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OBJECTIVE. This project aims to prospectively and objectively assess otolaryngological manifestations and quality of life of children with primary ciliary dyskinesia (PCD) and compare these findings with healthy pediatric controls. STUDY DESIGN. Cross-sectional. SETTING. Two high-volume pediatric PCD specialty centers. METHODS. Standardized clinical assessment; Sino-Nasal Outcome Test 22 (SNOT-22); Hearing Environment and Reflection Quality of Life (HEAR-QL); Reflux Symptom Index (RSI); standardized physical examination of the sinonasal, laryngeal, and otological systems; and investigations including pure-tone audiograms (PTAs) and sinonasal cultures were collected. RESULTS. Forty-seven children with PCD and 25 control participants were recruited. Children with PCD had more upper airway symptoms than healthy children. They had significantly higher scores in both SNOT-22 and RSI, indicating worse sinonasal and reflux symptoms, with worse quality of life on the HEAR-QL index compared to healthy children (P < .05). Fifty-two percent of children with PCD-related hearing loss were not aware of their hearing deficit that was present on audiological assessment, and only 23% of children who had ventilation tubes had chronic otorrhea, most of which was easily controlled with ototopic drops. Furthermore, although all children with PCD had chronic rhinosinusitis, only 36% of them were using topical nasal treatment. The most common bacteria cultured from the middle meatus were Staphylococcus aureus in 11 of 47 (23%), followed by Streptococcus pneumoniae in 10 of 47 (21%). CONCLUSION. This multisite cohort highlights the importance of otolaryngology involvement in the management of children with PCD. More rigorous otolaryngological management may lead to reductions in overall morbidity and improve quality of life for children with PCD.

Background Point-of-care ultrasonography of the airway is becoming a first-line noninvasive adjunct assessment tool of the pediatric airway. It is defined as a focused and goal-directed portable ultrasonography brought to the patient and performed and interpreted on the spot by the provider. Successful use requires a thorough understanding of airway anatomy and ultrasound experience. Aims To outline the many benefits, and some limitations, of airway ultrasonography in the clinical and perioperative setting. Materials and methods Expert review of the recent literature. Results Ultrasound assessment of the airway may provide the clinician with valuable information that is specific to the individual airway static and dynamic anatomy of the patient. Ultrasound can help identify vocal cord dysfunction and pathology, assess airway size, predict the appropriate diameter of endotracheal and tracheostomy tubes, differentiate tracheal from esophageal intubation, localize the cricothyroid membrane for emergency airway access and identify tracheal rings for US-guided tracheostomy. Ultrasonography is also a great tool for the intraoperative diagnosis of a pneumothorax, the visualization of the movement of the diaphragms, and quantifying the amount of gastric content. Ultrasonography signs, tips, and pearls that allow these diagnoses are highlighted. The major disadvantage of ultrasonography remains interobserver variability, and operator dependence, as it requires specific training and experience. Conclusion Although it is not standard of care yet, there is significant potential for the integration of ultrasound technology into the routine care of the airway.

OBJECTIVE To provide recommendations to otolaryngologists and allied physicians for the comprehensive management of children who present with signs and symptoms of congenital cholesteatoma. METHODS A two-iterative Delphi method questionnaire was used to establish expert recommendations by the members of the International Pediatric Otolaryngology Group, on the preoperative work-up, the perioperative considerations, and follow-up. RESULTS Twenty-two members completed the survey, in 14 tertiary-care center departments representing 5 countries. The main consensual recommendations were: a precise otoscopic description of the quadrants involved, extensive audiological workup (bilateral tonal, vocal audiometry, and BERA), and a CT scan are required. Facial nerve monitoring and a combination of microscope and telescope are recommended for surgical removal. Clinical and audiological follow-up should be pursued yearly for at least 5 years. First MRI follow-up should be done at 18 months postoperatively if the removal violated the matrix. MRI follow-up duration depends on the initial extent of the cholesteatoma. CONCLUSION The goal of preoperative and follow-up consensus from International Pediatric Otolaryngology Group participants is to help manage infants and children with congenital cholesteatoma. The operative techniques may vary, and experienced surgeons must perform these procedures.

Introduction Burkitt lymphoma (BL), an aggressive form of B-cell non-Hodgkin's lymphoma, arising from the nose and paranasal sinuses is relatively rare. It can present with various symptoms leading to potential misdiagnosis and delayed treatment. BL is fatal if left untreated, while early identification and treatment can improve prognosis. Objectives 1) To review clinical presentations and sites of involvement of six cases of pediatric BL with rhinologic manifestations and compare these with the current literature. 2) To raise awareness on the variety of presentations of BL in this particular anatomic location. Methods A series of six cases of pediatric (0–18 years) BL with rhinologic manifestations is presented. Age, sex, ethnicity, symptoms, imaging, staging, treatment and outcome were recorded. A systematic review of literature was also conducted using PRISMA guidelines. The search strategy used keywords related to rhinologic manifestations of BL (nasal cavity, nasopharynx, paranasal sinus etc.; Burkitt etc.) and included studies published in English and French describing patients 0–18 years of age. Results 42 patients were included (six from case series and 36 from current literature). Most common presenting symptoms were: nasal obstruction (29%), facial swelling (24%), headache (21%) and proptosis (19%). Most frequent sites of presentation were: nasopharynx (40%), maxilla (40%) and sphenoid (33%). More than half (60%) had systemic involvement, of which the most common locations were: kidney (19%), pancreas (17%) and liver (17%). Mortality from BL in children from this study population was correlated with a longer duration of symptoms prior to presentation, as well as a misdiagnosis preceding the final diagnosis of BL. Conclusions This study brings understanding to the numerous presentations of the same aggressive disease, promotes high clinical suspicion when evaluating common otolaryngologic symptoms and can guide healthcare providers in diagnosing pediatric BL with rhinologic manifestations.

Pediatric diseases of the vascular system include both vascular tumors and vascular malformations. Over 70% of these vascular anomalies involve the head and neck. Clinical findings, imaging, and numerous markers are helpful to distinguish various types of anomalies. The international society for the study of vascular anomalies (ISSVA) developed a classification system that is widely utilized [1].

Objective To provide guidance for home care tracheostomy management in the pediatric population. The mission of the IPOG is to develop expertise-based recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care. Methods Survey of expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). Results Survey results provide guidance for caregiver teaching, the reuse of tracheostomies and suction catheters while inpatient and following discharge, acceptable sterilization practices for tracheostomies, tracheitis workup and management, and outpatient follow-up practices. Conclusion This presentation of common home tracheostomy care practices are aimed at improving patient-centered care in the pediatric population.

In this report we describe a newborn with a rare case of Type II tracheal agenesis and bronchoesophageal fstula. Polyhydramnios and suspected esoph- ageal atresia were identifed during routine pre-natal ultrasound screening. Upon delivery, rigid bronchoscopy, esophagoscopy, and intraoperative fuoroscopy were performed, where both bronchi and the carina showed unusual horizontal orienta- tion making it diffcult to identify the fstula. However, a post mortem CT confrmed the diagnosis of an isolated Type II tracheal agenesis with bronchoesophageal fs- tula.

