). Effective collaboration is often missing, with parents lacking trust in professionals, with feelings of being expected to care and consequently unable to leave their child. This paper focuses on what parents want from their relationship with healthcare professionals. METHOD: An ethnographic approach was used, including in-depth interviews and informal discussions with nine parents of CYP with intellectual disability. Researcher voice poems are interspersed with quotations to illustrate key findings. RESULTS: Parents described the need for a genuine partnership with professionals. They identified seven elements that ideally characterize this partnership: Preparation, Accessibility, Reliability, Trust, Negotiation, Expertise and Respect (PARTNER). CONCLUSION: Children and young people with intellectual disability and their parents are frequent users of healthcare services. Getting it right from the outset is important to establish their trust in the system.