Factors Associated with Delayed Palatoplasty Before, During, and After the COVID-19 Pandemic.

BACKGROUND: Cleft palatoplasty is typically performed around 10 to 12 months of age in the US, and delays can negatively affect speech development. Early during COVID-19, elective surgeries were canceled. The aims of this study were to (1) identify overall risk factors for greater age at palatoplasty and (2) analyze delays in palatoplasty during COVID-19. METHODS: This study was part of a larger prospective, multicenter comparative study of speech outcomes in palatoplasty. Participants underwent palatoplasty between March 2019 and September 2022 at 18 pediatric hospitals in the United States. Ages were corrected for prematurity. Dates of palatoplasty were divided into 4 periods corresponding to different phases of the pandemic. Factors analyzed included region, language, adoption status, sex, ethnicity, race, rurality, health insurance type, and cleft type. Analyses were performed using ANOVA, Student's test, and multivariable linear regression, with a P value of ≤0.05 being significant. RESULTS: Nine hundred twenty-eight participants were included. Average corrected age at palatoplasty was 374 days. In univariable analysis, palatoplasty was performed later in children who were Hispanic (P=0.003), of a race other than White, Black, or Asian (P<0.001), and without private insurance (P<0.001). On multivariable regression, predictors of delayed palatoplasty were Hispanic ethnicity (P=0.015), from other race (P<0.001), and without private insurance (P<0.001). During COVID-19, disproportionate delays occurred in patients who were female, of other races, from nonrural areas, and on Medicaid. CONCLUSIONS: Palatoplasty was performed later in vulnerable populations. Some of these populations were also disproportionately affected by COVID-19 delays. Providers should be aware of these differences as they pertain to equitable access to craniofacial care. LEVEL OF EVIDENCE: III.

Correspondence between consonant inventories obtained using modified naturalistic listening in real time (NLRT) and parent report.

OBJECTIVE: The current study examined the correspondence between consonant inventories obtained using the modified NLRT approach and parent report. DESIGN: Prospective comparative study. SETTING: Multisite institutional. PARTICIPANTS: Participants included 70 children with repaired CP + L (mean age = 16 months) who were participating in the multicenter study. PROCEDURES: Parents of participants were asked to record approximately two hours of their child's vocalizations/words at home using a Language ENvironmental Analysis (LENATM) recorder. Four ten-minute audio-recorded samples of vocalizations were extracted from the original recording for each participant and analyzed for size of consonant inventory. Parent reported consonant inventory was compared to coder identified consonant inventory. Coders identified an in-inventory consonant using two different criteria: 2+ tokens of each consonant were required in the first analysis and 10+ tokens of each consonant were required in the second analysis. RESULTS: Coder identified consonant inventory was larger (mean = 7.90) than that reported by parents (mean = 6.06) when a minimum of two tokens per consonant was required for inclusion of a consonant in inventory, while the inventory transcribed by coders was smaller (mean = 4.46) than that reported by parents when inclusion criteria required a minimum of ten tokens per consonant. CONCLUSIONS: Although the mean number of consonants in inventory between coders and parents was slightly closer using the 10+ versus 2+ criterion for consonant inclusion, the difference was not significant enough to recommend one protocol over the other.

Standardization of Videorecorded Speech Samples for Children with Cleft Palate: Methods to Facilitate High-Quality Speech Outcomes Data Collection.

BACKGROUND: Collection of high-quality videorecorded speech samples is essential for speech outcomes research. SOLUTION: Cleft palate team SLPs were trained to collect standard videorecorded speech samples in the clinic setting across 20 sites in North America. Standard training and equipment was provided. WHAT WE DID THAT IS NEW: Quality management procedures were developed and utilized to verify video quality and protocol adherence. Over 97% of speech samples collected by trained SLPs met defined quality standards.

Stop Consonant Production in Children with Cleft Palate After Palatoplasty.

