388 results on '"Velopharyngeal Insufficiency diagnosis"'
Search Results
152. [Impaired wound healing following tonsillectomy].
- Author
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Zengel P, Betz CS, Berghaus A, and Leunig A
- Subjects
- Adult, Female, Humans, Cicatrix diagnosis, Cicatrix etiology, Tonsillectomy adverse effects, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency etiology, Wound Healing
- Abstract
Tonsillectomy is one of the most commonly performed surgical procedures in the field of otorhinolaryngology. The first tonsillectomy was done about 600 B.C. [3]. This operation is indicated for patients with recurrent tonsillitis, peritonsillar abscess, hypertrophy or asymmetry of the tonsils. Even though a routine procedure, it has a relatively high risk of complications such as post operative hemorrhage, infection or impaired wound healing. The reported case involves a 20 year old female patient who developed velopharyngeal insufficiency as a result of impaired wound healing after tonsillectomy. The patient was treated conservatively and is free of discomfort after 2 months.
- Published
- 2008
- Full Text
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153. Midface osteotomy versus distraction: the effect on speech, nasality, and velopharyngeal function in craniofacial dysostosis.
- Author
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Pereira V, Sell D, Ponniah A, Evans R, and Dunaway D
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- Adolescent, Articulation Disorders diagnosis, Child, Craniofacial Dysostosis complications, Female, Humans, Male, Prospective Studies, Speech Acoustics, Velopharyngeal Insufficiency diagnosis, Voice Disorders diagnosis, Articulation Disorders etiology, Craniofacial Dysostosis surgery, Maxilla surgery, Osteogenesis, Distraction, Osteotomy, Le Fort, Velopharyngeal Insufficiency etiology, Voice Disorders etiology
- Abstract
Objective: To assess speech outcomes following midface advancement and to explore whether the type of advancement surgery affects speech differently in patients with craniofacial dysostosis., Design: Prospective, before-after group design., Subjects: Fifteen consecutive patients were included in the study. Eight underwent advancement by osteotomy and seven by distraction. All patients were seen preoperatively and at least once postoperatively., Main Outcome Measures: Percentage of consonants correct, nature and type of articulation errors, nasalance score, severity ratings of resonance and of velopharyngeal function using nasendoscopy and lateral videofluoroscopy, and amount of forward advancement., Results: No statistically significant differences were found between groups for pre- and postoperative changes of percentage of consonants correct (p = .755, median difference 3.0, 95% confidence interval for median difference [-14.22, 20.22]) and nasalance (p = .171, median difference = -12.00, 95% confidence interval for median differences [-30.46, 6.46]). There was no statistically significant correlation between amount of forward advancement and nasalance (r = .87, p = .799) and percentage of consonants correct (r = -.550, p = .064). Findings from lateral videofluoroscopy and nasendoscopy are described. Individual changes of speech outcomes are reported., Conclusions: In view of the small sample size, results need to be interpreted with caution. However, the study adds to current limited knowledge with this clinical group. Further research with bigger sample sizes and randomization of patients into the different surgical groups is warranted.
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- 2008
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- View/download PDF
154. Multicenter interrater and intrarater reliability in the endoscopic evaluation of velopharyngeal insufficiency.
- Author
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Sie KC, Starr JR, Bloom DC, Cunningham M, de Serres LM, Drake AF, Elluru RG, Haddad J Jr, Hartnick C, Macarthur C, Milczuk HA, Muntz HR, Perkins JA, Senders C, Smith ME, Tollefson T, Willging JP, and Zdanski CJ
- Subjects
- Humans, Observer Variation, Prospective Studies, Reproducibility of Results, Single-Blind Method, Velopharyngeal Insufficiency classification, Videotape Recording, Endoscopy, Velopharyngeal Insufficiency diagnosis
- Abstract
Objective: To explore interrater and intrarater reliability (R (inter) and R (intra), respectively) of a standardized scale applied to nasoendoscopic assessment of velopharyngeal (VP) function, across multiple centers., Design: Multicenter blinded R (inter) and R (intra) study., Setting: Eight academic tertiary care centers., Participants: Sixteen otolaryngologists from 8 centers., Main Outcome Measures: Raters estimated lateral pharyngeal and palatal movement on nasoendoscopic tapes from 50 different patients. Raters were asked to (1) estimate gap size during phonation and (2) note the presence of the Passavant ridge, a midline palatal notch on the nasal surface of the soft palate, and aberrant pulsations. Primary outcome measures were R (inter) and R (intra) coefficients for estimated gap size, lateral wall, and palatal movement; kappa coefficients for the Passavant ridge, a midline palatal notch on the nasal soft palate, and aberrant pulsations were also calculated., Results: The R (inter) coefficients were 0.63 for estimated gap size, 0.41 for lateral wall movement, and 0.43 for palate movement; corresponding R (intra) coefficients were 0.86, 0.79, and 0.83, respectively. Interrater kappa values for qualitative features were 0.10 for the Passavant ridge; 0.48 for a notch on the nasal surface of the soft palate, 0.56 for aberrant pulsations, and 0.39 for estimation of gap size., Conclusions: In these data, there was good R (intra) and fair R (inter) when using the Golding-Kushner scale for rating VP function based on nasoendoscopy. Estimates of VP gap size demonstrate higher reliability coefficients than total lateral wall, mean palate estimates, and categorical estimate of gap size. The reliability of rating qualitative characteristics (ie, the presence of the Passavant ridge, aberrant pulsations, and notch on the nasal surface of the soft palate) is variable.
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- 2008
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155. Nasoendoscopy of velopharynx before and during diagnostic therapy.
- Author
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Pegoraro-Krook MI, Dutka-Souza Jde C, and Marino VC
- Subjects
- Air Pressure, Child, Child, Preschool, Cleft Lip complications, Cleft Palate complications, Female, Humans, Male, Nose, Velopharyngeal Insufficiency complications, Velopharyngeal Insufficiency etiology, Velopharyngeal Insufficiency physiopathology, Video Recording, Voice Disorders etiology, Endoscopy methods, Palate, Soft physiopathology, Pharynx physiopathology, Velopharyngeal Insufficiency diagnosis
- Abstract
Nasoendoscopy is an important tool for assessing velopharyngeal function. The purpose of this study was to analyze velar and pharyngeal wall movement and velopharyngeal gap during nasoendoscopic evaluation of the velopharynx before and during diagnostic therapy. Nasoendoscopic recordings of 10 children with operated cleft lip and palate were analyzed according to the International Working Group Guidelines. Ratings of movement of velum and pharyngeal walls, and size, location and shape of gaps were analyzed by 3 speech-language pathologists (SLPs). Imaging was obtained during repetitions of the syllable /pa/ during a single nasoendoscopic evaluation: (a) before diagnostic therapy, and (b) after the children were instructed to impound and increase intraoral air pressure (diagnostic therapy). Once the patients impounded and directed air pressure orally, the displacement of the velum, right, left and posterior pharyngeal walls increased 40, 70, 80, and 10%, respectively. Statistical significance for displacement was found only for right and left lateral pharyngeal walls. Reduction in gap size was observed for 30% of the patients and other 40% of the gaps disappeared. Changes in gap size were found to be statistically significant between the two conditions. In nasoendoscopic assessment, the full potential of velopharyngeal displacement may not be completely elicited when the patient is asked only to repeat a speech stimulus. Optimization of information can be done with the use of diagnostic therapy's strategies to manipulate VP function. Assuring the participation of the SLP to conduct diagnostic therapy is essential for management of velopharyngeal dysfunction.
- Published
- 2008
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156. [Different patterns of velopharyngeal dysfunction in cleft palate patients].
- Author
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Brunner M, Dockter S, Feldhusen F, Pröschel U, Plinkert P, Komposch G, and Müssig E
- Subjects
- Adolescent, Adult, Apraxias classification, Child, Cleft Palate classification, Female, Humans, Male, Speech Production Measurement, Velopharyngeal Insufficiency classification, Apraxias complications, Apraxias diagnosis, Cleft Palate complications, Cleft Palate diagnosis, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency etiology
- Abstract
Background: Velopharyngeal dysfunction (VPD) is generally known to be difficult to influence. Dysfunctional velopharyngeal motor patterns during speech were analyzed with the aim of optimizing the therapeutic strategies., Methods: Velopharyngeal dysfunctions were videotaped and contextually analyzed during 89 speech sequences in 25 patients. Distinctive features of the motor patterns formed the basis of categorization by three therapists experienced in nasopharyngoscopy. There was a high inter-rater reliability of 94%., Results: A total of four different function profiles were found: 1. VPD with retracted articulatory placement (compensatory articulation) (38%), 2. VPD with motor coordination problems characterized by mistiming of VP movements and voice onset/offset (15%), 3. VPD with verbal dyspraxia characterized by a silent positioning of VP closure before phonation started and a malregulation of muscle tonus (10%) and 4. phoneme-specific VPD (37%)., Conclusion: Specific knowledge regarding the characteristics of dysfunctional speech motor patterns enables specifically tailored therapy.
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- 2007
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157. [Velopharyngeal sequels in labial-alveolar-velopalatine clefts. Pharyngoplasty by pharynx Lipostructure].
