Velopharyngeal dysfunction and speech-related characteristics in craniofacial microsomia: a retrospective analysis of 223 patients.

This study aimed to document the prevalence, severity, and risk factors of velopharyngeal dysfunction (VPD) in craniofacial microsomia (CFM) and to analyse differences in VPD-related speech characteristics between CFM patients without cleft lip and/or palate (CL/P), CFM patients with CL/P, and CL/P patients without CFM (control). A total of 223 patients with CFM were included, of whom 59 had a CL/P. Thirty-four CFM patients had VPD, including 20 with a CL/P. VPD was significantly more prevalent in CFM with CL/P than in CFM without CL/P (odds ratio (OR) 4.1, 95% confidence interval (CI) 1.9-8.7; P < 0.001). Multivariate logistic regression showed a significant association between CL/P and VPD in CFM patients (OR 7.4, 95% CI 2.1-26.3; P = 0.002). The presence of VPD was not associated with sex, the laterality or severity of CFM. Speech problems related to VPD appeared to be similar among the different groups (CFM without CL/P, CFM with CL/P, CL/P without CFM). As 15.2% of all CFM patients and 8.5% of CFM patients without CL/P had VPD, it is proposed that all patients with CFM, with or without CL/P, should be assessed by a speech and language therapist for the potential risk of VPD.

Awake Craniotomy for Subcortical Brain Metastasis Beneath the Speech Center: A Technical Case Report.

BACKGROUND: To preserve language function, intraoperative functional brain mapping (IFBM) in and near the speech center is essential. CASE REPORT: We present a case of a 73-year-old right-handed woman with colon cancer. She presented with mild speech disturbance. Magnetic resonance imaging revealed a ringed enhancing lesion in the frontal operculum. The preservation of language function was critical; therefore, she underwent awake craniotomy using IFBM. Thus, the speech site was elicited by cortical electrical stimulation at the surface, near the location of the tumor. We made a safe corticotomy on the surface of the lesion and performed the resection of brain metastasis (BM) via a safety corridor. We achieved gross total resection of the BM while preserving the language function. After surgery, she recovered from speech disturbance. She returned to her normal life with improved language function. CONCLUSION: IFBM is a useful tool to undertake a safe approach via the speech center, avoiding permanent language deficits.

Speech and swallowing deficits in X-Linked Dystonia-Parkinsonism.

INTRODUCTION: X-linked Dystonia-Parkinsonism (XDP) is a progressive, disabling disease characterized by the devastating impairment of bulbar function, including speech and swallowing. Despite these detrimental impacts, bulbar impairments in this population are not well characterized. OBJECTIVES: To identify impairments in the bulbar system measured by oromotor performance in individuals with XDP relative to healthy controls. Secondarily, to detect diagnostic bulbar markers that are sensitive and specific to the initial years of XDP. METHODS: This case-control study included 25 healthy controls and 30 participants with XDP, divided into two subgroups based on the median of their disease length. Multiple clinical and instrumental oromotor tasks and measures were used to evaluate bulbar motor function. RESULTS: Differences were found between both the subgroups with XDP and healthy controls on almost all measures, including maximum performance tasks such as tongue strength, alternating motion rate (AMR), and sequential motion rate (SMR) (p < 0.05). Differences were found between the XDP subgroups and the control group for the percentage of pause time during the speech, a rating of speech severity, and a swallowing task (ps < 0.05). Scores on self-reported questionnaires, tongue strength, the number of repetitions produced during an AMR, percent pause, and speech severity demonstrated good sensitivity and specificity to differentiate the initial years of XDP onset from healthy controls. CONCLUSIONS: Our findings revealed impairments across bulbar functions in participants within the first 7 years of the XDP onset. Highly sensitive and specific bulbar impairment measures were detected in instrumental and self-reported measures that are fundamental for monitoring disease.

Functional maps of direct electrical stimulation-induced speech arrest and anomia: a multicentre retrospective study.