The current study examined stop consonant production in children with cleft lip and/or palate (CP ± L) 2-6 months following palatal surgery.Prospective comparative study.Multisite institutional.Participants included 113 children with repaired CP ± L (mean age = 16 months) who were participating in the multicenter CORNET study.Parents of participants were asked to record approximately two hours of their child's vocalizations/words at home using a Language ENvironmental Analysis (LENATM) recorder. Four ten-minute audio-recorded samples of vocalizations were extracted from the original recording for each participant and analyzed for presence of oral stop consonants. A minimum of 100 vocalizations were required for analysis.Preliminary findings indicate that at least one oral stop was evident in the consonant inventory for 95 of the 113 children (84%) at the time of their post-surgery 16-month recording, and 80 of these children (71%) were producing two or more different stops. Approximately 50% of the children (57/113) produced the three voiced stops, and eight of the children (7%) were producing all six stop consonants.The findings of this study suggest that the majority of children with repaired CP ± L from English-speaking homes are producing oral stops within six months following palatal surgery. Similar to same-age children without CL ± P, voiced stops were more frequently evident in the children's inventories than voiceless stops. In contrast to findings of previous reports suggesting place of articulation differences, a somewhat comparable percentage of children in this study produced voiced bilabial, alveolar, and velar stops.

Transcribing toddler vocalizations: comparison of a modified naturalistic listening approach with phonetic transcription.

Previous investigations have demonstrated that naturalistic listening in real time (NLRT) can be used reliability to assess prelinguistic vocalizations and is less time-consuming than phonetic transcription. The current investigation was performed to examine the correspondence of syllable and consonant information obtained using a modified naturalistic listening in real time (NLRT) methodology compared to broad phonetic transcription for 20 toddlers with repaired cleft palate. A forty-minute vocalization sample was obtained for each toddler. Two listeners listened to each recording independently and coded the number of syllables and consonant types produced by each toddler using NLRT. Each recording was phonetically transcribed by each listener 2 to 16 months following the NLRT analysis. High reliability was evident between the modified NSRT method and phonetic transcription for identification of both syllables and consonants. Differences were evident in the actual number of syllables and consonants identified between the two types of assessment across the 20 participants. Possible explanations for those differences are addressed. The results of this investigation indicate that the modified NLRT procedure is a reliable method for determining consonant/inventories of young children. It is less labor intensive than traditional phonetic transcription and may be useful clinically when documentation of early sound development is needed to support early intervention decisions.

Cleft Palate Repair Postoperative Management: Current Practices in the United States.

OBJECTIVE: To describe current postoperative management practices following cleft palate repair. DESIGN: A survey was administered to cleft surgeons to collect information on their demographic characteristics, surgical training, surgical practice, and postoperative management preferences. SETTING: Eighteen tertiary referral hospitals across the United States.Participants: Surgeons (n = 67) performing primary cleft palate repair. RESULTS: Postoperative diet restrictions were imposed by 92% of surgeons; pureed foods were allowed at one week after surgery by 90% of surgeons; a regular diet was allowed at one month by 80% of surgeons. Elbow immobilizers and/or mittens were used by 85% of surgeons, for a median duration of two weeks. There was significant disagreement about postoperative use of bottles (61% allow), sippy cups (68% allow), pacifiers (29% allow), and antibiotics (45% prescribe). Surgeon specialty was not associated with any aspect of postoperative management (p > 0.05 for all comparisons). Surgeon years in practice, a measure of surgeon experience, was associated only with sippy cup use (p < 0.01). The hospital at which the surgeon practiced was associated with diet restrictions (p < 0.01), bottle use (p < 0.01), and use of elbow immobilizers or mittens (p < 0.01); however, many hospitals still had disagreement among their surgeons. CONCLUSIONS: Surgeons broadly agree on diet restrictions and the use of elbow immobilizers or mittens following palate repair. Almost all other aspects of postoperative management, including the type and duration of diet restriction as well as the duration of immobilizer use, are highly individualized.

