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Supplemental Digital Content is available in the text., Objectives: Early detection of neonatal hearing impairment moderates the negative effects on speech and language development. Universal neonatal hearing screening protocols vary in tests used, timing of testing and the number of stages of screening. This study estimated the cost-effectiveness of various protocols in the preparation of implementation of neonatal hearing screening in Albania. Design: A micro-simulation model was developed using input on demography, natural history of neonatal hearing impairment, screening characteristics and treatment. Parameter values were derived from a review of the literature and expert opinion. We simulated multiple protocols using otoacoustic emissions (OAE) and automated auditory brainstem response (aABR), varying the test type, timing and number of stages. Cost-effectiveness was analyzed over a life-time horizon. Results: The two best protocols for well infants were OAE followed by aABR (i.e., two-stage OAE-aABR) testing in the maternity ward and single-aABR testing. Incremental cost-effectiveness ratios were €4181 and €78,077 per quality-adjusted life-year gained, respectively. Single-aABR screening led to more cases being detected compared to a two-stage screening program. However, it also resulted in higher referral rates, which increased the total costs of diagnostics. Multi-staged screening decreased referral rates but may increase the number of missed cases due to false-negative test results and nonattendance. Conclusions: Only the 2-stage OAE-aABR (maternity ward) protocol was below the willingness-to-pay threshold of €10,413 for Albania, as suggested by the World Health Organization, and was found to be cost-effective. This study is among the few to assess neonatal hearing screening programs over a life-time horizon and the first to predict the cost-effectiveness of multiple screening scenarios.

Purpose Prolonged endotracheal intubation may lead to laryngeal damage, with stridor being the most relevant clinical symptom. Our objective was to determine the incidence of post-extubation stridor and their clinical consequences in children within a tertiary referral center and to identify contributing factors. Methods 150 children, aged 0–16 years, intubated for more than 24 h were prospectively enrolled until discharge of the hospital. Potential relevant factors, thought to mediate the risk of laryngeal damage, were recorded and analyzed. Results The median duration of intubation was 4 days, ranging from 1 to 31 days. Stridor following extubation occurred in 28 patients (18.7%); 3 of them required reintubation due to respiratory distress and in 1 child stridor persisted for which a surgical intervention was necessary. In multivariate analyses, we found the following independent predictors of stridor: intubation on the scene, the use of cuffed tubes and lower age. Conclusion Despite a high incidence for post-extubation stridor, only few children need reintubation or surgical intervention as a result of post-extubation lesions. Intubation on the scene, the use of cuffed tubes and young age are associated with a significant increased risk of post-extubation stridor. Awareness of these factors gives the possibility to anticipate on the situation and to minimize laryngeal injury and its possible future consequences.

Introduction The pediatric voice handicap index (pVHI) has been developed to provide a better insight into the parents' perception of their child's voice related quality of life. The purpose of the present study was to validate the Dutch pVHI by evaluating its internal consistency and reliability. Furthermore, we determined the optimal cut-off point for a normal pVHI score. Methods All items of the English pVHI were translated into Dutch. Parents of children in our dysphonic and control group were asked to fill out the questionnaire. For the test re-test analysis we used a different study group who filled out the pVHI twice as part of a large follow up study. Internal consistency was analyzed through Cronbach's α coefficient. The test-retest reliability was assessed by determining Pearson's correlation coefficient. Mann-Whitney test was used to compare the scores of the questionnaire of the control group with the dysphonic group. By calculating receiver operating characteristic (ROC) curves, sensitivity and specificity we were able to set a cut-off point. Results We obtained data from 122 asymptomatic children and from 79 dysphonic children. The scores of the questionnaire significantly differed between both groups. The internal consistency showed an overall Cronbach α coefficient of 0.96 and an excellent test-retest reliability of the total pVHI questionnaire with a Pearson's correlation coefficient of 0.90. A cut-off point for the total pVHI questionnaire was set at 7 points with a specificity of 85% and sensitivity of 100%. A cut-off point for the VAS score was set at 13 with a specificity of 93% and sensitivity of 97%. Conclusions The Dutch pVHI is a valid and reliable tool for the assessment of children with voice problems. By setting a cut-off point for the score of the total pVHI questionnaire of 7 points and the VAS score of 13, the pVHI might be used as a screening tool to assess dysphonic complaints and the pVHI might be a useful and complementary tool to identify children with dysphonia.

Objectives: The Reflux Finding Score for Infants (RFS-I) was developed to assess signs of laryngopharyngeal reflux (LPR) in infants. With flexible laryngoscopy, moderate inter- and highly variable intraobserver reliability was found. We hypothesized that the use of rigid laryngoscopy would increase reliability and therefore evaluated the reliability of the RFS-I for flexible versus rigid laryngoscopy in infants. Methods: We established a set of videos of consecutively performed flexible and rigid laryngoscopies in infants. The RFS-I was scored twice by 4 otorhinolaryngologists, 2 otorhinolaryngology fellows, and 2 inexperienced observers. Cohen's and Fleiss' kappas (k) were calculated for categorical data and the intraclass correlation coefficient (ICC) was calculated for ordinal data. Results: The study set consisted of laryngoscopic videos of 30 infants (median age 7.5 (0-19.8) months). Overall interobserver reliability of the RFS-I was moderate for both flexible (ICC = 0.60, 95% CI 0.44-0.76) and rigid (ICC = 0.42, 95% CI 0.26-0.62) laryngoscopy. There were no significant differences in reliability of overall RFS-I scores and individual RFS-I items for flexible versus rigid laryngoscopy. Intraobserver reliability of the total RFS-I score ranged from fair to excellent for both flexible (ICC = 0.33-0.93) and rigid (ICC = 0.39-0.86) laryngoscopies. Comparing RFS-I results for flexible versus rigid laryngoscopy per observer, reliability ranged from no to substantial (k =-0.16-0.63, mean k = 0.22), with an observed agreement of 0.08-0.35. Conclusion: Reliability of the RFS-I was moderate and did not differ between flexible and rigid laryngoscopies. The RFS-I is not suitable to detect signs or to guide treatment of LPR in infants, neither with flexible nor with rigid laryngoscopy. (C) 2016 Elsevier Ireland Ltd. All rights reserved.

