Design  Retrospective review of patient medical records and histopathologic test results from January 1, 1998, through December 31, 2008.

Setting  Children's Hospital of Wisconsin.

Patients  Patients with a history of operation treated for recurrent respiratory papillomatosis.

Main Outcome Measures  The presence or absence of dysplasia identified in a papilloma specimen and patient characteristics, such as age of initial presentation, number of operations, tobacco exposure, treatment for reflux, and use of cidofovir, were quantified.

Results  Treatment for recurrent respiratory papillomatosis was identified in 21 patients. Age at initial diagnosis ranged from 8 months to 14 years. A total of 123 recurrent respiratory papillomatosis specimens in 20 patients were identified. Dysplasia was seen in less than 1% of samples (1/123), which represents 5% of total patients. Seven patients (35%) received cidofovir treatment and none of them developed dysplasia. These data demonstrate a lack of correlation between cidofovir treatment and dysplasia, with the P value being nonsignificant (Fisher exact test, P=.4).

Conclusion  Dysplasia is a rare event in pediatric recurrent respiratory papillomatosis, and there does not appear to be an association between the use of cidofovir and dysplastic changes.

Design  Retrospective study from January 1, 1990, through April 30, 2008, with a mean follow-up of 5.5 years (range, 1 month to 17 years).

Setting  Tertiary academic center.

Patients  Four hundred thirty-four patients younger than 16 years. Mean age at CI was 4.7 (range, 0.6-16.0) years. Forty-one children (9.4%) underwent CI when younger than 24 months. Forty-three (9.9%) had inner ear malformations.

Main Outcome Measures  Complications after CI, classified into early (0-8 days) or delayed (>8 days) and major or minor. Spontaneous failures of internal devices were excluded. Correlation to age at CI, local trauma, and inner ear malformations were analyzed using the ² test.

Results  Forty-three patients (9.9%) experienced complications. Delayed complications occurred in 28 patients (65.1%), with a mean delay of 2.2 (range, 0.1-8.4) years. Twenty-four patients (5.5%) had major complications, consisting of severe cutaneous infections (15 patients), magnet displacement (3), meningitis (2), cholesteatoma (2), cerebrospinal fluid leak (1), and electrode misplacement (1). Nineteen (4.4%) had minor complications, consisting of vertigo (9 patients), soft-tissue infection (5), persistent otitis media (4), and facial palsy (1). Complications led to reimplantation in 13 of the 43 patients (30.2%). Trauma to the mastoid area (14 patients) and inner ear malformations (51) were highly correlated with major delayed complications (P < .001) and early minor complications (P < .001), respectively. Young age at CI was not correlated with any type of complication.

Conclusions  Complications of CI in children are common, with trauma as a major factor. Inner ear malformations should prompt specific preventive management. Cochlear implantation in young children did not appear to be a risk factor in this study.

Design  Cross-sectional study.

Setting  Pediatric tertiary care cochlear implant center.

Patients  All children using bilateral cochlear implants who were followed up during a 4-month period at our implant center were invited to participate. Twenty-seven children (17 males and 10 females) aged 3 to 15 years were enrolled. Causes of deafness included congenital (n = 8), genetic (n = 8), meningitis (n = 3), cytomegalovirus (n = 2), and unknown (n = 6). The interval between first and second implantations ranged from simultaneous to 7.8 years (mean, 4.2 years).

Main Outcome Measures  Children completed acoustic voice testing using a Computerized Speech Lab and a Multi-Dimensional Voice Program. Acoustic results were compared with those of children receiving unilateral implants and with normative data.

Results  Compared with established pediatric normative data, children with bilateral implants demonstrated poor control of long-term frequency perturbation and long-term amplitude perturbation when vocalizing sustained phonations (P < .001 for both). This finding was consistent with data previously reported in children using unilateral cochlear implants. Long-term control of frequency perturbation improved as children used their bilateral cochlear implants over time and was significantly influenced by overall time in sound (P = .02).

Conclusions  Similar to unilateral cochlear implant users, children using bilateral implants have difficulty controlling long-term frequency perturbation and long-term amplitude perturbation during sustained phonations. These measures improved as the duration of usable hearing increases.