Non-Oral Compensatory Misarticulations Revisited.

Non-oral compensatory misarticulation patterns are often associated with the speech of children with cleft palate. Despite their saliency, the etiology, frequency, and treatment of these misarticulations have not been studied extensively. The purpose of this commentary is to review what we know about these atypical patterns of articulation and address clinical assumptions regarding their etiology and treatment.

The Implications of Nasal Substitutions in the Early Phonology of Toddlers With Repaired Cleft Palate.

OBJECTIVE: To examine the implications of nasal substitutions in the early words of toddlers with cleft palate. DESIGN: Retrospective. PATIENTS: Thirty-four toddlers with nonsyndromic cleft palate and 20 noncleft toddlers, followed from ages 13 to 39 months. MAIN OUTCOME MEASURES: The groups were compared for the percentage of toddlers who produced nasal substitutions in their early words. The percentage of toddlers with repaired cleft palate who produced nasal substitutions and were later suspected of having velopharyngeal dysfunction (VPD) was also examined. RESULTS: Seventy-six percent of the toddlers in the cleft group (n = 26) and 35% of toddlers in the noncleft group (n = 7) produced nasal substitutions on one or more of their early words. Only 38% (10/26) of the toddlers with cleft palate who produced nasal substitutions in their early words were later diagnosed as having moderate-severe hypernasality and suspected VPD. CONCLUSIONS: The presence of nasal substitutions following palatal surgery was not always an early sign of VPD. These substitutions were present in the early lexicon of children with and without cleft palate.

Genetic Candidate Variants in Two Multigenerational Families with Childhood Apraxia of Speech.

Childhood apraxia of speech (CAS) is a severe and socially debilitating form of speech sound disorder with suspected genetic involvement, but the genetic etiology is not yet well understood. Very few known or putative causal genes have been identified to date, e.g., FOXP2 and BCL11A. Building a knowledge base of the genetic etiology of CAS will make it possible to identify infants at genetic risk and motivate the development of effective very early intervention programs. We investigated the genetic etiology of CAS in two large multigenerational families with familial CAS. Complementary genomic methods included Markov chain Monte Carlo linkage analysis, copy-number analysis, identity-by-descent sharing, and exome sequencing with variant filtering. No overlaps in regions with positive evidence of linkage between the two families were found. In one family, linkage analysis detected two chromosomal regions of interest, 5p15.1-p14.1, and 17p13.1-q11.1, inherited separately from the two founders. Single-point linkage analysis of selected variants identified CDH18 as a primary gene of interest and additionally, MYO10, NIPBL, GLP2R, NCOR1, FLCN, SMCR8, NEK8, and ANKRD12, possibly with additive effects. Linkage analysis in the second family detected five regions with LOD scores approaching the highest values possible in the family. A gene of interest was C4orf21 (ZGRF1) on 4q25-q28.2. Evidence for previously described causal copy-number variations and validated or suspected genes was not found. Results are consistent with a heterogeneous CAS etiology, as is expected in many neurogenic disorders. Future studies will investigate genome variants in these and other families with CAS.

The Americleft Speech Project: A Training and Reliability Study.

OBJECTIVE: To describe the results of two reliability studies and to assess the effect of training on interrater reliability scores. DESIGN: The first study (1) examined interrater and intrarater reliability scores (weighted and unweighted kappas) and (2) compared interrater reliability scores before and after training on the use of the Cleft Audit Protocol for Speech-Augmented (CAPS-A) with British English-speaking children. The second study examined interrater and intrarater reliability on a modified version of the CAPS-A (CAPS-A Americleft Modification) with American and Canadian English-speaking children. Finally, comparisons were made between the interrater and intrarater reliability scores obtained for Study 1 and Study 2. PARTICIPANTS: The participants were speech-language pathologists from the Americleft Speech Project. RESULTS: In Study 1, interrater reliability scores improved for 6 of the 13 parameters following training on the CAPS-A protocol. Comparison of the reliability results for the two studies indicated lower scores for Study 2 compared with Study 1. However, this appeared to be an artifact of the kappa statistic that occurred due to insufficient variability in the reliability samples for Study 2. When percent agreement scores were also calculated, the ratings appeared similar across Study 1 and Study 2. CONCLUSION: The findings of this study suggested that improvements in interrater reliability could be obtained following a program of systematic training. However, improvements were not uniform across all parameters. Acceptable levels of reliability were achieved for those parameters most important for evaluation of velopharyngeal function.