To provide an overview of current practice patterns with regard to Robin sequence (RS) patients in Europe, a survey was conducted among European clinicians. This online survey consisted of different sections assessing characteristics of the respondent and clinic, definition, diagnosis, treatment, and follow-up. In total, surveys from 101 different European clinics were included in the analysis, and 56 different RS definitions were returned. The majority (72%) of the respondents used a sleep study system to determine the severity of the airway obstruction. A total of 63% used flexible endoscopy and 16% used rigid endoscopy in the diagnostic process. Treatment of the airway obstruction differed considerably between the different countries. Prone positioning for mild airway obstruction was the treatment modality used most often (63%). When prone positioning was not successful, a nasopharyngeal airway was used (62%). Surgical therapies varied considerably among countries. For severe obstruction, mandibular distraction was performed most frequently. Three-quarters of the respondents noted the presence of catch-up growth in their patient population. This first European survey study on definition and management of RS shows that there are considerable differences within Europe. Therefore, we would encourage the establishment of national (and international) guidelines to optimize RS patient care.

This research focuses on the numerical simulation of stridor; a high pitched, abnormal noise, resulting from turbulent airflow and vibrating tissue through a partially obstructed airway. Characteristics of stridor noise are used by medical doctors as indication for location and size of the obstruction. The relation between type of stridor and the various diseases associated with airway obstruction is unclear; therefore, simply listening to stridor is an unreliable diagnostic tool. The overall aim of the study is to better understand the relationship between characteristics of stridor noise and localization and size of the obstruction. Acoustic analysis of stridor may then in future simplify the diagnostic process, and reduce the need for more invasive procedures such as laryngoscopy under general anesthesia. In this paper, the feasibility of a coupled flow, acoustic and structural model is investigated to predict the noise generated by the obstruction as well as the propagation of the noise through the airways, taking into account a one-way coupled fluid, structure, and acoustic interaction components. The flow and acoustic solver are validated on a diaphragm and a simplified airway model. A realistic airway model of a patient suffering from a subglottic stenosis, derived from a real computed tomography scan, is further analyzed. Near the mouth, the broadband noise levels at higher frequencies increased with approximately 15-20 dB comparing the stridorous model with the healthy model, indicating stridorous sound.

Introduction To describe the characteristics and surgical outcome of 98 infants and children treated for an acquired laryngeal stenosis after intubation for respiratory support. Material and methods We retrospectively reviewed our data from the last 18 years (1994–2013) concerning infants and children with an acquired laryngotracheal stenosis who were treated in our hospital with a laryngotracheal reconstruction or a cricotracheal resection. Outcome was defined by decannulation ratio. Results Of the 98 infants and children who were studied, 54% were preterm, 18% neonates, 13% infants and 14% children. Ninety-one SS-LTR’s, two DS-LTR’s and five CTR’s were performed as primary surgery; three revision operations were performed (DS-LTR). Seventy-seven children had a tracheostomy prior to surgery; decannulation ratio was 93% after primary surgery and 95% after inclusion of revision surgery. For SS-LTR, the decannulation ratio was 93%, including grade III stenosis with comorbidities. Male sex and glottic involvement of the stenosis are correlated to failure of decannulation. Intubation in the term neonatal period is correlated to complicated post-operative course after SS-LTR. Conclusions Excellent results of surgery for acquired laryngotracheal stenosis can be obtained with a high decannulation rate. Even for higher grades of stenosis with comorbidities and glottic involvement, an SS-LTR is an effective surgical treatment for acquired laryngeal stenosis.

Objective To describe the course of obstructive sleep apnoea syndrome (OSAS) in children with syndromic craniosynostosis. Design Prospective cohort study. Setting Dutch Craniofacial Centre from January 2007 to January 2012. Patients A total of 97 children with syndromic craniosynostosis underwent level III sleep study. Patients generally undergo cranial vault remodelling during their first year of life, but OSAS treatment only on indication. Main outcome measures Obstructive apnoea-hypopnoea index, the central apnoea index and haemoglobin oxygenation-desaturation index derived from consecutive sleep studies. Results The overall prevalence of OSAS in syndromic craniosynostosis was 68% as defined by level III sleep study. Twenty-three patients were treated for OSAS. Longitudinal profiles were computed for 80 untreated patients using 241 sleep studies. A mixed effects model showed higher values for the patients with midface hypoplasia as compared to those without midface hypoplasia (Omnibus likelihood ratio test=7.9). In paired measurements, the obstructive apnoea-hypopnoea index (Z=−3.4) significantly decreased over time, especially in the first years of life (Z=−3.3), but not in patients with midface hypoplasia (Z=−1.5). No patient developed severe OSAS during follow-up if it was not yet diagnosed during the first sleep study. Conclusions OSAS is highly prevalent in syndromic craniosynostosis. There is some natural improvement, mainly during the first 3 years of life and least in children with Apert or Crouzon/Pfeiffer syndrome. In the absence of other co-morbid risk factors, it is highly unlikely that if severe OSAS is not present early in life it will develop during childhood. Ongoing clinical surveillance is of great importance and continuous monitoring for the development of other co-morbid risk factors for OSAS should be warranted.

