OBJECTIVE:To describe the prevalence and severity of pain experienced by children with Bell's palsy over the first 6 months of illness and its association with the severity of facial paralysis.METHODS:This was a secondary analysis of data obtained in a phase III, triple-blinded, randomised, placebo-controlled trial of prednisolone for the treatment of Bell's palsy in children aged 6 months to <18 years conducted between 13 October 2015 and 23 August 2020 in Australia and New Zealand. Children were recruited within 72 hours of symptom onset and pain was assessed using a child-rated visual analogue scale (VAS), a child-rated Faces Pain Score-Revised (FPS-R) and/or a parent-rated VAS at baseline, and at 1, 3 and 6 months until recovered, and are reported combined across treatment groups.RESULTS:Data were available for 169 of the 187 children randomised from at least one study time point. Overall, 37% (62/169) of children reported any pain at least at one time point. The frequency of any pain reported using the child-rated VAS, child-rated FPS-R and parent-rated VAS was higher at the baseline assessment (30%, 23% and 27%, respectively) compared with 1-month (4%, 0% and 4%, respectively) and subsequent follow-up assessments. At all time points, the median pain score on all three scales was 0 (no pain).CONCLUSIONS:Pain in children with Bell's palsy was infrequent and primarily occurred early in the disease course and in more severe disease. The intensity of pain, if it occurs, is very low throughout the clinical course of disease.TRIAL REGISTRATION NUMBER:ACTRN12615000563561.

Background There is limited evidence on the use of facial nerve function grading scales in acute facial nerve paralysis in children. Objective To investigate the agreement between and the usability of the House-Brackmann and Sunnybrook scales in children with idiopathic facial paralysis (Bell's palsy) and to compare their ease of administration. Methods Data from a randomized controlled trial in children aged 6 months to <18 years with Bell's palsy was used. Children were recruited within 72 hours of symptom onset and assessed using the House-Brackmann and the Sunnybrook scales at baseline and at 1, 3, and 6 months until recovered. Agreement between the scales was assessed using the intraclass correlation coefficient (ICC) at each time point and using a Bland-Altman plot. Ease of administration was assessed using an 11-point Likert scale. Results Comparative data were available for 169 of the 187 children randomized. The ICC between the 2 scales across all time points was 0.92 (95% confidence interval [CI] 0.91-0.93), at baseline 0.37 (95% 0.25, 0.51), at 1 month 0.91 (95% CI 0.89-0.94), at 3 months 0.85 (95% CI 0.80-0.89), and at 6 months 0.96 (95% CI 0.95-0.97). The median score for the ease of administration for the House-Brackmann and Sunnybrook scales was 3 (interquartile range [IQR]: 1-5) and 7 (IQR: 4-8) respectively (P < .001, Wilcoxon signed-rank test). Conclusions There was excellent agreement between House-Brackmann and Sunnybrook scales, with poorer agreement at baseline. Clinicians found the House-Brackmann scale easier to administer. These findings suggest that both scales can be applied in children.

Abstract Objective Currently there is no parent administered scale for facial nerve function in children. We set out to assess the agreement between a newly developed parent‐administered modified version of the House‐Brackmann (HB) scale and the standard clinician‐administered HB scale in children with Bell's palsy. Study Design Secondary analysis of a triple‐blind, randomized, placebo‐controlled trial of corticosteroids to treat idiopathic facial paralysis (Bell's palsy) in children (6 months to <18 years). Setting Multicenter study at pediatric hospitals with recruitment in emergency departments. Methods Children were recruited within 72 hours of symptom onset and assessed using the clinician‐administered and the parent‐administered modified HB scales at baseline, and at 1, 3, and 6 months until recovered. Agreement between the 2 scales was assessed using intraclass coefficient (ICC) and a Bland‐Altman plot. Results Data were available for 174 of the 187 children randomized from at least 1 study time point. The mean ICC between clinician and parent HB scores across all time points was 0.88 (95% confidence interval, CI: 0.86, 0.90). The ICC for the data collected at baseline was 0.53 (95% CI: 0.43, 0.64), at 1 month was 0.88 (95% CI: 0.84, 0.91), at 3 months was 0.80 (95% CI: 0.71, 0.87) and at 6 months was 0.73 (95% CI: 0.47, 0.89). A Bland‐Altman plot indicated a mean difference between the 2 scores (clinician‐reported minus parent‐reported) of only −0.07 (95% limits of agreement −1.37 to 1.23). Conclusion There was good agreement between the modified parent‐administered and the clinician‐administered HB scales.

