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To compare the prevalence of congenital cytomegalovirus (CMV) infection in Washington State in children with hearing loss (HL) and the general population and to compare the characteristics of HL in children with and without congenital CMV infection.Matched case-control; case cohort.Regional pediatric hospital, Washington State Department of Health (WSDOH).Cases were children 4 years and older with HL born in Washington State. Control individuals matched for demographic characteristics were identified at random through the WSDOH.Congenital CMV status determined using quantitative polymerase chain reaction testing on newborn heel stick blood spots archived by the WSDOH. Audiologic data were used to characterize HL.Congenital CMV testing was performed for 222 matched cases and controls. Congenital CMV infection was detected in 1.4% of controls and in 9.9% of cases (odds ratio, 10.5; 95% confidence interval, 2.6-92.4). An estimated 8.9% of HL in children in Washington can be attributed to CMV infection. After inclusion of an additional 132 children with HL (for a total of 354 cases in the case cohort), we observed that children with congenital CMV had more severe HL (P.001) and higher proportions of progressive (P = .02) and unilateral (P = .002) HL compared with children without congenital CMV infection. In the 35 children with congenital CMV infection, there was no relationship between neonatal CMV load and severity of HL.In Washington State, children with HL had a far higher prevalence of congenital CMV viremia than did the general pediatric population, and CMV infection seems to be responsible for an appreciable fraction of pediatric HL in Washington State.

Objective Describe airway management using nasopharyngeal airway in infants. Design Retrospective case series (1996 to 2006). Setting Tertiary pediatric hospital. Patients, Participants The craniofacial database of Seattle Children's Hospital was searched to identify patients with one of the following diagnoses: micrognathia, secondary cleft palate, branchial arch anomalies, Pierre Robin sequence (PRS), or velocardiofacial syndrome. Thirty-five (10.9%) of the 320 infants born between January 1, 1996, and March 31, 2006, identified using the criteria listed above were managed with nasopharyngeal airway (NPA) during infancy. Interventions Use of NPA. Main Outcome Measure Summary statistics describing the distribution of the infants' demographic characteristics, duration, and timing of their NPA placement, need for tracheotomy, feeding interventions, and death. Results Of the 35 patients included in this case series, 60% (21) were male. Eighteen (51.4%) patients had the diagnosis of PRS, 13 (37.1%) had secondary cleft palate and other craniofacial anomalies, and four (11.4%) had branchial anomalies and micrognathia (nonsyndromic or syndromic). Thirty-one children (88.6%) were born at term. Mean and median age at initial NPA placement was 3.2 and 1.3 weeks, respectively; median duration of NPA was 8.0 weeks. Nine children received tracheotomies. Feeding tubes were required in 85.7% of patients. Two children died; however, neither death was attributed to airway obstruction or the use of NPA. Conclusions NPA is one option in the management of patients with craniofacial anomalies and airway obstruction. The majority of nonsyndromic PRS patients treated with NPA during infancy did not require airway intervention beyond NPA.

To demonstrate the use of a state-maintained database (CHILD Profile) to monitor immunization status of pediatric cochlear implant recipients, and to assess compliance with current vaccination recommendations for cochlear implant users managed at Seattle Children's Hospital.Cross-sectional study.Tertiary academic pediatric hospital.Subjects were 260 patients with cochlear implants managed at Seattle Children's Hospital between July 1, 1995, and May 1, 2008. Patients were stratified by age groups (0-2 years, 2-5 years, 5-10 years,10 years). Using a statewide children's immunization registry (CHILD Profile), subjects were assessed with regard to their immunization status for Haemophilus influenzae type B vaccination (HiB), 7-valent pneumococcal conjugate vaccination (PCV-7), and 23-valent pneumococcal polysaccharide vaccine (PPV-23).Two hundred twenty-five of 260 subjects (87%) were registered in CHILD Profile; 126 of 225 (56%) were up to date with CDC recommendations for patients with cochlear implants. PPV-23 was the vaccination most likely to be incomplete. Age was predictive of immunization status only with HiB vaccination.A statewide immunization registry can be used to monitor the immunization status of cochlear implant recipients. Subjects were significantly more likely to be incomplete for PPV-23 than for either PCV-7 or HiB vaccinations.

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.