Objectives/Hypothesis: This study evaluated the accuracy of established obstructive sleep apnea syndrome (OSAS) questionnaires based on presenting symptoms and complaints as screening tools for OSAS in Treacher-Collins syndrome (TCS). Study Design: Cross-sectional cohort study. Methods: In 35 TCS patients (13 children, 22 adults) in whom diagnostic polysomnographic results on OSAS were available, the Brouillette score was evaluated in children and the Epworth Sleepiness Scale in adults. Results: The total Brouillette score showed a sensitivity of 50%, specificity of 71%, and positive and negative predictive values of 60% and 63%, respectively. The answer “No” to the question as to whether a child snored could rule out OSAS in children, and showed positive and negative predictive values of 55% and 100%, respectively. The Epworth Sleepiness Scale showed a sensitivity of 0%, specificity of 92%, and positive and negative predictive values of 0% and 57%, respectively. A positive answer to the question of whether a person falls asleep while sitting and talking to someone (sometimes or more) was able to predict OSAS in adults; this question had positive and negative predictive values of 100% and 72%, respectively. Conclusions: This cross-sectional cohort study showed that the Brouillette score and the Epworth Sleepiness Scale are of minimal usefulness in TCS. Diagnosis of OSAS based solely on complaints is not reliable, probably due to habituation. Therefore, for a good evaluation and optimal multidisciplinary treatment of this chronic disease in TCS, all newly referred pediatric and adult TCS patients should be screened for OSAS at least once with polysomnography. Laryngoscope, 2012

The purpose of this study was to present a simple and powerful fitting model that describes age-dependent changes of auditory brainstem responses (ABR) in a clinical population of normal hearing children. A total of 175 children (younger than 200 weeks postconceptional age) were referred for audiologic assessment with normal ABR results. ABR parameters of normal hearing children between 2003 and 2008 were included. The results of the right ears recorded at 90 dB nHL were analyzed. A simple and accurate fitting model was formulated based on these data. A very similar age-dependent effect was found for peaks III and V, and I III and 1 V intervals; latencies decrease as postconceptional age increases. It shows that the total age-dependent effect will be completed after 1.5-2 years. The age-dependent effect can be modeled by a relatively simple and accurate exponential function. This fitting model can be easily implemented to analyze ABR results of infants in daily clinical practice. We speculate about the underlying physiological processes.

Objective: To determine the inter-observer reliability in localization of recorded stridor sounds in children. Method: The stridor sounds of 28 children programmed for laryngobronchoscopy were recorded with a high quality digital recorder. Nineteen of these recordings with a diagnosis confirmed by endoscopy, were presented to otorhinolaryngology residents, academic specialists and non-academic consultants (n = 38) in different situations with and without additional information about the subject. The participants were requested to score the sounds as pharyngeal, supraglottic, glottic, subglottic or tracheal in both situations. The scores were analyzed per group of participants, per location of obstruction and per diagnosis in the different situations. Results: The performance of the total group was just above chance level with an average score of 29.6%. The total results improved slightly with the additional information present, although not significantly. No significant difference was found between the three categories of participants. The supraglottic sounds were significantly better differentiated from the other locations in both assessment types (p < 0.001). The tracheal region (p < 0.01) and the supraglottic region (p < 0.05) received significantly higher scores when the additional information was present. Laryngomalacia was significantly better differentiated (p < 0.001) from the other diagnoses. The improvement in assessment with and without additional information present was significant for laryngomalacia (p = 0.002) and tracheomalacia (p < 0.035). Conclusion: The clinical observation of stridor in children shows poor levels of localization. Even though the two most common diagnoses, laryngomalacia and tracheomalacia are localized more correctly, the general performance is not significantly higher than random. No evidence was found in this study for differences in results by the observer's level of experience. We would recommend that an observational policy is only adequate when clear clinical signs are present that indicate laryngomalacia as first differential diagnosis. In other cases rigid and flexible endoscopy is indicated to locate the site and nature of obstruction. (C) 2010 Elsevier Ireland Ltd. All rights reserved.

Objectives To evaluate independent etiologic factors associated with sensorineural hearing loss in infants who have been admitted to the neonatal intensive care unit compared to normal hearing controls. Method Between 2004 and 2009, 3366 infants were admitted to the neonatal intensive care unit of Sophia Children's Hospital, of which 3316 were screened with AABR. A total of 103 infants were referred for auditory brainstem response analysis after failure on neonatal hearing screening. We included all infants diagnosed with sensorineural hearing loss. Each patient was matched with two normal hearing controls from the neonatal intensive care unit of the same gender and postconceptional age. The following risk factors were studied: birth weight, dysmorphic features, APGAR scores (at 1, 5 and 10 min), respiratory distress (IRDS), CMV infection, sepsis, meningitis, cerebral bleeding, cerebral infarction, hyperbilirubinemia requiring phototherapy, peak total bilirubin level, furosemide, dexamethason, vancomycin, gentamycin and tobramycin administration. Results Fifty-eight infants were diagnosed with sensorineural hearing loss: 26 girls and 32 boys. The incidence of dysmorphic features ( P = 0.000), low APGAR score (1 min) ( P = 0.01), sepsis ( P = 0.003), meningitis ( P = 0.013), cerebral bleeding ( P = 0.016) and cerebral infarction ( P = 0.000) were significantly increased in infants with sensorineural hearing loss compared to normal hearing controls ( n = 116). Conclusion Dysmorphic features, low APGAR scores at 1 min, sepsis, meningitis, cerebral bleeding and cerebral infarction are associated with sensorineural hearing loss independent of neonatal intensive care unit admittance.

We report a girl with severe manifestations of Townes-Brocks syndrome (TBS) and a previously unreported serious congenital dysphagia. She is unable to swallow her saliva and consequently chokes frequently with desaturations still existing beyond the second year of life. Involvement of the feet was more extensive than is usually seen in TBS. Mutation analysis of the SALL1 gene, responsible for TBS, resulted in the identification of the de novo hot-spot mutation p.Arg276X. This report adds another rare, but serious manifestation to the multiorgan involvement found in TBS.