Direct electrical stimulation, the transient 'lesional' method probing brain function, has been utilized in identifying the language cortex and preserving language function during epilepsy and neuro-oncological surgeries for about a century. However, comparison of functional maps of the language cortex across languages/continents based on cortical stimulation remains unclear. We conducted a retrospective multicentre study including four cohorts of direct electrical stimulation mapping from four centres across three continents, where three indigenous languages (English, French and Mandarin) are spoken. All subjects performed the two most common language tasks: number counting and picture naming during stimulation. All language sites were recorded and normalized to the same brain template. Next, Spearman's correlation analysis was performed to explore the consistency of the distributions of the language cortex across centres, a kernel density estimation to localize the peak coordinates, and a hierarchical cluster analysis was performed to detect the crucial epicenters. A total of 598 subjects with 917 speech arrest sites (complete interruption of ongoing counting) and 423 anomia sites (inability to name or misnaming) were included. Different centres presented highly consistent distribution patterns for speech arrest (Spearman's coefficient r ranged from 0.60 to 0.85, all pair-wise correlations P < 0.05), and similar patterns for anomia (Spearman's coefficient r ranged from 0.37 to 0.80). The combinational speech arrest map was divided into four clusters: cluster 1 mainly located in the ventral precentral gyrus and pars opercularis, which contained the peak of speech arrest in the ventral precentral gyrus; cluster 2 in the ventral and dorsal precentral gyrus; cluster 3 in the supplementary motor area; cluster 4 in the posterior superior temporal gyrus and supramarginal gyrus. The anomia map revealed two clusters: one was in the posterior part of the superior and middle temporal gyri, which peaked at the posterior superior temporal gyrus; and the other within the inferior frontal gyrus, peaked at the pars triangularis. This study constitutes the largest series to date of language maps generated from direct electrical stimulation mapping. The consistency of data provides evidence for common language networks across languages, in the context of both speech and naming circuit. Our results not only clinically offer an atlas for language mapping and protection, but also scientifically provide better insight into the functional organization of language networks.

Robin Sequence: 5-Year Speech Outcomes-A Case-Control Study.

BACKGROUND: Pierre Robin sequence (Robin sequence) is defined as the triad of micrognathia, glossoptosis, and airway obstruction. It is frequently associated with palatal clefting. In recent years, increased interest in speech outcomes of cleft patients diagnosed with Robin sequence has been shown. METHODS: Speech outcomes of cleft patients with Robin sequence were assessed at age 5 in comparison with a cleft palate-only cohort. Speech parameters were evaluated according to the Cleft Audit Protocol for Speech-Augmented and analyzed using the National Audit Standards for Speech (United Kingdom). All patients were treated in the same institution during the same period (2005 to 2012). Subjects who needed nasopharyngeal airway support and those whose airway was managed by positioning only were eligible. RESULTS: Fifty-one cleft patients diagnosed with Robin sequence were included in this study. Outcomes were compared to those of 128 nonsyndromic cleft palate-only patients.Patients with Robin sequence were shown to present with a significantly higher rate of cleft speech characteristics in comparison to the reference cohort (p = 0.001). Furthermore, it was shown that Robin sequence is associated with a significantly higher rate of secondary speech surgery for velopharyngeal dysfunction before the age of 5 (p = 0.016). Robin sequence patients with a nasopharyngeal airway presented with a higher rate of cleft speech characteristics compared to Robin sequence patients managed with positioning only. CONCLUSION: Cleft patients with Robin sequence are more likely to need further surgery to correct velopharyngeal dysfunction before the age of 5 and are more prone to present with cleft speech characteristics at the age of 5. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Disfluencias típicas del habla en niños fluentes de Santiago de Chile / Typical speech dysfluencies in fluent children from Santiago de Chile

El estudio de la fluidez del habla, en distintas etapas del ciclo vital, es relevante porque contribuye tanto a la comprensión del desarrollo típico del habla como a la comprensión del habla patológica, lo que aporta al diagnóstico y al tratamiento. Los objetivos del trabajo son: a) determinar la presencia y distribución de las disfluencias en distintos grupos etarios y b) determinar si existe variación en la distribución de las disfluencias en relación con el género de los informantes. La muestra estuvo compuesta por 60 niños y adolescentes entre los 4.01 años y 15.0 años distribuidos en tres grupos. Los resultados indican que la prolongación de vocal fue la disfluencia más frecuente, mientras que la repetición de sílaba fue la menos presente en los grupos analizados. En el grupo de mujeres de menor edad hubo una cantidad significativamente alta de repetición de palabras. No se encontraron otras diferencias en relación con el género de los informantes. Finalmente, se discuten los resultados y algunas consideraciones relacionadas con la metodología en este tipo de investigaciones.