Design  Patients were presented with narrowband noise and speech-in-noise tasks while undergoing fMRI of the brain. In the narrowband noise task, 5 chirps at center frequencies of 250 Hz, 500 Hz, 1 kHz, 2 kHz, and 4 kHz were presented monaurally for 1 second in a randomized order to children in both groups. In the speech-in-noise task, Bamford-Kowal-Bench (BKB) sentences were presented over 4-talker babble to both ears, and scans were acquired after each stimulus. We compared fMRI data across groups using independent component analysis and Bayesian (hierarchical) linear models.

Setting  Tertiary referral center.

Patients  Twelve children with USNHL and 23 normally hearing controls.

Interventions  Perform fMRI while subject listens to narrowband and speech-in-noise tasks.

Main Outcome Measures  Neuroanatomic differences in fMRI.

Results  In the narrowband noise task, children with USNHL had less activation of auditory areas and failed to activate auditory association areas and attention networks compared with normally hearing controls. In the speech-in-noise task, children with USNHL activated only secondary auditory processing areas in the left hemisphere, while controls activated these areas bilaterally. Children with right-sided USNHL failed to activate attention areas that were activated in controls and in children with left-sided USNHL. Only children with left-sided USNHL activated bilateral visual association areas.

Conclusions  Results show significant differences in the cortical processing of sound between children with severe to profound USNHL and normally hearing children. These differences may account for the functional auditory problems that children with USNHL experience.

Design  Prospective cohort study.

Setting  Tertiary care children's hospital.

Patients  Consecutive series of 36 children with tracheostomy and 72 general pediatric otolaryngology patients without tracheostomy.

Interventions  A pediatric constipation questionnaire (from previously published references) was given to parents of the study participants. Data were collected regarding patient medications, neurological status, and use of positive pressure airway assistance, speaking valves, and feeding tubes. Data were evaluated using ² and t tests. Logistic regression analysis was used to search for independent variables impacting presence of constipation.

Results  The mean ages for the tracheostomy and control groups were 6.8 and 4.7 years, respectively (P = .07). A history of constipation was elicited in 60% of children with tracheostomy compared with only 16.7% of controls (P < .001). More patients with tracheostomy tubes (80.0%) than controls (20.8%) were taking medication to treat constipation (P < .001). Constipation was also significantly associated with older age (P = .02), use of medications with constipation as a known adverse effect (P = .02), and the presence of neurodevelopmental impairment (P < .001). Constipation was still independently associated with the presence of a tracheostomy tube when correcting for age and the use of constipation-causing medications. When controlling for neurodevelopmental impairment, the presence of a tracheostomy tube was not proven to be an independent predictor of constipation.

Conclusion  Children with tracheostomy tubes are more likely to have a history of constipation, although a causal relationship between tracheostomy and constipation could not be determined due to the potentially confounding variable of neurodevelopmental delay.

Design  Retrospective medical record review.

Setting  Tertiary care university hospital.

Patients  Thirty-eight children with salivary gland disorders undergoing diagnostic and interventional sialendoscopy between January 1, 2003, and November 30, 2008.

Intervention  Diagnostic and interventional sialendoscopy using general anesthesia.

Main Outcome Measures  Demographic, clinical, and surgical variables, including age, sex, date of first symptoms, parotid or submandibular location of disease, preoperative ultrasonographic results, sialendoscopy technique, sialendoscopy observations, and complications.

Results  Pediatric sialendoscopy was performed on the parotid gland in 23 patients (61%) and on the submandibular gland in 15 patients (39%). The most frequent indication for sialendoscopy was recurrent salivary gland swelling. Thirty-two of 38 procedures (84%) were performed endoscopically, whereas a combined intervention was necessary for 3 patients and a submandibular gland excision for another 3 patients. Sialendoscopy allowed the diagnosis of 12 patients with salivary duct lithiasis, 21 with salivary duct stenosis, and 2 with both submandibular lithiasis and stenosis, and findings from 3 sialendoscopies were normal. Preoperative ultrasonographic results were confirmed by sialendoscopy in only 7 patients. Of the 10 patients with lithiasis found using sialendoscopy, only 4 had been detected using preoperative ultrasonography.

Conclusions  Sialendoscopy is a pertinent technique for the diagnosis and treatment of salivary gland disorders in children. It also allows the most conservative treatment of sialolithiasis and juvenile recurrent parotitis.

Design  In vitro mMEEC model.