Objectives The purpose of this study is to evaluate the long-term health related quality of life (HRQoL) in a cohort of children surgically treated for laryngotracheal stenosis (LTS). Study design Prospective cohort study. Methods Parents of children between 4 and 18 years at follow-up completed the Child Health Questionnaire Parent Form (CHQ-PF50). Children between 11 and 18 years at follow-up completed the Child Health Questionnaire Child Form (CHQ-CF87). Biographical and pre-operative data were extracted from the hospital records. Post-operative measurements consisted of the Bruce treadmill test and pulmonary function testing (PFT). Results Fifty-four parents completed the CHQ-PF50; twenty-one children completed the CHQ-CF87. The CHQ-PF50 was significantly worse than the norm population on the subscales physical functioning, role functioning: emotional/behavior, general health perceptions, family activities, parental impact: emotional, and time. CHQ-CF87 was significantly worse than the norm population on physical functioning and better on mental health. After multivariate analysis, presence of co-morbidities and glottic stenosis are the most important pre-operative factors for worse scores on general health. As post-operative measurements, the Bruce treadmill test and peak expiratory flow (PEF) correlate well with HRQoL physical subscales. Conclusions At long-term follow-up after treatment for LTS, deficits in HRQoL may still exist. Presence of co-morbidities and glottic stenosis are important negative factors for long-term HRQoL. The Bruce treadmill test and peak expiratory flow on pulmonary function testing correlate well with physical subscales on HRQoL. A long-term multidisciplinary follow-up with assessment of HRQoL is advised in patients treated for LTS. Level of evidence 2B, individual prospective cohort study.

Context: Recently, several cases of sudden death in GH-treated and non-GH-treated, mainly young Prader-Willi syndrome (PWS), patients were reported. GH treatment in PWS results in a remarkable growth response and an improvement of body composition and muscle strength. Data concerning effects on respiratory parameters, are however, limited. Objective: The objective of the study was to evaluate effects of GH on respiratory parameters in prepubertal PWS children. Design: Polysomnography was performed before GH in 53 children and repeated after 6 months of GH treatment in 35 of them. Patients: Fifty-three prepubertal PWS children (30 boys), with median (interquartile range) age of 5.4 (2.1–7.2) yr and body mass index of +1.0 sd score (−0.1–1.7). Intervention: Intervention included treatment with GH 1 mg/m2·d. Results: Apnea hypopnea index (AHI) was 5.1 per hour (2.8–8.7) (normal 0–1 per hour). Of these, 2.8 per hour (1.5–5.4) were central apneas and the rest mainly hypopneas. Duration of apneas was 15.0 sec (13.0–28.0). AHI did not correlate with age and body mass index, but central apneas decreased with age (r = −0.34, P = 0.01). During 6 months of GH treatment, AHI did not significantly change from 4.8 (2.6–7.9) at baseline to 4.0 (2.7–6.2; P = 0.36). One patient died unexpectedly during a mild upper respiratory tract infection, although he had a nearly normal polysomnography. Conclusions: PWS children have a high AHI, mainly due to central apneas. Six months of GH treatment does not aggravate the sleep-related breathing disorders in young PWS children. Our study also shows that monitoring during upper respiratory tract infection in PWS children should be considered.

Summary Objective: The postoperative management of children undergoing single stage laryngotracheoplasty (SSLTP) includes intubation and muscle paralysis to secure the airway and protect the wound. We reduced the period of postoperative muscle paralysis in an attempt to decrease the incidence of pulmonary complications. The objective of this study was to evaluate the influence of the duration of muscle paralysis on pulmonary complications and outcome. Methods: Medical records of all children admitted, between 1994 and 2002, to the pediatric intensive care unit following SSLTP were analysed. Children were grouped according to the number of days muscle paralysis was used. Results: Thirty-six children (15 male, 21 female, mean age 32 months (9–162 months)) underwent SSLTP for laryngeal stenosis. Prior to surgery 29 needed a tracheotomy (mean duration 11.1 months). Shorter muscle paralysis leads to shorter intubation and mechanical ventilation and therefore PICU and hospital length of stay were 12.4 and 9.9 days shorter in the group with short use of muscle paralysis ( p p = 0.002, respectively). There was no significant difference in postoperative complications, but a trend towards fewer atelectases in children with short muscle paralysis could be recognised. Postoperatively we observed no auto-extubations in either group and success rate of SSLTP was comparable in both groups (94 and 95%). Conclusion: Children undergoing SSLTP can safely benefit from a postoperative strategy using a short duration of muscle relaxants. They have fewer days on mechanical ventilation with a concomitant decrease in duration of hospital stay.

Background/Purpose: The aim of this study was to add to the experience of slide tracheoplasty in infants with congenital tracheal stenosis (CTS), to review the recent literature on this subject, and to evaluate the diagnostic workup in infants with CTS. Methods: A retrospective review of 3 infants with CTS treated with slide tracheoplasty was conducted at our institution. They all underwent bronchoscopy, tracheobronchography, and echocardiography. Therapy consisted of slide tracheoplasty and simultaneous correction of associated vascular malformations. Results: A pulmonary artery sling was missed initially on esophagography in 2 patients. Stridor became worse in 2 patients after tracheobronchography. After surgical therapy, 2 patients survived and were discharged 12 days after surgery. Both are without symptoms at follow-up of 12 and 20 months. One patient had inoperable restenosis and died 27 days after the operation. Conclusions: Esophagography is not a reliable tool to depict associated vascular anomalies for patients with CTS. According to the literature, computer tomography has become more reliable to depict the tracheobronchial tree and is useful to elucidate associated vascular anomalies as well. Compared with other surgical techniques for infants with CTS reported in the literature, slide tracheoplasty has fewer postoperative complications and comparable survival rates.