Esophageal atresia (EA) is the most common congenital esophageal malformation. Airway pathology, in particular, tracheomalacia and laryngotracheal anomalies is a major cause of morbidity and mortalilty in patients with EA. The aim of this study was to report the incidence and type of laryngotracheal anomalies seen in a large series of patients with EA, and to evaluate their impact on the management of children with EA. Retrospective study. Retrospective cohort including all patients referred to the EA National Reference Center from January 2002 to December 2014. Airway assessment was based on endoscopy performed before, during and/or after esophageal surgery. One-hundred and fifty-eight patients were included in the study. Endoscopy revealed tracheomalacia in 141 cases (89.2%) and other laryngotracheal anomalies in 43 patients (27.2%). Ninety-six patients (60.7%) presented with persistent respiratory symptoms, including acute life-threatening events in 21 cases, leading to death in 6 cases. A correlation was observed between degree of tracheal collapse and presence of acute life-threatening events. Laryngotracheal surgery was required in 35 cases (22%). Laryngotracheal anomalies are frequently associated with EA and represent an important etiology of morbidity and mortality that can be prevented by early and systematic diagnosis and aggressive management. An early systematic endoscopic evaluation is recommended to coordinate the airway management with the EA surgery.

Objective Temporary conductive hearing loss due to amniotic fluid accumulation in the middle ear cavity may lead to failure (false positive) in newborn hearing screening tests. The aim of this study was to identify whether amniotic fluid index has association with failure of the initial newborn otoacoustic emission (OAE) screening test. Methods A cohort study in a tertiary hospital center (Royal Victoria Hospital, Montreal) was constructed from 70 newborns that failed the OAE test, but passed a subsequent auditory brainstem response (ABR) test, and 75 randomly selected newborns that passed initial otoacoustic emission testing. Maternal (including the amniotic fluid index in the third trimester) and newborn clinical data were extracted from medical records. Statistical association models were built to determine variables that influenced hearing screen passage or failure. Results The two arms of the cohort had no significant differences in maternal or child clinical indices, including in amniotic fluid index. Calculated as individual odds ratios, maternal tobacco [95% CI of odds ratio: 0.04, 0.59, p = 0.0078], and drug use [95% CI of odds ratio: 0.0065, 0.72, p = 0.058] [borderline significance] were associated with failing the otoacoustic emission testing. Conclusions Amniotic fluid index was not found to be associated with failure of otoacoustic emission screening in newborns. However, our study unveiled an interesting unexpected association of OAE failure with maternal smoking and/or drug use. This finding can help alleviate some of the time, cost and parental anxiety related to failed OAE screening. In selected cases of maternal smoking or drug use we might want to replace or add OAE to the ABR test in newborn hearing screening protocols, that don't perform both tests before discharge.

Background Chronic suppurative otitis media can be recalcitrant and difficult to treat, particularly with the increasing occurrence of antibiotic resistance. Lactobacillus plantarum is a probiotic that has been shown to decrease S. aureus and P. aeruginosa growth in wounds, making it a good candidate for the treatment of chronic suppurative otitis media. However, before it can be applied in the ear, its ototoxicity potential must be evaluated. Methods A prospective controlled trial was conducted in a chinchilla animal model at the Animal care research facilities of the Montreal Children’s Hospital Research Institute to determine whether Lactobacillus plantarum is ototoxic when applied transtympanically. Ten chinchillas each had one ear randomly assigned to receive 109 CFU/mL of Lactobacillus plantarum solution, while the contralateral ear received saline. Auditory brainstem responses were measured bilaterally at 8, 20, 25 kHz before, at 7–10 days after application, and at 28 days after application of probiotic or saline. Facial nerve and vestibular function were assessed clinically. Results There were no statistically significant differences in hearing thresholds between control and experimental ears at 28 days after application. A difference of 11 dB was noted in the 25 kHz range at day 7–10, but resolved by day 28. No animals receiving probiotics developed vestibular nerve dysfunction. There was no histologic evidence of auditory hair cell damaged evidenced by scanning electron microscopy. Conclusion Our study suggests that a single application of Lactobacillus plantarum at 109 CFU/mL does not cause ototoxicity in a chinchilla animal model. These preliminary safety evaluations and the pathogen inhibitory effects of L. plantarum demonstrated by previous studies present this probiotic as a candidate of interest for further investigation. Electronic supplementary material The online version of this article (10.1186/s40463-017-0242-y) contains supplementary material, which is available to authorized users.

Background Leukodystrophies consist of degenerative neurogenetic diseases often associated with comorbidities that extend beyond the neurological system. Despite their impacts on patients' quality of life and risks of complications, head and neck symptomology is poorly reported in the literature. The objective of this study was to identify and quantify the main head and neck complaints among a cohort of patients diagnosed with leukodystrophies and define the role of the otolaryngologist as part of a multidisciplinary team for treating these patients. Methods During the First Canadian National Conference on Leukodystrophies held at the Montreal's Children Hospital, a cohort of 12 patients diagnosed with leukodystrophies were recruited and evaluated by a multidisciplinary team. An otolaryngology-focused assessment was done through history and physical examination, and included a screening questionnaire for 23 common otolaryngology issues. If families reported a history of sialorrhea, a validated questionnaire (Drool Quality of Life Assessment Questionnaire (DroolQoL)) was subsequently distributed. Results from the questionnaires were then compiled and analyzed. Results Of the 12 recruited patients, 83% (10/12) were known to an otolaryngologist. Drooling affected 67% (8/12) of patients although only 37.5% (3/8) of patients had undergone medical or surgical therapies for this issue. Four patients experienced at least one aspiration pneumonia. 58% (7/12) of the patients had dysphagia, of whom 43% (3/12) were fed exclusively via gastrostomy tube and 28% (2/7) required thickening of feeds. Two patients, despite suspicion of dysphagia and aspiration, had never undergone evaluation. As for otologic issues, it was noted that 25% (3/12) of patients had a history of pressure equalizing tubes (PETs) and one patient had a history of hearing loss. Conclusion Head and neck comorbidities affect children with leukodystrophies. Therefore, the otolaryngologist should be part of the multidisciplinary team, specifically for the management of dysphagia and sialorrhea.