Early lexical characteristics of toddlers with cleft lip and palate.

Objective : To examine development of early expressive lexicons in toddlers with cleft palate to determine whether they differ from those of noncleft toddlers in terms of size and lexical selectivity. Design : Retrospective. Patients : A total of 37 toddlers with cleft palate and 22 noncleft toddlers. Main Outcome Measures : The groups were compared for size of expressive lexicon reported on the MacArthur Communicative Development Inventory and the percentage of words beginning with obstruents and sonorants produced in a language sample. Differences between groups in the percentage of word initial consonants correct on the language sample were also examined. Results : Although expressive vocabulary was comparable at 13 months of age for both groups, size of the lexicon for the cleft group was significantly smaller than that for the noncleft group at 21 and 27 months of age. Toddlers with cleft palate produced significantly more words beginning with sonorants and fewer words beginning with obstruents in their spontaneous speech samples. They were also less accurate when producing word initial obstruents compared with the noncleft group. Conclusions : Toddlers with cleft palate demonstrate a slower rate of lexical development compared with their noncleft peers. The preference that toddlers with cleft palate demonstrate for words beginning with sonorants could suggest they are selecting words that begin with consonants that are easier for them to produce. An alternative explanation might be that because these children are less accurate in the production of obstruent consonants, listeners may not always identify obstruents when they occur.

Cognitive and language issues associated with cleft lip and palate.

Language and cognitive performance of children with cleft palate with/without cleft lip (CP ± L) have received relatively little attention in the literature to date. The lack of attention directed toward these areas is probably attributed to two factors. First, the speech characteristics associated with velopharyngeal inadequacy are undoubtedly the most salient problems encountered by clinicians treating these children and thus have taken priority in research for many years. Second, early findings suggesting little difference between these children and their typically developing peers in language and cognition simply discouraged additional study. More recent investigations, however, have expanded the scope of study to include toddlers and adults and utilized alternative methodologies to study this population. In this article, we will summarize the literature and explore recent findings associated with language and cognition in children with CP ± L. In addition, we will also examine performance of children with velocardiofacial syndrome-a common syndrome associated with CP ± L.

An analysis of the frame-content theory in babble of 9-month-old babies with cleft lip and palate.

UNLABELLED: The aim of this study was to examine the consonant-vowel co-occurrence patterns predicted by the Frame-Content theory in 16 nine-month-old babies with unrepaired cleft palate (±cleft lip) and 16 age-matched non-cleft babies. Babble from these babies was phonetically transcribed and grouped according to the intrasyllabic predictions of the theory (labial-central, alveolar-front, and velar-back). Both groups demonstrated the three consonant-vowel co-occurrence patterns predicted by the Frame-Content theory. Other patterns not predicted by the Frame-Content theory emerged as strong patterns as well. LEARNING OUTCOMES: The reader will be able to: • Describe consonant-vowel co-occurrence patterns produced by babies with and without cleft palate. • Describe vowel inventories of babies with cleft palate. • Identify possible therapy targets for babies with cleft palate.

The relationship between early reading skills and speech and language performance in young children with cleft lip and palate.