We report on a family with a not earlier described multiple congenital malformation. Several male family members suffer from laryngeal obstruction caused by bilateral vocal cord paralysis, outer and middle ear deformity with conductive and sensorineural hearing loss, facial dysmorphisms, and underdeveloped shoulder musculature. The affected female members only have middle ear deformity and hearing loss. The pedigree is suggestive of an X-linked recessive inheritance pattern. SNP-array revealed a deletion and duplication on Xq28 in the affected family members. A possible aetiology is a neurocristopathy with most symptoms expressed in structures derived from branchial arches.

Objective: To assess the occurrence of hearing loss in children due to neonatal exposure to long courses of tobramycin and/or high tobramycin serum concentrations. Methods: This was a pilot case–control study in 3–4-year old children. Data on tobramycin administration were abstracted from the patient files of an earlier study. Patients exposed in the neonatal period to either long courses (>7 days) or high serum concentrations of tobramycin constituted the study group. The control group consisted of patients without tobramycin exposure. Patients were matched for other risk factor according to criteria of the joint committee on infant hearing. All patients underwent the following investigations: otoscopy and pneumatic otoscopy, followed by impedance audiometry, to exclude middle ear effusion. Click-evoked oto-acoustic emissions (ce-OAE) as well as distortion product oto-acoustic emissions (dp-OAE), tested at f2 frequencies ranging from 1 to 10 kHz, were measured to assess hearing. All patients with abnormal ce-OAE results underwent brainstem electric response audiometry (BERA) as well. Since aminoglycoside ototoxicity is usually bilateral, results were compared per patient and not per ear. Results: A total of 29 patients were tested. Eleven patients were excluded due to middle ear effusion. Data for 18 patients were analyzed. In the tobramycin treated group ( n =9) both ce-OAE and dp-OAE (at all tested frequencies) were not detectable in six ears of three patients. All other patients had normal ce-OAE's as well as normal dp-OAE's in this frequency range. Difference between the tobramycin treated and control group for OAE as well as dp-OAE showed a trend ( P =0.08). In all three patients with undetectable emissions BERA confirmed a cochlear loss of 60–70 dB at 3 kHz in both ears. These three patients had the longest total exposure to tobramycin: 20–24 days and 84–92 mg/kg, respectively. No relation to either peak or trough serum concentrations could be detected. Conclusion: There was no statistical relation between hearing loss and tobramycin exposure, probably due to sample size. Our results do indicate a need for a case–control follow-up study of hearing in neonates exposed to long courses of aminoglycosides.

OBJECTIVE: It is hypothesized that laryngeal edema is caused by laryngopharyngeal reflux (LPR) (ie, gastroesophageal reflux extending into the larynx and pharynx). The validated reflux finding score (RFS) assesses LPR disease in adults. We, therefore, aimed to develop an adapted RFS for infants (RFS-I) and assess its observer agreement.STUDY DESIGN: Visibility of laryngeal anatomic landmarks was assessed by determining observer agreement. The RFS-I was developed based on the RFS, the found observer agreement, and expert opinion. An educational tutorial was developed which was presented to 3 pediatric otorhinolaryngologists, 2 otorhinolaryngologists, and 2 gastroenterology fellows. They then scored videos of flexible laryngoscopy procedures of infants who were either diagnosed with or specifically without laryngeal edema.RESULTS: In total, 52 infants were included with a median age of 19.5 (0-70) weeks, with 12 and 40 infants, respectively, for the assessment of the laryngeal anatomic landmarks and the assessment of the RFS-I. Overall interobserver agreement of the RFS-I was moderate (intraclass correlation coefficient = 0.45). Intraobserver agreement ranged from moderate to excellent agreement (intraclass correlation coefficient = 0.50-0.87).CONCLUSION: A standardized scoring instrument was developed for the diagnosis of LPR disease using flexible laryngoscopy. Using this tool, only moderate interobserver agreement was reached with a highly variable intraobserver agreement. Because a valid scoring system for flexible laryngoscopy is lacking up until now, the RFS-I and flexible laryngoscopy should not be used solely to clinically assess LPR related findings of the larynx, nor to guide treatment.

Little is known about the cellular infiltrates in the nasal mucosa of children. This study was set up to compare the nasal cellular infiltrates in biopsy specimens from allergic children and controls. Atopic children were distinguished from controls on the basis of symptoms of allergic rhinitis and/or asthma, total serum immunoglobulin (Ig)E, family history and specific serum IgE to food and aeroallergens. Fifteen allergic patients (median age 4.3 yrs) and 15 age-matched nonallergic control subjects were evaluated. The number of cells positive for CD1a, CD4, CD8, CD19, CD68, chymase, tryptase, IgE and major basic protein was determined in the mucosa of the inferior turbinate. A significantly higher number of IgE-positive cells and mast cells was found in the epithelia of the allergic group. In the lamina propria, higher numbers of IgE-positive cells and eosinophils were found. Langerhans' cells positive for IgE were only seen in allergic children with specific serum IgE against aeroallergens. These children also had a higher number of IgE-positive mast cells compared to controls and atopic children without specific serum IgE. These results show that the nasal cellular infiltrates of allergic children differ from nonallergic control subjects. Prior to the detection of specific serum immunoglobulin E, cellular changes can be found in the nasal mucosa of atopic children.

