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To determine predictors of success following Veau 1 and 2 cleft palate repair in patients with and without syndromes.Retrospective review of prospectively collected data.Tertiary care children's hospital.All children18 months of age undergoing Furlow palatoplasty for Veau 1 and 2 cleft repair between 2000 and 2014 with postoperative perceptual speech assessment (PSA).In total, 368 consecutive patients were identified; 95 were excluded, resulting in 273 patients. Median age at surgery was 13.0 months (interquartile range [IQR], 11-15 months) with postoperative PSA at a median of 32.3 months (IQR, 26.3-44.5 months). Fifty patients (18.3%) had syndrome diagnosis; 59 patients (21.6%) had nonsyndromic Robin sequence. Velopharyngeal insufficiency (VPI) occurred in 27 patients (10.5%); 13 underwent secondary speech surgery. Cleft-related speech errors occurred in 46 patients (17.6%). Non-cleft-related speech errors occurred in 155 patients (59.6%) and reduced intelligibility in 127 patients (47.9%). Oronasal fistula occurred in 23 patients (8.8%) and was exclusive to Veau 2 clefts. In multivariate analysis, age13 months at palatoplasty demonstrated a 6-fold higher rate of VPI (hazard ratio [HR], 6.64;VPI occurred in 10% of patients undergoing Furlow palatoplasty repair of Veau 1 or 2 clefts. Age13 months at palatoplasty was associated with improved speech outcomes and lower VPI incidence (2.8% vs 16.2%). Syndromic diagnosis was associated with noncleft speech errors and reduced intelligibility on univariate analysis but not velopharyngeal function after palatoplasty.

OBJECTIVES: To evaluate the performance of 4-dimensional computed tomography (4D-CT) in assessing upper airway obstruction (UAO) in patients with Robin Sequence (RS) and compare the accuracy and reliability of 4D-CT and flexible fiberoptic laryngoscopy (FFL). STUDY DESIGN: Prospective survey of retrospective clinical data. SETTING: Single, tertiary care pediatric hospital. METHODS: At initial and 30-day timepoints, a multidisciplinary group of 11 clinicians who treat RS rated UAO severity in 36 sets of 4D-CT visualizations and FFL videos (dynamic modalities), and static CT images. Raters assessed UAO at the velopharynx and oropharynx (1-none to 5-complete) and noted confidence levels (LOC) of each rating. Intraclass correlation and Krippendorff’s Alpha were used to assess intra- and inter-rater reliability, respectively. Accuracy was assessed by comparing clinician ratings with Quantitative Percent Constriction (QPC) Ratings, calculated based on 4D-CT airway cross-sectional area. Results were compared using Wilcoxon rank-sum and signed-rank tests. RESULTS: 4D-CT and FFL demonstrated similar intra-rater agreement (moderate to substantial) and both demonstrated fair inter-rater agreement. Both modalities underestimated UAO severity, though 4D-CT ratings were significantly more accurate, as determined by QPC similarity, than FFL (−1.06 and −1.46 vs QPC Ratings, p=0.004). Overall LOC was similar for 4D-CT and FFL, but other specialists were significantly less confident in FFL ratings than were otolaryngologists (2.25 and 3.92, p

(1) To define the minimal clinically important difference (MCID) of the Velopharyngeal Insufficiency (VPI) Effects on Life Outcomes (VELO) instrument, and (2) to test for the change in quality of life (QOL) after VPI surgery.Prospective observational cohort.VPI clinic at a tertiary pediatric medical center.Children with VPI and their parents completed the VELO instrument (higher score is better QOL) at enrollment and then underwent VPI surgery (Furlow palatoplasty or sphincter pharyngoplasty, n = 32), other treatments (obturator or oronasal fistula repair, n = 7), or no treatment (n = 18). They completed the VELO instrument again and an instrument of global rating of change in QOL at 1 year. The MCID was anchored to the global change instrument scores corresponding to "a little" or "somewhat" better. Within-group (paired t test) and between-group (Student t test) changes in VELO scores were tested for the VPI surgery and no treatment groups. The association between treatment group and change in VELO scores was tested with multivariate linear regression, adjusting for confounders.Follow-up was obtained for 37 of 57 (65%) patients. The mean (±standard deviation) change in VELO scores corresponding to the MCID anchor was 15 ± 13. The VELO score improved significantly more in the VPI surgery group (change, 22 ± 15; P.001) than in the no treatment group (change, 9 ± 12; P = .04), after adjusting for confounders (P = .007 between groups).VPI surgery using the Furlow palatoplasty or sphincter pharyngoplasty improves VPI-specific QOL, and the improvement is clinically important.

