Can MRI Replace Nasopharyngoscopy in the Evaluation of Velopharyngeal Insufficiency?

OBJECTIVE: To investigate whether flexible nasopharyngoscopy, when performed in addition to magnetic resonance imaging (MRI), influences the type of surgery selected or success of surgery in patients with velopharyngeal insufficiency (VPI). DESIGN: Cohort study. SETTING: A metropolitan children's hospital. PATIENTS: Patients with non-syndromic, repaired cleft palate presenting for management of VPI. INTERVENTIONS: MRI and nasopharyngoscopy or MRI alone for preoperative imaging of the velopharyngeal mechanism. MAIN OUTCOME MEASURES: (1) Surgical selection and (2) resolution of hypernasality. All speech, MRI, and nasopharyngoscopy measurements were performed by raters blinded to patients' medical and surgical history. RESULTS: Of the 25 patients referred for nasopharyngoscopy, 76% completed the exam. Of the 41 patients referred for MRI, the scan was successfully completed by 98% of patients. Completion of nasopharyngoscopy was significantly (p=0.01) lower than MRI. Surgical selection did not significantly differ (p=0.73) between the group receiving MRI and nasopharyngoscopy and the group receiving MRI alone, nor was there a significant difference between these groups in the proportion of patients achieving resolution of hypernasality postoperatively (p=0.63). Percent total velopharyngeal closure assessments on nasopharyngoscopy and MRI were strongly correlated (r=0.73). CONCLUSIONS: In patients receiving MRI as part of their preoperative VPI evaluation, the addition of nasopharyngoscopy did not result in a difference in surgical selection or resolution of hypernasality. Routine inclusion of nasopharyngoscopy may not be necessary for the evaluation of velopharyngeal anatomy when MRI is available.

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.

Preoperative Velopharyngeal Closure Predicts Hypernasality Outcomes of Secondary Furlow Double-Opposing Z-Plasty.

OBJECTIVE: To determine if preoperative velopharyngeal closure percentage (VCP) is predictive of successful Furlow double opposing Z-plasty (DOZP) and subsequently determine the optimal velopharyngeal closure cutoff for successful DOZP. DESIGN: Retrospective study. SETTING: Tertiary academic center. PATIENTS: 110 patients with repaired cleft lip and palate having hypernasality treated with DOZP. INTERVENTIONS: Speech videofluoroscopy images were used to obtain the preoperative VCP and other measurements. MAIN OUTCOME MEASURES: Changes in hypernasality scores using the Cleft Audit Protocol for Speech-Augmented-Americleft Modification (CAPS-A-AM) rating system were used as the primary outcome measure. A successful DOZP was defined as a postoperative hypernasality score of ≤ 1 or an improvement of 2 or more scores from baseline. A receiver operating characteristic (ROC) curve was calculated to determine preoperative VCP cutoff. RESULTS: There were 110 patients who underwent DOZP for treatment of velopharyngeal insufficiency. Of these patients, 94 (85%) had successful surgery as determined by their postoperative CAPS-A-AM hypernasality score. Preoperative VCP was a statistically significant predictor of successful DOZP (P < .0001). The ROC curve with Youden index (J) determined a cutoff (c*) of 55% preoperative VCP or greater to optimize surgical success rate. Grouping by preoperative VCP showed that surgical success increases directly with preoperative VCP, and patients with low VCP had above a 50% success rate in reducing hypernasality scores. CONCLUSIONS: Preoperative VCP was significantly associated with improved hypernasality ratings postoperatively. A preoperative VCP of ≥55% may be used to help predict success of Furlow palatoplasty treatment. Patients with lower VCP can still benefit from secondary DOZP.

Rehabilitation of Combined Hard and Soft Palate Defects with a Soft Relining Prosthesis: A Clinical Report.