Setting  Center for Genetic Medicine Research, Children's National Medical Center, Washington, DC.

Participants  Cells from the immortalized mMEEC line.

Main Outcome Measures  Cell viability, the quantity of Muc5b messenger RNA abundance, and Muc5b promoter activity.

Results  The 3-(4,5-dimethylthiazoyl-2-yl)-2,5-diphenyltetrazolium bromide assays demonstrated an acidic dose-dependent decrease in cell survival, with pH less than 4 significantly decreasing viability at 1 hour. Pepsin had a mild protective effect up to 8 hours, with greater cell viability, in the pH range of 5.0 to 7.6. Reverse-transcriptase polymerase chain reaction demonstrated induction of Muc5b messenger RNA levels over controls after exposure to acidic pH levels of 5.7, and 4 with and without pepsin. Similarly, a pH of 4.0 significantly increased Muc5b promoter activation 5.4-fold. Pepsin at neutral or acidic pH values did not significantly alter Muc5b expression or promoter activity.

Conclusions  Despite decreasing cell viability, acidic pH drives middle ear epithelial Muc5b gene expression in vitro, which perhaps explains how laryngopharyngeal reflux can contribute to otitis media. Pepsin at neutral or acidic pH levels had minimal effects on Muc5b gene expression; thus, although pepsin may be a useful marker for detecting the presence of reflux, our results suggest that acid itself is a more likely pathologic component of gastric juice in the middle ear.

Design  Diagnostic lateral cephalometric imaging study that took place from January 1, 2006, to December 31, 2007.

Setting  American University of Beirut Medical Center.

Patients  Children with chronic mouth breathing referred by a pediatric otolaryngologist for cephalometric evaluation by participating orthodontists.

Main Outcome Measures  Two distances were measured on the digitized lateral cephalographs between the adenoid and soft palate: the shortest adenoid distance (SAD) and the most convex adenoid distance (CAD). The palatal airway was assessed on a grade-1 to grade-3 scale independently by the referring otolaryngologist and an orthodontist.

Results  A total of 200 children were included in the study (127 boys and 73 girls; mean age, 6 years; age range, 1.71-12.62 years). High correlations were observed between the airway ratings gathered by both examiners (r = 0.96) and between SAD and CAD (r = 0.92). Significant correlations were noted between the palatal airway grade and the SAD and CAD measurements (r = –0.73 and r = –0.79, respectively). Shortest adenoid distance measures of 2 mm or less corresponded mostly to grade 3 obstruction and were more prevalent in patients younger than 6 years. Age was inversely proportional to both the grade and SAD (P < .001).

Conclusions  Both methods are reliable for assessment of airway obstruction by the adenoid. Because SAD and CAD are highly correlated, we recommend the use of SAD as a more readily identifiable distance on cephalometric radiographs. Removal of adenoids when SAD is less than 2 mm may be indicated because this condition reflects a severe airway obstruction associated with potential changes in dentofacial structure.

Design  Guinea pig model.

Setting  University of Utah otolaryngology research labs.

Participants  Thirty-one Hartley guinea pig pups were divided into 4 groups. Group 1 pups were delivered from pregnant dams inoculated with 1 x 10⁵ plaque-forming units (PFU) of guinea pig CMV (gpCMV). Group 2 and group 3 pups were delivered from pregnant dams inoculated with higher doses of 2 and 4 x 10⁵ PFU of gpCMV, respectively. Group 4 pups, the control group, were delivered from uninoculated dams.

Main Outcome Measures  All groups underwent weekly auditory brainstem response studies. Six weeks after delivery, the brain, cochlea, salivary glands, lungs, liver, and kidneys were harvested. All tissue except the cochlea was analyzed for histologic evidence of the virus. All tissue underwent polymerase chain reaction (PCR) to detect gpCMV.

Results  Seven of the 19 (37%) inoculated pups developed a 30-dB hearing loss; none of the animals in the control group had a worse click threshold than 20 dB. Group 1 pups demonstrated statistically significant asymmetric hearing loss. All 3 inoculated groups showed evidence of progressive hearing loss over time. The control group did not demonstrate evidence of progressive threshold worsening. The PCR testing detected gpCMV in the cochleas of group 2 and group 3 animals.