- Author
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Bardot J, Salazard B, Casanova D, Pech C, and Magalon G
- Subjects
- Adult, Articulation Disorders diagnosis, Buttocks, Child, Humans, Injections instrumentation, Pulmonary Ventilation physiology, Plastic Surgery Procedures instrumentation, Speech physiology, Subcutaneous Fat, Abdominal, Tissue and Organ Harvesting methods, Transplantation, Autologous, Treatment Outcome, Velopharyngeal Insufficiency diagnosis, Adipose Tissue transplantation, Cleft Lip complications, Cleft Palate complications, Pharynx surgery, Plastic Surgery Procedures methods, Velopharyngeal Insufficiency surgery
- Abstract
Advancing the posterior pharyngeal wall is a classic technique to treat velopharyngeal insufficiency. Injection of autologous fat behind the posterior pharyngeal mucosa according to the Coleman Lipostructure technique is a recent development. The authors report their experience in six cases using this modification. The preoperative work-up was performed by a speech therapist with a physical examination and measurement of the nasal air loss was performed using an aerophonometer. Fat was harvested either on the abdominal wall or on a buttock and then centrifuged. Fat injection was performed using a curved blunt cannula under the mucosa of the lateral and posterior pharyngeal walls. Injecting fat is an autologous graft of fat tissue: after a postoperative period of three months, the volume of fat becomes permanently stable. In five out of the six patients who presented moderate velopharyngeal insufficiency, speech improvement was significant. The single failure was a patient with bilateral cleft lip and palate sequels after previous pharyngoplasty using the Orticochea procedure. Treatment of moderate velopharyngeal insufficiency using fat injection is an efficient method. The advantages are its innocuousness and that scaring of the pharynx is avoided.
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- 2007
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158. [Velopharyngeal sequels in labial-alveolar-velopalatine clefts. Analysis, conclusions and speech therapy management].
- Author
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Gaillot A, Mondie JM, Buffard F, Barthelemy I, and Sannajust JP
- Subjects
- Age Factors, Child, Diagnosis, Differential, Follow-Up Studies, Humans, Palate, Soft physiopathology, Palate, Soft surgery, Pharynx physiopathology, Pharynx surgery, Phonation physiology, Speech Disorders diagnosis, Speech Disorders therapy, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency therapy, Voice Disorders diagnosis, Voice Disorders therapy, Cleft Lip complications, Cleft Palate complications, Speech Disorders etiology, Speech Therapy, Velopharyngeal Insufficiency etiology, Voice Disorders etiology
- Abstract
The association of voice and speech defines phonation. Phonation depends on various organs: the lungs for air, the larynx as a vibration generator, and cavities of resonance modeling articulation and tone. The role of the soft palate is important in French and for everyday conversation. Velopharyngeal incompetence is defined as a voice and articulation disruption by anatomic and/or functional deficiency between the soft palate and pharynx. Hypernasality is studied on an acoustic, phonetic, and vocal level and graded in various degrees of hypernasality. The various clinical and perceptive tests of velopharyngeal incompetence are described according to age. The difficulty to modelize hypernasality is discussed based on a recent study published in 2007. The differential diagnosis with hyper-closed nasality is discussed. The treatment for this condition is essentially speech therapy associated with pharyngoplasty in some cases. The assessment of children born with a labial-alveolar-velopalatine cleft is mainly clinical and perceptive. Therapeutic management is necessary for the child and his family. The follow-up requires a multidisciplinary approach.
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- 2007
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159. [Open nasal speech and velopharyngeal insufficiency in adenoidectomy and tonsillectomy].
- Author
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Brosch S and Haase S
- Subjects
- Adolescent, Age Factors, Child, Child, Preschool, Cleft Palate complications, Cleft Palate epidemiology, Humans, Infant, Prevalence, Risk Factors, Surgical Flaps, Adenoidectomy, Speech Disorders diagnosis, Speech Disorders etiology, Tonsillectomy, Velopharyngeal Insufficiency complications, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency surgery
- Abstract
Adenoidectomy and tonsillectomy are the most frequently recommended childhood ENT operations. Although current standards of practice are high, note still needs to be taken of indications, especially in patients with palatal anomalies. These are not always easily estimated or recognised. The origin and therapy of velopharyngeal insufficiency are of particular importance.
- Published
- 2007
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160. Validity of the nasometer measuring the temporal characteristics of nasalization.
- Author
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Bae Y, Kuehn DP, and Ha S
- Subjects
- Adult, Female, Humans, Linear Models, Male, Reproducibility of Results, Tape Recording, Time Factors, Velopharyngeal Insufficiency diagnosis, Rhinometry, Acoustic instrumentation, Speech Production Measurement instrumentation, Voice Disorders diagnosis
- Abstract
Objective: To examine the validity of the Nasometer (KayPENTAX, Lincoln Park, NJ) in measuring the temporal characteristics of nasalization by comparing the Nasometer measures to the measures from an external criterion procedure., Design: Speech samples consisted of three rate-controlled nonsense syllables, which varied in their vowel compositions: /izinizi/, /azanaza/, and /uzunuzu/. Acoustic data were recorded simultaneously through the Nasometer and an external criterion procedure (a specialized microphone set that collected acoustic signals separately for the nasal and oral channels). Speech segment durations measured from the two instrumental conditions were compared on the Nasometer display and the Computerized Speech Lab (KayPENTAX, Lincoln Park, NJ) display. Five durational variables were measured: total utterance duration, nasal onset interval, nasal consonant duration, nasal offset interval, and total nasalization duration., Participants: Fourteen normal adults who speak American English as their first language participated in the study., Results: No significant differences were found between the measures from the Nasometer and those from an external criterion procedure in all the durational variables pertinent to nasalization. Different vowels, however, yielded significantly different patterns in these durational variables, in which the low vowel /a/ context revealed significantly longer total nasalization duration than did the high vowel /i/ and /u/ contexts., Conclusions: The results suggest that the Nasometer can be used as a valid tool to measure the temporal characteristics underlying nasalization and confirm significant vowel effects on the temporal patterns of nasalization.
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- 2007
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161. Magnetic resonance imaging of the levator veli palatini muscle in speakers with repaired cleft palate.
- Author
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Ha S, Kuehn DP, Cohen M, and Alperin N
- Subjects
- Adult, Cleft Palate complications, Humans, Magnetic Resonance Imaging, Male, Palatal Muscles physiology, Prospective Studies, Reproducibility of Results, Speech Articulation Tests, Velopharyngeal Insufficiency etiology, Cleft Palate rehabilitation, Palatal Muscles anatomy & histology, Velopharyngeal Insufficiency diagnosis
- Abstract
Objective: To obtain detailed anatomic and physiologic information on the levator veli palatini muscle from MRI in individuals with repaired cleft palate and to compare the results with those from normal subjects reported by Ettema et al. (2002)., Design: Prospective study., Setting: University-based hospital., Participants: Four men (ages 22 to 43 years) with repaired cleft lip and palate., Main Outcome Measures: Four quantitative measurements of the levator veli palatini muscle from rest position and dynamic speech magnetic resonance images were obtained: the distance between the origins of the muscle, angle of origin of the muscle, muscle length, and muscle thickness., Results: The length and thickness of the levator veli palatini muscle varied among the subjects and were different from measurements obtained from normal subjects in a previous study. The distance between origin points, length, and thickness of the levator veli palatini muscle were smaller than those of the normal subjects. There were systematic changes of the levator veli palatini muscle, depending upon vowel and consonant types. Levator veli palatini muscle angle of origin and length became progressively smaller from rest, nasal consonants, low vowels, high vowels, and fricative consonants. These changes are consistent with those of the normal subjects., Conclusions: This study contributes to a better understanding of cleft palate anatomy in comparison with normal anatomy of the levator veli palatini muscle. The use of MRI shows promise as an important tool in the diagnosis and eventual aid to treatment decisions for individuals born with cleft palate.
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- 2007
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162. Selective IgM deficiency and 22q11.2 deletion syndrome.
- Author
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Kung SJ, Gripp KW, Stephan MJ, Fairchok MP, and McGeady SJ
- Subjects
- Adolescent, Blood Cell Count, Child, Congenital Abnormalities diagnosis, Congenital Abnormalities pathology, Developmental Disabilities diagnosis, Developmental Disabilities genetics, DiGeorge Syndrome blood, Dysgammaglobulinemia blood, Dysgammaglobulinemia genetics, Female, Humans, Immunoglobulin M blood, Immunoglobulins blood, Immunoglobulins immunology, Male, Otitis Media diagnosis, Otitis Media genetics, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency genetics, DiGeorge Syndrome immunology, Dysgammaglobulinemia diagnosis, Immunoglobulin M deficiency
- Abstract
Background: The 22q11.2 deletion syndrome is a common chromosomal disorder with highly variable phenotypic expression and immunologic defects. Humoral immunity is mostly unaffected, but selective IgA deficiency occurs in up to 13% of patients. Selective IgM deficiency associated with 22q11.2 deletion has been reported in 1 patient., Objective: To describe another 2 patients with 22q11.2 deletion syndrome and IgM deficiency., Methods: Patient 1 was a 6-year-old boy with recurrent otitis media, sinopulmonary infections, wheezing, and speech delay. His serum IgM level was 18 mg/dL, and his IgA and IgG levels were normal. Antibody titers to protein and carbohydrate antigens were protective. Workup for velopharyngeal insufficiency resulted in the diagnosis of 22q11.2 deletion syndrome 3 years later. Patient 2 was a 14-year-old girl diagnosed as having 22q11.2 deletion at 9 years of age after presenting with neonatal seizures, atrial and ventricular septal defects, recurrent otitis media, mental retardation, and asthma. Her serum IgM level was 11 mg/dL, with normal IgG and IgA levels. Antibody titers to protein and carbohydrate antigens were protective. Patient 3 was a previously described 15-year-old girl with persistently draining ears, 22q11.2 deletion, and an IgM level less than 6 mg/dL. Her clinical and laboratory features are summarized., Results: Results of further testing on the patients, including lymphocyte enumeration, were normal. The literature is reviewed regarding decreased IgM levels in 22q11.2 deletion syndrome., Conclusions: Fluorescence in situ hybridization analysis for chromosome 22q11.2 deletion should be considered in patients with selective IgM deficiency, especially if concurrent chronic otitis media, developmental delay, velopharyngeal insufficiency, or dysmorphic features are present.