The study of fluency of speech, at different stages of the life cycle, is relevant in that it contributes both to the understanding of typical speech development and to the understanding of pathological speech, which is useful for diagnosis and treatment. The objectives of this work are a) to determine the presence and distribution of the disfluencies present in different age groups and b) to determine if there is variation in the distribution of disfluencies in relation to the gender of the informants. Thesample consisted of 60 children and adolescents between 4.01 years old and 15.0 years old distributed in three groups. The results indicate that vowel prolongation was the most frequent disfluency, while syllable repetition was the least present inthe groups analyzed. In the group of younger women, there was a significantly high amount of word repetition when comparing the presence of this disfluency in the other two groups. There were no more differences reported in relation to the gender ofthe informants. Finally, the results and some considerations related to the methodology in this type of research are discussed

Comparison between deep brain stimulation and magnetic resonance-guided focused ultrasound in the treatment of essential tremor: a systematic review and pooled analysis of functional outcomes.

The current gold standard surgical treatment for medication-resistant essential tremor (ET) is deep brain stimulation (DBS). However, recent advances in technologies have led to the development of incisionless techniques, such as magnetic resonance-guided focused ultrasound (MRgFUS) thalamotomy. The authors perform a systematic review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement to compare unilateral MRgFUS thalamotomy to unilateral and bilateral DBS in the treatment of ET in terms of tremor severity and quality of life improvement. PubMed, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials and SCOPUS databases were searched. 45 eligible articles, published between 1990 and 2019, were retrieved. 1202 patients were treated with DBS and 477 were treated with MRgFUS thalamotomy. Postoperative tremor improvement was greater following DBS than MRgFUS thalamotomy (p<0.001). A subgroup analysis was carried out stratifying by treatment laterality: bilateral DBS was significantly superior to both MRgFUS and unilateral DBS (p<0.001), but no significant difference was recorded between MRgFUS and unilateral DBS (p<0.198). Postoperative quality of life improvement was significantly greater following MRgFUS thalamotomy than DBS (p<0.001). Complications were differently distributed among the two groups (p<0.001). Persistent complications were significantly more common in the MRgFUS group (p=0.042). While bilateral DBS proves superior to unilateral MRgFUS thalamotomy in the treatment of ET, a subgroup analysis suggests that treatment laterality is the most significant determinant of tremor improvement, thus highlighting the importance of future investigations on bilateral staged MRgFUS thalamotomy.

Development, behaviour and sensory processing in Marshall-Smith syndrome and Malan syndrome: phenotype comparison in two related syndromes.

BACKGROUND: Ultrarare Marshall-Smith and Malan syndromes, caused by changes of the gene nuclear factor I X (NFIX), are characterised by intellectual disability (ID) and behavioural problems, although questions remain. Here, development and behaviour are studied and compared in a cross-sectional study, and results are presented with genetic findings. METHODS: Behavioural phenotypes are compared of eight individuals with Marshall-Smith syndrome (three male individuals) and seven with Malan syndrome (four male individuals). Long-term follow-up assessment of cognition and adaptive behaviour was possible in three individuals with Marshall-Smith syndrome. RESULTS: Marshall-Smith syndrome individuals have more severe ID, less adaptive behaviour, more impaired speech and less reciprocal interaction compared with individuals with Malan syndrome. Sensory processing difficulties occur in both syndromes. Follow-up measurement of cognition and adaptive behaviour in Marshall-Smith syndrome shows different individual learning curves over time. CONCLUSIONS: Results show significant between and within syndrome variability. Different NFIX variants underlie distinct clinical phenotypes leading to separate entities. Cognitive, adaptive and sensory impairments are common in both syndromes and increase the risk of challenging behaviour. This study highlights the value of considering behaviour within developmental and environmental context. To improve quality of life, adaptations to environment and treatment are suggested to create a better person-environment fit.

Surgical maxillary advancement and speech resonance: comparison among cleft types. / Avanço cirúrgico de maxila e ressonância de fala: comparação entre os tipos de fissura.