Conclusions  We have successfully demonstrated elevated auditory brainstem response click thresholds with characteristics of progressive and asymmetric loss that have been reported in clinical reports of congenital CMV infection. We also detected gpCMV via PCR testing in the cochleas of inoculated pups.

Design  Randomized controlled animal study.

Subjects  Eight Sus scrofa piglets (15-20 kg) randomly intubated with either a standard or a modified uncuffed ETT.

Interventions  The modified ETT was handcrafted by gluing and then trimming dry polyvinyl acetate foam circumferentially to the distal end of a standard uncuffed ETT. After intubation, the foam quickly self-expanded as it absorbed the secretions of the laryngopharynx and adopted the shape of the intraluminal airway. This conforming shape also sealed the larynx to allow for positive pressure ventilation. Both groups were intubated for 4 hours under constant hypoxic conditions (mean oxygen saturation <70%) to enhance and accelerate intubation damage. They were then humanely killed, and the larynx and trachea were harvested for histologic examination.

Main Outcome Measures  The severity of laryngeal injury graded on a scale from 0 to 4 (0 indicates normal; 1, epithelial compression; 2, epithelial loss; 3, subepithelial and glandular necrosis; and 4, perichondrium involvement).

Results  All of the specimens histologically demonstrated areas of inflammation and epithelial loss. The standard ETT caused substantial deep damage, with a mean (SD) severity score of 2.79 (0.74). The modified ETT caused mainly superficial damage, with a mean (SD) severity score of 1.65 (0.56) (P < .001).

Conclusion  The modified ETT objectively caused less laryngotracheal damage compared with the standard ETT and may be of potential clinical benefit.

Design  Retrospective medical record review.

Setting  Tertiary care children's hospital.

Patients  Seventy-one patients underwent 84 procedures (22 ssLTRs and 62 dsLTRs).

Intervention  Review of preoperative disease severity and surgical outcomes for patients who underwent ssLTR vs dsLTR.

Main Outcome Measure  Operation-specific and overall decannulation rates.

Results  Regarding ssLTRs, the mean grade of subglottic stenosis was 2.1 and the overall and operation-specific decannulation rates were 100% and 91%, respectively. The mean grade of subglottic stenosis for double-stage procedures was 2.9, and the overall and operation-specific decannulation rates were 93% and 68%, respectively. Patients who underwent ssLTR and dsLTR were further divided into early and late groups based on whether the posterior graft was sutured in place (early) or not (late). Overall and operation-specific decannulation rates were 100% and 89%, respectively, for the early single-stage group and 100% and 92% for the late group. Regarding the dsLTR group, overall and operation-specific decannulation rates were 88% and 42%, respectively, for the early group and 95% and 79% for the late group. Among all groups, there was no significant difference in overall decannulation rates (P > .05). Single-stage LTR offered an increased rate of operation-specific decannulation over dsLTR (P < .05). However, that difference was not significant between the late ssLTR and the late dsLTR groups (P > .05).

Conclusion  Careful assessment of preoperative disease severity and overall medical status will help surgeons choose between ssLTR and dsLTR, maximizing patient outcomes for both modalities.

Design  A 6-year retrospective medical record review.

Setting  Tertiary care hospital.

Patients  Thirty-one infants younger than 1 year with complete data from preoperative FFLT and microlaryngoscopy and bronchoscopy (MLB) were examined.

Main Outcome Measures  Results from 241 MLB procedures were reviewed.

Results  Laryngomalacia (LM) and tracheomalacia (TM) were identified more often by FFLT than by MLB. In particular, the detection rate for LM and TM by MLB, as seen preoperatively by FFLT, was 79% and 61%, respectively. Compared with FFLT, MLB accurately diagnosed the severity of LM and TM only 55% and 65%, respectively, of the time. Fiberoptic laryngoscopy revealed synchronous airway lesions in 62% of infants with LM, while MLB discovered synchronous airway lesions in 54%. Static airway lesions were more frequently diagnosed with MLB. No respiratory events occurred during FFLT.

Conclusions  In an appropriate patient, FFLT is a safe and effective diagnostic tool for common infant tracheal and laryngeal abnormalities. Detection and characterization of dynamic airway lesions is better achieved by FFLT than by MLB.

Design  Retrospective case note study (2005-2009).

Setting  Department of Otolaryngology–Head and Neck Surgery, Armand Trousseau Children's Hospital, Paris, France.