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- 2007
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163. [The effectiveness of an individual mandibular advancement splint (MAS) in subjects with obstructive tongue-base-snoring].
- Author
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Mahl N, Schuff S, Mattheis S, and Siegert R
- Subjects
- Dental Prosthesis Design, Endoscopy, Humans, Polysomnography, Sleep Apnea, Obstructive diagnosis, Treatment Outcome, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency therapy, Occlusal Splints, Sleep Apnea, Obstructive therapy
- Abstract
Objective: Besides operation the mandibular advancement splint (MAS) is one of the main options for treatment of snoring. Especially the MAS by Hinz, which exclusively blocks mandibular retrusion, has been established as an effective alternative. The aim of this study is to examine the effectiveness of this MAS and to evaluate the role of obstruction at the level of the tongue base as a successful pretherapeutic predictor by using the sleep nasal endoscopy., Subjects and Methods: From August 2001 until August 2006 we have examined over 450 patients with sleep disorders at our hospital. All patients have had an ENT examination, a standardized polysomnography and a sleep nasal endoscopy with therapy simulation. The application of a MAS is indicated if an obstruction at the level of the tongue base is detected and the RDI (respiratory disturbance index) is below 10/h. 10 patients are examined by PSG and partly by sleep nasal endoscopy before and after adjustment of the MAS. The patient group is classified in three velum- (n = 3), isolated tongue base-snorers, five combined tongue base-/velum- and two tongue base-/epiglottis-snorers., Results: The subjective sleep recovery and the snoring-intensity can be improved by the MAS in eight of ten patients. In addition the long-term acceptance of this therapy is very high., Conclusions: According to our experiences the MAS by Hinz is an effective treatment option for patients with an obstruction level at the tongue base confirmed by sleep nasal endoscopy.
- Published
- 2007
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164. Acoustic analysis and detection of hypernasality using a group delay function.
- Author
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Vijayalakshmi P, Reddy MR, and O'Shaughnessy D
- Subjects
- Humans, Nasal Cavity, Reproducibility of Results, Sensitivity and Specificity, Velopharyngeal Insufficiency complications, Voice Disorders etiology, Voice Quality, Algorithms, Diagnosis, Computer-Assisted methods, Pattern Recognition, Automated methods, Sound Spectrography methods, Speech Production Measurement methods, Velopharyngeal Insufficiency diagnosis, Voice Disorders diagnosis
- Abstract
In this paper, we describe a group delay-based signal processing technique for the analysis and detection of hypernasal speech. Our preliminary acoustic analysis on nasalized vowels shows that, even though additional resonances are introduced at various frequency locations, the introduction of a new resonance in the low-frequency region (around 250 Hz) is found to be consistent. This observation is further confirmed by a perceptual analysis carried out on vowel sounds that are modified by introducing different nasal resonances, and an acoustic analysis on hypernasal speech. Based on this, for subsequent experiments the focus is given only to the low-frequency region. The additive property of the group delay function can be exploited to resolve two closely spaced formants. However, when the formants are very close with considerably wider bandwidths as in hypernasal speech, the group delay function also fails to resolve. To overcome this, we suggest a band-limited approach to estimate the locations of the formants. Using the band-limited group delay spectrum, we define a new acoustic measure for the detection of hypernasality. Experiments are carried out on the phonemes /a/, /i/, and /u/ uttered by 33 hypernasal speakers and 30 normal speakers. Using the group delay-based acoustic measure, the performance on a hypernasality detection task is found to be 100% for /a/, 88.78% for /i/ and 86.66% for /u/. The effectiveness of this acoustic measure is further cross-verified on a speech data collected in an entirely different recording environment.
- Published
- 2007
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165. Velopharyngeal dysfunction in perspective: a commentary on the Smith and Kuehn article.
- Author
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Scheuerle J
- Subjects
- Child, Child, Preschool, Cleft Palate surgery, Humans, Oral Surgical Procedures adverse effects, Speech Disorders diagnosis, Speech Disorders etiology, Speech Disorders therapy, Speech Production Measurement economics, Speech Production Measurement instrumentation, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency etiology, Velopharyngeal Insufficiency physiopathology, Velopharyngeal Insufficiency therapy
- Published
- 2007
- Full Text
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166. Speech evaluation of velopharyngeal dysfunction.
- Author
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Smith BE and Kuehn DP
- Subjects
- Adolescent, Child, Child, Preschool, Female, Humans, Male, Speech Disorders etiology, Speech-Language Pathology education, Velopharyngeal Insufficiency complications, Velopharyngeal Insufficiency physiopathology, Velopharyngeal Insufficiency therapy, Voice Quality, Speech Disorders diagnosis, Speech Production Measurement instrumentation, Speech Production Measurement methods, Velopharyngeal Insufficiency diagnosis
- Abstract
This article reviews concepts basic to the evaluation of the speech of persons with velopharyngeal dysfunction. It defines velopharyngeal dysfunction as well as reviews normal and abnormal velopharyngeal function for speech. It defines the common speech characteristics of persons with velopharyngeal dysfunction, including hypernasality, hyponasality, nasal emission, compensatory articulations, and weak pressure consonants. Speech sounds commonly impacted by velopharyngeal dysfunction are discussed. This article identifies the components of a complete speech evaluation as well as identifies anatomic and physiologic measurements of palatal function used to corroborate perceptual speech judgments indicating palatal problems. It identifies special considerations in the evaluation of persons with suspected velopharyngeal dysfunction. It briefly discusses management of velopharyngeal dysfunction. Review questions follow the article.
- Published
- 2007
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167. [Perceptual and instrumental assessment of velopharyngeal function in asymptomatic submucous cleft palate].
- Author
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Miguel HC, Genaro KF, and Trindade IE
- Subjects
- Adolescent, Adult, Child, Female, Humans, Male, Middle Aged, Pressure, Rhinometry, Acoustic, Speech Acoustics, Cleft Palate physiopathology, Speech Production Measurement methods, Velopharyngeal Insufficiency diagnosis
- Abstract
Background: submucous cleft palate (SMCP) may or may not be associated to symptoms of velopharyngeal dysfunction (VPD)., Aim: to verify whether patients with SMCP, who were considered as asymptomatic on a perceptual speech evaluation, present absence of hypernasality and adequate velopharyngeal closure on an instrumental assessment., Method: 22 patients with SMCP and with no VPD symptoms, of both genders, aged 6 to 46 years, underwent speech acoustic assessment (nasometry)--to determine nasalance scores--and speech aerodynamic assessment (pressure-flow technique)--to verify velopharyngeal closure. The complete agreement between the findings on the perceptual and instrumental assessments was the tested null hypothesis., Results: the pressure-flow technique confirmed the perceptual findings, that is, all patients were diagnosed as having adequate velopharyngeal function on both types of assessment. Nasometry results confirmed the perceptual findings in 15 out of the 20 analyzed patients (75% of the cases). The remaining 5 patients (25%) presented nasalance scores that suggest hypernasality (nasometry). This symptom was not identified by the perceptual evaluation, yielding to the rejection of the null hypothesis., Conclusion: results indicate the importance of the combined use of perceptual and instrumental assessments for the diagnosis of VPD in SMCP cases. The follow-up of patients diagnosed as asymptomatic by a perceptual speech evaluation and presenting evidences of VPD on instrumental assessment (e.g. nasometry) is recommended, particularly when dealing with children who are more prone to develop symptoms with aging.
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- 2007
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168. [Role of MR imaging on the diagnosis of velopharyngeal insufficiency preoperatively].
- Author
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Huang CY, Cheng T, Lü W, Xiong B, Zhao M, Yin NB, and Zhao ZM
- Subjects
- Adolescent, Adult, Child, Female, Humans, Male, Velopharyngeal Insufficiency surgery, Young Adult, Magnetic Resonance Imaging, Velopharyngeal Insufficiency diagnosis
- Abstract
Objective: To assess the feasibility and actuality of MR imaging on diagnosis and evaluation of velopharyngeal insufficiency preoperatively., Methods: Since 2002, six patients with velopharyngeal insufficiency were examined with MRI using midsagittal, coronal, and axial images, contrast with radiography, to affirm configuration and movements of soft palate, posterior and lateral pharyngeal walls, and velopharyngeal opening for the choices of following surgeries., Results: MRI visualizes directly and measures objectively the shorter soft palate, confined movements of soft palate, lateral or/and posterior pharyngeal wall, and deeper and enlarged velopharyngeal opening, which were coincided with clinical symptoms and affirmed by observations in the following operations, surpassing radiography., Conclusions: MRI is effective, actual, and uninvasive in imaging and measuring the velopharyngeal insufficiency, thus has a potential role in investigation and planning surgical repairs.
- Published
- 2007
169. The prognostic value of simulated snoring in awake patients with suspected sleep-disordered breathing: introduction of a new technique of examination.
