International Journal of Pediatric Otorhinolaryngology

International Journal of Pediatric Otorhinolaryngology

Factors affecting persistent tympanic membrane perforation after tympanostomy tube removal in children

02-12-2019 – Clarice Brown, Philomena Behar

Journal Article

Objectives

Evaluate and compare the rates of persistent tympanic membrane (TM) perforations between short-term vs long-term tympanostomy tubes. In addition, to determine which demographic, anatomical, and physical factors affect ear drum healing after tympanostomy tube (TT) removal and simultaneous paper patch myringoplasty (PPM) in children.

Study design

Retrospective chart review.

Methods

Charts were reviewed from the Women and Childrens of Buffalo hospital and our pediatric otolaryngology practice electronic medical record. Data was retrieved from patients less than 18 years old who underwent surgical removal of a TT and concomitant PPM between January 2005 and January 2017.

Results

343 ear drums were studied that underwent tympanostomy tube removal and paper patch myringoplasty. 45/343 (13%) of ears had a persistent perforation after PPM. The rate of persistent perforation with short-term tubes and long-term tubes was significantly different (6.6% and 20% respectively). Patient characteristics significantly associated with persistent perforations were: age at time of tube removal and number of tubes that patient received. Ear drum characteristics that significantly impacted persistent perforation included: size of ear drum perforation, and presence of tympanosclerosis. Length of intubation, ear drum atrophy, retraction, granulation tissue, middle ear effusion and thickened ear drum were not found to be significant factors.

Conclusion

Overall, we found an 87% perforation closure rate after surgical removal of the TT and PPM. Persistent perforations occurred significantly more often in patients with long-term tubes than short term tubes. Our data also suggests that several patient and ear drum characteristics may be important factors that contribute to persistent perforation after tympanostomy tube placement and removal.

Efficacy of crenotherapy by Politzer in the treatment of otitis media with effusion in children with down syndrome

04-12-2019 – Massimo Mesolella, Brigida Iorio, Filippo Ricciardiello, Gaetano Motta

Journal Article

Safety of nasal stenting in pharyngeal flap surgery for pediatric velopharyngeal dysfunction

04-12-2019 – R. Araslanova, R. Instrum, A. Dzioba, M. Husein

Journal Article

Introduction

The pharyngeal flap procedure is an effective surgery for velopharyngeal dysfunction (VPD) yet carries approximately a 3.2% post-operative airway obstruction risk. Life threatening airway compromise occurs in first 24 h post-operatively. Nasopharyngeal airway has been shown to decrease these complications but its routine use is not commonplace. At our centre, surgical technique involves routine placement of bilateral nasopharyngeal airway referred to as nasal stenting. Our objective was to demonstrate safety of nasal stenting for children with VPD undergoing pharyngeal flap surgery.

Methods

A retrospective review of pediatric patients aged 1 through 18 years at the time of VPD diagnosis, who underwent superiorly based pharyngeal flap surgery at London Health Sciences Centre (LHSC), was conducted. Patients were admitted to a regular ward with bihourly oxygen saturation checks. Nasal stents were removed on post-operative day two. Demographic data along with patient outcomes and perioperative complications were collected and analyzed.

Results

Eighty-five pediatric patients underwent superiorly based pharyngeal flap surgery at LHSC from November 2004 through February 2017 that met inclusion criteria. Mean age at the time of surgery was 11.0 years, whereas average age at diagnosis was 8.7 years. The majority of patients (60.0%) had history of cleft palate repair. Only 28.2% had additional comorbidities, the most common being Pierre-Robin Sequence. Average length of hospital stay was 2.7 days. No patients required admission to an intensive care unit. Surgical complication rate was 8.2%. No post-operative airway obstruction events were encountered. Two patients (2.3%) underwent pharyngeal flap reversal for obstructive sleep apnea and one for persistent nasal obstruction. Five (5.9%) minor stent-related complications were captured. Two patients developed self-limiting epistaxis. Two patients had partially extruded stents prior to their removal. One patient developed nasopharyngeal port granuloma which resolved with a course of intranasal corticosteroids.

Conclusion

Nasal stenting for pharyngeal flap surgery is safe. Stent related complications were minor and did not influence length of hospital stay. A prospective study to determine if routine stenting would lead to decreased serious airway complications is warranted.