Purpose This study investigated the influence of the cleft type on the appearance of hypernasality after surgical maxillary advancement (MA). Methods Nasality was determined by measurement of nasalance (acoustic correlate of nasality) by nasometry. The study involved analysis of the nasalance scores of 17 individuals with isolated cleft palate (CP), 118 with unilateral cleft lip and palate (UCLP) and 69 with bilateral cleft lip and palate (BCLP), of both sexes, aged 18 to 28 years, after MA. Only individuals with normal nasalance scores indicating balanced resonance before MA were included in this study. Nasometry was performed 3 days before and 15 months after MA, on average. The proportion of patients who presented nasalance scores indicating hypernasality after surgery was calculated by the ANOVA test, and comparison among the different cleft types was evaluated by the chi-square test (p < 0.05). Results No significant difference was found in the proportions of individuals with hypernasality among the cleft types. Conclusion Nasometry showed that the appearance of hypernasality after MA in individuals with cleft palate with or without cleft lip occurred in similar proportions, regardless of the cleft type.

Longitudinal Speech Change After Subthalamic Nucleus Deep Brain Stimulation in Parkinson's Disease Patients: A 2-Year Prospective Study.

BACKGROUND: Speech disorders are among the most common adverse effects after subthalamic nucleus deep brain stimulation (STN-DBS) in Parkinson's disease (PD) patients. However, longitudinal speech changes after STN-DBS are not fully understood. OBJECTIVE: We performed a two-year prospective study on PD patients who underwent STN-DBS and analyzed changes in speech function to clarify factors predicting for speech deterioration. METHODS: Twenty-five PD patients were assessed before and up to two years after STN implantation. Speech function was evaluated in the on-stimulation condition and 30 min after stimulation cessation using auditory-perceptual assessment. Patients who experienced overall worsening in speech intelligibility or naturalness ≥1 point during follow-up were classified into a deteriorated group (n = 16), with the remaining subjects being classified into a stable group (n = 9). Cognitive and motor functions were also assessed. RESULTS: The stable group had significantly better values of low volume, monoloudness, and asthenic voice subscores of the auditory-perceptual assessment in the on-stimulation condition compared with the off-stimulation condition. Imprecise consonants, excess loudness variation, and strained voice subscores were improved via cessation of stimulation in both groups. Before surgery, the deteriorated group had significantly lower scores in the Stroop Color-Word Test and Digit Span compared to the stable group. CONCLUSIONS: During follow-up, some subscores showed significant worsening in the on-stimulation condition in both groups. However, beneficial effects of STN-DBS on speech appeared to counterbalance negative effects of STN-DBS on speech function only in the stable group. Worse cognitive function may be a potential predictor for speech deterioration after STN-DBS in PD patients.

Caracterização do escape posterior tardio na deglutição / Characterization of posterior leakage in swallowing

RESUMO Objetivo Descrever e caracterizar um achado, o escape posterior tardio de resíduo alimentar na deglutição, segundo idade, gênero e consistência do alimento, que ocorreu no evento. Método A coleta de dados ocorreu por meio da análise de cada exame de videonasoendoscopia funcional da deglutição anteriormente gravado em um ambulatório especializado. A população do estudo contemplou 200 pacientes de ambos os gêneros, na faixa etária entre 46 e 87 anos, com e sem patologia de base para disfagia. As imagens foram estudadas individualmente pelo pesquisador e analisadas por juízes, com o objetivo de identificar e selecionar imagens que constatassem a presença ou ausência do evento em estudo. Resultados Verificou-se o escape tardio em 45 exames do total de 200 analisados. Os exames selecionados para o estudo apresentaram o escape residual posterior tardio em pelo menos uma consistência. A maior frequência do escape posterior tardio ocorreu com o líquido. A análise mostrou significância do evento em estudo com a população que apresentava idades mais avançadas da nossa amostra. Conclusão O escape posterior tardio ocorre predominantemente na consistência líquida, em população mais idosa e sem predomínio de gênero.

ABSTRACT Purpose To describe and characterize a finding, i.e., delayed posterior leakage of food residue during swallowing, according to age, gender and food consistency, which occurred in the event. Methods Data were collected through the analysis of each functional videonasoendoscopy test of swallowing previously recorded in a specialist outpatient clinic. The study population included 200 patients, both males and females, aged between 46 and 87 years, with and without an underlying pathology for dysphagia. The images were studied individually by the researcher and analyzed by judges in order to identify and select images that would confirm the presence or absence of the study event. Results Delayed escape was found in 45 out of the 200 analyzed tests. The tests selected for the study showed delayed posterior bolus leakage in at least one consistency. The highest frequency of delayed posterior leakage occurred with fluids. The analysis showed the significance of the study event with the population related to older ages in the sample analyzed. Conclusion Delayed posterior leakage occurs predominantly in the liquid consistency in older populations with no gender predominance.