- Author
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Herzog M, Metz T, Schmidt A, Bremert T, Venohr B, Hosemann W, and Kaftan H
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- Female, Humans, Male, Middle Aged, Pharynx physiopathology, Polysomnography, Prognosis, Sleep Apnea, Obstructive physiopathology, Tongue physiopathology, Velopharyngeal Insufficiency physiopathology, Endoscopy, Palate, Soft physiopathology, Physical Examination, Sleep Apnea, Obstructive diagnosis, Snoring physiopathology, Uvula physiopathology, Velopharyngeal Insufficiency diagnosis, Wakefulness physiology
- Abstract
Study Objective: A clinical examination of the upper airway in patients with suspected sleep-disordered breathing (SDB) is frequently performed before nighttime polysomnography. In recent years, the findings of "static" examinations, such as dorsalization of the tongue base, the Malampatti index, and Mueller maneuver, have been determined to be of low predictive value., Design: We developed a new method of "dynamic" examination of the upper airway during simulated snoring in awake patients and analyzed the method in terms of the predictive value for suspected SDB., Setting: N/A PATIENTS: One hundred thirty-one patients were examined prior to night-time polysomnography, and the results were correlated with the apneahypopnea-index (AHI)., Interventions: N/A RESULTS: A significant correlation was detected between an increased dorsal movement of the tongue base, as well as with pharyngeal collapse at the level of the tongue base and the AHI. Pharyngeal collapse at the level of the velum did not correlate with the AHI. The patient's body position during simulated snoring did not influence the results. The "static" examinations, such as the dorsalization of the tongue base, tonsil size, Malampatti index, and Mueller maneuver, did not correlate with the AHI. Patients with a high degree of pharyngeal collapse at the level of the tongue base, in combination with dorsal movement of the tongue base during simulated snoring, revealed a probability of 75% to have an AHI more than 10 and of 92% for an AHI more than 5., Conclusion: The "dynamic" examination of the upper airway under simulated snoring in awake patients is an easy-to-perform method to predict the probability of SDB prior to nighttime polysomnography.
- Published
- 2006
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170. Interrater and intrarater reliability in the evaluation of velopharyngeal insufficiency within a single institution.
- Author
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Yoon PJ, Starr JR, Perkins JA, Bloom D, and Sie KC
- Subjects
- Adolescent, Child, Child, Preschool, Endoscopy, Female, Humans, Male, Nasal Cavity, Observer Variation, Palate, Soft physiopathology, Pharynx physiopathology, Reproducibility of Results, Velopharyngeal Insufficiency physiopathology, Velopharyngeal Insufficiency diagnosis
- Abstract
Objective: To explore the interrater and intrarater reliability in nasoendoscopic assessment of velopharyngeal (VP) function using the standardized reporting method described by Golding-Kushner within a single institution., Design: Prospective blinded study., Setting: Academic, tertiary care, pediatric hospital., Participants: Six health care providers (2 pediatric otolaryngology faculty members, 2 pediatric otolaryngology fellows, and 2 speech pathologists) independently rated 50 videotaped nasoendoscopy segments twice. The segments on the videotape were obtained in a clinical setting., Main Outcome Measures: The Golding-Kushner rating system was used to rate VP function. Raters described VP closure quantitatively by rating palatal and lateral pharyngeal wall movement for each segment. They also qualitatively described characteristics of the VP gap, rated gap size as none, small, medium, or large, and estimated the percentage gap size relative to the resting position. Reliability coefficients were calculated for the data sets., Results: Fairly good interrater and intrarater reliability was seen in the quantitative measures. Faculty otolaryngologists rated segments more similarly to each other than did pediatric otolaryngology fellows, but intrarater reliability was similar for both the experienced and less experienced otolaryngologists. Less consistency was seen in the ratings of the speech pathologists. Raters tended to rate with less consistency when describing qualitative characteristics of the VP gap than when making quantitative measurements., Conclusions: The Golding-Kushner scale is a reasonably reliable tool for reporting nasoendoscopic findings at our institution. However, these data also indicate that there exists room for improvement and that rater training may increase reliability.
- Published
- 2006
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171. Analysis of velopharyngeal morphology in adults with velopharyngeal incompetence after surgery of a cleft palate.
- Author
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Lu Y, Shi B, Zheng Q, Xiao W, and Li S
- Subjects
- Adolescent, Adult, Cephalometry, Female, Humans, Male, Observer Variation, Severity of Illness Index, Velopharyngeal Insufficiency epidemiology, Cleft Palate surgery, Palate, Soft anatomy & histology, Palate, Soft physiopathology, Pharynx anatomy & histology, Pharynx physiopathology, Postoperative Complications, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency etiology
- Abstract
This study analyzed the relationship of velopharyngeal morphology and velopharyngeal function among 13 adults with velopharyngeal incompetence (VPI), 14 adults with velopharyngeal competence (VPC) after primary surgical treatment of cleft palate, and 20 noncleft adults. The measurements included velar length, pharyngeal depth, pharyngeal height, and the need ratio of pharyngeal depth to velar length. In addition, the cranial base, cervical vertebrae, posterior nasal spine, and also the position of the posterior pharyngeal wall (PPW) in the pharyngeal triangle were analyzed. All data were subjected to the Student t test of statistical significance. The results showed that the VPI group had normal pharyngeal depth and a significantly shorter velar length, resulting in a greater depth/length ratio than those of the VPC group and normal control subjects. The position of PPW in the pharyngeal triangle was located significantly more superior in the VPI group compared with the VPC group and normal control subjects. Measurements of the anteroposterior and the vertical dimensions in the regions of the cranial base and cervical vertebrae revealed no significant difference among the 3 groups. According to this study, the velopharyngeal morphology of adults with VPI is characterized by a shorter palate, greater need ratio, slightly counterclockwise-rotated pharyngeal triangle, and superiorly positioned PPW.
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- 2006
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172. Diagnosis and treatment of velopharyngeal insufficiency: clinical utility of speech evaluation and videofluoroscopy.
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Dudas JR, Deleyiannis FW, Ford MD, Jiang S, and Losee JE
- Subjects
- Adolescent, Child, Child, Preschool, Female, Humans, Male, Retrospective Studies, Severity of Illness Index, Speech Disorders etiology, Velopharyngeal Insufficiency complications, Fluoroscopy instrumentation, Plastic Surgery Procedures methods, Speech Disorders diagnosis, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency surgery, Video-Assisted Surgery instrumentation
- Abstract
Introduction: The workup of velopharyngeal insufficiency (VPI) includes speech pathology evaluation and examination of velopharyngeal anatomy and physiology. This study sought to determine whether perceptual speech symptoms were predictive of velopharyngeal closure., Patients and Methods: A retrospective chart review of patients with VPI following primary palatoplasty was performed. All patients underwent perceptual speech evaluation using the Pittsburgh Weighted Speech Scale (PWSS) and examination of velopharyngeal anatomy by videofluoroscopy. PWSS scores were correlated to velopharyngeal closure., Results: All patients exhibited clinical VPI (PWSS = 5-27). No patient demonstrated complete velopharyngeal closure on videofluoroscopy. Velopharyngeal closure on the lateral view showed a statistically significant, moderate correlation with both the PWSS total score (rs = -0.424; P = 0.03) and the phonation subscore (rs = -0.405; P = 0.04)., Conclusions: Although certain aspects of speech are related to velopharyngeal anatomy, speech and videofluoroscopic studies each provide unique information in the workup of VPI. Selection of surgical approach often depends on anatomic factors, and improvement in speech postoperatively indicates successful treatment.
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- 2006
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173. Malignancy in chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome).
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McDonald-McGinn DM, Reilly A, Wallgren-Pettersson C, Hoyme HE, Yang SP, Adam MP, Zackai EH, and Sullivan KE
- Subjects
- Child, Child, Preschool, DiGeorge Syndrome diagnosis, Diagnosis, Differential, Female, Humans, Infant, Infant, Newborn, Male, Neoplasms diagnosis, Velopharyngeal Insufficiency diagnosis, Chromosome Deletion, Chromosomes, Human, Pair 22 genetics, DiGeorge Syndrome genetics, Neoplasms genetics, Velopharyngeal Insufficiency genetics
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- 2006
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174. A comparison of nasendoscopy and multiview videofluoroscopy in assessing velopharyngeal insufficiency.
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Lam DJ, Starr JR, Perkins JA, Lewis CW, Eblen LE, Dunlap J, and Sie KC
- Subjects
- Adolescent, Adult, Age Factors, Child, Child, Preschool, Cleft Palate surgery, Cohort Studies, Female, Humans, Male, Nose physiopathology, Palatal Muscles physiopathology, Palate, Soft physiopathology, Pharynx physiopathology, Phonation physiology, Retrospective Studies, Speech Intelligibility physiology, Velopharyngeal Insufficiency physiopathology, Cineradiography methods, Endoscopy methods, Fluoroscopy methods, Velopharyngeal Insufficiency diagnosis
- Abstract
Objectives: 1) To compare nasendoscopy (NE) and multiview fluoroscopy (MVF) in assessing velopharyngeal gap size; and 2) to determine the relationship between these assessments and velopharyngeal insufficiency (VPI) severity., Study Design and Setting: Retrospective review of consecutive patients with VPI at a tertiary care children's hospital, assessed with NE and MVF between 1996 and 2003., Results: 177 subjects. NE and MVF gap areas were correlated (R = 0.34, 95% CI 0.26-0.41). In adjusted analysis, VPI severity was associated with: 1) NE gap area (OR = 2.78, 95% CI 1.96-3.95), 2) MVF gap area (OR 1.64, 95% CI 1.37-1.95), 3) age <5 years (OR 3.30, 95% CI 1.47-7.38), and 4) previously repaired cleft palate (OR 0.48, 95% CI 0.25-0.94)., Conclusions and Significance: NE and MVF assessments provide complementary information and are correlated. Both are associated with VPI severity. However, the "bird's-eye view" provided by NE has a stronger correlation with VPI severity than MVF., Ebm Rating: B-2b.