Pediatric adenoidectomy in the very young child and indications for postoperative inpatient admission

04-12-2019 – Stephen R. Chorney, Julia F. Dailey, Karen B. Zur

Journal Article

Objectives

To determine the rate of significant respiratory events following adenoidectomy in young patients and to identify factors that would prompt inpatient admission postoperatively.

Methods

A retrospective chart review was performed of consecutive adenoidectomy surgeries at a high-volume, tertiary-care childrens hospital between 2016 and 2018. Children under 3.5 years of age who had surgery for obstructive symptoms were included. Patients were grouped by age (youngest ≤1.5 years, middle 1.6–2.5 years, and oldest 2.6–3.5 years). We excluded patients having revision surgery, a concomitant tonsillectomy, or additional major surgical procedure.

Results

There were 353 patients that met inclusion criteria. The three age groups were similar with respect to all characteristics except age (p < .001), body mass index (p < .001), and percentage of Black or African American children (p = .02). Patients under 1.5 years more often had preoperative polysomnography (p = .02) with a lower oxygen saturation nadir (p = .04), and were more likely to have surgery for obstructive sleep apnea (p < .001). No differences were found between age groups with respect to recovery room issues, nurse triage calls, or readmissions within 30 days of surgery. An elective admission rate in the cohort was 35.1%, and this was age-group dependent with 79.5% of the youngest group being admitted (p < .001). On admission, 16.9% of all patients had admission events requiring positive pressure support, intensive care unit admission, or prolonged hospitalization, which was similar across all age groups (p = .67). Events were more common in younger patients (17 mos. vs 20 mos., p = .07), those with more comorbidities (74.8% vs 51.5%, p = .06) and significantly higher in those with severe preoperative polysomnogram variables (p < .001). Based on multivariate regression analysis, younger children (OR: 13.7, 95% CI: 6.5 – 29.0, p < .001) or children with an AHI over 5 events/hr (OR: 32.3, 95% CI: 3.4 – 303.2, p = .005) were more likely to have significant events on admission.

Conclusions

Significant respiratory events are uncommon after adenoidectomy for obstructive symptoms, even in very young children. However, for children under 1.5 years of age or those with AHI scores above 5 events/hr, postoperative admission for monitoring is recommended. Clinical judgement should be used when considering outpatient surgery for older children or those with comorbidities.

National questionnaire on skills and techniques in pediatric tracheotomy surgery in Turkey

01-12-2019 – Murat Gumussoy, Ibrahim Cukurova, Sinan Atmaca

Journal Article

Objective

The aim of the study was to determine the differences in surgical preferences of ENT Surgeons in Turkey with regard to pediatric tracheotomy.

Design

Questionnaire study.

Participants

ENT Surgeons.

Materials and methods

The national multiple-choice questionnaire study included a total of 16 questions about physicians technical preferences, different methods and complications in pediatric tracheotomy surgery. It was planned and implemented with the support of a professional survey company (www.surveymonkey.com).

Results

The questionnaire was answered by 591 ENT Surgeons; the percentage of the physicians performing pediatric tracheotomies in the previous year was 59.6%. Forty point four percent (40.4%) of the physicians had not performed tracheotomies in the pediatric age group and 57.9% had not performed tracheotomies in patients under one year old. Seventy point six percent (70.6%) of the physicians who had performed tracheotomies had made vertical skin incisions, 69.5% of them had removed subcutaneous adipose tissue, 81.4% of them had retraction the thyroid isthmus area from their field of view; 83.9% of them had made a vertical incision to the trachea, 82.5% of them had applied a stay suture to the trachea and 4.7% of them had used additional techniques for stoma maturation. The intraoperative mortality, early complication, late complication, and total complication rates were 3.9%, 32.7%, 21.2% and 53.9 respectively.

Conclusions

This is the first large-scale questionnaire study with data on pediatric tracheotomy techniques and the practices of ENT Surgeons at a national level. Common approaches were observed among the.