Objective To delineate the auditory functional improvement and peri-operative outcomes of the Sophono™ transcutaneous bone conduction device. Methods Eligible articles presenting patients implanted with the Sophono™ were identified through a comprehensive search of PubMed and Embase electronic databases. All relevant articles were reviewed to justify inclusion independently by 2 authors. Studies that successfully passed critical appraisal for directness of evidence and risk of bias were included. Results From a total of 125 articles, 8 studies encompassing 86 patients using 99 implants were selected. Most patients (79.1%) were children. Ear atresia (67.5%) was the most frequently reported indication for Sophono™ implantation. Overall pure tone average auditory improvement was 31.10 (±8.29) decibel. During a mean follow-up time of 12.48 months, 25 patients (29%) presented with post-operative complications from which 3 were deemed as serious implant-related adverse events (3.5%). Conclusions The Sophono™ transcutaneous bone conduction device shows promising functional improvement, no intra-operative complications and minor post-operative skin related complications. If suitable, the device could be a proposed solution for the rehabilitation of hearing in children meeting eligibility criteria. A wearing schedule must be implemented in order to reduce magnet-related skin complications.

Objective To provide recommendations for the comprehensive management of airway obstruction in patients with Robin Sequence. Methods Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). Results The consensus statement provides recommendations for medical specialists who manage infants with Robin Sequence including: evaluation and treatment considerations for commonly debated issues in post-natal airway obstruction, assessment of antenatal obstruction and perinatal airway management. Conclusion Consensus recommendations are aimed at improving management of airway obstruction in patients with Robin Sequence.

Objective 1) To assess the feasibility of reconstructing 2-cm-long circumferential tracheal defects with a 3D printed polycaprolactone (PCL) implant in rabbits. 2) To evaluate endoscopic, histologic, and functional characteristics of a PCL tracheal implant over time. Methods Ten New Zealand rabbits were included in this study. A 2-cm-long 3D printed PCL tracheal implant was created. All rabbits underwent surgical excision of a 2-cm-long cm segment of cervical trachea, which was reconstructed with the implant. Rabbits were sacrificed at the following time points: 0, 4, 5, 6, and 7 weeks postoperatively. At these time points, a rigid bronchoscopy was performed, and blinded evaluators calculated the percentage of airway stenosis. The tracheas were then harvested and prepared for histologic analysis. Results All rabbits survived to their date of sacrifice except for one. Rabbits were euthanized between 0 to 54 days postoperatively with a median of 30 days. All rabbits developed significant granulation tissue with an average percentage stenosis of 92.3% ± 6.1%. On histology, granulation was present with extensive neovascularization and mixed inflammatory cells. There was re-epithelialization present on the luminal surface of the PCL implant near the anastomoses but absent at the center of the implant. Conclusion This study demonstrates that our 2-cm-long 3D printed PCL tracheal implant can be used to reconstruct a tracheal defect of equivalent size in a New Zealand rabbit model in the short term. However, significant granulation tissue formation limits long-term survival. Further research is warranted to limit the granulation tissue overgrowth. Level of evidence NA Laryngoscope, 2019.

Medulloblastoma is the most common pediatric malignant brain tumor and carries a relatively grim prognosis despite recent advances in multimodality therapy. Delays in diagnosis and treatment initiation may contribute to worst outcomes. Signs of increased intracranial pressure and ataxia are known presentations of posterior fossa tumors, but sensorineural hearing loss (SNHL) is a seldom reported symptom. We report the case of a 2-year-old girl who had progressive unilateral SNHL since birth, which was later found to have a posterior fossa medulloblastoma when a head magnetic resonance imaging was ordered following subsequent progression to bilateral hearing impair. We further report our review of 17 additional cases of medulloblastoma presenting with SNHL and their associated head and neck findings. The present study provides insight into the current state of the literature on this rare symptom of a commonly encountered diagnosis, while highlighting the need to consider dedicated brain imaging in pediatric unilateral SNHL when a syndromic etiology or inner ear anomaly is not readily identified on initial investigations.

Objective To provide recommendations to otolaryngologists and allied physicians for the comprehensive management of young infants who present with signs or symptoms of choanal atresia. Methods A two-iterative delphi method questionnaire was used to establish expert recommendations by the members of the International Otolaryngology Group (IPOG), on the diagnostic, intra-operative, post-operative and revision surgery considerations. Results Twenty-eight members completed the survey, in 22 tertiary-care center departments representing 8 countries. The main consensual recommendations were: nasal endoscopy or fiberscopy and CT imaging are recommended for diagnosis; unilateral choanal atresia repair should be delayed after at least age 6 months whenever possible; transnasal endoscopic repair is the preferred technique; long term follow-up is recommended (minimum one year) using nasal nasofiberscopy or rigid endoscopy, without systematic imaging. Conclusion Choanal atresia care consensus recommendations are aimed at improving patient-centered care in neonates, infants and children with choanal atresia.

Introduction Tracheal pathology can be life-threatening if not managed appropriately. There are still some surgical limitations today for certain pathologies, such as in severe tracheomalacia, or when long segments of trachea need to be resected. Poly(e-caprolactone) (PCL) is a polymer that has recently gained popularity for its use in tracheal surgeries in animal models and in certain human pediatric cases in hopes of addressing these difficult situations. PCL can be 3D printed or manufactured through molds to create tracheal stents, splints, patches and even to reconstruct full circumferential tracheal defects. Objective To perform a scoping review, and explore insights into the applications of PCL for tracheal surgeries in-vivo. Methods A literature search in Embase, MEDLINE, and BIOSIS was performed to include all articles available prior to December 21, 2018 without any language restrictions. We included all original research that investigated the use of a PCL implant, stent, splint, scaffold, or graft in tracheal surgeries in-vivo. Assessment of all articles were performed by two independent authors prior to inclusion for analysis. Results A total of 27 articles were included in the study. All articles were original research studies, primarily consisting of interventional studies (92.4%), there was also 2 case reports (7.4%). Articles were published in the last decade, publications range from 2009 to 2019. The most common animal model used for the tracheal surgeries were the New Zealand rabbits (n = 19, 70%). Two studies (7%) also described the use PCL in a total of 4 human cases. To investigate the PCL reconstructed airways, histology and bronchoscopy were the most commonly implemented methods of analysis in 88.9% and 70.4% respectively. Airway analysis was also done using imaging modalities including CT scan (n = 9, 33.3%), MRI (n = 2, 7.4%), X-ray (n = 1, 3.7%). 17 (62.9%) of the studies used 3D printing processes to create their PCL implants. Conclusions Overall, this review demonstrates the feasibility of PCL in tracheal reconstruction and tracheal stenting/splinting. It highlights common trends and the limitations of the literature thus far on this topic.

We hereby present the first-reported pediatric case of a hard palate neurovascular hamartoma in a male newborn, in which the diagnosis was established following an initial nondiagnostic biopsy, extensive radiological investigations, and eventual wide local excision. These benign lesions can easily be mistaken for malignant diagnoses, leading to increased parental and child anxiety as well as avoidable diagnostic and therapeutic interventions.