Correlation between the cephalometric measurements and acoustic properties of /s/ sound in Turkish

Abstract Objectives To evaluate the acoustic properties of the /s/ sound in individuals with different occlusion types and to investigate relationships between these properties and cephalometric measurements. Methodology Sixty patients were divided into three groups based on malocclusion. Group 1 included 20 patients (mean age: 14.85±2.01 years) with Class I skeletal and dental relationships. Group 2 included 20 patients (mean age: 13.49±1.78 years) with Class II skeletal and dental relationships. Group 3 included 20 patients (mean age: 12.46±2.62 years) with Class III skeletal and dental relationships. Cephalometric tracings were obtained from cephalometric radiographs. All included patients were native speakers of Turkish. The /s/ sound was selected for center of gravity analysis. Correlations between cephalometric values and acoustic parameters were also investigated. Results The center of gravity of the /s/ sound had the lowest value in Group 2 (p<0.05). For the /s/ sound in Group 3, moderate positive correlations were found between center of gravity and Sella-Nasion to Gonion-Gnathion angle (p<0.05, r=0.444) Lower incisor to Nasion-B point (p<0.023, r=0.505), and Lower incisor to Nasion-B point angle (p<0.034; r=0.476). No correlation was found in other cephalometric measurements. Conclusions The /s/ sound was affected by malocclusion due to the changing place of articulation. Therefore, referral to an orthodontist for malocclusion treatment especially patients with class III in the early period is suggested for producing acoustically ideal sound.

Contribuições da CIF para uma abordagem integral na atenção à Saúde de Crianças e Adolescentes / Contributions of ICF to an integral approach in the Health Care for Children and Adolescents

RESUMO Objetivo: Investigar a participação e funcionalidade de crianças e adolescentes com alterações de fala utilizando a Classificação Internacional de Funcionalidade, Incapacidade e Saúde. Método: Pesquisa descritiva-analítica de abordagem qualitativa-quantitativa com 30 participantes com alteração de fala (CAF) e 30 com desenvolvimento típico de fala (DTF). Para a coleta de dados, realizaram-se entrevistas semiestruturadas com os participantes, estudo de prontuário, observação dos participantes quanto aos aspectos de fala e aplicação de questionário estruturado com os responsáveis. Os dados coletados foram utilizados para seleção de qualificadores das categorias da Classificação Internacional de Funcionalidade, Incapacidade e Saúde. Para comparação dos qualificadores entre grupos, utilizou-se o Teste Mann-Whitney, e a análise de conteúdo temática, para as entrevistas. Resultados: Os participantes do grupo CAF relataram maior grau de dificuldade do que o grupo DTF. As alterações de fala repercutiram significativamente em Funções do Corpo (articulação e fluência), Atividades e Participação (conversa, relacionamentos, realização de rotinas e lidar com estresse) e Fatores Ambientais (atitudes de familiares, amigos e conhecidos). Conclusão: Os resultados evidenciam o impacto das alterações de fala e suas implicações sociais para estas crianças e adolescentes. A CIF permitiu compreender a saúde em complexidade e integralidade, possibilitando assim o planejamento de estratégias para amenizar o impacto dessas alterações individual e coletivamente. Podendo ser base, futuramente, para a criação de políticas públicas e ações que poderão melhorar a qualidade de vida e promover a saúde dessa população.

ABSTRACT Purpose: To investigate participation, and functionality of children and adolescents with speech disorders using the International Classification of Functioning, Disability and Health (ICF). Method: Descriptive and analytical research of qualitative and quantitative approach. The sample was composed of 30 participants with speech disorders and 30 with typical speech development. For data collection, it was conducted semi-structured interviews with the participants, medical record review, observation of speech aspects' participants, and analyzed study of medical records. The collected data were used to qualify the framework codes. The Mann-Whitney Test was used for comparison between groups, and thematic content analysis for the interviews. Results: Participants with speech disorders reported more difficulties than the participants with typical speech development. Speech disorders significantly impacted on the Body Functions (articulation and fluency), Activities and Participation (conversation, relationships, carrying out the routine and handling of stress) and Environmental Factors (attitudes of family, friends and acquaintances). Conclusion: The results show the impact of speech disorders and their social consequences for these children and adolescents. The ICF allowed us to comprehend health in all its complexity and integrality, making possible to plan strategies to soften the disorders impact in an individual and collective perspective. Then, ICF can be used, in the future, for elaboration of public policies and actions that will improve the quality of life and promote the health of this population.