OBJECTIVE: To examine the early reading skills of young children with cleft lip and palate and to examine the relationship between early reading skills and speech and language performance. PARTICIPANTS: A total of 56 children participated in the study: 28 children with cleft lip and palate and 28 noncleft children matched for age (mean age, 5 years 7 months), gender, and months of formal schooling. MAIN OUTCOME MEASURES: The two groups of children were compared (t tests) on the Test of Early Reading-3. Pearson product moment correlations were performed to examine separately the relationship between early reading skills and speech production abilities and between early reading skills and receptive and expressive language abilities for the two groups. RESULTS: Statistically significant group differences were noted on the Test of Early Reading-3. The mean score of 99 obtained by the group of children with cleft lip and palate was within normal limits compared with the norms for the Test of Early Reading-3; however, 14% of the children with cleft lip and palate scored outside the normal range on the Test of Early Reading-3. Statistically significant correlations were obtained between early reading skills and speech production abilities and between early reading skills and language abilities. CONCLUSIONS: Children with cleft lip and palate differed from noncleft peers in speech and early reading skills. Children with the most severe speech problems were the children with the poorest performance on the Test of Early Reading-3. Management of children with cleft lip and palate should include early identification of and intervention for delays in speech, language, and reading.

Speech-language characteristics of children with neurofibromatosis type 1.

Delays in speech and articulation development have been found in school-aged children and adolescents with neurofibromatosis type 1 (NF1). This report examines speech and language skills of preschool children with NF1. Nineteen 3- to 5-year-old children diagnosed with NF1 were assessed using measures of articulation (GFTA-2), and receptive and expressive language (CELF-P2). Significant differences were observed between mean scores obtained by the group of children with NF1 compared to the validated controls from the speech and language instruments (P < or = 0.009). Sixty-eight percent of the children exhibited delays in speech and/or language. Thirty-two percent demonstrated delays in articulation, 37% percent demonstrated delays in receptive language, and 37% exhibited delays in expressive language. Sixteen percent of the children exhibited a voice disorder and 42% were judged to have a resonance problem. No significant differences were observed on any of the measures of speech and language for children with non-familial versus familial NF1. Results of this study support the need for early assessment of speech and language problems for children diagnosed with NF1 and implementation of appropriate timely intervention as needed.

Timing of palatal surgery and speech outcome.

OBJECTIVE: To examine the impact of age and lexical status at the time of primary palatal surgery on speech outcome of preschoolers with cleft palate. PARTICIPANTS: Forty children (33 to 42 months) with nonsyndromic cleft palate participated in the study. Twenty children (Group 1) were less lexically advanced and younger (mean age = 11 months) and 20 children (Group 2) were more lexically advanced and older (mean age = 15 months) when palatal surgery was performed. MAIN OUTCOME MEASURES: Samples of the children's spontaneous speech were compared on 11 speech production measures (e.g., size of consonant inventory, total consonants correct, % correct for manner of articulation categories, compensatory articulation usage, etc.). Next, listeners rated a 30-second sample of each child's connected speech for articulation proficiency and hypernasality, separately, using direct magnitude estimation (DME). RESULTS: Group differences were noted for 4 of the 11 speech production measures. Children in Group 1 exhibited larger consonant inventories (and true consonant inventories) and more accurate production of nasals and liquids compared to children in Group 2. On the DME task, significant group differences were found for ratings of articulation proficiency and hypernasality. Children in Group 1 exhibited better articulation and less hypernasality than children in Group 2. CONCLUSIONS: The findings suggested that children who were less lexically advanced and younger at the time of palatal surgery exhibited better articulation and resonance outcomes at 3 years of age.

The impact of early intervention on speech and lexical development for toddlers with cleft palate: a retrospective look at outcome.