Cisplatin is a commonly used chemotherapeutic agent and causes serious side effects, including progressive and irreversible hearing loss. No treatment is currently available for cisplatin-induced ototoxicity. We have previously demonstrated that erdosteine, a potent antioxidant, partially protected the cochlea against cisplatin toxicity in vivo. The aims of this study were to (1) evaluate the protein profiles of the cochlea following cisplatin administration and (2) evaluate the impact of erdosteine on the protein profile using a proteomics-based approach. Thirty Sprague-Dawley rats were injected intraperitoneally with saline (n = 10), cisplatin (n = 10) or with cisplatin and erdosteine (n = 10). The cisplatin dosage was 14 mg/kg and for erdosteine, 500 mg/kg. Following euthanasia, protein lysates were obtained from fresh-frozen cochleae and were processed for mass spectrometry and western blotting. We detected 445 proteins that exhibited a twofold change or greater in the cisplatin group as compared to the control group. Of these, 18 proteins showed a fourfold or greater change in expression associated with cisplatin administration, including ras-related protein Rab-2A, Rab-6A, cd81, ribosomal protein S5, and myelin basic protein, which were downregulated, while Ba1-647 and fibrinogen (alpha chain), amongst others, were upregulated. Co-administration of erdosteine revealed a reversal of these changes in the expression of ras-related protein Rab-2A, ribosomal protein S5, myelin basic protein, and fibrinogen (alpha chain); erdosteine also upregulated glutathione reductase. In this study, we identified various proteins that may play a role in cisplatin-induced ototoxicity. We also observed the changes resulting from co-treatment with an antioxidant.

The authors describe the conservative management of 2 rare patients of uncomplicated pneumosinus dilatans of the frontal sinus with minimal bossing of the forehead. Regular nasal hygiene in combination with topical corticosteroids was administered with following complete resolution symptoms.

Objective To provide recommendations for the comprehensive management of young infants who present with signs or symptoms concerning for laryngomalacia. Methods Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). Results Consensus recommendations include initial care and triage recommendations for health care providers who commonly evaluate young infants with noisy breathing. The consensus statement also provides comprehensive care recommendations for otolaryngologists who manage young infants with laryngomalacia including: evaluation and treatment considerations for commonly debated issues in laryngomalacia, initial work-up of infants presenting with inspiratory stridor, treatment recommendations based on disease severity, management of the infant with feeding difficulties, post-surgical treatment management recommendations, and suggestions for acid suppression therapy. Conclusion Laryngomalacia care consensus recommendations are aimed at improving patient-centered care in infants with laryngomalacia.

Objective To make recommendations on the identification, routine evaluation, and management of fetuses at risk for airway compromise at delivery. Methods Recommendations are based on expert opinion by members of the International Pediatric Otolaryngology Group (IPOG). A two-iterative Delphi method questionnaire was distributed to all members of the IPOG and responses recorded. The respondents were given the opportunity to comment on the content and format of the survey, which was modified for the second round. “Consensus” was defined by >80% respondent affirmative responses, “agreement” by 51–80% affirmative responses, and “no agreement” by 50% or less affirmative responses. Results Recommendations are provided regarding etiologies of perinatal airway obstruction, imaging evaluation, adjunct evaluation, multidisciplinary team and decision factors, micrognathia management, congenital high airway obstruction syndrome management, head and neck mass management, attended delivery procedure, and delivery on placental support procedure. Conclusions Thorough evaluation and thoughtful decision making are required to optimally balance fetal and maternal risks/benefits.

Introduction: Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal recessive disease affecting the cytotoxic pathway. Due to the recent advances in molecular diagnosis, immuno-chemo therapy, and hematopoietic stem cell transplantation treatment, FHL survival rates have drastically increased. Case Presentation: Herein, we describe a case of FHL type 5 presenting with low-frequency sensorineural hearing loss. Alongside our reported case, 6 additional patients were identified in the literature. Management and Outcome: The progressive nature of FHL disorder may cause bilateral, low-frequency, irreversible sensorineural hearing loss. This type of hearing loss should be considered among the long-term sequelea presenting with FHL5. Discussion: We recommend audiological evaluation at initial FHL5 diagnosis to assess for hearing functions. Follow-up in audiology should be part of the long-term monitoring of patients with FHL5 as hearing loss could develop long after diagnosis.

Summary Primary ciliary dyskinesia (PCD) is a genetically heterogeneous, rare lung disease resulting in chronic oto‐sino‐pulmonary disease in both children and adults. Many physicians incorrectly diagnose PCD or eliminate PCD from their differential diagnosis due to inexperience with diagnostic testing methods. Thus far, all therapies used for PCD are unproven through large clinical trials. This review article outlines consensus recommendations from PCD physicians in North America who have been engaged in a PCD centered research consortium for the last 10 years. These recommendations have been adopted by the governing board of the PCD Foundation to provide guidance for PCD clinical centers for diagnostic testing, monitoring, and appropriate short and long‐term therapeutics in PCD patients. Pediatr Pulmonol. 2016;51:115–132. © 2015 The Authors. Pediatric Pulmonology Published by Wiley Periodicals, Inc.

Sialolithiasis should be considered in the differential diagnosis of any patient with persitent or recurring salivary gland swelling or inflammation. It occurs most frequently in the Wharton duct followed by the Stensen duct, and rarely in the sublingual glands. Sialolithiasis typically presents with pain and swelling of the involved gland and is aggravated by eating. In the acute setting, patients with sialadenitis secondary to calculi are treated conservatively with antibiotics, and stones less than 2 mm may pass spontaneously. Sialendoscopy is now well established as a novel minimally invasive diagnostic and therapeutic modality for salivary gland stones. Sialoendoscopes can also be used in combination with several open surgical approaches to guide the extraction of large calculi. This article highlights the open surgical interventions that are frequently used in sialolithiasis management, when sialoendoscopy is not the most appropriate option, has failed, or is not available. They range from minimally invasive to open techniques. Mastering the anatomy of the salivary ducts and surrounding structures is of exquisite importance. The management of sialolithiasis should aim for gland-sparing procedures before considering gland excision.

The present study reviewed all cases reported of isolated Langerhans cell histiocytosis bone lesions in the head and neck of pediatric patients. The objective was to create a treatment algorithm to facilitate the diagnosis and management of these lesions.Eligible articles were identified through a comprehensive search of the following electronic databases: PubMed, Ovid Medline, Embase, and Cochrane Library.Two authors independently reviewed the titles and abstracts retrieved by the electronic search concordant with the criteria for study eligibility. The lists of articles from each author were jointly reviewed and a common list created. All relevant articles were reviewed in hard copies and as full texts to justify inclusion.A total of 201 patients (67% male, mean age: 8.1 ± 4.3 years) were included from 45 studies. Clinical presentations included swelling (64%), pain (9%), or both (18%). The skull (61%) and the orbit (24%) were the most common location for isolated bone lesions in the head and neck. The most frequently documented management option was resection, followed by observation, chemotherapy, and intralesional steroid injection.Due to its rarity and variability in presentation and severity, treatment of these lesions has yet to be standardized. Based on the review, a diagnosis and treatment algorithm was created for head and neck surgeons when encountering these types of lesions.