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- 2006
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175. Acute onset of velopharyngeal insufficiency and Dysphagia after sternocleidomastoid myotomy for congenital muscular torticollis.
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Sönmez A, Ersoy B, and Numanoğlu A
- Subjects
- Acute Disease, Adult, Deglutition Disorders diagnosis, Female, Follow-Up Studies, Humans, Laryngoscopy methods, Magnetic Resonance Imaging, Monitoring, Physiologic methods, Postoperative Complications diagnosis, Remission, Spontaneous, Severity of Illness Index, Time Factors, Velopharyngeal Insufficiency diagnosis, Deglutition Disorders etiology, Neck Muscles surgery, Torticollis congenital, Torticollis surgery, Velopharyngeal Insufficiency etiology
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- 2006
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176. Cleft palate speech and velopharyngeal insufficiency: surgical approach.
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Sie KC
- Subjects
- Adenoidectomy, Airway Obstruction diagnosis, Articulation Disorders diagnosis, Child, Preschool, Cineradiography, Decision Making, Endoscopy, Fluoroscopy, Humans, Palate, Soft physiopathology, Palate, Soft surgery, Pharyngeal Muscles physiopathology, Pharyngeal Muscles surgery, Speech physiology, Speech Disorders etiology, Speech Intelligibility physiology, Surgical Flaps, Tonsillectomy, Treatment Outcome, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency etiology, Voice Quality physiology, Cleft Palate complications, Speech Disorders surgery, Velopharyngeal Insufficiency surgery
- Abstract
Velopharyngeal insufficiency is the main morbidity associated with clefting of the secondary palate. Therefore, it is important to monitor speech production in all children with a history of cleft palate. Diagnosis and management of velopharyngeal insufficiency is an important function of the cleft palate team. The surgical approach used in the Craniofacial Center at Children's Hospital and Regional Medical Center, Seattle, Washington, USA is presented. Assessment of velopharyngeal function, as it relates to surgical intervention and measurement of outcome, is reviewed. Surgical management using Furlow palatoplasty and sphincter pharyngoplasty is discussed.
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- 2006
177. SISL (ScreeningsInstrument Schisis Leuven): assessment of cleft palate speech, resonance and myofunction.
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Breuls M, Sell D, Manders E, Boulet E, and Vander Poorten V
- Subjects
- Articulation Disorders diagnosis, Articulation Disorders etiology, Facial Expression, Humans, Language Development, Nose physiopathology, Palatal Muscles physiopathology, Palate, Soft physiopathology, Patient Care Planning, Phonetics, Speech physiology, Speech Disorders etiology, Velopharyngeal Insufficiency etiology, Voice physiology, Voice Disorders etiology, Voice Quality physiology, Cleft Palate complications, Speech Disorders diagnosis, Velopharyngeal Insufficiency diagnosis, Voice Disorders diagnosis
- Abstract
This paper presents an assessment protocol for the evaluation and description of speech, resonance and myofunctional characteristics commonly associated with cleft palate and/or velopharyngeal dysfunction. The protocol is partly based on the GOS.SP.ASS'98 and adapted to Flemish. It focuses on the relevant aspects of cleft type speech necessary to facilitate assessment, adequate diagnosis and management planning in a multi-disciplinary setting of cleft team care.
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- 2006
178. Velopharyngeal dysfunction: evolving developments in evaluation.
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Rowe MR and D'Antonio LL
- Subjects
- Ear Diseases complications, Ear, Middle, Endoscopy, Humans, Pharynx surgery, Sleep Apnea, Obstructive surgery, Speech, Surgical Flaps, Treatment Outcome, Velopharyngeal Insufficiency physiopathology, Velopharyngeal Insufficiency surgery, Video Recording, Magnetic Resonance Imaging, Velopharyngeal Insufficiency diagnosis
- Abstract
Purpose of Review: Otolaryngologists are increasingly being called upon to assist in the differential diagnosis of velopharyngeal valving disorders for speech, assisting in treatment planning and the assessment of treatment outcomes., Recent Findings: The most commonly used methods for direct visualization of velopharyngeal function remain nasendoscopy and videofluoroscopy. Literature supporting the use of either nasendoscopy followed by videofluoroscopy or the reverse can be found. Several studies also suggest that magnetic resonance imaging can make important contributions to the evaluation of velopharyngeal anatomy and function. The routine use of magnetic resonance imaging for evaluating the velopharynx is neither practical nor probable at the present time. Although magnetic resonance imaging may be as effective as videofluoroscopy or nasendoscopy, the cost of magnetic resonance imaging and the radiation exposure of videofluoroscopy will likely dictate that nasendoscopy continues to be the most common technique for evaluating velopharyngeal function during speech. Several recent studies have documented the use of instrumental assessment of velopharyngeal function for assigning patients to a given surgical procedure, predicting surgical success or complications, and evaluating treatment outcomes., Summary: There are still discussions and conflicting results regarding the best method for evaluating velopharyngeal function. Opinions conflict regarding the various methods, which suggests that no single method is best. The decision regarding the most appropriate evaluation protocol should be guided by the information that the clinician is attempting to obtain and the relative benefits and risks of each method. Increasingly, these evaluation methods are used by the otolaryngologist to assist in the differential diagnosis of velopharyngeal valving disorders, the assignment of patients to various treatment options, and the evaluation of treatment outcomes.
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- 2005
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179. [Finding the appropriate therapy with the help of sleep endoscopy].
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Baisch A, Hein G, Gössler U, Maurer JT, and Hörmann K
- Subjects
- Aged, Catheter Ablation, Continuous Positive Airway Pressure, Humans, Hyoid Bone surgery, Male, Palate, Soft surgery, Polysomnography, Postoperative Complications etiology, Sleep Apnea, Obstructive diagnosis, Tongue surgery, Treatment Failure, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency surgery, Endoscopy methods, Sleep Apnea, Obstructive surgery, Video Recording
- Abstract
Nasal continuous positive airway pressure (nCPAP) ventilation is the gold standard in the treatment of obstructive sleep apnea (OSA). For patients with CPAP-intolerance and for those tolerating the CPAP-mask but without success, surgical strategies are of growing interest. There are several surgical concepts which combine different surgical procedures on both the velopharyngeal and the retro-lingual section of the upper airway. The case presented here describes the failed CPAP-therapy in an OSAS-patient who tolerates the CPAP-mask. The apneas and hypopnoes could not be reduced by using the CPAP-mask, even more they increased under CPAP-ventilation. Performing a video-documented sleep-endoscopy of the upper airway (under physiological sleep patterns) the location of the obstruction could be identified. After performing a hyoid suspension in combination with a radiofrequency therapy of the base of tongue the apneas and hypopneas decreased. Only by using the sleep endoscopy the reason for the failed CPAP-therapy could be identified and therefore the surgical protocol succeed. To what extend the sleep video endoscopy becomes a standardized procedure in sleep labs warrants further investigations.
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- 2005
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180. Evaluation of VPI-assessment with videofluoroscopy and nasoendoscopy.
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Havstam C, Lohmander A, Persson C, Dotevall H, Lith A, and Lilja J
- Subjects
- Adolescent, Adult, Child, Child, Preschool, Cleft Lip complications, Cleft Palate complications, Endoscopy, Fluoroscopy, Humans, Reproducibility of Results, Retrospective Studies, Velopharyngeal Insufficiency etiology, Video Recording, Velopharyngeal Insufficiency diagnosis
- Abstract
Unlabelled: The purpose of this study was to investigate how different amounts of visual assessment information influence the recommended treatment for velopharyngeal insufficiency (VPI). Is a patient presented with videofluoroscopy (VF) in lateral projection recommended the same treatment as when frontal projection, nasoendoscopy, or both, are added? Retrospective material with video recorded assessment of VPI was blinded and copied in random order. Each patient was then presented in four separate combinations: VF in lateral projection; VF in lateral and frontal projection; VF in lateral projection and nasoendoscopy; and VF in lateral and frontal projection and nasoendoscopy (all of the available assessment material). The cleft palate team of Göteborg, Sweden, mutually rated velopharyngeal function and recommended action based on the presented material., Subjects: Nineteen consecutive patients (median age 7:5 years, range 4:4-19:7) investigated with VF in lateral and frontal projection and nasoendoscopy during 1997-99 at the cleft palate centre in Göteborg, Sweden. Post operative assessments were excluded. Percent agreement and Kappa calculations were used to compare the different combinations of parts of information to all of the available information., Results: Thirteen of the 19 patients (68%) were recommended the same action regardless of the amount of presented information. Percent agreement (Kappa) between parts and all of the available information: VF in lateral projection 84% (0.75), VF in lateral and frontal projection 79% (0.74), and VF in lateral projection and nasoendoscopy 84% (0.72)., Conclusions: VF in lateral projection is recommended to be the first step in visualising velopharyngeal function, and nasoendoscopy the next when further investigation is required.
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- 2005
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181. Evaluation of VPI-assessment with videofluoroscopy and nasoendoscopy.
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Sommerlad BC
- Subjects
- Endoscopy, Fluoroscopy, Humans, Nasal Cavity, Video Recording, Velopharyngeal Insufficiency diagnosis
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- 2005
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182. Effects of genioglossal muscle advancement on speech: an acoustic study of vowel sounds.