It remains a significant challenge in prosthetic rehabilitation for combined hard and soft palate defects on account of two primary reasons. At first, conventional impressions can hardly get an accurate analogue and usually bring about a terrible experience for the patients. Secondly, conventional hard denture base resins used in obturator prostheses exhibit limitations in marginal sealing, undercut retention, and elastic buffering when in contact with the soft palate. This article presents a case where combined hard and soft palate defects were successfully and rapidly reconstructed by using digital intraoral impression technology and denture soft reline material.

Do Palatoplasty Procedures Resolve Hypernasality as Effectively as Pharyngoplasty Procedures in Patients with 22q11.2 Deletion Syndrome?

OBJECTIVE: Compare the effectiveness of palatoplasty and pharyngoplasty procedures at resolving hypernasality in patients with 22q11.2 deletion syndrome (22q). DESIGN: Retrospective cohort study. SETTING: Metropolitan children's hospital. PATIENTS: Fourteen patients with 22q presenting for management of velopharyngeal insufficiency. INTERVENTIONS: Palatoplasty or pharyngoplasty procedure. MAIN OUTCOME MEASURE: Resolution of hypernasality 12 months postoperatively. RESULTS: Both procedure groups had a mean preoperative velopharyngeal gap of 6.2 mm during phonation. No patient who underwent palatoplasty achieved resolution of hypernasality; 1/7 patients had worse hypernasality, 4/7 had no change, and 2/7 had improved hypernasality. In contrast, hypernasality was resolved in 6/7 patients in the pharyngoplasty group, which was significantly (P = .03) higher than the palatoplasty group. CONCLUSIONS: In patients with 22q, palatoplasty procedures may be less effective than pharyngoplasty procedures at resolving hypernasality. This may be due to underlying anatomic or physiologic differences, such as increased pharyngeal depth and hypodynamic muscles.

Perceptions of employability related to severity of hypernasality: a pilot study.

The primary aim of this investigation was to evaluate listener auditory-perceptual assessment of employability for individuals with hypernasal speech. Using an online survey platform, listeners with managerial experience evaluated speech samples from individuals with varying hypernasal resonance disorder severity to determine auditory-perceptual judgements regarding intelligence and employability. Speech samples of individuals with hypernasal speech were rated lower on scales of intelligence and employability, and more likely to be selected for jobs with infrequent rates of communication and lower levels of responsibility. Additionally, males with hypernasal speech were perceived as less intelligent, less employable, and more likely to be selected for a job with infrequent communication in comparison to females with hypernasal speech. Results of this preliminary investigation suggest that individuals with hypernasal speech may face employment barriers. The conclusions collected from this initial investigation open the doors for further research addressing linguistic considerations and aspects of employability. This is an important consideration for individuals with either acquired or congenitally related hypernasal resonance disorder.

Effect of adenoid size on the post-adenoidectomy hypernasality in children with a normal palate.

PURPOSE: Adenoidectomy, either alone or with tonsillectomy, is a common surgical procedure in the field of pediatric otorhinolaryngology. Resonance function may be altered postoperatively in the form of hypernasality, which is usually transient. This study aimed to investigate the effect of adenoid size on post-adenoidectomy hypernasality in children with a normal palate. METHODS: Seventy-one children with different degrees of adenoid hypertrophy were included in this prospective observational study. Endoscopic assessment of the adenoid size and preoperative and postoperative evaluation of speech (at 1 and 3 months) with auditory perceptual assessment (APA) and nasometry were performed. RESULTS: APA showed preoperative hyponasality in 59.1% of children and was found to be significantly related to the adenoid size, with more hyponasality in grades 3 and 4. One month postoperatively, hypernasality was detected in 26.7% of patients and was found to be related to the preoperative adenoid size with higher hypernasality in grades 3 and 4. Three months postoperatively, all patients had gained normal nasality except one (1.4%) who was subjected to a longer follow-up period. Nasometric assessment showed significant differences at the three visits (pre, 1, and 3 months postoperatively), with a negative correlation between the grade of adenoid size and nasalance scores preoperatively and a significant positive correlation between them at 1 month postoperatively. However, no significant correlation was detected at 3 months postoperatively. CONCLUSION: Transient hypernasality may develop in some patients after adenoidectomy, especially in children with a larger preoperative adenoid size. However, transient hypernasality generally resolves spontaneously within 3 months.