Objective To determine if laryngeal contrast pooling on a videofluoroscopic swallowing study increases the risk for pneumonia in the following 6 months in children with dysphagia. Secondarily, to determine in the same population, if laryngeal abnormalities or syndromic disorders increase the risk for pneumonia in the same timeframe. Study design Retrospective cohort study. Methods A chart review of pediatric patients that presented to the swallowing and dysphagia clinic at the Montreal Children's Hospital for a videofluoroscopic swallowing study in the last three years was conducted. Videofluoroscopic findings, patient characteristics, demographic data, and pneumonias occurring within 6 months after the study were recorded for all patients. Patients with unsuccessful swallowing studies, incomplete charts, extra-laryngeal etiologies for recurrent pneumonia, or who were lost to follow up were excluded. Results Of the 287 children who presented to the clinic, 239 patients remained after exclusion, of which 40 (16.7%) exhibited pooling and 199 (83.3%) did not. Children with pooling on videofluoroscopic swallowing study did not have significantly more pneumonias than patients without pooling (22.5% vs 17.1%, P = 0.42). Secondary analyses revealed that laryngeal abnormalities were a significant independent risk factor (P = 0.02) for pneumonia at 6 months, while being diagnosed with a syndrome was not (P = 0.18). Conclusion In this study of contrast pooling in videofluoroscopic swallowing study, there was no significant difference in pneumonia occurrence in patients with and without pooling at 6 months post study. Future prospective studies should be conducted to confirm these findings. The present review showed that feeding changes should not be made based on pooling alone.

Objectives Management of recurrent thyroglossal duct cysts (TGDC) remains a clinical challenge to otolaryngologists – head and neck surgeons. The purpose of this systematic review is to determine the best surgical management for recurrent TGDC. Methods A comprehensive search for relevant articles was carried out on electronic databases named Ovid Medline, Ovid Medline in process and Other Non-Indexed Citations, Embase, Ovid OldMedline, and Ovid Medline Daily. Articles published in English until 2014 were eligible for review. Using predefined inclusion criteria, published articles on surgical outcomes in the management of recurrent thyroglossal duct cyst, were selected, reviewed, and their findings synthesized. Results Nine studies met the inclusion criteria for this systematic review comprising a total of 66 patients who underwent 114 secondary surgeries. Better outcomes were observed with en bloc neck dissection vs. a revision Sistrunk with a recurrence rate of 20% vs. 30.12% respectively. In addition two new surgical approaches, suture-guided transhyoid pharyngotomy and Koempel's suprahyoid technique reported 100% success rate. Conclusion Recurrence after primary surgical management remains a clinical challenge. We highlight the outcomes of the 4 main surgical techniques reported in the literature, repeat Sistrunk procedure, en bloc neck dissection, suture-guided transhyoid pharyngotomy, and Koempel's suprahyoid technique. Although this review reports a 100% success rate with the 2 latter techniques, further prospective studies and additional experience with these same techniques by other surgeons/institutions with or without a randomized trial could provide additional confirmation of improved outcomes using these specific surgical procedures.

Patients undergoing radiotherapy (RT) often present with serious bacterial infections requiring the use of antibiotic treatment. Gentamicin is a commonly used aminoglycoside antibiotic, whose ototoxicity remains a major problem in clinical use. The objective of this study was to determine whether radiation exposure can influence gentamicin-induced ototoxicity.Prospective animal study.Animal care facilities of the Montreal Children's Hospital Research Institute.Sixteen guinea pigs received low-dose RT unilaterally for 4 weeks (total: 48 Gy). Animals then received low or high doses of gentamicin (40 mg/kg/d and 80 mg/kg/d) for 10 days. The ears were divided into 4 groups: gentamicin 40 mg, gentamicin 80 mg, gentamicin 40 mg + RT, and gentamicin 80 + RT. Auditory brainstem responses and distortion products otoacoustic emissions were assessed at baseline and before and after gentamicin treatment. Cochlear morphology using light and scanning electron microscopy were evaluated.High-dose gentamicin caused significant auditory brainstem response threshold shifts (P = .020), with greater hearing loss in the irradiated ear (difference of 23.6 + 7.5 dB). All animals exposed to high-dose gentamicin had head tilts toward the radiated side. Cochlear morphology revealed the greatest hair cell damage in the gentamicin 80 + RT group followed by gentamicin 80.Results suggest that radiation can exacerbate the ototoxicity of gentamicin at high doses.

Thyroglossal duct cyst (TGDC) is one of the most common congenital anterior midline neck masses in children. Sistrunk operation is considered as the cornerstone in TGDC removal. Nevertheless, 5% to 7 % of patients have been reported to show a recurrence even after adequate resection. This necessitates at times multiple resections with potential complications. One interesting emerging technique is sclerotherapy of TGDC remnants post resection. We present here a case of a child who had a successfully treated recurrent TGDC using sclerotherapy with doxycycline.

Background: This document presents the American Thoracic Society clinical practice guidelines for the diagnosis of primary ciliary dyskinesia (PCD). Target Audience: Clinicians investigating adult and pediatric patients for possible PCD. Methods: Systematic reviews and, when appropriate, meta-Analyses were conducted to summarize all available evidence pertinent to our clinical questions. Evidence was assessed using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach for diagnosis and discussed by amultidisciplinary panelwith expertise in PCD. Predetermined conflict-of-interest management strategies were applied, and recommendations were formulated, written, and graded exclusively by the nonconflicted panelists. Three conflicted individuals were also prohibited from writing, editing, or providing feedback on the relevant sections of the manuscript. Results: After considering diagnostic test accuracy, confidence in the estimates for each diagnostic test, relative importance of test results studied, desirable and undesirable direct consequences of each diagnostic test, downstream consequences of each diagnostic test result, patient values and preferences, costs, feasibility, acceptability, and implications for health equity, the panel made recommendations for or against the use of specific diagnostic tests as compared with using the current reference standard (transmission electron microscopy and/or genetic testing) for the diagnosis of PCD. Conclusions: The panel formulated and provided a rationale for the direction as well as for the strength of each recommendation to establish the diagnosis of PCD.

Background Concha bullosa mucocele is a rare diagnosis that presents as a nasal mass. It impinges on surrounding structures and can easily be mistaken for a neoplasm. Objective The objective of this study was to shed light on this rare entity and report its diagnostic features and treatment outcomes. Methods A case series conducted in a tertiary health care center. Demographic data, clinical presentation, imaging, cultures, and treatments were recorded. Operative video illustration and key images were obtained. A review of the literature was also performed. Results A total of five cases were reviewed, four of which were concha bullosa mucoceles and one was a mucopyocele. Three of the patients had some form of previous nasal trauma. Headache and nasal obstruction were the most common symptoms with a nasal mass finding on physical examination. Computed tomography was used in all the patients, and magnetic resonance imaging was used in four of the five patients. Four patients had coexistent chronic rhinosinusitis, and three had positive bacterial cultures. All these patients were treated endoscopically either with middle turbinate marsupialization or subtotal resection. No recurrence has been noted thus far. Conclusion Concha bullosa mucocele is a rare diagnosis. Imaging characteristics are helpful in considering the diagnosis, although surgical intervention is often necessary to confirm the diagnosis and treat concha bullosa mucocele.