Summary Objectives (1) Identify the existence of semicircular canal bony dehiscence in the pediatric population; (2) determine the frequency of this finding in children over 3 years of age who have had temporal bone computed tomography (CT) imaging; (3) correlate the clinical history and audiological data to CT findings in this population. Design and setting Retrospective review of temporal bone CT scans performed at a tertiary care children's hospital and retrospective chart review. Patients Children over 3 years of age who underwent a temporal bone CT scan between January 2006 and December 2006. Results Review of 131 temporal bone CT scans using multiplanar imaging techniques revealed evidence of semicircular canal bony dehiscence in 18 children older than 3 years of age. Dehiscence of the superior semicircular canal was identified in 14 patients while dehiscence of the posterior semicircular canal was identified in 5 patients. One patient had both the superior and posterior semicircular canal dehiscences. Retrospective chart review comparing children with and without semicircular canal dehiscence showed no significant difference in clinical history or audiological data. Conclusions Semicircular canal dehiscence exists in the pediatric population. In this series, 18 of 131 temporal bone CT scans were positive for bony dehiscence of the superior or posterior semicircular canals. Further study is required to determine the clinical significance of this radiographic finding.

Velopharyngeal closure is required for normal speech production. Incomplete velopharyngeal closure manifests as resonance disorders and nasal air escape. Management of velopharyngeal insufficiency requires a general knowledge of speech production as well as a more detailed understanding of the velopharyngeal mechanism. Comprehensive evaluation by a velopharyngeal insufficiency team includes medical assessment focusing on airway obstructive symptoms, perceptual speech analysis, and instrumental assessment, which is utilized to characterize the velopharyngeal gap. Options for intervention include speech therapy, intraoral prosthetic devices, and surgery. Surgical interventions can be categorized as palatal, palatopharyngeal, or pharyngeal procedures. The therapeutic challenge lies in achieving velopharyngeal closure during speech production while maintaining patency of the upper airway. We present our protocol for evaluation of velopharyngeal function with a focus on indications for palatoplasty and pharyngoplasty. We also discuss surgical modifications of sphincter pharyngoplasty.

Objectives: We present an experience in the management of primary and recurrent thyroglossal duct cysts (TGDCs) and describe a novel method for recurrent TGDC removal. Methods: We performed a retrospective review of TGDC surgery at Children's Hospital in Seattle from 1980 to 2003. The surgical techniques for primary and recurrent TGDCs and the factors associated with TGDC recurrence were evaluated and analyzed. Results: During the study period, 231 patients underwent 296 TGDC surgeries. Thirty-four of the 231 patients (15%) underwent a total of 88 procedures for recurrent TGDCs. Successful procedures used for secondary TGDC management included central neck dissection with directed base of tongue (BOT) excision in 6 of 9 patients (67%), secondary Sistrunk operation with limited BOT resection in 12 of 27 patients (44%), revision Sistrunk operation with BOT dissection in 7 of 11 patients (64%), and suture-guided transhyoid pharyngotomy in 8 of 8 patients (100%). Ten of the 231 patients (4%) had initial TGDC incision and drainage and then underwent a total of 21 procedures, excluding the incision and drainage. The factors associated with TGDC recurrence were inaccurate initial diagnosis (17 of 34 or 50%), infection (5 of 34 or 15%), unusual TGDC presentation (5 of 34 or 15%), and lack of BOT musculature removal (7 of 34 or 20%). The level of surgeon training affected the surgical outcome. Conclusions: Successful TGDC treatment requires consideration of factors associated with recurrence. Recurrent TGDCs can be treated by several methods, including suture-guided transhyoid pharyngotomy.

The objectives of the study were to describe speech outcomes in a large series of patients undergoing Furlow palatoplasty for management of velopharyngeal insufficiency and to test whether preoperative velopharyngeal gap size and other patient characteristics significantly affect those outcomes.Data collected included age at the time of surgery, surgeon, type of cleft, syndrome diagnosis, preoperative velopharyngeal gap size as determined by videonasendoscopy, and preoperative and postoperative perceptual speech assessments. Descriptive statistics were generated and ordinal logistic regression on the outcome variable, postoperative velopharyngeal insufficiency severity score, was performed.In this series of 154 patients, 148 had complete perceptual speech data. Of these 148 patients, 72 percent had improvement in velopharyngeal insufficiency severity after the procedure and 56 percent had complete resolution of velopharyngeal insufficiency. Postoperative insufficiency was scored as none or minimal (i.e., resolution) in 38 of 52 patients (73 percent) with a small preoperative velopharyngeal gap, 26 of 51 patients (51 percent) with a moderate preoperative gap, and four of 21 patients (19 percent) with a large preoperative gap. Preoperative velopharyngeal gap size was significantly associated (p0.0001) with postoperative insufficiency on ordinal multivariate logistic regression after controlling for preoperative insufficiency and other covariates. There was not a significant association between syndrome diagnosis, age at Furlow palatoplasty (younger than 5 years versus older), gender, surgeon, or presence of submucous cleft palate and postoperative speech outcome, in either the unadjusted or adjusted analyses.Preoperative velopharyngeal gap size, as determined with nasendoscopy, was significantly associated with postoperative velopharyngeal insufficiency severity after Furlow palatoplasty. Small gap size is associated with a greater likelihood of resolution.

