Considering the role of speech processing in cleft-related speech sound disorders: Implications for causal pathways and classification systems.

BACKGROUND: Classification systems in healthcare support shared understanding of conditions for clinical communication, service monitoring and development, and research. Children born with cleft palate with or without cleft lip (CP+/-L) are at high risk of developing cleft-related speech sound disorder (SSD). The way cleft-related SSD is represented and described in SSD classification systems varies. Reflecting on the potential causal pathways for different cleft-related speech features, including the role of speech processing skills, may inform how cleft-related SSD is represented in classification systems. AIM & APPROACH: To explore and reflect on how cleft-related SSD is represented in current SSD classification systems in the context of considering how speech processing skills and other factors may be involved in causal pathways of cleft speech characteristics (CSCs). MAIN CONTRIBUTION: Variation in the representation of cleft-related SSD in classification systems is described. Potential causal pathways for passive cleft- related speech features and different active CSCs are explored. The factors involved in the development and/or persistence of different active CSCs may vary. Some factors may be specific to children born with CP+/-L, but if speech processing skills are also involved, this is an overlap with other SSD subtypes. Current evidence regarding relationships between different speech processing skills and active CSCs is limited. Implications for the representation of cleft-related SSD in SSD classification systems are discussed. CONCLUSION: There are different categories of cleft-related speech features which are essential to understand and identify in children with cleft-related SSD to ensure appropriate management. Representation of these feature categories in classification systems could support understanding of speech in this population. Speech processing skills could be involved in the development and/or persistence of different active CSCs in individual children. Reflection and discussion on how cleft-related SSD is represented in classification systems in relation to other SSD subtypes may inform future iterations of these systems. Further work is needed to understand factors influencing the development and/or persistence of active CSCs, including speech processing skills. WHAT THIS PAPER ADDS: What is already known on the subject Cleft-related speech sound disorder (SSD) is commonly described as being of known origin. The features of cleft-related SSD have been described extensively and several authors have also examined factors which may contribute to speech development and outcomes in children born with cleft palate +/- lip. There is limited evidence regarding the role of speech processing in the development and persistence of cleft-related SSD. What this study adds This paper reflects on how cleft-related SSD is represented in SSD classification systems in relation to key feature categories of cleft-related SSD and possible causal pathways for passive features and active cleft speech characteristics (CSCs). The role of cognitive speech processing skills is specifically considered alongside other factors that may contribute to the development of active CSCs. What are the clinical implications of this work? Causal pathways for different features of cleft-related SSD may vary, particularly between passive and active features, abut also between different active CSCs. Speech and language therapists (SLTs) need to differentially diagnose passive speech features and active CSCs. Consideration of the role of different speech processing skills and interactions with other potentially influencing factors in relation to active CSCs may inform clinical hypotheses and speech and language therapy (SLT) intervention. Representing key features of cleft-related SSD in classification systems may support understanding of cleft-related SSD in relation to other SSD subtypes.

Timing of Primary Surgery for Cleft Palate.

BACKGROUND: Among infants with isolated cleft palate, whether primary surgery at 6 months of age is more beneficial than surgery at 12 months of age with respect to speech outcomes, hearing outcomes, dentofacial development, and safety is unknown. METHODS: We randomly assigned infants with nonsyndromic isolated cleft palate, in a 1:1 ratio, to undergo standardized primary surgery at 6 months of age (6-month group) or at 12 months of age (12-month group) for closure of the cleft. Standardized assessments of quality-checked video and audio recordings at 1, 3, and 5 years of age were performed independently by speech and language therapists who were unaware of the trial-group assignments. The primary outcome was velopharyngeal insufficiency at 5 years of age, defined as a velopharyngeal composite summary score of at least 4 (scores range from 0 to 6, with higher scores indicating greater severity). Secondary outcomes included speech development, postoperative complications, hearing sensitivity, dentofacial development, and growth. RESULTS: We randomly assigned 558 infants at 23 centers across Europe and South America to undergo surgery at 6 months of age (281 infants) or at 12 months of age (277 infants). Speech recordings from 235 infants (83.6%) in the 6-month group and 226 (81.6%) in the 12-month group were analyzable. Insufficient velopharyngeal function at 5 years of age was observed in 21 of 235 infants (8.9%) in the 6-month group as compared with 34 of 226 (15.0%) in the 12-month group (risk ratio, 0.59; 95% confidence interval, 0.36 to 0.99; P = 0.04). Postoperative complications were infrequent and similar in the 6-month and 12-month groups. Four serious adverse events were reported (three in the 6-month group and one in the 12-month group) and had resolved at follow-up. CONCLUSIONS: Medically fit infants who underwent primary surgery for isolated cleft palate in adequately resourced settings at 6 months of age were less likely to have velopharyngeal insufficiency at the age of 5 years than those who had surgery at 12 months of age. (Funded by the National Institute of Dental and Craniofacial Research; TOPS ClinicalTrials.gov number, NCT00993551.).