- Author
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Vähätalo K, Laaksonen JP, Tamminen H, Aaltonen O, and Happonen RP
- Subjects
- Articulation Disorders diagnosis, Follow-Up Studies, Humans, Male, Middle Aged, Phonetics, Postoperative Complications diagnosis, Sleep Apnea, Obstructive diagnosis, Sound Spectrography, Velopharyngeal Insufficiency diagnosis, Articulation Disorders etiology, Chin surgery, Mandibular Advancement, Masticatory Muscles surgery, Osteotomy, Postoperative Complications etiology, Sleep Apnea, Obstructive surgery, Speech Acoustics, Tongue surgery, Velopharyngeal Insufficiency surgery
- Abstract
Objective: The effects of the genioglossal muscle advancement on phonetic quality of speech were studied analyzing the acoustic features of vowel sounds., Study Design and Setting: The study group consisted of 5 men suffering from partial upper airway obstruction during sleep. To prevent tongue base collapse, genioglossal muscle advancement was made with chin osteotomy without hyoid myotomy and suspension. The speech material consisted of 8 vowels produced in sentence context repeated 10 times before the operation, and 10 days and 6 weeks after the operation. The acoustic features of vowels were analyzed., Results: The operation had no significant effects on vowel quality. Only for 2 of the subjects the pitches changed systematically due to the operation., Conclusion: According to the acoustic analysis, genioglossal muscle advancement with chin osteotomy has no effects on vowel production. Some short-term changes were observed, but these changes were highly individual., Significance: The operation seems to have no potential to change vowel production.
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- 2005
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183. Speech and swallowing following radial forearm flap reconstruction of major soft palate defects.
- Author
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McCombe D, Lyons B, Winkler R, and Morrison W
- Subjects
- Carcinoma, Squamous Cell rehabilitation, Carcinoma, Squamous Cell surgery, Dysarthria diagnosis, Dysarthria etiology, Female, Humans, Male, Oropharyngeal Neoplasms rehabilitation, Tongue physiopathology, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency etiology, Deglutition, Oropharyngeal Neoplasms surgery, Palate, Soft surgery, Speech Intelligibility, Surgical Flaps
- Abstract
Velopharyngeal function is often compromised by the resection and reconstruction of oropharyngeal and palatal tumours. While free tissue transfer has improved the outcomes of head and neck reconstruction. In general, palatal reconstruction remains a challenge. Velopharyngeal function was analysed in eight patients following microsurgical reconstruction of defects of between 50 and 100% of the soft palate. The radial forearm fasciocutaneous free flap was used in all cases. The outcome of reconstruction was analysed by patient questionnaire and with standardised tests of speech and swallowing function. Velopharyngeal function post-operatively ranged from poor to near normal. Poor function appeared due to the loss of active elevation and contracture of the reconstructed palate producing failure of velopharyngeal closure during swallowing and speech. The results emphasise the limitations of reconstruction of a dynamic structure such as the soft palate with the static fold of skin and soft tissue produced by a fasciocutaneous flap. The relatively poor results obtained suggest that an anatomical approach to soft palate reconstruction is inadequate and reduction of the calibre of the velopharyngeal aperture is required to compensate for the lack of mobility in the reconstructed palate.
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- 2005
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184. Dynamic near-real-time magnetic resonance imaging for analyzing the velopharyngeal closure in comparison with videofluoroscopy.
- Author
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Beer AJ, Hellerhoff P, Zimmermann A, Mady K, Sader R, Rummeny EJ, and Hannig C
- Subjects
- Adolescent, Adult, Child, Child, Preschool, Feasibility Studies, Female, Fluoroscopy methods, Humans, Male, Phonation physiology, Reference Values, Magnetic Resonance Imaging methods, Pharynx physiopathology, Velopharyngeal Insufficiency diagnosis, Videotape Recording methods
- Abstract
Purpose: To demonstrate the feasibility of dynamic magnetic resonance imaging (MRI) with near-real-time temporal resolution ("real-time MRI") for analyzing the velopharyngeal closure in comparison with multiview videofluoroscopy., Materials and Methods: Seven patients (three females and four males, 5-21 years old, mean age=11.3 years) with suspected velopharyngeal insufficiency, and one healthy volunteer were examined with videofluoroscopy and real-time MRI using a turbo-spin-echo (TSE) sequence (TR=170 msec, TE=21 msec, slice thickness=6 mm, six images per second). Imaging was done during phonation in all three image planes. The results were analyzed by two radiologists in comparison with videofluoroscopy as the standard of reference for overall image quality and the pattern of velopharyngeal closure., Results: Real-time MRI correctly depicted the pattern of velopharyngeal closure in correspondence to videofluoroscopy in all cases. Concerning the movement of the pharyngeal walls, real-time MRI falsely depicted a general movement of the dorsal pharyngeal wall in one case, whereas videofluoroscopy showed no movement. In one patient, real-time MRI provided additional information by showing an asymmetric movement of the lateral pharyngeal walls that could not be depicted by videofluoroscopy due to technical limitations. Concerning image quality, the coronal plane was more difficult to evaluate with real-time MRI compared to videofluoroscopy. The axial plane was easier to analyze in real-time MRI compared to videofluoroscopy., Conclusion: Real-time MRI has the potential to depict the pattern of velopharyngeal closure in close correlation with videofluoroscopy, and may deliver additional information in selected cases.
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- 2004
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185. Velopharyngeal dysfunction: speech characteristics, variable etiologies, evaluation techniques, and differential treatments.
- Author
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Dworkin JP, Marunick MT, and Krouse JH
- Subjects
- Adolescent, Child, Child, Preschool, Humans, Male, Palate, Soft physiopathology, Patient Care Team, Speech Production Measurement, Articulation Disorders etiology, Phonation physiology, Velopharyngeal Insufficiency complications, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency etiology, Velopharyngeal Insufficiency therapy, Vocal Cords physiology
- Abstract
This tutorial addresses the complex pathophysiology; various structural and neurologic causes; methods of clinical appraisal; and alternative behavioral prosthetic, and medicosurgical treatments of velopharyngeal dysfunction. To balance the primary focus of this review on hypernasal resonance and articulation disorders, discussions concerning hyponasal speech characteristics and underlying etiologies are also rendered.
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- 2004
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186. [An evaluation of the speech therapy methods for patients after pharyngoplasty.].
- Author
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Jiang LP, Wang GM, Yang YS, Chen Y, and Wu YL
- Subjects
- Adolescent, Adult, Articulation Disorders diagnosis, Articulation Disorders surgery, Articulation Disorders therapy, Child, Child, Preschool, Humans, Speech, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency surgery, Young Adult, Pharynx surgery, Plastic Surgery Procedures methods, Speech Therapy methods
- Abstract
Purpose: Speech therapy was carried out for patients with speech disorders after pharyngoplasty, and the comparison of speech articulation before and after treatment, was done to evaluate the new method., Methods: 82 patients with the varying age from 4 to 31 years were included in the current study. The patients' speeches including syllables, word, phrase and sentence were compared before and after treatment according to Chinese Speech Intelligibility Testing Form. The systematic speech training was carried out according to the compensatory articulation characteristics. The steps were as follows. Firstly, the function of velopharyngeal closure should be strengthened. Secondly, the patients were trained with an average duration of 3.83 months (range from 1.5-12 months) according to the following three sequences: One was from phoneme, syllables, phrase, sentence to short paragraph or dialogue, another was from anterior to posterior according to the speech position, and the other was from easy to difficult according to the articulation method, such as from aspirated syllables to non-aspirated syllables, from plosive, fricative to affricative., Results: There were 71 patients with compensatory articulation characteristic of velopharyngeal incompetence, the speech articulation of patients with glottis plosive increased from preoperative 46.27% to postoperative 97.16% (P<0.001). The speech articulation of patients with pharynx fricative increased from preoperative 57.19% to postoperative 97.72% (P<0.001). There were 11 patients with compensatory articulation characteristic of velopharyngeal adequacy, and the speech articulation of 11 patients with palatalized and lateral misarticulation increased from preoperative 71.10% to postoperative 98.55% (P<0.001)., Conclusions: The training of velopharyngeal closure should be emphasized on the patients with compensatory articulation resulting from velopharyngeal incompetence after pharyngoplasty, then the correct speech position and method was established. While to the patients with compensatory articulation without velopharyngeal incompetence after pharyngoplasty, the correct speech position and method could be directly established.
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- 2004
187. A comparison of oral-nasal balance patterns in speakers who are categorized as "almost but not quite" and "sometimes but not always".
- Author
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Jones DL, Morris HL, and Van Demark DR
- Subjects
- Adolescent, Articulation Disorders etiology, Child, Child, Preschool, Cleft Lip complications, Cleft Palate complications, Humans, Multivariate Analysis, Pulmonary Ventilation, Rhinomanometry, Signal Processing, Computer-Assisted, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency etiology, Voice Disorders etiology, Articulation Disorders diagnosis, Speech Acoustics, Speech Production Measurement methods, Velopharyngeal Insufficiency physiopathology, Voice Disorders diagnosis
- Abstract
Objective: The purpose of this study was to determine whether amplitude or temporal patterns of oral-nasal balance differentiate speakers with cleft palate who are classified as belonging to the "almost but not quite" (ABNQ) and "sometimes but not always" (SBNA) subgroups of marginal velopharyngeal inadequacy., Design: The nasal accelerometric vibrational index (NAVI) was used to measure amplitude and temporal aspects of oral-nasal balance during the productions of oral and nasal syllables, words, and sentences. NAVI measures obtained include mean amplitude, time integral (area under the curve), duration, rise time, and fall time., Setting: Tertiary care center for patients with cleft palate-craniofacial anomalies., Participants: Seventeen patients with repaired cleft palate who were assigned by perceptual assessment to the ABNQ subgroup and 17 patients who were assigned to the SBNA subgroup., Results: No differences were found between the ABNQ and SBNA subgroups with regard to patterns of nasalization. Further analysis as a function of level of production and phonetic context revealed no differences between the subgroups., Conclusions: Although clinicians may report perceived differences in the resonance patterns of speakers who fall within the category of marginal velopharyngeal inadequacy, further division into the ABNQ and SBNA subgroups has yet to be validated.