Assessing the Agreement of Hypernasality and Audible Nasal Emission Ratings Between Audio-Recordings and a Clinic Setting.

Assess agreement of hypernasality and audible nasal emission (ANE) ratings between audio-recordings and a clinic setting.Cross-sectional study using retrospective clinical recordings.Audio-recording ratings by two trained speech language pathologists.Percent agreement and intra- and inter-rater reliability of perceptual ratings.Intra-rater reliability (AC2) of 167 audio-recorded speech samples for the primary and secondary raters, respectively, was 0.82 and 0.79 for hypernasality; for ANE, it was 0.57 and 0.75. Inter-rater reliability was 0.77 for hypernasality and 0.63 for ANE. When comparing ratings made from audio-recording versus the original clinical ratings, intra-rater reliability was 0.85 and 0.61 (primary and secondary rater, respectively) for hypernasality and 0.21 and 0.34 for ANE.Ratings for hypernasality made from audio recordings were consistent with clinical evaluation, while ratings of ANE were not. ANE ratings made from audio recordings may not be a valid measure of velopharyngeal insufficiency speech characteristics.

Relationship Between Auditory-Perceptual and Objective Measures of Resonance in Children with Cleft Palate: Effects of Intelligibility and Dysphonia.

To investigate the relationship between auditory-perceptual ratings of resonance and nasometry scores in children with cleft palate. Factors which may impact this relationship were examined including articulation, intelligibility, dysphonia, sex, and cleft-related diagnosis.Retrospective, observational cohort study.Outpatient pediatric cranio-facial anomalies clinic.Four hundred patients <18 years of age identified with CP ± L, seen for auditory-perceptual and nasometry evaluations of hypernasality as well as assessments of articulation and voice.Relationship between auditory-perceptual ratings of resonance and nasometry scores.Pearson's correlations indicated that auditory-perceptual resonance ratings and nasometry scores were significantly correlated across oral-sound stimuli on the picture-cued portion of the MacKay-Kummer SNAP-R Test (r values .69 to.72) and the zoo reading passage (r = .72). Linear regression indicated that intelligibility (p ≤ .001) and dysphonia (p = .009) significantly impacted the relationship between perceptual and objective assessments of resonance on the Zoo passage. Moderation analyses indicated that the relationship between auditory-perceptual and nasometry values weakened as severity of speech intelligibility increased (P < .001) and when children presented with moderate dysphonia (p ≤ .001). No significant impact of articulation testing or sex were observed.Speech intelligibility and dysphonia alter the relationship between auditory-perceptual and nasometry assessments of hypernasality in children with cleft palate. SLPs should be aware of potential sources of auditory-perceptual bias and shortcomings of the Nasometer when following patients with limited intelligibility or moderate dysphonia. Future study may identify the mechanisms by which intelligibility and dysphonia affect auditory-perceptual and nasometry evaluations.

Double-opposing Z-Plasty Extended with a Pedicled Buccal Fat Pad Flap for Correcting Velopharyngeal Insufficiency after Primary Palatoplasty.

INTRODUCTION: Furlow double-opposing Z-plasty (DOZ) lengthens the soft palate; however, this lengthening is achieved at the expense of increased mucosal flap tension. Thus, its use is limited in patients with severe tension applied on mucosal flap after DOZ. In this study, DOZ was combined with a buccal fat pad (BFP) flap to maximize palatal lengthening and muscle repositioning. METHODS: This study included patients who underwent surgical correction for velopharyngeal insufficiency between December 2016 and February 2019. Patients with more than moderate degree hypernasality following primary palatoplasty were included in the study. Patients younger than 4 years of age, those with a submucous cleft palate, or syndromic patients were excluded. Speech outcomes were investigated for those who underwent DOZ only (DOZ group, n = 17) and those in whom a BFP was used (BFP group, n = 15) pre- and postoperatively. The velopharyngeal gaps between the uvula and pharyngeal wall were measured before and immediately after surgery to estimate the palatal length. RESULTS: Most patients who received a BFP showed improvement in hypernasality. However, the hypernasality of the DOZ group was more severe than that of the BFP group (p = 0.023). The extent of palatal lengthening was 4.4 ± 1.7 mm and 7.5 ± 2.1 mm in the DOZ and BFP groups, respectively (p = 0.001). CONCLUSIONS: BFPs reduced the tension of the DOZ mucosal flap and maximized palatal lengthening and muscle repositioning. They promoted velopharyngeal closure in patients with moderate and moderate-to-severe velopharyngeal insufficiency. Hence, our method improves the surgical outcomes of patients with velopharyngeal insufficiency after primary palatoplasty.