Inter-rater reliability in classification of canonical babbling status based on canonical babbling ratio in infants with isolated cleft palate randomised to Timing of Primary Surgery for Cleft Palate (TOPS).

Canonical babbling (CB) is commonly defined as present when at least 15% of all syllables produced are canonical, in other words a canonical babbling ratio (CBR) ≥0.15. However, there is limited knowledge about inter-rater reliability in classification of CB status based on CBR and inter-rater differences in assessment of CBR. We investigated inter-rater reliability of experienced Speech Language Therapists (SLTs) on: classification of CB status based on CBR ≥ 0.15, CBRs and the total number of syllables per infant used to calculate CBR.Each infant (n = 484) was video-recorded at a clinical site in play interaction with their parent as part of the randomised controlled trial Timing of Primary Surgery for Cleft Palate. Each recording was subsequently assessed by three independent SLTs, from a pool of 29 SLTs. They assessed the recordings in real time.The three assessing SLTs agreed in classification of CB status in 423 (87.4%) infants, with higher complete agreement for canonical (91%; 326/358) than non-canonical (77%; 97/126). The average difference in CBR and total number of syllables identified between the SLT assessments of each infant was 0.12 and 95, respectively.This study provided new evidence that one trained SLT can reliably classify CB status (CBR ≥ 0.15) in real time when there is clear distinction between the observed CBR and the boundary (0.15); however, when the observed CBR approaches the boundary multiple SLT assessments are beneficial. Thus, we recommend to include assessment of inter-rater reliability, if the purpose is to compare CBR and total syllable count across infants or studies.Trial registration number here: www.clinicaltrials.gov, identifier NCT00993551.

A retrospective analysis of speech and hearing in patients receiving surgery for submucous cleft palate.

Submucous cleft palate (SMCP) is an uncommon subtype of cleft palate that is associated with symptoms of velopharyngeal insufficiency (VPI), the most common being hypernasal speech. A high proportion of patients also suffer from conductive hearing loss, which is thought to be due to eustachian tube dysfunction. A number of surgical techniques have been proposed to correct the anatomical defect that is responsible for VPI. This exploratory study aims to describe surgical techniques and clinical outcomes in a series of patients who underwent surgical repair of SMCP at a single regional specialist cleft centre between 1999 and 2018. Through a retrospective case note review, records of 57 patients who underwent SMCP repair between the ages of 6 months and 16 years were examined. Patients underwent one of the three surgical techniques: Intravelar Veloplasty, Furlow or a novel technique we have termed as "Hemi-Furlow". Hypernasality, measured on the Great Ormond Street Speech Assessment, showed evidence of improvement post-operatively in all three surgical groups (P<0.005), with no evidence to favour any specific approach. Post-operative complications, including wound dehiscence and fistulae, occurred in nine patients (15.8%). Nine patients (15.8%) required revisional surgery, either due to post-operative complications or persistent speech problems. Otological disease was present in 54.4% of patients, comprising conductive hearing loss due to otitis media with effusion (52.6%) and sensorineural hearing loss (1.8%). There is now a need for large, multi-centre studies with robust outcomes measures to further examine relationships between surgical techniques and clinical outcomes in people born with SMCP.

Parent/Caregiver Views of the Effectiveness of Speech-Language Pathology for Children Born With Cleft Palate Delivered via Telemedicine During COVID-19.

PURPOSE: The purpose of this study was to describe and examine parent views of speech-language pathology (SLP) for children born with cleft palate delivered via telemedicine during the COVID-19 pandemic in the United Kingdom (UK). METHOD: Parents were asked whether they found this method of delivery "very effective," "somewhat effective," or "not at all effective." Free text was then invited. There were 212 responses. Ordinal chi-square, Kruskal-Wallis, or Fisher's exact tests examined associations between parent views of effectiveness and biological variables and socioeconomic status. Free text responses were analyzed using qualitative content analysis. RESULTS: One hundred and forty (66.0%) respondents reported that SLP delivered via telemedicine was "somewhat effective," 56 (26.4%) "very effective," and 16 (7.6%) "not at all effective." There was no evidence of an association between parent reported effectiveness and any of the explanatory variables. Parent-reported challenges impacting on effectiveness included technology issues and keeping their children engaged with sessions. Importantly, telemedicine was viewed as "better than nothing." CONCLUSIONS: Most parents reported that they felt SLP delivered via telemedicine during the first few months of the COVID-19 pandemic in the UK was at least "somewhat effective." It is important to interpret this in the context of there being no other method of service delivery during this time and that this study only represents families who were able to access SLP delivered via telemedicine. Further work is needed to identify which children with cleft palate might benefit from SLP delivered via telemedicine to inform postpandemic service provision.