Objectives/Hypothesis The airway management of children with Robin sequence is controversial. This study provides an overview of a single-center experience with the diagnosis and treatment of 59 children with Robin sequence. Study Design Retrospective cohort study. Methods We conducted a retrospective cohort study of 59 children (

This cohort study in 35 patients (13 children) evaluates the prevalence, severity and anatomical cause of obstructive sleep apnoea syndrome (OSAS) in patients with Treacher Collins syndrome. Ambulatory polysomnography was performed cross-sectionally to determine OSAS prevalence and severity. All upper airway related surgical interventions were evaluated retrospectively. In 11 patients, sleep endoscopy, and flexible and rigid endoscopy were applied to determine the level of anatomical obstruction of the upper airway. The overall prevalence of OSAS in Treacher Collins patients was 46% (54% in children; 41% in adults). Thirty-eight upper airway related surgical interventions were performed in 17 patients. Examination of the upper airway revealed various anatomical levels of obstruction, from the nasal septum to the trachea. Most significant obstruction was found at the level of the oro/hypopharynx. OSAS in Treacher Collins patients is an important problem so all patients should be screened for OSAS by polysomnography. Endoscopy of the upper airways was helpful in determining the level of obstruction. Surgical treatment at one level will not resolve OSAS in most patients because OSAS in Treacher Collins has a multilevel origin. Non-invasive ventilation (continuous positive airway pressure or bilevel positive airway pressure) or tracheotomy should be considered as a treatment modality.

Infants admitted to neonatal intensive care units (NICUs) have a higher incidence of perinatal complications and delayed maturational processes. Parameters of the auditory brainstem response (ABR) were analyzed to study the prevalence of delayed auditory maturation or neural pathology. The prevalence of prolonged I-V interval as a measure of delayed maturation and the correlation with ABR thresholds were investigated. All infants admitted to the NICU Sophia Children's Hospital between 2004 and 2009 who had been referred for ABR measurement after failing neonatal hearing screening with automated auditory brainstem response (AABR) were included. The ABR parameters were retrospectively analyzed. Between 2004 and 2009, 103 infants were included: 46 girls and 57 boys. In 58.3% (60 infants) of our population, the I-V interval was recordable in at least one ear at first diagnostic ABR measurement. In 4.9%, the I-V interval was severely prolonged. The median ABR threshold of infants with a normal or mildly prolonged I-V interval was 50 dB. The median ABR threshold of infants with a severely prolonged I-V interval was 30 dB. In conclusion, in case both peak I and V were measurable, we found only a limited (4.9%) incidence of severely prolonged I-V interval (a parts per thousand yen0.8 ms) in this high-risk NICU population. A mild delay in maturation is a more probable explanation than major audiologic or neural pathology, as ABR thresholds were near normal in these infants.

Objective: Infants admitted to neonatal intensive care units have a higher incidence of significant congenital hearing loss. We classified audiologic diagnoses and follow-up in infants who had been admitted to our neonatal intensive care unit. Methods: We included all infants admitted to the neonatal intensive care unit at Sophia Children's Hospital between 2004 and 2009 who had been referred for auditory brainstem response measurement after failing neonatal hearing screening with automated auditory brainstem response. We retrospectively analyzed the results of auditory brainstem response measurement. Results: Between 2004 and 2009 3316 infants admitted to our neonatal intensive care unit had neonatal hearing screening. 103 infants failed neonatal hearing screening: 46 girls and 57 boys. After first auditory brainstem response measurement we found 18% had normal hearing or a minimal hearing loss. The remainder had a type of hearing loss, distributed as follows: 15% conductive, 32% symmetric sensorineural, 14% asymmetric sensorineural, and 21% absent auditory brainstem responses. Repeated auditory brainstem response measurement showed a shift in hearing outcome. The main difference was an improvement from symmetric sensorineural hearing loss to normal hearing. However, in a small percentage of children, the hearing deteriorated. Conclusions: As many as 58% of infants in this high-risk population who failed the neonatal hearing screening were diagnosed with sensorineural hearing loss or absent auditory brainstem responses. An initial overestimation of sensorineural hearing loss of about 10% was seen at first auditory brainstem response measurement. This may be partially explained by a conductive component that has resolved. Finally, in a small percentage of children the hearing deteriorated. (c) 2010 Elsevier Ireland Ltd. All rights reserved.

Twenty-five complications (of which 2 were lethal) occurred in 1332 rigid laryngobronchoscopies performed under general anesthesia in the Sophia Children's Hospital during an 8 year period (1982–1990). The nature and circumstances of these complications were studied retrospectively. Important intraoperative complications were hemorrhage and cardiac arrhythmia; postoperative complications were intoxication and respiratory complications. Three risk factors: tetralogy of Fallot, biopsy/drainage, and extraction of an aspirated foreign body appeared to be significantly associated with complications. The results of this study were compared with those reported in studies of rigid and flexible laryngobronchoscopies in children.

Objective: Little is known about the long-term prevalence of elevated intracranial pressure (ICP), obstructive sleep apnoea (OSA), level of education, language and motor skills, impaired sight and hearing in craniosynostosis syndromes. The objective of this study was to define the prevalence per syndrome of elevated ICP, OSA, impaired sight and impaired hearing. Methods: A retrospective study was undertaken on 167 consecutive patients diagnosed with Apert, Crouzon, Pfeiffer, Muenke or Saethre-Chotzen syndrome, aged 1-25 years and treated between 1983 and 2008. The mean age at time of referral and review was 1 years and 2 months and 10 years and 3 months, respectively. Results: Patients with Apert and Crouzon/Pfeiffer syndromes had the highest prevalence of elevated ICP (33% and 53%, respectively) and OSA (31% and 27%, respectively), while Saethre-Chotzen syndrome was also associated with a fair risk for elevated ICP (21%). The prevalence of impaired sight (61%) and hearing (56%) was high in all syndromes. Conclusion: Based on these data, a syndrome-specific risk profile with suggestions for screening and treatment is presented. (C) 2009 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Almost 50% of patients with Apert, Crouzon or Pfeiffer syndrome develop obstructive sleep apnea (OSA), mainly due to midface hypoplasia. Midface advancement is often the treatment of choice, but the few papers on long-term outcome report mixed results. This paper aimed to assess the long-term respiratory outcome of midface advancement in syndromic craniosynostosis with OSA and to determine factors contributing to its efficacy. A retrospective Study was performed on 11 patients with moderate or severe OSA, requiring oxygen, continuous positive airway pressure (CPAP), or tracheostomy. Clinical symptoms, results of polysomnography, endoscopy and digital volume measurement Of the upper airways oil CT scan before and after midface advancement were reviewed. Midface advancement had a good respiratory outcome in the short term in 6 patients and was ineffective in 5. In all patients without respiratory effect or with relapse, endoscopy showed obstruction of the rhino- or hypopharynx. The volume measurements supported the clinical and endoscopic outcome. Despite midface advancement, long-term dependence on, or indication for, CPAP or tracheostomy was maintained in 5 of 11 patients. Pharyngeal collapse appeared to play a role in OSA. Endoscopy before midface advancement is recommended to identify airway obstruction that may interfere with respiratory improvement after midface advancement.