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- 2004
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188. Velopharyngeal incompetence: the need for speech standards.
- Author
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Pannbacker M
- Subjects
- Certification, Humans, Palate, Soft physiopathology, Pharynx physiopathology, Practice Guidelines as Topic, United States, Clinical Competence standards, Speech-Language Pathology standards, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency therapy
- Abstract
Speech-language pathologists are often involved in the diagnosis and treatment of velopharyngeal incompetence (VPI). Some speech-language pathologists have extensive educational and clinical experience with VPI; others have limited training and experience. Thus, the quality of speech-language services for people with VPI is heterogenous, and it ranges from poor to excellent. There are, as yet, no specific guidelines for speech-language pathologists providing services to people with VPI. Optimal services require specific guidelines for training and experience. The purpose of this article was to (a) review speech-language pathology standards and qualifications, (b) provide reasons for identifying qualified speech-language pathologists, and (c) identify strategies for reduction of risks involved in the delivery of speech-language services for people with VPI.
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- 2004
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189. Pediatric myasthenia gravis and velopharyngeal incompetence.
- Author
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Rieder AA, Conley SF, and Rowe L
- Subjects
- Adolescent, Child, Preschool, Female, Follow-Up Studies, Humans, Infant, Newborn, Male, Palatal Obturators, Retrospective Studies, Severity of Illness Index, Speech Intelligibility, Speech Therapy, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency therapy, Myasthenia Gravis complications, Velopharyngeal Insufficiency etiology
- Abstract
Objective: To determine the clinical course of velopharyngeal incompetence in children with myasthenia gravis (MG)., Methods: A 30-year retrospective study was performed using the medical records of 538 children who presented with velopharyngeal inadequacy (VPI) to a tertiary care academic pediatric center. Children with velopharyngeal incompetence due to myasthenia gravis were identified and their clinical courses were reviewed., Results: Four children were identified with velopharyngeal incompetence associated with myasthenia gravis. All four children required intervention for improvement of speech intelligibility. A speech prosthesis was the uniform intervention., Conclusion: Neonatal myasthenia gravis patients should be followed long-term as symptoms may recur as speech impairment. In addition, a high index of suspicion for this entity is required for early diagnosis due to the highly variable presentation and clinical course.
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- 2004
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190. The effects of adenoidectomy and tonsillectomy on speech and nasal resonance.
- Author
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Maryn Y, Van Lierde K, De Bodt M, and Van Cauwenberge P
- Subjects
- Adenoids pathology, Child, Humans, Hyperplasia diagnosis, Hyperplasia surgery, Palatine Tonsil pathology, Sound Spectrography, Velopharyngeal Insufficiency diagnosis, Adenoidectomy, Speech Acoustics, Speech Disorders diagnosis, Tonsillectomy
- Abstract
This article presents a review of literature concerning speech and nasal resonance following adenoidectomy and/or tonsillectomy. In order to comprehend speech-related postoperative changes, the role of the adenoid and the tonsils in speech is discussed as well as the concept of veloadenoidal closure separating oral and nasal cavities. Moreover, the effects of adenoidal and tonsillar hypertrophy on speech and nasal resonance are debated., (Copyright 2004 S. Karger AG, Basel)
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- 2004
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191. Correlation between the perceptual rating of speech in Dutch patients with velopharyngeal insufficiency and composite measures derived from mean nasalance scores.
- Author
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Keuning KH, Wieneke GH, and Dejonckere PH
- Subjects
- Adolescent, Adult, Aged, Aged, 80 and over, Child, Child, Preschool, Female, Humans, Male, Middle Aged, Phonetics, Sound Spectrography, Speech Acoustics, Speech Articulation Tests, Speech Disorders diagnosis, Speech Intelligibility, Speech Production Measurement, Velopharyngeal Insufficiency diagnosis, Voice Quality
- Abstract
Objective: To evaluate the potential clinical use of composite measures derived from mean nasalance scores., Procedure: Speech samples with a normal distribution of phonemes (normal text, NT) and speech samples free of nasal consonants (denasal text, DT) of 43 patients with perceived hypernasality were used. The overall grade of severity, hyperrhinophonia, audible nasal emission, misarticulations associated with velopharyngeal insufficiency and intelligibility were perceptually rated on separate visual analog scales. Mean nasalance scores were computed by the Nasometer for the same speech samples on which the perceptual ratings were performed. From the mean nasalance scores computed for the NT and DT passages the difference and the quotient were calculated. The advantage could be that the derived measures provide some normalization with regard to the performance of the individual speaker. Spearman correlation coefficients were computed between these composite measures and the perceptually rated parameters. The results were compared with the correlation coefficients between the mean nasalance scores and the ratings., Setting: The Institute of Phoniatrics, Utrecht University Hospital, The Netherlands., Results: The correlations between the composite measures and the perceptual ratings were generally lower than the correlations between mean nasalance scores and the ratings., Conclusion: Normalization of the nasalance scores did not enhance the correlation with the perceptual ratings in this study., (Copyright 2004 S. Karger AG, Basel)
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- 2004
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192. The evaluation and management of velopharyngeal dysfunction.
- Author
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Marsh JL
- Subjects
- Airway Obstruction prevention & control, Algorithms, Humans, Palate, Soft anatomy & histology, Palate, Soft physiology, Pharynx anatomy & histology, Pharynx physiology, Treatment Outcome, Velopharyngeal Insufficiency complications, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency physiopathology, Airway Obstruction etiology, Otorhinolaryngologic Surgical Procedures adverse effects, Plastic Surgery Procedures adverse effects, Velopharyngeal Insufficiency surgery
- Abstract
Several interventions are available for management of VPD. Using an approach of differential management based on differential diagnosis, VPD can be effectively treated in most patients. The current challenge for the VPD care team, however, is to resolve the signs and symptoms of VPD without exchanging them for a different but an equally or more morbid set of signs and symptoms.
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- 2004
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193. Pressure-flow measurements for selected oral sound segments produced by normal children and adolescents: a basis for clinical testing.
- Author
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Smith BE, Patil Y, Guyette TW, Brannan TS, and Cohen M
- Subjects
- Adolescent, Age Factors, Child, Child, Preschool, Discriminant Analysis, Female, Humans, Male, Pharynx anatomy & histology, Pressure, Prospective Studies, Reference Values, Rhinomanometry, Speech Acoustics, Speech Production Measurement instrumentation, Transducers, Pressure, Respiratory Function Tests statistics & numerical data, Speech Production Measurement statistics & numerical data, Velopharyngeal Insufficiency diagnosis
- Abstract
Despite advances in surgery, a significant number of patients who undergo cleft palate repair have residual velopharyngeal insufficiency. Maxillary advancement may also result in velopharyngeal openings during speech. Instrumental approaches providing objective measures of palatal function assisting in the accurate diagnosis of these patients include pressure-flow measurements of velopharyngeal valving during speech. There is little information to guide clinicians in interpreting pressure-flow data when testing pediatric patients, however. The primary purpose of this study was to develop a method for categorizing pressure-flow data used in the diagnosis of children and adolescents with suspected velopharyngeal insufficiency. This prospective study involved 56 male and female subjects 5 to 18 years of age. Subjects had normal speech and resonance at the time of testing, no history of speech therapy, no upper respiratory infections or allergies at the time of testing, and no orofacial anomalies. Subjects repeated oral syllables and the word "hamper" after an examiner. Mean pressures, airflows, and velopharyngeal orifice areas were obtained for each utterance produced by each subject. A discriminate function analysis was performed to determine whether data could be grouped by age, gender, or utterance type. Results indicated significant differences in data for age groups 5 to 8 years, 9 to 13 years, and 14 to 18 years. There were no significant differences between data for male subjects versus female subjects or for different utterance types. Pressures generally decreased, whereas airflows and orifice areas increased with age. Results for 14 to 18 year olds were like those for adults. Using these data, a categorization scheme for velopharyngeal function was proposed for use in clinical testing.