Speech input processing in children born with cleft palate: A systematic literature review with narrative synthesis.

BACKGROUND: Speech development requires intact and adequately functioning oral anatomy and cognitive 'speech processing' skills. There is evidence that speech input processing skills are associated with speech output problems in children not born with a cleft. Children born with cleft palate ± lip (CP±L) are at high risk of developing disordered speech output. Less is known about their speech input processing skills and whether they are associated with cleft-related speech sound disorder (SSD). AIMS: (1) To collate and evaluate studies reporting evidence regarding the speech input processing skills of children born with cleft palate in comparison with data from typically developing children or other comparison groups; and (2) to identify any available evidence regarding relationships between speech input processing skills and speech output in children born with CP±L. METHODS & PROCEDURES: Potentially relevant studies published up to November 2019 were identified from the following databases: Medline via Ovid, Embase via Ovid, Cinahl via Ebscohost, PsycInfo via Ebscohost, BNI via ProQuest, AMED via Ovid, Cochrane Library and Scopus. Inclusion criteria were: peer-reviewed articles published in scientific journals, any design, published in English, participants born with a CP±L aged up to age 18 years who completed speech input processing assessments compared with normative data and/or a control or other comparison group. Critical Appraisal Skills Programme (CASP) checklists were used to quality appraise included studies. OUTCOMES & RESULTS: Six studies were retained in the final review. There is some evidence that children born with CP±L perform less well than non-cleft controls on some speech input processing tasks and that specific input processing skills may be related to errors in the children's speech. Heterogeneity in relation to study groups and assessments used, as well as small sample sizes, limits generalization of findings. CONCLUSION & IMPLICATIONS: There is limited evidence regarding the speech input processing skills of children born with CP±L. There are indications that children born with CP+/L may have difficulty in some aspects of speech input processing in comparison with children not born with a cleft, and that difficulties with some speech input processing tasks may be specific to errors in children's speech output. Further research is required to develop our understanding of these skills in this population and any associations with speech output. WHAT THIS PAPER ADDS: What is already known on the subject Few studies have been published that examine aspects of speech input processing in children born with CP±L. Theoretical models of speech processing, and published studies, propose that speech input processing skills are associated with SSD in children who were not born with a cleft. However, it is less clear whether there is any association between speech input processing and cleft-related SSD. What this paper adds to existing knowledge This review systematically collates and evaluates the published, peer-reviewed evidence regarding speech input processing skills in children born with CP±L. The collated evidence indicates that some speech input processing skills differ between children with and without CP±L. There is some evidence, from a single study, that speech input processing of specific cleft speech characteristics (CSCs) may be associated with the presence of these CSCs in the speech output of some children born with CP±L. What are the potential or actual clinical implications of this work? While the evidence is currently limited, increasing our knowledge of speech input processing skills in children born with CP±L contributes to our clinical understanding of the nature of cleft-related SSD. The current evidence suggests that speech and language therapists should consider speech input processing skills when assessing children with cleft-related SSD to support intervention planning. Considering these skills in relation to literacy development in these children may also be important.

The Cleft Multidisciplinary Collaborative: Establishing a Network to Support Cleft Lip and Palate Research in the United Kingdom.

BACKGROUND: As a growing paradigm of health research, trainee collaboratives can influence clinical practice through the generation of cost-effective multicenter audit and research projects. The aims of the present article are to outline and discuss the establishment of a multidisciplinary collaborative in the context of cleft lip and/or palate (CL/P). METHODS: The Cleft Multidisciplinary Collaborative (CMC) was formed in April 2016 under the overarching supervision of the National Institute for Health Research. Membership of the CMC is open to all members of the CL/P multidisciplinary team, who are encouraged to submit ideas for new research projects that will benefit clinical practice. RESULTS: To date, 48 clinical participants are involved in the CMC. These participants represent all 17 cleft teams from the United Kingdom and encompass a wide range of disciplines. The CMC has undertaken 2 major projects so far. The first involved collection of phenotype data to support a national cohort study. The second, still in progress, is a systematic review investigating factors associated with outcomes for velopharyngeal competence following cleft palate repair. CONCLUSIONS: The concept of a multidisciplinary collaborative in CL/P has been demonstrated through the generation of a United Kingdom-wide network of committed clinicians and researchers and the effective undertaking of 2 large research projects. As the CMC gathers momentum, it hopes to attract funding to support its activities, to promote more involvement from the allied health and nursing professions, to encourage a more ingrained research culture within the CL/P community, and to promote the wider ambition of a global collaborative.