Avanço cirúrgico de maxila e ressonância de fala: comparação entre os tipos de fissura / Surgical maxillary advancement and speech resonance: comparison among cleft types

RESUMO Objetivo Investigar a influência do tipo de fissura sobre o aparecimento da hipernasalidade após o avanço cirúrgico da maxila (AM). Método A nasalidade foi determinada por meio da medida de nasalância (correlato acústico da nasalidade) utilizando-se a nasometria. Foi realizada a análise dos escores de nasalância de 17 indivíduos com fissura isolada de palato (FP), 118 com fissura de lábio e palato unilateral (FLPU) e 69 com fissura de lábio e palato bilateral (FLPB), de ambos os sexos, com idades entre 18 e 28 anos, submetidos ao AM. Apenas indivíduos com escores de nasalância indicativos de ressonância equilibrada previamente ao AM foram incluídos neste estudo. A nasometria foi realizada, em média, três dias antes e 15 meses após o AM. A proporção de pacientes que apresentaram escores de nasalância indicativos de hipernasalidade após o AM foi calculada por meio do teste ANOVA e a comparação entre os diferentes tipos de fissura foi realizada utilizando-se o teste Qui-quadrado (p < 0,05). Resultados Não foi observada diferença significante entre as proporções de indivíduos com hipernasalidade, de acordo com o tipo de fissura. Conclusão A nasometria mostrou que o aparecimento da hipernasalidade após o AM, em indivíduos com fissura de palato envolvendo ou não o lábio, ocorreu em proporções similares independentemente do tipo de fissura.

ABSTRACT Purpose This study investigated the influence of the cleft type on the appearance of hypernasality after surgical maxillary advancement (MA). Methods Nasality was determined by measurement of nasalance (acoustic correlate of nasality) by nasometry. The study involved analysis of the nasalance scores of 17 individuals with isolated cleft palate (CP), 118 with unilateral cleft lip and palate (UCLP) and 69 with bilateral cleft lip and palate (BCLP), of both sexes, aged 18 to 28 years, after MA. Only individuals with normal nasalance scores indicating balanced resonance before MA were included in this study. Nasometry was performed 3 days before and 15 months after MA, on average. The proportion of patients who presented nasalance scores indicating hypernasality after surgery was calculated by the ANOVA test, and comparison among the different cleft types was evaluated by the chi-square test (p < 0.05). Results No significant difference was found in the proportions of individuals with hypernasality among the cleft types. Conclusion Nasometry showed that the appearance of hypernasality after MA in individuals with cleft palate with or without cleft lip occurred in similar proportions, regardless of the cleft type.

Le Fort I osteotomy in cleft patients: Maxillary advancement and velopharyngeal function.

BACKGROUND: Maxillary advancement may affect speech in cleft patients. AIMS: To evaluate whether the amount of maxillary advancement in Le Fort I osteotomy affects velopharyngeal function (VPF) in cleft patients. METHODS: Ninety-three non-syndromic cleft patients (51 females, 42 males) were evaluated retrospectively. All patients had undergone a Le Fort I or bimaxillary (n = 24) osteotomy at Helsinki Cleft Palate and Craniofacial Center. Preoperative and postoperative lateral cephalometric radiographs were digitized to measure the amount of maxillary advancement. Pre- and postoperative speech was assessed perceptually and instrumentally by experienced speech therapists. Student's t-test and Mann-Whitney's U-test were used in the statistical analyses. Kappa statistics were calculated to assess reliability. RESULTS: The mean advancement of A point was 4.0 mm horizontally (range: -2.8-11.3) and 3.9 mm vertically (range -14.2-3.9). Although there was a negative change in VPF, the amount of maxillary horizontal or vertical movement did not significantly influence the VPF. There was no difference between the patients with maxillary and bimaxillary osteotomy. CONCLUSIONS: The amount of maxillary advancement does not affect the velopharyngeal function in cleft patients.

Timing Of Primary Surgery for cleft palate (TOPS): protocol for a randomised trial of palate surgery at 6 months versus 12 months of age.