Evaluation of Velopharyngeal Closure Function With 4-Dimensional Computed Tomography and Assessment of Radiation Exposure in Pediatric Patients: A Cross-Sectional Study.

OBJECTIVE: Some patients with cleft palate (CP) need secondary surgery to improve functionality. Although 4-dimensional assessment of velopharyngeal closure function (VPF) in patients with CP using computed tomography (CT) has been existed, the knowledge about quantitative evaluation and radiation exposure dose is limited. We performed a qualitative and quantitative assessment of VPF using CT and estimated the exposure doses. DESIGN: Cross-sectional. SETTING: Computed tomography images from 5 preoperative patients with submucous CP (SMCP) and 10 postoperative patients with a history of CP (8 boys and 7 girls, aged 4-7 years) were evaluated. PATIENTS: Five patients had undergone primary surgery for SMCP; 10 received secondary surgery for hypernasality. MAIN OUTCOME MEASURES: The presence of velopharyngeal insufficiency (VPI), patterns of velopharyngeal closure (VPC), and cross-sectional area (CSA) of VPI was evaluated via CT findings. Organ-absorbed radiation doses were estimated in 5 of 15 patients. The differences between cleft type and VPI, VPC patterns, and CSA of VPI were evaluated. RESULTS: All patients had VPI. The VPC patterns (SMCP/CP) were evaluated as coronal (1/4), sagittal (0/1), circular (1/2), and circular with Passavant's ridge (2/2); 2 patients (1/1) were unevaluable because of poor VPF. The CSA of VPI was statistically larger in the SMCP group (P = .0027). The organ-absorbed radiation doses were relatively lower than those previously reported. CONCLUSIONS: Four-dimensional CT can provide the detailed findings of VPF that are not possible with conventional CT, and the exposure dose was considered medically acceptable.

Comparing the Temporal Aspects of Velopharyngeal Closure in Children with and without Cleft Palate.

INTRODUCTION: Children with cleft palate exhibit differences in the 4 temporal components of nasalization (nasal onset and offset intervals, nasal consonant duration, and total speech duration), with various patterns having been noted based on different languages. Thus, the current study aimed to examine the temporal aspects of velopharyngeal closure in children with and without cleft palate; this is the first study to do so in the Turkish language. METHODS: This study evaluated and compared the 4 temporal characteristics of velopharyngeal closure in children (aged 6-10 years) with (n = 28) and without (n = 28) cleft palate using nonword consonant and vowel speech samples, including the bilabial nasal-to-stop combination /mp/ and the velar nasal-to-stop combination /ηk/. Acoustic data were recorded using a nasometer, after which acoustic waveforms were examined to determine the 4 temporal components of nasalization. Flexible nasoendoscopy was then used to evaluate velopharyngeal closure patterns. RESULTS: With regard to the 4 closure patterns, significant differences in the nasal offset interval (F4-25 = 10.213, p = 0.04; p < 0.05) and the nasal consonant duration ratio (F4-25 = 12.987, p = 0.02; p < 0.05) were observed for only /ampa/. The coronal closure pattern showed the longest closure duration (0.74 s). Children with cleft palate showed prolonged temporal parameters in all 4 characteristics, reflecting oral-nasal resonance imbalances. In particular, the low vowel sound /a/ was significantly more prolonged than the high vowel sounds /i/ and /u/. CONCLUSIONS: The examined temporal parameters offer more accurate characterizations of velopharygeal closure, thereby allowing more accurate clinical assessments and more appropriate treatment procedures. Children with cleft palate showed longer nasalization durations compared to those without the same. Thus, the degree of hypernasality in children with cleft palate may affect the temporal aspects of nasalization.