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.Retrospective review of consecutive patients with VPI at a tertiary care children's hospital, assessed with NE and MVF between 1996 and 2003.177 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) age5 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).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.B-2b.

Objectives/Hypothesis: Congenital severe to profound sensorineural hearing loss (SNHL) is found in higher proportions of children with minority and/or lower socioeconomic status (SES). Cochlear implants were approved by the U.S. Food and Drug Administration for use in children with bilateral severe to profound SNHL in 1990. The objectives of the study were as follows: 1) to study the epidemiology of pediatric cochlear implantation, assessing whether cochlear implant technology is provided to children with severe to profound SNHL in proportion to their racial/ethnic or SES, and 2) to compare data provided by a national health care database with data provided by cochlear implant manufacturers. Study Design: Patients aged 0 to 18 years who underwent cochlear implantation in 1997 using a cross-sectional study design. Methods: Analyses were made of pediatric cochlear implant patients, using data from the 1997 Health Care and Utilization Project/Kids' Inpatient Database. Relative rates of implantation compared with rates of severe to profound SNHL were calculated using national estimates generated from census and Galludet Research Institution data. Logistic regression analysis was carried out to compare implanted children of different racial/ethnic backgrounds. A surrogate measure of socioeconomic status was used based on the median household income of the patient's home ZIP code. Information was also obtained from the two companies producing U.S. Food and Drug Administration–approved cochlear implants in 1997 and used to determine whether the data obtained from the Health Care and Utilization Project/Kids' Inpatient Database were representative of the national cohort of implanted children. Results: The Health Care and Utilization Project/Kids' Inpatient Database identified 124 children who underwent cochlear implant surgery in 1997. White and Asian children were implanted at higher rates than Hispanic and black children. Furthermore, white and Asian children received implants at greater rates than would be expected based on prevalence of severe to profound SNHL. The relative rate (RR) of implantation, defined as the proportion of children who received cochlear implants divided by the proportion of children with severe to profound SNHL (in each race/ethnicity group compared with the same ratio in white children), was similar in white (RR = 1.00) and Asian (RR = 0.93) children but markedly different in Hispanic (RR = 0.28) and black (RR = 0.10) children. Comparison of SES information from the Health Care and Utilization Project/Kids' Inpatient Database population with the manufacturers' database suggested that the Health Care and Utilization Project/Kids' Inpatient Database is representative of all implanted children in the United States. Both sources of information suggested that children receiving cochlear implants in the United States in 1997 resided in above-average SES areas. Conclusion: White and Asian children with severe to profound SNHL had higher proportionate rates of cochlear implantation than black and Hispanic children in 1997. Implanted children were more likely to live in areas (represented by ZIP codes) with higher median incomes. Although there was a disparity in rate of cochlear implantation based on race/ethnicity and surrogate measures of SES, these data did not allow the authors to determine the causes for these differences.

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.