PURPOSE: The purpose of the present investigation was to examine the impact of early intervention on speech and lexical measures for toddlers with cleft palate. METHOD: Speech measures of ten 27-month-old toddlers with cleft palate who had been referred for therapy at 17 months of age were compared to those of 10 toddlers with cleft palate who had been referred but did not receive therapy. Both groups were compared to 2 other groups of children who had never been referred for therapy. RESULTS: Multivariate analysis of variance revealed only 1 significant difference between the children who received therapy and those who did not receive therapy. Children who received therapy produced a significantly greater percentage of glides (p < .001) than did children who did not receive therapy. CONCLUSION: The findings of this study indicated that early intervention as conducted by the speech-language pathologists in this study was not as effective as expected for this group of toddlers with cleft palate.

Conversational skills of children with cleft lip and palate: a replication and extension.

OBJECTIVE: To replicate and to extend a previous study examining the conversational skills of children with cleft lip and palate. PARTICIPANTS: Thirty-four children (33 to 44 months) participated: 17 children with cleft lip and palate and 17 noncleft children. METHODS: The children were observed during an interaction with caregivers in their homes. Samples of caregiver-child interactions were coded as assertive or responsive, for type of conversational act, and for discourse level categories. Profiles of conversational activity were determined for each child based on the coding. Correlations were performed to examine the relationship between assertiveness and speech variables (articulation and resonance) for the children with cleft lip and palate. RESULTS: Group comparisons revealed that the children with cleft lip and palate produced fewer assertive utterances, were less likely to respond adequately to comments by caregivers, and produced more topic maintaining and fewer topic extending utterances than did their noncleft peers during conversational interactions. Examination of individual child data indicated that 35% of the children with cleft lip and palate exhibited conversational profiles characterized by either low assertiveness or low responsiveness. Finally, a significant positive correlation was noted between conversational assertiveness and speech production skills. CONCLUSION: These findings suggested that the children with cleft lip and palate were less conversationally assertive than their noncleft peers. Further, there appeared to be a relationship between speech production skills and conversational skills, suggesting that poor speech may be impacting language performance for these children.

Is presurgery and early postsurgery performance related to speech and language outcomes at 3 years of age for children with cleft palate?

This study examined the relationship between presurgery speech measures and speech and language performance at 39 months as well as the relationship between early postsurgery speech measures and speech and language performance at 39 months of age. Fifteen children with cleft lip and palate participated in the study. Spontaneous speech samples were obtained in the children's homes at presurgery/ 9 months, postsurgery/13 months and again at 39 months of age. Correlational analyses revealed a lack of association between speech measures presurgery and early postsurgery and speech outcome measures at 39 months. Significant negative correlations were noted for true canonical babbling ratio presurgery and mean length of utterance (MLU) at 39 months, as well as for size of true consonant inventory presurgery, and both language outcome measures at 39 months (MLU and number of different words). Significant positive correlations were noted for percentage of true stops produced postsurgery and both language measures at 39 months. Variables that may have influenced speech and language development from presurgery to 39 months are discussed.

The impact of cleft type on early vocal development in babies with cleft palate.

OBJECTIVE: The purpose of the present investigation was to examine the impact of cleft type on early sound development in children with cleft palate (with or without cleft lip). PARTICIPANTS: The participants included 53 babies with unrepaired cleft palate aged 8 to 10 months. Thirty-five babies had cleft lip and palate (CLP) and 18 had cleft palate only (CPO). MAIN OUTCOME MEASURES: Spontaneous vocalizations of the CLP and CPO groups were compared to determine whether differences were evident in canonical babbling, size of consonant inventory as well as place and manner of consonant production, and frequency of vocalization. RESULTS: Student's t tests revealed no significant differences between the groups in canonical babbling, size of consonant inventory, place and manner of consonant production, or frequency of vocalization. However, there was a trend for babies in the CPO group to produce fewer compensatory stop consonants and more anterior place features. In addition, a larger percentage of children in the CPO group had entered the canonical babbling stage at the time of this study. CONCLUSIONS: In general, the findings of this study suggested that cleft type does not appear to play a significant role in early consonant development for babies with cleft palate. Additional study using a larger CPO group is recommended to further investigate the trend toward better performance noted in this group.