Evaluation of noise excitation as a method for detection of hypernasality.

Hypernasality is a disorder where excess nasal resonance is perceived during speech, often as a result of abnormal coupling between the oral and nasal tracts known as velopharyngeal insufficiency (VPI). The most common cause of VPI is a cleft palate, which affects around 1 in 1650 babies, around ⅓ of whom have persistent speech problems after surgery. Current equipment-based assessment methods are invasive and require expert knowledge, and perceptual assessment methods are limited by the availability of expert listeners and differing interpretations of assessment scales. Spectral analysis of hypernasality within the academic community has resulted in potentially useful spectral indicators, but these are highly variable, vowel specific, and not commonly used within clinical practice. Previous works by others have developed noise excitation technologies for the measurement of oral tract transfer functions using resonance measurement devices (RMD). These techniques provide an opportunity to investigate the structural system abnormalities which lead to hypernasality, without the need for invasive measurement equipment. Thus, the work presented in this study adapts these techniques for the detection of hypernasality. These adaptations include augmentation of the hardware and development of the software, so as to be suitable for transfer function measurement at the nostrils rather than the mouth (nRMD). The new method was tested with a single participant trained in hypernasal production, producing 'normal' and hypernasal vowels, and the recordings validated through a listening test by an expert listener and calculation of nasalance values using a nasality microphone. These validation stages indicated the reliability of the captured data, and analysis of the nRMD measurements indicated the presence of a systematic difference in the frequency range 2 to 2.5 kHz between normal and hypernasal speech. Further investigation is warranted to determine the generalisability of these findings across speakers, and to investigate the origins of differences manifesting in the transfer functions between conditions. This will provide new insights into the effects of nasal tract coupling on voice acoustics, which could in turn lead to the development of useful new tools to support clinicians in their work with hypernasality.

Furlow Palatoplasty: Should We Also Focus on the Size of the Nasopharynx?

OBJECTIVES: (1) To determine the incidence rate of velopharyngeal dysfunction (VPD) according to 7 speech criteria post-Furlow palatoplasty. (2) To find an anatomical measurement of the cleft palate (or combination of measurements) associated with the occurrence of VPD. STUDY DESIGN: Retrospective cohort study. PARTICIPANTS AND METHODS: Fifty-six patients with cleft palate ± cleft lip underwent palatoplasty with the Furlow technique at the age of 10 months. Pre-and post-palatoplasty cleft palate measurements were collected during the procedure. Three blinded speech-language pathologists (SLPs) retrospectively scored the patients from the chart data at age 4. Student t test and receiver operating characteristic curve analysis were used to evaluate the association and predictive capacity between cleft measurements and parameters (M&P) with all VPD criteria. RESULTS: The SLPs found an incidence of VPD according to 7 criteria: hypernasality (11%), audible nasal emission (4%), nasal rustle (14%), compensatory errors (4%), impairment of speech understandability (7%), and impairment of speech acceptability (16%). The SLPs recommended a secondary surgical procedure in 5 patients (9%). A statistically significant association was found between, respectively, 17 and 5 M&P and the occurrence of compensatory errors and audible nasal emission. Our data suggest that the length of the cleft, the cleft area, and the postoperative transversal size of the nasopharynx are the best indicators of the future positivity of VPD criteria. CONCLUSION: The size of the postoperative transverse nasopharyngeal area during the primary cleft palate procedure may become the focus of the next generation of cleft surgeons to reduce the incidence of VPD.

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.

Nasalance-Based Preclassification of Oral-Nasal Balance Disorders Results in Higher Agreement of Expert Listeners' Auditory-Perceptual Assessments: Results of a Retrospective Listening Study.