Background In otologic surgery good visualization is paramount, and patients with bleeding diatheses or who need to be anti-coagulated can present a significant challenge. Here, we determine whether Floseal™, a hemostatic matrix, is ototoxic in a validated animal model. Methods Nine chinchillas housed in the animal care facilities of the Montreal Children’s Hospital Research Institute were used for the study. After a myringotomy incision was made in each tympanic membrane, baseline auditory brainstem response measurements were performed at 8, 20, and 25 kHz. In each animal one ear was randomized to receive Floseal™ to the middle ear cavity, whereas the other ear served as the control and received 0.9% sodium chloride. Outcome measures included early (day 7) and late (day 30) auditory brainstem response, clinical evidence of facial nerve or vestibular disturbance and histological evidence of ototoxity. Results There was no significant hearing threshold shift on auditory brainstem response across all tested frequencies for both experimental and control ear. No animals receiving Floseal™ developed facial or vestibular nerve dysfunction and there was no histological evidence of ototoxicity. Conclusion Based on the preliminary ototoxicity assessment on nine chinchillas, transtympanic Floseal™ does not appear to be ototoxic. More studies are warranted to assess the safety and applicability of the product in humans.

Objectives/Hypothesis Auralgan (benzocaine and antipyrine) is an over-the-counter otic drug commonly used for otalgia. Nevertheless, there is limited evidence about the effects of the drug on hearing function and cochlear morphology in the presence of a tympanic membrane perforation. The aim of the present study was to assess the cytotoxicity of Auralgan using cultured auditory cells (HEI-OC1) and to examine its effects on hearing function and cochlear morphology after intratympanic administration in a chinchilla model. Study Design Animal experiment. Methods Cell viability and DNA labeling assays were conducted to investigate the cytotoxic effect of the drug on cultured auditory cells (HEI-OC1). To examine the possible drug ototoxic effect in vivo, chinchillas received intratympanic injection of Auralgan in one ear, whereas the contralateral control ear received saline. Outcome measures included auditory brainstem response and postmortem cochlear morphology. Results A dose-dependent toxic effect of Auralgan was noted on cultured cells. Animal experiments showed an inflammatory reaction in the experimental ears and facial paralysis in 80% of the animals on the side receiving transtympanic injection of Auralgan (P

Objective The aim of the present study is to examine the effects of tea tree oil on hearing function and cochlear morphology after intratympanic administration in a chinchilla animal model. Methods Nine chinchillas received intratympanic injection of 3% tea tree oil dissolved in olive oil in one ear, whereas the contralateral control ear received olive oil only. Outcome measures included auditory brainstem responses conducted before treatment and at 10 days and 30 days following the injection. Post-mortem cochlear morphology was assessed using scanning electron microscopy. Results At 10 and 30 days following the injection, there was no significant change in auditory brain response thresholds at 8, 16, 20 or 25 kHz. Scanning electron microscopy imaging showed no damage to auditory hair cells. Conclusion Tea tree oil (3%) does not appear to be ototoxic in a chinchilla animal model. Future preclinical and clinical studies are required to establish the effectiveness of TTO in treating otitis.

OBJECTIVES Cisplatin, one of the most effective and widely used chemotherapeutic agents in the treatment of head and neck malignancies, has severe dose-limiting side effects including ototoxicity. This study evaluates the effectiveness of nanoencapsulated curcumin and dexamethasone in preventing degenerative changes in inner ear cells caused by cisplatin. STUDY DESIGN Prospective study, animal experiment. METHODS Cultured auditory cells [House Ear Institute Organ of Corti-1 (HEI-OC1)] and a guinea pig model were used for in vitro and in vivo experiments, respectively. Cell viability assays were conducted to compare the direct toxicity of cisplatin against auditory cells in the presence or absence of pretreatment with nanoencapsulated curcumin and dexamethasone. To recapitulate these effects in vivo, 68 guinea pigs received cisplatin either alone, or along with dexamethasone, nanoencapsulated curcumin, or the combination of both products. Outcome measures included auditory brainstem response, cochlear morphology under both light and scanning electron microscopy, and antioxidant enzyme assays. RESULTS Pretreatment of auditory cells with naonoencapsulated curcumin and dexamethasone resulted in significant attenuation of cisplatin toxicity. Similarly, in the corresponding animal model (guinea pig), cisplatin caused an average hearing loss of 50 dB, which was attenuated by nanoencapsulated curcumin and dexamethasone across all of the hearing frequencies. There was also greater preservation of histologic structures in this group. Superoxide dismutase and catalase activities were increased in cisplatin-treated animals, whereas the nanoencapsulated curcumin with dexamethasone led to a diminution of this effect. CONCLUSION Nanoencapsulated curcumin administered in combination with dexamethasone provides a partial but marked protection against cisplatin-induced hearing loss, likely because of reduced toxic damage to auditory cells.

Objectives/Hypothesis The purpose of this study is to assess the effect of fractionated radiotherapy on sensorineural hearing loss using an animal model. Study Design In vivo animal study. Methods Ears of 25 guinea pigs were divided into three groups: control, irradiated with a total of 48 gray (Gy), and 71 Gy. Unilateral exposure of 48 Gy and 71 Gy fractionated irradiation was given for a 4-week period. Auditory brainstem response and distortion products otoacoustic emissions (DPOAE) were tested prior to irradiation and 1, 6, 10, and 16 weeks after completion of radiotherapy to assess the hearing threshold shift postradiotherapy over time. Results No significant differences in hearing thresholds between the low dose radiation (48 Gy) and the control group (no radiation) underlined that 48 Gy caused no hearing deficits (P = 0.37). The higher dose (71 Gy) showed progressive deterioration of the hearing function over time. Three-way ANOVA interactions revealed significant group-time effects (F= 9.261; P

Background The antineoplastic agents cisplatin and carboplatin are widely-used and highly-effective against a variety of pediatric cancers. Unfortunately, ototoxicity is a frequently encountered side effect of platinum-based chemotherapy. There is currently no treatment or prevention for platinum-induced ototoxicity and development of hearing loss may lead to devastating consequences on the quality of life of pediatric cancer survivors. The objective of this study is to determine the incidence of platinum-induced ototoxicity in a large series of pediatric patients and to evaluate the incidence of progression of ototoxicity after completion of treatment. Procedures A retrospective chart review of pediatric patients treated with cisplatin or carboplatin between 2000 and 2012 was conducted. The incidence of ototoxicity was determined based on the American-Speech-Language-Hearing Association (ASHA) criteria and severity was based on the Chang classification. Results Four hundred and sixty-six patients received platinum-based chemotherapy. Patients were excluded due to congenital hearing loss (n = 1) and insufficient data for calculating the platinum dose (n = 24) or for assessing ototoxicity (n = 135). Three hundred and six patients were included in the analysis. Post-chemotherapy ototoxicity was detected in 148 (48%) patients, and clinically-significant ototoxicity was present in 91 (30%). In addition, based on the ASHA criteria, 48% of patients (97/204) with long-term follow-up had further deterioration of their hearing after completion of treatment. Conclusions Ototoxicity following chemotherapy with cisplatin or carboplatin is common and can frequently progress after the completion of treatment. Long-term follow-up is strongly recommended. Pediatr Blood Cancer 2014;61:2012–2017. © 2014 Wiley Periodicals, Inc.