Background and Objectives Corticosteroids are used to treat the early stages of idiopathic facial paralysis (Bell palsy) in children, but their effectiveness is uncertain. We set out to determine whether prednisolone improves the proportion of children with Bell palsy with complete recovery at 1 month. Methods We conducted a double-blind, placebo-controlled, randomized trial of prednisolone in children presenting to emergency departments with Bell palsy. Patients aged 6 months to younger than 18 years were recruited within 72 hours after the symptom onset and were randomly assigned to receive 10 days of treatment with oral prednisolone (approximately 1 mg/kg) or placebo. The primary outcome was complete recovery of facial function at 1 month rated on the House-Brackmann scale. Secondary outcomes included facial function, adverse events, and pain up to 6 months. Target recruitment was n = 540 (270 per group). Results Between October 13, 2015, and August 23, 2020, 187 children were randomized (94 to prednisolone and 93 to placebo) and included in the intention-to-treat analysis. At 1 month, the proportions of patients who had recovered facial function were 49% (n = 43/87) in the prednisolone group compared with 57% (n = 50/87) in the placebo group (risk difference −8.1%, 95% CI −22.8 to 6.7; adjusted odds ratio [aOR] 0.7, 95% CI 0.4 to 1.3). At 3 months, these proportions were 90% (n = 71/79) for the prednisolone group vs 85% (n = 72/85) for the placebo group (risk difference 5.2%, 95% CI −5.0 to 15.3; aOR 1.2, 95% CI 0.4 to 3.0) and, at 6 months, 99% (n = 77/78) and 93% (n = 76/82), respectively (risk difference 6.0%, 95% CI −0.1 to 12.2; aOR 3.0, 95% CI 0.5 to 17.7). There were no serious adverse events and little evidence for group differences in secondary outcomes. Discussion In children with Bell palsy, the vast majority recover without treatment. This study, although underpowered, does not provide evidence that early treatment with prednisolone improves complete recovery. Trial Registration Information Registered with the Australian New Zealand Clinical Trials Registry ACTRN12615000563561, registered June 1, 2015. anzctr.org.au/Trial/Registration/TrialReview.aspx?id=368505&isReview=true. Classification of Evidence This study provides Class I evidence that for children with Bell palsy, prednisolone does not significantly change recovery of complete facial function at 1 month. However, this study lacked the precision to exclude an important harm or benefit from prednisolone.

BACKGROUND AND OBJECTIVES:Corticosteroids are used to treat the early stages of idiopathic facial paralysis (Bell palsy) in children, but their effectiveness is uncertain. We set out to determine whether prednisolone improves the proportion of children with Bell palsy with complete recovery at 1 month. METHODS:We conducted a double-blind, placebo-controlled, randomized trial of prednisolone in children presenting to emergency departments with Bell palsy. Patients aged 6 months to younger than 18 years were recruited within 72 hours after the symptom onset and were randomly assigned to receive 10 days of treatment with oral prednisolone (approximately 1 mg/kg) or placebo. The primary outcome was complete recovery of facial function at 1 month rated on the House-Brackmann scale. Secondary outcomes included facial function, adverse events, and pain up to 6 months. Target recruitment was n = 540 (270 per group). RESULTS:Between October 13, 2015, and August 23, 2020, 187 children were randomized (94 to prednisolone and 93 to placebo) and included in the intention-to-treat analysis. At 1 month, the proportions of patients who had recovered facial function were 49% (n = 43/87) in the prednisolone group compared with 57% (n = 50/87) in the placebo group (risk difference -8.1%, 95% CI -22.8 to 6.7; adjusted odds ratio [aOR] 0.7, 95% CI 0.4 to 1.3). At 3 months, these proportions were 90% (n = 71/79) for the prednisolone group vs 85% (n = 72/85) for the placebo group (risk difference 5.2%, 95% CI -5.0 to 15.3; aOR 1.2, 95% CI 0.4 to 3.0) and, at 6 months, 99% (n = 77/78) and 93% (n = 76/82), respectively (risk difference 6.0%, 95% CI -0.1 to 12.2; aOR 3.0, 95% CI 0.5 to 17.7). There were no serious adverse events and little evidence for group differences in secondary outcomes. DISCUSSION:In children with Bell palsy, the vast majority recover without treatment. This study, although underpowered, does not provide evidence that early treatment with prednisolone improves complete recovery. TRIAL REGISTRATION INFORMATION:Registered with the Australian New Zealand Clinical Trials Registry ACTRN12615000563561, registered June 1, 2015. anzctr.org.au/Trial/Registration/TrialReview.aspx?id=368505&isReview=true. CLASSIFICATION OF EVIDENCE:This study provides Class I evidence that for children with Bell palsy, prednisolone does not significantly change recovery of complete facial function at 1 month. However, this study lacked the precision to exclude an important harm or benefit from prednisolone.