OBJECTIVE: Reliable perceptual and instrumental assessment of oral-nasal balance disorders is a persistent problem in speech-language pathology. The goal of the study was to evaluate whether nasalance-based preclassification of oral-nasal balance disorders improves listener agreement. DESIGN: Retrospective listening study. SETTING: Tertiary university hospital. PARTICIPANTS: Fifty-four randomly selected recordings of patients with repaired unilateral cleft lip and palate (UCLP). Three experienced speech-language pathologists participated as expert listeners. INTERVENTIONS: Two listening experiments were based on nasalance scores and audio recordings of speakers with repaired UCLP. The speakers were preclassified as normal, hypernasal, hyponasal, or mixed based on their nasalance scores. Initially, the listeners determined the diagnostic category of the oral-nasal balance for 62 audio recordings (8 repeats). Six months later, they listened to 38 of the recordings (6 repeats) along with a spreadsheet indicating the nasalance-based categories for the oral-nasal balance. The listeners confirmed, or rejected and corrected, the nasalance-based preclassification. MAIN OUTCOME MEASURES: Intralistener, interlistener agreement, and agreement between listener categories and nasalance-based oral-nasal balance categories. RESULTS: In the first study, the agreement between the listeners' diagnostic category and the nasalance-based category was 45.1% and the interlistener agreement was 36.7%. In the second study, the agreement between the listeners' category and the nasalance-based category was 67.1% (75% agreement for the correct nasalance-based categories and 41.7% for the misclassifications), and the interlistener agreement was 85.4%. CONCLUSIONS: Preclassification of oral-nasal balance disorders based on nasalance scores may help listeners achieve better diagnostic accuracy and higher agreement.

Speech-language therapy students' auditory-perceptual judgements of simulated concurrent hypernasality and articulation disorders.

Auditory-perceptual judgements are regarded as the standard method for assessing speech disorders. However, the results of auditory-perceptual evaluations and rater reliability can be affected by various factors, such as concurrent problems in multiple speech subsystems. This study investigated the effect of a co-occurring articulation disorder on auditory-perceptual judgements of hypernasality and the effect of co-occurring hypernasality on judgements of an articulation disorder. The speech stimuli were sentences produced by a male speaker who simulated four levels of hypernasality (typical nasality, and mild, moderate, and severe hypernasality) at four levels of disordered articulation (typical articulation, and mild, moderate, and severe articulation disorder). Thirty speech and language therapy students used visual analogue scales to rate the severity of hypernasality and articulation disorder for each speech sample. Results showed that the hypernasality ratings were significantly higher when articulation disorder co-occurred compared to those without. However, there was no significant difference between mild, moderate and severe concurrent articulation disorder on hypernasality ratings. The speech samples with typical articulation and those with severe articulation disorder were rated as more severe in terms of articulation problem when combined with severe hypernasality. However, there was no significant hypernasality effect on articulation ratings for speech with mild or moderate articulation disorder. The present results generally agreed with previous findings regarding the effect of co-occurring speech problems on auditory-perceptual judgements. Clinicians are advised to be cautious of the potential impact. If possible, speech evaluation using instrumental techniques should be used to supplement auditory-perceptual judgements.

Palatal evaluation and treatment in 22q11.2 deletion syndrome.