Children with craniofacial syndromes, especially those with midfacial hypoplasia, micrognathia, or deformation of the cranial base, are frequently suffering from obstructive sleep apnea syndrome (OSAS). It is important to recognize this condition. Diagnostic methods and therapeutic developments are discussed. Experience with 31 patients in the Sophia Children’s Hospital is presented. The majority of these infants suffered from moderate or severe OSAS. Treatment varied from symptomatic (e.g. continuous positive airway pressure) to curative. These therapies could often prevent a tracheotomy. Still more curative treatment options are needed. © 1999 Elsevier Science Ireland Ltd. All rights reserved.

Six children with a Subglottic hemangioma were treated in the Sophia Children's Hospital in the period 1982–1987 by means of intralesional corticosteroid injection, followed by intubation. After treatment all children were symptomfree. In 3 patients this result was attained after 1 injection, in 2 after 3 injections. One patient needed 5 injections. The average duration of intubation was 19 days (7–36). Three months ( 1 2 –7 1 2 ) after the onset of therapy all patients were free of symptoms. No serious complications were observed. No patient needed a tracheotomy. The average follow-up period was 3.3 years (1 1 2 –5 1 2 ). The authors feel that not only the effect of intralesional corticosteroids, but also local gentle pressure by the tube is of therapeutic importance. The above-mentioned treatment of subglottic hemangioma in children is now the treatment of choice in our clinic.

In the Sophia Children's Hospital we perform fiberoptic laryngoscopy in neonates under general anesthesia without the use of muscle relaxants in the diagnostics of functional laryngeal disorders. The necessary diagnostic and anesthetic equipment is described. Special attention is paid to the way in which the fiberscope is introduced into the breathing circuit and into the child's airway. Several advantages over other methods are discussed. The procedure has proved to be easy, safe and informative.

Objectives: Insertion of ventilation tubes in children with otitis media with effusion (OME) is an accepted and common treatment procedure. The majority of patients require general anesthesia. Although laser myringotomy can be performed in local anesthesia, evidence is lacking that this treatment modality is an alternative for tubes, and outcome predictors for laser myringotomy are not available. Study Design: Prospective randomized trial. Methods: We screened 1,403 children with chronic OME that were indicated for placement of ventilation tubes. In the eligible patients, we performed laser myringotomy in one ear and placed a tube in the other ear, both within the same patient. Follow-up was scheduled each month for 6 months. Success was defined as absence of effusion or aural discharge. A logistic regression model was used with success of the therapy as binary outcome. This model was based on base-line variables, asked for in a parent's questionnaire. Results: Two hundred eight children received the allocated intervention, and no complications occurred. The mean closure time of the laser perforation was 2.4 weeks, and the mean patency time of the ventilation tube was 4.0 months. The mean success rate was 40% for laser and 78% for tubes. Ten known variables were found to predict middle ear status after therapy. Conclusion: Laser myringotomy is a safe but less-effective procedure than insertion of a ventilation tube in the treatment of chronic OME. The prognostic model enables the otolaryngologist to choose the surgical treatment for the child that benefits most: laser myringotomy or ventilation tube.

Aspirated foreign bodies in children: why are they more commonly found on the left? The objective was to present our experience in the brochoscopic removal of foreign bodies in children. This was carried out through a retrospective analysis of the medical records of 96 patients in whom endoscopic removal of an inhaled foreign body was required, at the department of paediatric otorhinolaryngology, Eramus Medical Centre, Rotterdam. The participants were all children who underwent bronchoscopy after suspected foreign body aspiration from 1990 to 2000. The main outcome measures were confirmed diagnosis of foreign body aspiration and localization of aspirated foreign body. It was seen that in children up to 3 years of age, there was no significant difference in foreign body distribution and in children aged 3 and older foreign bodies were more commonly found in the right main bronchus. It was concluded that aspirated foreign bodies are equally distributed between the left and right main bronchus in children younger than 3 years. This could be explained by the handling of choking infants by their parents.

Clinically relevant cartilaginous subglottic stenosis was found in 2 patients with Shwachman-Diamond syndrome (SDS) for which tracheotomy was required in one case. Considering the pathogenesis of SOS, including deficient chondrogenesis, we hypothesise that subglottic stenosis may be a rare symptom of SDS. Otorhinolaryngologist and paediatricians should be aware of the risk of airway pathology in patients with SDS. (C) 2012 Elsevier Ireland Ltd. All rights reserved.