Background: Computed tomography (CT) is the accepted imaging modality to assess choanal atresia (CA) but carries the risks of ionising radiation.Methods: In this proof-of-concept retrospective study, the imaging findings in children under 12 months of age with bilateral or unilateral CA who underwent both CT and magnetic resonance imaging (MRI) prior to surgical repair, were evaluated using a checklist of descriptive and quantitative parameters considered relevant in pre-operative planning.Results: Seven patients were included. MRI was able to identify CA as unilateral or bilateral in all cases. An intact skull base as identified on CT was confirmed on MRI in all cases. Choanal height, width and skull base slope were able to be assessed in most MRI scans, however, quantitative correlation between CT and MRI could not be established.Conclusions: MRI was able to correctly report presence of CA and intact skull base in all cases. MRI appears to be less sensitive in identifying bony intranasal and mid face abnormalities. While this limited study demonstrated that MRI could assess some relevant surgical points, correlation between the gold-standard of CT is yet to be established and further prospective studies are required. It is unknown what impact the variability between imaging modalities has on surgical planning.

BACKGROUND:A significant number of surgeries in children are being performed in the private setting. Our aim was to determine the rate of unplanned inter-hospital transfers (IHTs) for paediatric patients undergoing elective surgical procedures in a private hospital without a paediatric intensive care unit to a tertiary hospital, and to investigate the reasons for these transfers.METHODS:A retrospective clinical audit was performed searching hospital coded data of all patients aged 18 years or less at the date of admission, who underwent elective surgery between 1 January 2013 and 31 October 2018 at St Vincent's East Melbourne Private Hospital.RESULTS:A total of 17 366 patients were identified, of whom 23 required IHT, with an overall transfer rate of 0.13%. Adenotonsillectomy had the highest IHT rate of 0.26%; however, operative specialty had no statistical correlation with IHT (P = 0.24) with a comparable transfer rate across all specialties. Hypoxia was the most frequent reason for IHT and was the cause in 16 out of 23 transfers (69%). Nine cases (39%) were transferred due to hypoxia while awake and seven (30%) due to hypoxia only while asleep. Three patients requiring IHT were identified as having preoperative acute respiratory illness.CONCLUSION:Elective paediatric surgery undertaken at St Vincent's East Melbourne Private Hospital is safe and has a low IHT rate, with surgery involving the upper airway having a higher risk. In the paediatric population, hypoxia while awake is the most frequent cause for IHT.

Aim Transfer of patients from a regional to a tertiary paediatric facility is sometimes required for management of otolaryngological conditions when the expertise available locally is insufficient to deal with the complexity of the case. Methods A retrospective analysis was carried out over a 3‐year period of all patients under 18 years of age who were transferred to the Royal Children's Hospital Melbourne, primarily for otolaryngology management, from a regional hospital with an inpatient paediatric service and located 100 km or far from the Royal Children's Hospital. Results Thirty‐nine patients (30 male, 9 female) were identified. The majority of patients (29, 74%) required surgical management, with the most common procedures being rigid bronchoscopy with or without foreign body removal (7 patients, 24%), drainage of deep neck space infection (7 patients, 24%) and bilateral choanal atresia repair (4 patients, 13%). Conclusion Notwithstanding the cost and inconvenience, transfer was necessary in essentially all cases in this study for either specialist surgical management or medical management of conditions that could have otherwise escalated in acuity. By inference, it would appear that most routine acute paediatric otolaryngology conditions are managed successfully in a regional setting.