Palatal involvement occurs commonly in patients with 22q11.2 Deletion Syndrome (22qDS), and includes palatal clefting and velopharyngeal dysfunction in the absence of overt or submucous clefts. The reported incidence and distribution of palatal abnormalities vary in the literature. The aim of this article is to revisit the incidence and presenting features of palatal abnormalities in a large cohort of patients with 22qDS, summarize the surgical treatments performed in this cohort, and provide an overview of surgical treatment protocols and management guidelines for palatal abnormalities in this syndrome. Charts of 1,121 patients seen through the 22q and You Center at the Children's Hospital of Philadelphia were reviewed for palatal status, demographic factors, deletion size, and corrective surgical procedures. Statistical analysis was performed using Pearson's chi-squared test to identify differences between gender, deletion size, and palatal abnormality. Of the patients with complete evaluations, 67% were found to have a palatal abnormality. The most common finding was velopharyngeal dysfunction in 55.2% of patients, and in 33.3% of patients, this occurred in the absence of palatal clefting. There was no significant difference in the incidence of palatal abnormalities by gender; however, a difference was noted among race (p < 0.01) and deletion sizes (p < 0.01). For example, Caucasian and Asian patients presented with a much higher prevalence of palatal abnormalities, and conversely those with nested deletions presented with a much lower rate of palatal defects. Overall, 26.9% of patients underwent palatal surgery, and the most common indication was velopharyngeal dysfunction. Palatal abnormalities are a hallmark feature of 22q11.2 Deletion Syndrome; understanding the incidence, presenting features, and treatment protocols are essential for practitioners counseling and treating families affected with this disorder.

Outcomes following TORS for HPV-positive oropharyngeal carcinoma: PEGs, tracheostomies, and beyond.

OBJECTIVES: To review swallowing, airway and speech outcomes following transoral robotic surgery (TORS) ±â€¯adjuvant therapy for human papillomavirus associated oropharyngeal squamous cell carcinoma (HPV[+]OPSCC). METHODS: Patients underwent TORS ± standard adjuvant therapy from 5/1/2007-5/31/2015. Clinical data were recorded and descriptive analysis was performed. RESULTS: 267 patients met criteria. All patients underwent surgery at Mayo, however, only 41/81 and 71/119 patients received RT and CRT at a Mayo Clinic site. A PEG was placed in 77 patients (3 prior to any treatment, 74 reactively during adjuvant therapy), with 3 PEG dependent and 3 partially PEG reliant at last follow-up. Tracheostomy was performed in 30 (11%) patients; 28 were decannulated. Swallow evaluations were completed for 20/81 undergoing RT and 50/119 undergoing CRT at a median of 3.8 and 7.6 months post-treatment, respectively. An unrestricted oral diet was reported by 5% following RT and 12% following CRT on the Functional Oral Intake Scale. HN-PSS normalcy of diet scores indicated a diet beyond soft chewable foods for 27% following RT and 46% following CRT. No restriction of place, food, or companion was reported for the HN-PSS for public eating in 13% after RT and 33% after CRT. Aspiration of thin liquid was present in 17% and 28% following RT and CRT, respectively. HN-PSS understandability of speech was "always understandable" in 60% and 63%, following RT and CRT, respectively. Hoarseness was reported in 56% and 45% following RT and CRT respectively. CONCLUSION: Long-term PEG and tracheostomy dependence in this cohort is low. However, these outcomes under-represent the decrement in patient speech and swallowing following TORS ± standard adjuvant therapy for HPV(+)OPSCC.

Associations Between Hypernasality, Intelligibility, and Language and Reading Skills in 10-Year-Old Children With a Palatal Cleft.

BACKGROUND: This study investigated the associations between hypernasality and intelligibility, and language and reading skills in 10-year old children with a cleft palate ± lip. DESIGN: Cross-sectional data collected during routine assessments of speech and language in a centralized treatment setting. PARTICIPANTS: Children aged 10, born with cleft palate ± lip from 4 birth cohorts (N = 123). OUTCOME MEASURES: Hypernasality and intelligibility: Swedish Articulation and Nasality Test-N; language: Language 6-16 (Sentence recall, Serial recall, Vocabulary); reading: word chain test and reading comprehension test. RESULTS: A total of 71.3% of the children had no occurrence of hypernasality and 82.8% had intelligibility scores within the normal range. For all children with hypernasality and intelligibility within the normal range, reading and language scores were also within normal ranges. Children with presence of hypernasality had significantly lower language skills, with mean scores within the lower normal range. Children with reduced intelligibility had lower scores on reading comprehension. CONCLUSIONS: The findings highlight a possible association between hypernasality and language skills, and intelligibility and reading skills. Cleft teams should consider routine assessments of language and reading skills in children with speech impairment, in order to identify potential needs for intervention as early as possible.