INTRODUCTION: Cleft palate is among the most common birth abnormalities. The success of primary surgery in the early months of life is crucial for successful feeding, speech, hearing, dental development and facial growth. Over recent decades, age at palatal surgery in infancy has reduced. This has led to palatal closure in one-stage procedures being carried out around the age of 12 months, but in some cases as early as 6 months. The primary objective of the Timing Of Primary Surgery for Cleft Palate (TOPS)trial is to determine whether surgery for cleft palate performed at 6 or 12 months of age is most beneficial for speech outcomes. METHODS AND ANALYSIS: Infants with a diagnosis of non-syndromic isolated cleft palate will be randomised to receive standardised primary surgery (Sommerlad technique) for closure of the cleft at either 6 months or 12 months, corrected for gestational age. The primary outcome will be perceived insufficient velopharyngeal function at 5 years of age. Secondary outcomes measured across 12 months, 3 years and 5 years will include growth, safety of the procedure, dentofacial development, speech, hearing level and middle ear function. Video and audio recordings of speech will be collected in a standardised age-appropriate manner and analysed independently by multiple speech and language therapists. The trial aims to recruit and follow-up 300 participants per arm. Data will be analysed according to the intention-to-treat principle using a 5% significance level. All analyses will be prespecified within a full and detailed statistical analysis plan. ETHICS AND DISSEMINATION: Ethical approval has been sought in each participating country according to country-specific procedures. Trial results will be presented at conferences, published in peer-reviewed journals and disseminated through relevant patient support groups. TRIAL REGISTRATION NUMBER: NCT00993551; Pre-results.

"Accent issue": foreign accent syndrome following ischemic stroke.

BACKGROUND: Foreign accent syndrome (FAS) is arare syndrome associated with altered speech rhythm and prosody, which listeners perceive as foreign; cerebrovascular accidents, tumors and multiple sclerosis are reported as possible causes of FAS. The pathophysiology of FAS is not yet understood. CASE PRESENTATION: A 68-year-old Italian man was admitted to the EmergencyDepartment for non-fluent aphasia and dysarthria. Computed tomography (CT) scan did not show abnormalities; the patient was treated with systemic thrombolysis. A repeated brain CT and magnetic resonance imaging (MRI) confirmed an infarct in the left primary motor cortex and mild extension to cortico-subcortical frontal regions. In the following days he gradually improved, speaking Italian fluently with a typical German accent. In conclusion, FAS is a rare motor speech disorder, often related to cerebrovascular accidents involving critical regions in the dominant hemisphere. In addition, the present case adds further evidence to the role of the left primary motor cortex in modulation of prosody. In rare cases FAS can be the only sign of stroke or can appear after recovery from post-stroke aphasia.

Speech difficulties and patient health communication mediating effects on worry and health-related quality of life in children, adolescents, and young adults with Neurofibromatosis Type 1.

The objective was to investigate the serial mediating effects of speech difficulties, patient health communication, and disease-specific worry in the relationship between neurofibromatosis (NF) symptoms (pain and skin symptoms) and total generic health-related quality of life (HRQOL) in children, adolescents, and young adults with NF Type 1 (NF1) from the patient perspective. The Speech, Communication, Worry, Pain, Skin Itch Bother, and Skin Sensations Scales from the Pediatric Quality of Life Inventory (PedsQL) NF1 Module and the PedsQL 4.0 Generic Core Scales were completed in a multi-site national study by 305 patients ages 5-25 years. A serial multiple mediator model analysis was conducted to test the hypothesized sequential mediating effects of speech difficulties, health communication, and worry as intervening variables in the association between NF1 symptoms and HRQOL. Symptoms predictive effects on total generic HRQOL were serially mediated by speech difficulties, patient health communication, and worry. In predictive analytics models utilizing hierarchical multiple regression analyses with age and gender demographic covariates, the pain, skin itch bother, and skin sensations multiple mediator models accounted for 61%, 59%, and 56% of the variance in generic HRQOL (p < .001), reflecting large effect sizes. Speech difficulties, patient health communication, and disease-specific worry explain in part the mechanism of symptoms predictive effects on total generic HRQOL in pediatric patients with NF1. Identifying NF1-specific predictors and serial mediators of total generic HRQOL in pediatric patients with NF1 from the patient perspective enables a patient-centered comprehensive care approach for children, adolescents, and young adults with NF1.