Patients  Eleven patients who underwent endoscopic cleft closure as a primary (n = 8) or secondary (n = 3) procedure among 22 patients treated for laryngotracheal clefts. We report patients' demographics, symptoms leading to the diagnosis, endoscopic evaluation method, medical examination results, and surgical techniques.

Intervention  Endoscopic closure of the cleft under spontaneous ventilation via 2-layer interrupted sutures after excision of the mucosal edge using a carbon dioxide laser in 10 patients and a thulium laser in 1.

Main Outcome Measures  Analysis of postoperative complications, revision surgery, need for intensive care unit admission, closure of the cleft, and long-term symptom results.

Results  Successful closure of the 11 clefts (with revision surgery in 3 patients) without the need for intubation or intensive care unit admission enabled the elimination of aspiration in 10 patients and significant improvement in 1 patient with bilateral vocal cord paralysis. There were no significant postoperative complications.

Conclusions  Endoscopic closure of laryngotracheal clefts is a reliable technique that significantly reduces perioperative and postoperative morbidity. The results of this technique are entirely satisfactory, and we suggest that it is suitable as a primary procedure for the treatment of type I, II, and III clefts extending to the cervical trachea, including in neonates.

Design  A retrospective medical chart review was conducted from 2001 to 2006.

Setting  Tertiary care center.

Patients  Pediatric patients aged 3 to 17 years (mean age, 11 years) who presented with prolonged dysphonia. All patients were previously examined by flexible laryngoscopy and treated with speech therapy for a presumed diagnosis of vocal cord nodules.

Interventions  Flexible or rigid VLS was performed.

Main Outcome Measure  The diagnosis per patient established after VLS.

Results  Eighty patients were included in the study: 50 underwent rigid VLS; 28 underwent flexible VLS; and 2 did not tolerate either procedure. A total of 132 diagnoses were made, including 68 benign mucosal diseases (41 nodules, 15 polyps, 8 cysts, and 4 sulci), 41 inflammatory disorders, 11 functional disorders, 6 congenital disorders, 4 traumatic injuries, and 2 neurologic disorders. Many patients received more than 1 intervention for their dysphonia, including antireflux medication and speech therapy, but 16 patients also underwent phonomicrosurgery.

Conclusions  Patients with a history of prolonged dysphonia for whom treatment has failed should be referred for evaluation by VLS. Videolaryngostroboscopy elucidates subtle features of different disease processes; clarifies the differences between benign mucosal disorders that might require surgical intervention; and helps identify inflammatory processes that contribute to dysphonia. To our knowledge, these findings have not previously been reported in the pediatric population. Although most pediatric dysphonia can be attributed to benign nodules, our results show that inflammatory conditions and benign lesions other than nodules contribute to dysphonia and are often overlooked and undertreated.

Design  Prospective, observational study.

Setting  Tertiary care children's hospital.

Patients  Infants and children with sensorineural hearing loss (SNHL).

Intervention  Sequencing of the GJB2 (connexin 26) gene.

Main Outcome Measures  Degree and progression of SNHL.

Results  From December 1, 1998, through November 30, 2006, 126 children with biallelic GJB2 mutations were identified. Of the 30 different mutations identified, 13 (43%) were truncating and 17 (57%) were nontruncating; 62 patients had 2 truncating, 30 had 1 truncating and 1 nontruncating, and 17 had 2 nontruncating mutations. Eighty-four patients (67%) initially had measurable hearing in the mild to severe range in at least 1 of 4 frequencies (500, 1000, 2000, or 4000 Hz). Of these 84 patients with residual hearing, 47 (56%) had some degree of progressive hearing loss. Patients with 2 truncating mutations had significantly worse hearing compared with all other groups. Patients who had 1 or 2 copies of either an M34T or a V37I allele had the mildest hearing loss.

Conclusions  Hearing loss owing to GJB2 mutations ranges from mild to profound and is usually congenital. More than 50% of patients will experience some hearing loss progression, generally gradually but occasionally precipitously. Hearing loss severity may be influenced by genetic factors, such as the degree of preserved protein function in nontruncating mutations, whereas hearing loss progression may be dependent on factors other than the connexin 26 protein. Genetic counseling for patients with GJB2 mutations should include the variable audiologic phenotype and the possibility of progression.