Objective : To evaluate speech outcomes and complications of sphincter pharyngoplasty and pharyngeal flap performed for management of velopharyngeal insufficiency (VPI). Design : Case series. Setting : Tertiary care children’s hospital. Patients : All patients who underwent pharyngeal flap or sphincter pharyngoplasty from 1990 to 1995. Methods : Perceptual speech analysis was used to assess severity of VPI, presence of nasal air emissions and quality of nasal resonance (hyper, hypo, or normal). Pre-operative measures of velopharyngeal function were based upon nasendoscopy and videofluoroscopic speech assessment. Recommendations for management were made by the attending surgeon. Complications of hyponasality and obstructive sleep symptoms (OSS) were noted. Patient characteristics were compared using univariate analysis. Results : Sixteen patients underwent sphincter pharyngoplasty and 18 patients underwent superiorly based pharyngeal flap. Patients were similar in terms of lateral pharyngeal wall medial motion and palatal elevation. The groups were also similar with regard to VPI severity, though there was a trend for more severe VPI in patients undergoing sphincter pharyngoplasty than pharyngeal flap (50 vs. 33.3%, respectively). Patients with pharyngoplasty had a higher rate of resolution of VPI than those who had pharyngeal flap (50 vs. 22.2%, respectively), although this was not statistically significant. Post-operative hyponasality and obstructive sleep symptoms were present in both groups. However, only patients who underwent PF and had postoperative OSS had obstructive sleep apnea (OSA). Conclusions : There were no detectable anatomic differences between treatment groups implying that treatment selection during the study period was not guided by strict anatomic criteria. Sphincter pharyngoplasty may have a higher success rate with a lower risk of OSS.

Destruction of the cochlea causes secondary changes in the central auditory pathway through transynaptic regulation. These changes appear to be mediated by an activity-dependent process and can be detected in the avian auditory system as early as 30 minutes after deafferentation. We compared the early changes in cochlear nucleus neurons following deafferentation by cochlea ablation with those seen following activity deprivation by perilymphatic tetrodotoxin (TTX) exposure. Protein synthesis and size of large spherical cells in the anteroventral cochlear nucleus (AVCN) of 14-day-old gerbils were measured during the first 48 hours after the manipulations. Both cochlea ablation and TTX produced a reliable decrease in protein synthesis by AVCN neurons (30–-40%) by 1 hour. The magnitude of change in tritiated leucine incorporation was similar at all survival times, in both experimental groups. In contrast to the rapid changes in protein synthesis, the decrease in cell size was first evident 18 hours after TTX exposure and 48 hours after cochlea ablation. There was no significant change in protein synthesis or cell size in control groups at any of the survival times. These findings are consistent with changes in the avian auditory system in response to deafferentation and TTX exposure. Cochlea ablation and TTX exposure induced similar transneuronal changes, supporting the hypotheses that activity of auditory afferents in young mammals plays a regulatory role in the metabolism and morphology of their target neurons in the central auditory pathway, and that early changes following destruction of the peripheral receptor are due to reduction of activity-dependent interactions of presynaptic and postsynaptic cells.

To study routine culture-negative persistent cervical lymphadenitis in children treated surgically during a 10-year period (December 26, 1997, to October 1, 2007) at a single institution.Retrospective case series.Tertiary university-based pediatric referral center.Patients 18 years or younger with cervical lymphadenitis managed surgically (incision and drainage, curettage, and/or excisional lymphadenectomy) and medically (antibiotic therapy), culture-negative after 48 hours, and subsequently diagnosed using the polymerase chain reaction, extended culture incubation, and/or histopathologic evaluation.Number of surgical interventions, causative organisms, histopathologic features, and resolution of lymphadenitis.Ninety surgical procedures were performed in 60 patients. The cure rate was 23% (approximately 14 patients) with incision and drainage, 58% (approximately 35 patients) with curettage, and 95% (57 patients) with excisional lymphadenectomy. Nontuberculous mycobacteria were the most prevalent causative organisms, followed by Bartonella and Legionella organisms. Four of 6 patients with Bartonella infection had a history of cat exposure, and 4 of 6 patients with Legionella infection had a history of hot tub exposure.Excisional lymphadenectomy is the preferred treatment of mycobacterial persistent cervical lymphadenitis in children. Sufficient data are lacking for similar recommendations in patients with disease caused by Bartonella organisms, whereas for neck disease caused by Legionella organisms, excisional lymphadenectomy may be superior to incision and drainage. The polymerase chain reaction is useful for pathogen identification in pediatric cervical lymphadenitis, although it is less sensitive in identification of mycobacteria. To our knowledge, our study is the first to report multiple cases of legionellosis in otherwise healthy children. Legionella seems to be a previously unrecognized but relatively common pathogen in culture-negative persistent cervical lymphadenitis in children.

Gorham-Stout syndrome, or disappearing bone disease, is characterized by the proliferation of thin-walled vascular channels associated with regional osteolysis. There have been fewer than 150 cases reported in the literature. In this clinical report, we describe 2 additional cases of Gorham-Stout syndrome affecting the maxillofacial skeleton. We provide a review of the clinical diagnosis of this syndrome and describe treatment options.