Background. Recurrent respiratory papillomatosis is a rare but morbid disease caused by human papillomavirus (HPV) types 6 and 11. Infection is preventable through HPV vaccination. Following an extensive quadrivalent HPV vaccination program (females 12-26 years in 2007-2009) in Australia, we established a method to monitor incidence and demographics of juvenile-onset recurrent respiratory papillomatosis (JORRP) cases.Methods. The Australian Paediatric Surveillance Unit undertakes surveillance of rare pediatric diseases by contacting practitioners monthly. We enrolled pediatric otorhinolaryngologists and offered HPV typing. We report findings for 5 years to end 2016.Results. The average annual incidence rate was 0.07 per 100 000. The largest number of cases was reported in the first year, with decreasing annual frequency thereafter. Rates declined from 0.16 per 100 000 in 2012 to 0.02 per 100 000 in 2016 (P =.034). Among the 15 incident cases (60% male), no mothers were vaccinated prepregnancy, 20% had maternal history of genital warts, and 60% were first born; 13/15 were born vaginally. Genotyped cases were HPV-6 (n = 4) or HPV-11 (n = 3).Conclusion. To our knowledge, this is the first report internationally documenting decline in JORRP incidence in children following a quadrivalent HPV vaccination program.

The advent of the slide tracheoplasty technique and a multi-disciplinary approach has improved outcomes of congenital tracheal stenosis. However, tracheal surgery in younger patients with pulmonary malformations, especially low birth-weight neonates, has been associated with increased mortality. Patients with very low birth-weight, pulmonary malformations and prematurity may be palliated prior to definitive tracheal surgery due to the poor prognosis. We report a successful and unique approach of delaying tracheal reconstruction to allow growth and development in the premature, very low birth-weight neonate (1046g) with left lung agenesis.

Introduction: Bilateral choanal atresia (BCA) is associated with a high incidence of congenital abnormalities that include skull base anomalies and defects. Surgical repair of BCA is necessary in the early neonatal period and any altered anatomy of the adjacent skull base will heighten the risk of intracranial injury. This risk may be further increased in patients with CHARGE syndrome. Objectives: To measure surgically relevant nasal and skull base dimensions in neonates with BCA in order to determine whether any difference exists between isolated and CHARGE syndrome associated subgroups, thereby optimizing the safety of surgical repair. Methods: A retrospective review of medical charts and computed tomography was undertaken at a tertiary pediatric hospital of all neonates diagnosed with BCA between 2004 and 2016. Isolated and CHARGE syndrome subgroups of BCA were identified from clinical records and CT data was analyzed and compared between the two. The skull base parameters measured were choanal width, choanal height, mid-nasal skull base height and skull base slope. Results: Of the 13 patients included, 3 had CHARGE syndrome and 10 had isolated BCA. Whilst the difference in mid-nasal height approached significance for the two groups, numbers were too small for a statistical difference to be identified. The mean value for choanal width in the isolated BCA group was significantly less the largest series of normative data available in the literature for comparison (p < 0.001). No skull base anomalies were noted in either group. Conclusion: While this is a small study with limited numbers, it is the first that has attempted to identify and measure the posterior nasal and skull base anatomy most pertinent to avoiding skull base injury in the surgical management of BCA.