Acute mastoiditis is an uncommon but challenging condition when it occurs in children with cochlear implant. The literature is scarce as to the management of this condition with regards to explantation. The objective of the study is to determine the need for explantation in patients with cochlear implants who suffer from acute mastoiditis.Online medical databases-PubMed, Ovid Medline, Ovid Medline in process, Embase, Cochrane Library, CINAHL, Biosis, Google Scholar, and Scopus.A systematic review of all publications addressing the treatment of mastoiditis in cochlear implant children prior to November 2013 was conducted. Data were collected from online medical databases-PubMed, Ovid Medline, Ovid Medline in process, Embase, Cochrane Library, CINAHL, Biosis, Google Scholar, and Scopus. The review was performed in 3 phases; an initial screening review of abstracts was performed, followed by a detailed review of full articles based on inclusion and exclusion criteria, and lastly a final review to extract data from selected articles.Twelve articles were found eligible for this systematic review including a total of 43 patients. Subperiosteal abscess was present in 14.3%. All patients received intravenous antibiotics as an initial treatment, and if needed, surgical intervention was performed. Only 1 patient required explantation (2.3%).Prompt, aggressive medical and if needed surgical therapy can help in saving the implant and result in a favorable outcome.

There is currently no treatment available to prevent radiation-induced sensorineural hearing loss. Metformin has antineoplastic effects and is able to regulate the mitochondrial production of reactive oxygen species after cellular stress, which is one of the mechanisms involved in apoptosis after radiation damage. The objective of this study was to determine the safety and radioprotective properties of metformin against radiation-induced cochlear damage both in vitro and in vivo.In vitro and prospective animal study.Animal Care Facilities of the Montreal Children's Hospital Research Institute.Cultured auditory hair cells (HEI-OC1) were exposed to different concentrations of metformin to determine its safety. Cells were incubated with different metformin concentrations and subjected to radiation. Cell viability after experiments was determined with the 3-(4,5-dimethylthiazol-2-yl)-5-(3-carboxymethoxyphenyl)-2-(4-sulfophenyl)-2H-tetrazolium (MTS) assay. Sixteen guinea pigs were divided in 2 groups: drinking tap water (n = 8) and drinking water containing metformin (n = 8). The animals were unilaterally irradiated for 20 days (total dose 70 Gy), and the ears were divided in 4 groups: control (n = 8), irradiated (n = 8), metformin (n = 8), and experimental (n = 8). Auditory brainstem responses were assessed before and 1, 6, and 16 weeks after completion of radiotherapy.Metformin was not cytotoxic or radioprotective in cultured auditory hair cells. Experimental ears had less hearing loss than radiated ones; however, differences were not statistically significant (P.05).Metformin is not ototoxic or radioprotective in vitro or in vivo. Ears solely subjected to metformin had better hearing thresholds than the rest of the groups.

Objectives/hypothesis: The aim of this study was to systematically and quantitatively review the available evidence on the effects of type 2 diabetes mellitus on hearing function. Data sources and review methods: Eligible studies were identified through searches of eight different electronic databases and manual searching of references. Articles obtained were independently reviewed by two authors using predefined inclusion criteria to identify eligible studies. Meta-analysis was performed on pooled data using Cochrane's Review Manager. Results: Eighteen articles fulfilled the inclusion criteria. Hearing loss (HL) was defined by all studies as pure tone average greater than 25 dB in the worse ear. The incidence of HL ranged between 44% and 69.7% for type 2 diabetics, significantly higher than in controls (OR 1.91; 95% confidence interval 1.47-2.49). The mean PTA (pure tone audiometry) thresholds were greater in diabetics than in controls for all frequencies [test or overall effect Z = 3.68, P = 0.0002]. Auditory brainstem response (ABR) wave V latencies were also statistically significantly longer in diabetics when compared to control groups [OR 3.09, 95% CI 1.82- 4.37, P < 0.00001]. Conclusions: Type 2 diabetic patients had significantly higher incidence for at least the mild degree of HL when compared with controls. Mean PTA thresholds were greater in diabetics for all frequencies but were more clinically relevant at 6000 and 8000 Hz. Prolonged ABR wave V latencies in the diabetic group suggest retro-cochlear involvement. Age and duration of DM play important roles in the occurrence of DM-related HL.

Objectives/Hypothesis To determine the ototoxic potential of aluminum acetate (0.5%)/benzethonium chloride (0.03%) otic solution in the chinchilla animal model. Study Design A randomized, prospective, controlled study was conducted in the chinchilla animal model. Methods Fourteen female chinchillas were used. After an incision was made in each tympanic membrane, baseline auditory brainstem response measurements were performed at 16, 20, and 25 kHz. One ear was randomized to receive aluminum acetate (0.5%)/benzethonium chloride (0.03%), whereas the other ear served as the control and received 0.9% NaCl. Solutions were applied transtympanically once a day for 3 consecutive days. Postapplication auditory brainstem response measurements were done at day 7 and day 30. In half of the animals, scanning electron microscopy was done following the last auditory brainstem response measurement. Results A statistically significant difference was found between the mean threshold shifts in the experimental and control ears at all frequencies on both 7 and 30 days postapplication (P < .02). Increased ossification of the cochleae was seen in experimental ears (n = 12). Scanning electron microscopy study demonstrated outer hair cell damage and/or loss in the experimental ears. No significant morphological changes were detected on scanning electron microscopy obtained from the control ears. Conclusions Application of aluminum acetate (0.5%)/benzethonium chloride (0.03%) otic solution causes hearing loss in chinchillas when a tympanic membrane perforation exists. Further studies are needed to test the effect of this otic solution on hearing. Level of Evidence 4. Laryngoscope, 123:2521–2525, 2013