One hundred and fifteen patients, between 6 months and 12 years of age, had bronchoscopy on suspicion of foreign body aspiration. The histories of these patients were studied to examine the diagnostic value of signs, symptoms and examinations, and to determine the time that passed between aspiration and removal of the foreign body. The sensitivity of the symptoms choking and coughing was fairly high (81 and 78%), but the specificity was poor. The sensitivity of a chest radiograph was 82%, the specificity 44%. The sensitivity of radiographs on inspiration and expiration was 80%, the specificity 55%. The patients had been referred with the initial diagnosis foreign body aspiration (80), pneumonia (34), or subglottic laryngitis (1). In 85 patients a foreign body was identified and extracted. The other 30 patients had respiratory tract infections. The initial diagnosis of foreign body aspiration was correct in 61 out of 85 patients. In these cases, the period between aspiration and extraction of the foreign body was a mean 6 days, compared with 55 days, if the initial diagnosis was pneumonia or sub-glottic laryngitis. We conclude that the diagnosis of foreign body aspiration is too often missed, and that, apart from bronchoscopy, diagnostic tools are of little value.

The infantile subglottic hemangioma can be treated in various ways. The results of the treatment used in the Sophia Children's Hospital, intralesional steroids and intubation (IS + I), are discussed and compared with the results of other current treatment methods: CO2 laser vaporization, submucous resection and intubation alone. A total of 18 infants were treated for subglottic hemangioma in our hospital: ten with IS + I alone, five were first treated with systemic therapy and later with IS + I alone and three with various therapies. IS + I was effective in 14 of the 15 patients, one patient was lost from follow up. The remaining three infants were treated with (combinations of) various therapies, because IS + I failed or was not tried. Two patients were finally cured, one still has a tracheotomy. Of other current therapies, CO2 laser vaporization is reported to be effective. In all 30% of the infants treated in Boston Children's Hospital with CO2 laser needed a tracheotomy. Moreover subglottic stenosis is a serious complication. Submucous resection is often successful. It may be complicated by subglottic stenosis and in some cases, depending on the localization of the hemangioma, it may be contraindicated. Intubation alone is less effective than intubation combined with intralesional steroids. Management of subglottic hemangioma in Sophia Children's Hospital is primarily intralesional steroids and intubation and secondarily submucous resection or tracheotomy. CO2 laser vaporization is seldom applied because of the risk of subglottic stenosis.

Thirty young patients with acquired laryngeal stenosis were treated by means of a laryngofissure and stenting. The age distribution suggested two subgroups: infants aged 0-2 years (n = 24), and children aged 6-16 years (n = 6). The causes of the stenosis and the treatment results were different in these subgroups. Treatment resulted in successful decannulation in 22 of 24 infants, and in 5 of 6 children. Treatment included a re-operation in two patients. In 1 patient the therapy failed, and 2 patients died after fatal complications. Decannulation rate, duration of the tracheotomy, and the number of re-operations compare favorably to the results of other centers. The longer duration of stenting, and the high mortality rate may be considered disadvantages.

The failure to extubate a preterm infant after prolonged intubation is often caused by laryngotracheal injury. This condition is treated by tracheotomy, anterior cricoid split, or often, by reintubation and subsequent extubation attempts in a later stage. To assess the value of reintubation as treatment of post-intubation injury, we retrospectively studied a group of preterm infants from the neonatal intensive care unit in the Sophia Children's Hospital. Three categories of injury were distinguished according to the findings at laryngobronchoscopy: (a) edema or superficial lesions, (b) ulcerations and edema and (c) granulations. Twenty-three infants were therapeutically reintubated after post-intubation injury was diagnosed, for a mean period of 17 days. The therapy was successful in 22 patients, and a failure in one. The follow-up period was a mean 34 months. The result and the duration of the treatment vary with the category of the injury and the condition of the patient. Therapeutic reintubation is compared with alternatives such as anterior cricoid split and tracheotomy. We conclude that reintubation is a valuable therapy that should precede the decision for surgery.

Laryngobronchoscopy (LBS), using both rigid and flexible bronchoscopes, has become a frequently performed operation in children. A data base was established to enable retrospective evaluation of a large number of LBSs carried out in a pediatric center. Experience with 1332 cases of LBS involving 808 patients over an 8-year period is presented. The main indications for LBS were inspiratory stridor, atelectasis, and suspected foreign body aspiration. Most frequent diagnoses at LBS were bronchopneumonia, intubation trauma, tracheomalacia, laryngomalacia, and foreign body aspiration. Only 25 complications occurred (1.9%) including two cases of xylometazoline intoxication.

In an experimental study in growing rabbits an endolaryngeal injury to the subglottis resulted in the development of a stenosis due to the formation of scar tissue containing ectopic cartilage. For comparison, biopsies taken from the subglottic stenosis in 8 children were studied histologically. In 6 cases ectopic cartilage was observed; all patients had a history of endotracheal intubation. In 3 children the diagnosis hamartoma was made. In the remaining 3 cases the formation of ectopic cartilage might have been a direct reaction to the endolaryngeal intubation. The observations suggest that the formation of ectopic cartilage in acquired subglottic stenosis is not always due to a developmental aberration such as a hamartoma.

Little information is available regarding the auditory function in Pompe patients. Hearing loss has been reported in classic infantile patients, but it is still unknown whether central nervous system involvement interferes with auditory function and whether enzyme replacement therapy can improve hearing. Auditory function has not been studied in children with milder forms of the disease. We analyzed repetitive auditory brainstem response measurements and pure tone audiometry in 24 children with Pompe disease. Only 1 of 13 patients with milder phenotypes showed recurrent conductive hearing loss, while 10 out of 11 classic infantile patients had sensorineural hearing defects. These patients also had a high prevalence of conductive hearing loss. Five patients showed evidence of mild retrocochlear pathology, suggestive of glycogen accumulation in the central nervous system. Hearing loss persisted during therapy in all patients. The results emphasize the need for careful monitoring of auditory function in classic infantile Pompe patients, and for early implementation of hearing aids to protect speech and language development.