Introduction To estimate and monitor national incidence of Juvenile onset Recurrent Respiratory Papillomatosis (JoRRP) in Australia following the extensive quadrivalent HPV vaccine catch up program (females aged 12–26 years in 2007–2009, which included women of child bearing age) and to assess demographics and risk factors of incident cases. Methods The Australian Paediatric Surveillance Unit (APSU) undertakes surveillance of rare paediatric diseases by contacting practitioners monthly to report cases. We utilised this well established methodology to undertake prospective population based surveillance of JoRRP by enrolment in APSU of paediatric ENT surgeons, designing a JoRRP case reporting form, and offering clinicians HPV typing of incident cases. Surveillance commenced Oct 2011 and we report here findings for the five-year period to end 2016. Results Using Australian Bureau of Statistics population estimates for children 0–15 years, the average annual incidence rate over the period was 0.12 per 1 00 000. The largest number of cases was reported in the first year, with a decreasing frequency each year thereafter. The rate declined from 0.3 per 1 00 000 in 2012 to 0.04 per 1 00 000 in 2016. Among incident cases, no mothers had been vaccinated prior to pregnancy, 20% had a past history of genital warts, 60% of cases were male, and 60% were first born. The majority were born by vaginal delivery. Four incident cases were genotyped; all were positive for HPV6 (n=1) or HPV11 (n=3). Conclusion To our knowledge this is the first report internationally documenting a decline in JoRRP incidence in a population of children following the introduction of a quadrivalent HPV vaccination program. Support: I Professor Suzanne Garland, have received Grants to my institution from Commonwealth Department of Health for HPV genoprevalance surveillance post vaccination, Merck and GSK (GlaxoSmithKline) to perform phase 3 clinical vaccine trials: Merck to evaluate HPV in RRP post vaccination programme, CSL for HPV in cervical cancer study, and VCA (Victoria Cancer Agency) for a study on effectiveness of public health HPV vaccine study plus a study on associations of early onset cancers. I have received speaking fees from MSD and SPMSD for work performed in my personal time. Merck paid for travel and accommodation to present at HPV Advisory board meetings.

Bell’s palsy or acute idiopathic lower motor neurone facial paralysis is characterized by sudden onset paralysis or weakness of the muscles to one side of the face controlled by the facial nerve. While there is high level evidence in adults demonstrating an improvement in the rate of complete recovery of facial nerve function when treated with steroids compared with placebo, similar high level studies on the use of steroids in Bell’s palsy in children are not available. The aim of this study is to assess the utility of steroids in Bell’s palsy in children in a randomised placebo-controlled trial.

Background: Unilateral tonsillar enlargement (UTE) is not an uncommon incidental finding. Lymphoma is the most common malignancy of the tonsils in children and presents with tonsillar enlargement. Methods: This is a retrospective case series of all cases of histopathological analysis of tonsillectomy specimens at a single pediatric tertiary hospital between 1996 and 2015. Patients either had a preoperative indication (n = 53) for pathological testing or indications based on intraoperative findings (n = 88). Of the preoperative group, 17 patients had tonsillectomy for UTE alone. In 72% of cases, the side of perceived unilateral enlargement was confirmed at histopathological assessment. Results: Zero cases of suspected malignancy were identified on testing. Conclusion: There is a very low prevalence of unsuspected and suspected malignancy in routine and targeted examination of tonsillar specimens. It is reasonable to test based on other clinical findings and risk factors such as immunosuppression.

To review the scientific literature on the relationship between Kölliker-Fuse nucleus (KF) and cranial nerve function in animal models, with view to evaluating the potential role of KF maturation in explaining age-related normal physiologic parameters and developmental and acquired impairment of cranial nerve function in humans. Medical databases (Medline and PubMed). Studies investigating evidence of KF activity responsible for a specific cranial nerve function that were based on manipulation of KF activity or the use of neural markers were included. Twenty studies were identified that involved the trigeminal (6 studies), vagus (9), and hypoglossal nerves (5). These pertained specifically to a role of the KF in mediating the dive reflex, laryngeal adductor control, swallowing function and upper airway tone. The KF acts as a mediator of a number of important functions that relate primarily to laryngeal closure, upper airway tone and swallowing. These areas are characterized by a variety of disorders that may present to the otolaryngologist, and hence the importance of understanding the role played by the KF in maintaining normal function.

Dear Sir, I greatly enjoyed reading the paper by David and Lim on “Congenital Bilateral Vocal Fold Paralysis”1 and complement the authors on their excellent surgical outcome. They also very appropriately underscored the primacy of careful clinical assessment over costly investigations, particularly in an environment of scarce resources. We have previously reported on the MRI findings in 23 children with bilateral vocal fold dysfunction (BVFD) as an isolated abnormality that was present at birth. We found MRI abnormalities in 35%, but these were all non-specific.2 This study indicated that MRI was of research and clinical value, but did not identify any major CNS structural abnormalities in this patient group. However, the case reported by David and Lim involved the delayed onset of symptoms at 2 years of age that may have increased over the next 3 years. This acquired and possibly progressive disease process differs from that of congenital BVFD. The likelihood of Arnold-Chiari malformation or another significant structural CNS abnormality being present would appear to be higher when symptoms of BVFD are acquired later in life compared to when they are present at birth. Anecdotally, I have been involved with a case of acquired BVFD presenting at a similar age where the underlying cause was found to be a posterior fossa tumour. The 3 year history of symptoms in the child in the case report would very likely preclude this diagnosis, but certainly another CNS cause needs to be considered to explain the development of delayed-onset brainstem dysfunction, and this would require MRI. Robert Berkowitz Department of Otolaryngology Royal Children’s Hospital Melbourne, Australia