Ototoxicity associated with cis-platinum administration commonly presents as hearing loss and tinnitus. The hearing loss is usually an irreversible, high-frequency sensorineural loss. Histologic studies in humans and animals suggest that the outer hair cells (OHCs) are most susceptible to cis-platinum. Evoked otoacoustic emissions (EOAE), as a measure of outer hair cell function, are potentially useful in following ototoxic insults involving OHCs. Distortion-product otoacoustic emissions (DPOAE) test frequency-specific regions of the cochlea and therefore may be particularly well suited for monitoring ototoxic injuries. We measured distortion product otoacoustic emissions, at f2 = 2, 4, 6, 8, 10, and 12 kHz, in gerbils after a single large dose of cis-platinum. Animals treated with saline served as controls. The findings were compared to auditory brain stem evoked response (ABR) thresholds, using tone pips of the same frequencies. The DPOAE and ABR thresholds were measured before treatment and again 2, 5, and 14 days after drug administration. The changes in DPOAE were compared with the changes in ABR. No treatment effect was noted in the 2-day group. Animals treated with c/s-platinum demonstrated significant elevation of DPOAE and ABR thresholds compared with control animals at 5 and 14 days. There was no significant difference between the threshold changes in the 5-and 14-day groups.

Objective: In a study of patients with velopharyngeal insufficiency (VPI) diagnosed by pediatric speech pathologists, test the VPI Effects on Life Outcomes (VELO) instrument for criterion validity, construct validity, reliability, and responsiveness (ability to detect change in quality of life).Method: VPI subjects/parents completed the VELO at baseline, 2 weeks later, and 3 months posttreatment. Criterion-validity tested correlation between VELO score and VPI severity; construct-validity tested correlation with speech intelligibility. Test-retest reliability tested intraclass-correlation between baseline VELO score and 2-week score. Responsiveness tested mean baseline and posttreatment scores with the paired t test.Results: Parental responses are reported (n = 83); youth responses (obtained in 8+ year-olds only; n = 24) showed similar results. VPI severity (minimal n = 11, mild n = 31, moderate n = 27, severe n = 14) and speech intelligibility were rated by pediatric speech pathologists. The VELO instrum...

OSB can occur in the absence of an obvious contiguous source of infection. When a patient has persistent unilateral headache, elevated ESR, and radiographic evidence of a lytic skull-base lesion, the clinician should consider OSB as a potential diagnosis. A baseline gallium scan should be obtained before biopsy, since surgery or trauma can also produce positive results on radionuclide scans. Technetium-phosphate bone scans should also be performed before any surgical manipulation. However, positive results from a gallium or technetium scan in this setting are not conclusive evidence of infection. At biopsy, the otolaryngologist-head and neck surgeon should consider sending a specimen to the microbiology department for culture in addition to the specimen sent for routine pathologic study; this procedure could minimize delay in diagnosis. Establishing the diagnosis in these patients without obvious contiguous infection can be difficult, demanding perseverance and an appropriate index of suspicion. Once the diagnosis is confirmed, intravenous antibiotic therapy should begin immediately. The duration of therapy must be individualized; patients may require from 4 weeks to several months of treatment. Response to therapy is indicated by resolution of symptoms, normalization of ESR, and reversal of abnormalities on radionuclide scans. Serial gallium scans are particularly useful in following response to treatment.

Problem: Pediatric cochlear implant recipients have been historically assessed postoperatively with audiometric testing in a soundproof booth by trained audiologists. Assessment of functional outcome after cochlear implantation must also consider a number of other factors including speech perception, speech intelligibility, mode of communication, educational placement, as well as the social and psychological factors that affect one’s ability to function in the mainstream environment. No uniform tool for assessing effective functional outcome in the daily life of pediatric cochlear implant recipients has been developed. Methods: Using a cross-sectional design, the COCHLEA (Children’s Outcome for Cochlear Implant Hearing, Language, and Environmental Assessment) questionnaire, designed to measure functional outcomes in the pediatric cochlear implant population, was administered. The questionnaire was tested for reliability and validity, while investigating for any possible respondent or administrative burden. The parents of children with cochlear implants completed the questionnaire. The subjects were individuals with nonsyndromic sensorineural hearing loss (SNHL) implanted at Children’s Hospital and Regional Medical Center before 12/31/2001, aged 3–12 years. Implantation must have occurred before age 7. Non-English-speaking children/families were excluded secondary to a lack of resources. Results: Thirty-one patients were enrolled in the study. Preliminary results indicate that absolute scores of this outcome measure correlate with higher global health measures, including the Child Health Questionnaire (CHQ), and predict better audiometric scores. Conclusion: The COCHLEA functional outcome measure appears to be a reliable and valid method for assessing effective cochlear implantation. More research is necessary to determine this questionnaire’s sensitivity to longitudinal change. Significance: The COCHLEA questionnaire represents the first disease-specific functional outcome measure for the pediatric cochlear implant patient population. Ultimately it may be used to compare the effectiveness of cochlear implants and rehabilitation strategies. Support: None reported.