- Published
- 2004
- Full Text
- View/download PDF
194. Effects of mandibular advancement on airway curvature and obstructive sleep apnoea severity.
- Author
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Tsuiki S, Lowe AA, Almeida FR, Kawahata N, and Fleetham JA
- Subjects
- Adolescent, Adult, Airway Obstruction diagnosis, Airway Obstruction physiopathology, Humans, Male, Middle Aged, Palate, Soft physiopathology, Pharynx physiopathology, Polysomnography, Sleep Apnea, Obstructive diagnosis, Sleep Apnea, Obstructive physiopathology, Treatment Outcome, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency physiopathology, Velopharyngeal Insufficiency therapy, Vertical Dimension, Airway Obstruction therapy, Mandibular Advancement instrumentation, Sleep Apnea, Obstructive therapy
- Abstract
In a curved tube, the amount of airflow appears to be influenced by the amount of curvature. The purpose of this study was to investigate changes in obstructive sleep apnoea (OSA) severity and awake velopharyngeal curvature in response to an anteriorly titrated mandibular position in 20 male OSA patients. Baseline supine cephalometry was obtained before the initial insertion of a titratable oral appliance and follow-up supine cephalometry was undertaken after titration of the mandibular position with the appliance in place. The mean apnoea/hypopnea index (AHI) before treatment (31.6 +/- 13.0 events x h(-1)) was significantly reduced (9.8 +/- 7.4 events x h(-1)) after titration of the mandibular position in all 20 patients. There was a significant increase in the anteroposterior calibre and the radius of the curvature of the anterior wall of the velopharynx in 14 good responders who exhibited an AHI reduction to < or = 15. Similar observations were not found in six poor responders. To conclude, an anteriorly titrated mandibular position reduced obstructive sleep apnoea severity, enlarged the velopharynx and diminished the curvature of the anterior velopharyngeal wall in good responders. It is proposed that this change in the upper airway curvature associated with mandibular advancement may effect obstructive sleep apnoea severity through its effect on airflow dynamics.
- Published
- 2004
- Full Text
- View/download PDF
195. [The Tübingen palatal plate--an innovative therapeutic concept in Pierre-Robin sequence].
- Author
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von Bodman A, Buchenau W, Bacher M, Arand J, Urschitz MS, and Poets CF
- Subjects
- Dental Prosthesis Design, Female, Follow-Up Studies, Humans, Infant, Infant, Newborn, Pierre Robin Syndrome complications, Polysomnography, Sleep Apnea, Obstructive diagnosis, Sleep Apnea, Obstructive therapy, Treatment Outcome, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency therapy, Palatal Obturators, Pierre Robin Syndrome therapy, Sleep Apnea, Obstructive congenital, Velopharyngeal Insufficiency congenital
- Abstract
Infants with Pierre-Robin sequence (PRS) may suffer severe upper airway obstruction resulting in hypoxemia that is difficult to treat. We are currently evaluating a new therapeutic approach involving an oral appliance that widens the pharynx by pulling the base of the tongue forward using a preepiglottic baton. Here we present a patient treated with this device who showed a decrease in his desaturation index from 50 to < 1.
- Published
- 2003
- Full Text
- View/download PDF
196. Velopharyngeal incompetence: a guide for clinical evaluation.
- Author
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Johns DF, Rohrich RJ, and Awada M
- Subjects
- Humans, Speech Disorders diagnosis, Speech Disorders physiopathology, Velopharyngeal Insufficiency etiology, Velopharyngeal Insufficiency physiopathology, Velopharyngeal Insufficiency diagnosis
- Abstract
Various causes of velopharyngeal disorders and the myriad of diagnostic methods used by speech-language pathologists and plastic surgeons for assessment are described in this article. Velopharyngeal incompetence occurs when the velum and lateral and posterior pharyngeal walls fail to separate the oral cavity from the nasal cavity during speech and deglutination. The functional goals of cleft palate operations are to facilitate normal speech and hearing without interfering with the facial growth of a child. Basic and helpful techniques are presented to help the cleft palate team identify preoperative or postoperative velopharyngeal incompetence. This information will enable any member of the multidisciplinary cleft palate team to better assist in the differential diagnosis and management of patients with speech disorders.
- Published
- 2003
- Full Text
- View/download PDF
197. Velopharyngeal insufficiency.
- Author
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Willging JP
- Subjects
- Adenoidectomy adverse effects, Child, Humans, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency genetics, Velopharyngeal Insufficiency prevention & control, Velopharyngeal Insufficiency therapy
- Abstract
Purpose of Review: Velopharyngeal insufficiency is a niche within our specialty, but patients with hypernasality present who have never been diagnosed previously. Otolaryngologists should be familiar with current trends in diagnosis and treatment of hypernasality., Recent Findings: Velopharyngeal insufficiency has been associated with genetic conditions and identifiable syndromes. Multiple surgical techniques are available for the treatment of this condition, the results of which vary widely in the literature. There is difficulty in interpreting the success of surgical outcomes on speech intelligibility and resonance because of the heterogeneity of the patient population and the subjective nature of assessing results. More studies are now available for the evaluation of associations of comorbid conditions and their impact on speech results., Summary: Velopharyngeal insufficiency must be diagnosed properly. Syndromes and comorbid conditions must be identified. No single specialty can care appropriately for these patients. A team approach is the ideal method of evaluating and managing patients with velopharyngeal insufficiency. Specialists with a particular interest and training in the management of patients with clefts of the palate and velopharyngeal insufficiency must collaborate to obtain the maximal functional outcome for these patients.
- Published
- 2003
- Full Text
- View/download PDF
198. [Idiopathic palatopharyngeal hemiparalysis].
- Author
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González Alvarez V, Costa Orvay JA, Guardia Camí MT, Garrido Romero R, Pineda Marfá M, and Luaces Cubells C
- Subjects
- Child, Preschool, Female, Glossopharyngeal Nerve Diseases complications, Humans, Paralysis complications, Velopharyngeal Insufficiency etiology, Vocal Cord Paralysis complications, Velopharyngeal Insufficiency diagnosis
- Abstract
Idiopathic velopalatine palsy is a condition of unknown etiology and is rarely seen in childhood. Consequently, diagnosis requires a high degree of suspicion. We report a case of sudden onset dysfunction of the lower cranial pairs (IX and X) in a 5-year-old girl who was previously asymptomatic. The clinical course was favorable and the results of complementary investigations were normal and the patient was diagnosed with velopalatine palsy. Based on this case, we aim to report our experience of this condition and provide a review of the literature. This disease should be suspected in patients aged between 5 and 15 years old who present a palsy of the IX and X cranial nerves of sudden onset and without any other symptoms in order to rationalize diagnostic and therapeutic tools. Treatment is based on support measures. The prognosis is excellent, with a high percentage of complete recovery and absence of recurrences.
- Published
- 2003
- Full Text
- View/download PDF
199. Management of velopharyngeal dysfunction: differential diagnosis for differential management.
- Author
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Marsh JL
- Subjects
- Airway Obstruction etiology, Airway Obstruction surgery, Algorithms, Articulation Disorders etiology, Articulation Disorders surgery, Child, Child, Preschool, Decision Trees, Diagnosis, Differential, Humans, Palatal Obturators, Palate, Soft abnormalities, Palate, Soft surgery, Pharyngeal Muscles innervation, Pharynx abnormalities, Pharynx surgery, Velopharyngeal Insufficiency complications, Velopharyngeal Insufficiency etiology, Oral Surgical Procedures methods, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency surgery
- Abstract
A single surgeon's 20 year experience in one cleft center with differential diagnosis for differential management of velopharyngeal dysfunction (VPD) is reviewed. The specific diagnostic and functional status of each affected individual is determined to select the method of VPD management. Two types of diagnostic evaluation of velopharyngeal function, perceptual and instrumental, are used to make that determination. There are four broad etiologic categories of VPD: anatomic deficiency, myoneural deficiency, anatomic and myoneural deficiency, and neither anatomical nor myoneural deficiency. The type of VPD management is specific for each etiologic category. The management options are between prosthetic appliances (lift, obturator, "liftorator") and operations (intravelar veloplasty, velar Z-plasty, pharyngeal flap, sphincter pharyngoplasty, posterior pharyngeal wall augmentation). The objective of differential management based on differential diagnosis is to optimize the function of the velopharynx for speech tasks while minimizing the morbidity of the intervention on the upper airway. A personal experience, in the context of an interdisciplinary cleft team, with such an approach over the past 20 years validates the assumption that differential management of VPD based on differential diagnosis can achieve this goal.
- Published
- 2003
- Full Text
- View/download PDF
200. Vasomotor instability in neonates with chromosome 22q11 deletion syndrome.
- Author
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Shashi V, Berry MN, and Hines MH
- Subjects
- Adult, DiGeorge Syndrome diagnosis, DiGeorge Syndrome genetics, Fatal Outcome, Female, Humans, Infant, Newborn, Male, Phenotype, Syndrome, Velopharyngeal Insufficiency diagnosis, Velopharyngeal Insufficiency genetics, Chromosomes, Human, Pair 22 genetics, Gene Deletion, Heart Defects, Congenital genetics, Hypotension genetics, Vasomotor System abnormalities
- Abstract
Approximately 70% of individuals with chromosome 22q11 deletion syndrome (22q11DS) have congenital heart defects. A host of other vascular problems in these patients, such as tortuous carotid arteries, Raynaud's phenomenon, unexplained hypotension, hypertension, and hypothermia, raise the possibility that there may be abnormal autonomic regulation of the vascular system. So far, however, there has been no formal report of autonomic dysfunction in patients with 22q11 deletion. We present two infants with 22q11DS, who had profound hypotension after uncomplicated surgeries for congenital heart disease. The hypotension was not responsive to vasopressor treatment (and extracorporeal membrane oxygenation in one infant) and resulted in death, due to multiorgan system failure. Obvious causes, such as poor cardiac contractility, prolonged circulatory arrest, neurological abnormality, sepsis and blood loss were excluded. On autopsy, no abnormalities were found that could explain the hypotension. We hypothesize that these infants died of severe hypotension due to abnormal vascular tone and that this is a variable feature in individuals with 22q11 deletion. The autonomic nervous system, which is responsible for the regulation of vasomotor tone, may be variably affected in 22q11DS. This could have implications for the surgical management of patients with 22q11DS. Further studies on this topic would establish or refute the association between 22q11DS and dysautonomia.
- Published
- 2003
- Full Text
- View/download PDF
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