Objective: Tinnitus in adults is generally investigated by contrast-enhanced magnetic resonance imaging (MRI) to rule out the diagnosis of acoustic neuroma. Acoustic neuroma is rare in children and, therefore, the role of MRI in children with tinnitus is unclear. This study was undertaken to determine the value of MRI in the investigation of tinnitus in children. Methods: Retrospective study of children younger than 18 years who underwent MRI for the investigation of tinnitus over a 10-year period. Results: Sixty-five patients were identified, but there were only 34 who had also undergone audiologic assessment. Among the 25 patients with normal audiology, MRI abnormalities were present in 9, but these were all thought to be nonspecific. Nine patients had abnormal audiograms and the MRI was abnormal in 4 of these cases, which included 3 children who were found to have multiple sclerosis. Conclusion: Magnetic resonance imaging would appear to be mandatory in the investigation of tinnitus in children who are found to have sensorineural hearing loss, particularly to rule out the diagnosis of multiple sclerosis. Although our study does not support the routine use of MRI in children with normal audiology, the numbers in our series are too small for a conclusive recommendation.

Many respiration‐related interneurons and motoneurons receive a catecholaminergic input, but the extent and distribution of this input to recurrent laryngeal motoneurons that innervate intrinsic muscles of the larynx are not clear. In the present study, we examined the catecholaminergic input to expiratory laryngeal motoneurons in the caudal nucleus ambiguus by combining intracellular labeling of single identified motoneurons, with immunohistochemistry to reveal tyrosine hydroxylase immunoreactive (catecholaminergic) terminal varicosities. Close appositions were found between the two structures, with 18 ± 5 close appositions per motoneuron (n = 7). Close appositions were more frequently observed on distal rather than proximal dendrites. Axosomatic appositions were not seen. In order to determine the source of this input, microinjections of cholera toxin B subunit (1%, 20 nl) were made into the caudal nucleus ambiguus. Retrogradely labeled neurons, located in the ipsilateral nucleus tractus solitarius and the area postrema, were tyrosine hydroxylase‐positive. Our results not only demonstrate details of the extent and distribution of potential catecholamine inputs to the expiratory laryngeal motoneuron, but further indicate that the inputs, at least in part, originate from the dorsomedial medulla, providing a potential anatomical basis for previously reported catecholaminergic effects on the laryngeal adductor reflex. J. Comp. Neurol. 523:381–390, 2015. © 2014 Wiley Periodicals, Inc.

Objectives: We studied the findings of brain magnetic resonance imaging (MRI) in infants with idiopathic congenital bilateral vocal fold dysfunction (CBVFD). Methods: We performed a retrospective investigation of a case series. Results: We identified 26 children (14 male, 12 female) over 11 years. Three children were excluded. Thirteen patients required airway interventions, including continuous positive airway pressure (4 patients), endotracheal intubation (1), and tracheostomy (8). The findings on brain MRI were abnormal in 8 patients (35%). Tracheostomy was required in 3 patients (38%) with abnormal MRI findings, as compared with 5 of 15 patients (33%) with normal MRI findings. The MRI abnormalities involved evidence of white matter injury (2), abnormal white matter signal (1), subdural blood (3), cerebral swelling (1), and perisylvian polymicrogyria (1). The cranial ultrasound findings were abnormal in 4 of 11 patients. The MRI findings were abnormal in 2 of 7 children in whom the cranial ultrasound findings were normal, and in 2 of the 4 patients in whom the cranial ultrasound findings were abnormal. Conclusions: The MRI abnormalities were nonspecific; however, they may indicate unrecognized perinatal intracranial injury as being related to CBVFD. In addition, MRI may reveal an underlying structural brain anomaly. Cranial ultrasound has poor sensitivity and specificity. Hence, MRI should be considered as part of the routine assessment of neonates with CBVFD.