Objectives/Hypothesis Olive oil is often used by patients to soften ear wax or to relieve ear canal obstruction. It is also sold in drugstores as a cerumenolytic. To date, no study has assessed the safety of ototopical olive oil on hearing in the presence of tympanic membrane perforation. The present study aimed to assess the safety of ototopic olive oil on hearing in the presence of tympanic membrane perforation. Study Design Prospective, randomized, controlled trial in a chinchilla animal model. Materials and methods Eleven chinchillas underwent bilateral myringotomy. In each animal, one ear was randomly assigned to receive olive oil (experimental ear), while the contralateral control ear received normal saline. Auditory brain response (ABR) test was performed at baseline and then 7, 14, and 30 days following the application. Results At 30 days follow-up, there was no significant change in auditory brain response thresholds at 8, 16, 20, or 25 kHz. Scanning electron microscope imaging showed no damage to the hair cells. Conclusion Olive oil does not seem to cause hearing loss in chinchillas with perforated tympanic membranes. Future clinical studies are required. Level of Evidence N/A Laryngoscope, 123:2009–2012, 2013

Purpose of review The management of primary or recurrent tracheoesophageal fistula (TEF) remains an important challenge for airway surgeons. Recent findings The accuracy of prenatal detection can be significantly improved in specialized centers. Routine preoperative and postoperative airway endoscopy is recommended to detect a proximal fistula and evaluate vocal cord motility. Minimally invasive thoracoscopic approaches have equal success and improved cosmesis and visualization as compared with thoracostomy. Novel open approaches for complex TEF include a transcervical, transtracheal approach, and slide tracheoplasty.Endoscopic closure of TEF carries less morbidity. Options include de-epithelialization of the tract, interposed material, and combinations. The mean operative time is 30 min; however multiple treatments are required (average 2.1). Use of continuous positive airway pressure in the immediate postoperative period was not associated with increased leak or recurrence. Children post-TEF repair continue to have frequent gastrointestinal and respiratory symptoms. Summary Prenatal diagnosis is beneficial both for prenatal counseling and for planning care. The ideal endoscopic approach is undecided but remains an interesting alternative to open surgery provided failures are anticipated and prompt repeated treatments initiated to preclude ongoing respiratory complications. Transtracheal approaches and slide tracheoplasty are well tolerated and effective in complex/recurrent cases. Long-term follow-up of patients with TEF is important.

The antineoplastic agent's cisplatin and carboplatin are widely used as they are highly effective. Unfortunately, ototoxicity is a frequently encountered side effect of platinum-based chemotherapy. Clinically, patients generally develop a progressive, bilateral, and irreversible sensorineural hearing loss. With rising cancer survival rates, a greater proportion of patients are living with the side effects of their chemotherapy treatments. Consequently, the quality of life of cancer survivors has now become a major concern for clinicians. Various classification systems are currently available to grade side effects and provide a guideline for subsequent treatments. An extensive review of the literature revealed that a variety of criteria are used worldwide for grading platinum-induced hearing loss in children and adults, including the National Cancer Institute criteria, Brock's grading system, the American Speech-Hearing-Language Association criteria, the World Health Organization criteria, the Pediatric Oncology Group criteria, and the Muenster classification. Less commonly used criteria include the Chang classification, the Functional Hearing Loss scale, the HIT system (German Hirntumor study grading system), and most recently, the International Society of Pediatric Oncology Boston ototoxicity grading scale. The objective of this review is to evaluate the commonly used ototoxicity criteria and discuss their benefits and limitations.

Background Patients receiving radiotherapy (RT) for head and neck tumors are at risk of developing sensorineural hearing loss. The objective of this study was to analyze the literature regarding sensorineural hearing loss after RT for head and neck cancer. Methods Seven databases were searched to identify eligible studies. Eligible articles were independently assessed for quality by 2 authors. Results Fourteen articles were evaluated. There was considerable heterogeneity among studies. The reported incidence of sensorineural hearing loss (SNHL) varied from 0% to 85% for the speech frequencies and from 27% to 95% for high frequencies. As the follow-up increased, an increase in the incidence of SNHL was observed. The minimum cochlear dose reported to be a risk factor for SNHL was 45 Gy. Conclusions High quality literature in the topic is lacking. Radiation-induced SNHL is progressive, permanent, and dose-dependent. Total dose and follow-up time are important factors affecting incidence rates. © 2012 Wiley Periodicals, Inc. Head Neck, 35: 1662–1668, 2013

Ototoxicity is a common side effect of cisplatin chemotherapy. This study was undertaken to determine the potential protective effects of a systemic administration of dexamethasone against cisplatin-induced ototoxicity. A prospective controlled trial conducted in an animal model. The setting was Animal care research facilities of the Montreal Children's Hospital Research Institute. An experimental guinea pig model was used. The animals were divided as follows: group 1 (n = 10): 12 mg/kg intraperitoneal (IP) cisplatin, group 2 (n = 14): 15 mg/kg/day dexamethasone IP for 2 days followed by cisplatin 12 mg/kg IP, group 3 (n = 14): 10 mg/kg/day dexamethasone IP for 2 days, on day 3, they received cisplatin 12 mg/kg IP followed by 20 mg/kg/day dexamethasone for 2 days and group 4 (n = 5): 10 ml of saline IP twice a day for 3 days. Auditory brainstem response (ABR) threshold shifts were measured at four frequencies (8, 16, 20 and 25 kHz) for groups 1, 2 and 3. Histological changes in the organ of Corti, the stria vascularis, the spiral ligament and the spiral ganglion neurons as well as scanning electron microscopy for outer hair cells were completed. Immunohistochemistry for tumour necrosis factor-alpha (TNF-α) was performed. ABR threshold shifts were similar in all groups. Histological and scanning electron findings demonstrate that dexamethasone has greater protective effect on the stria vascularis. Systemic dexamethasone administration in a guinea pig model did not provide significant protection against cisplatin-induced ototoxicity. Dexamethasone may be useful in future applications as a complementary treatment.

Objective To conduct an updated systematic review on the outcome of Bell's palsy (BP) in children following steroid treatment. Data sources MEDLINE, EMBASE, Cochrane Library and BIOSIS Previews electronic databases were searched obtaining articles published between 2000 and 2010 without any language restriction. Review methods Articles describing children aged 0–18 years with BP treated solely with corticosteroids were included. In studies including various etiologies for facial palsy; cases of BP treated with steroids were selected and when available, untreated patients as well for comparison. The outcome measure was facial movements following steroidal treatment based on different clinical scales. Controlled clinical trials, prospective and historical cohort studies, cross sectional studies and case series were included. Results A total of 2293 papers were initially identified. Following review by two authors, 68 papers were analyzed in a hard-copy format. Finally, 6 studies were eligible to be included in the systematic review. Four of the studies included children with BP exclusively while the remaining studies described various etiologies of facial palsy. Type of steroid and duration of treatment were inconsistently specified. Outcome measures used include the House–Brackmann scale, Yanagihara grading system and clinical evaluation. Studies analyzed were retrospective cohorts or case-series and were categorized as level 4 of evidence. Conclusion There were no controlled trials and level 4 publications predominate. Therefore, the role of steroid treatment for BP in children is still inconclusive. Further studies are required.