Cochlear implant outcomes in children with attention-deficit/hyperactivity disorder: Comparison with controls

01-12-2019 – Shaghayegh Omidvar, Zahra Jeddi, Afsaneh Doosti, Seyed Basir Hashemi

Journal Article

Objectives

The coincidence of attention-deficit/hyperactivity disorder (ADHD) and hearing loss in the children has adverse effects in speech, cognition, communication and motor development. This can influence cochlear implant (CI) outcomes negatively. The aim of this study was to compare auditory, language, speech, cognition, communication and motor outcomes between CI children with versus without ADHD.

Methods

Nineteen CI children with ADHD and twenty-three age and gender matched children without ADHD at the Shiraz CI center ranging in age from 37 to 60 months were participated in this cross-sectional study. The developmental quotient in auditory, receptive and expressive language, speech and cognition skills was evaluated through Newsha Developmental Scale. The Persian version of the Ages and Stages Questionnaire (ASQ) was used to assess childrens developmental status in fine and gross movements, communication, problem solving, and personal-social domains. A comparison of the results between two groups was made by the Mann-Whitney test.

Results

CI children with ADHD had significantly lower Newsha developmental quotients in cases of auditory, receptive and expressive language, speech and cognition skills compared to the control group (P = 0.027 to <0.001). A significant difference was observed between children with and without ADHD in fine and gross movements, communication, problem solving, and personal-social domains of ASQ at 60 months (P = 0.029 to 0.003).

Conclusion

Children with ADHD showed decreased ability in auditory, language, speech, cognition, motor and communication skills following CI compared to children without ADHD. It can guide clinician to provide these children with more specific rehabilitation program to improve their skills.

The immunology of the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; what can the tonsils reveal. A literature review

02-12-2019 – Jostein Førsvoll, Einar Klæboe Kristoffersen, Knut Øymar

Journal Article, Review

Objectives

Tonsillectomy (TE) or adenotonsillectomy (ATE) may have a beneficial effect on the clinical course in children with the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. However, an immunological reason for this effect remains unknown. This literature review summarizes the current knowledge regarding the immunological role of the tonsils in the PFAPA syndrome.

Methods

We searched PubMed, Medline, EMBASE and Cochrane for papers written in English dated from 1 January 1987 to 30 April 2019. The search included all studies reporting histological, immunological or microbiological workup of tonsil specimens from children aged 0–18 years with PFAPA.

Results

Thirteen articles reported histological, immunological or microbiological workup of tonsil specimens in children with PFAPA. The histology of tonsil specimens from children with PFAPA displayed chronic tonsillar inflammation with lymphoid hyperplasia. No uniform immunological pattern was identified, but some studies found fewer B-lymphocytes and smaller germinal centers in PFAPA compared to controls. A difference in tonsillar microbiota between PFAPA and controls was found in one study.

Conclusion

A uniform immunological or microbiological pattern explaining the clinical effect of TE in children with PFAPA has not been revealed. Future targeted immunological studies of tonsils in PFAPA patients could possibly illuminate the understanding of the immunology in this disease.

Genetic identification and molecular modeling characterization of a novel POU3F4 variant in two Italian deaf brothers

02-12-2019 – Sara Giannantonio, Emanuele Agolini, Alessandro Scorpecci, Roberta Anzivino, Emanuele Bellacchio, Dario Cocciadiferro, Antonio Novelli, Maria Cristina Digilio, Pasquale Marsella

Journal Article

In this report, we describe a novel, probably pathogenic hemizygous variant c.870G > T (p.
Lys290Asn) in the POU3F4 gene in two deaf brothers from one Italian family with identical inner ear abnormalities specific to X-linked deafness-2 (DFNX2). In addition, we performed homology modeling to predict the effect of the missense variant on the protein structure showing a possible disruption of the normal folding. The identification of pathogenic variants causing X-linked recessive deafness will improve molecular diagnosis, genetic counseling, and knowledge of the molecular epidemiology of hearing loss among Italian individuals. Taken together, we recommend preoperative gene mutation analysis in patients who have DFNX2 diagnosed on the basis of characteristic radiological findings, in order to provide with better prognostic information, the risk of recurrence, and improved rehabilitation options. Finally, the present work strengthens the hypothesis that DFNX-2 could be considered as a syndromic deafness, since mixed hearing loss is associated with other dysfunctions of the neuropsychological profile of the patients.

Fibrous dysplasia mimicking a pediatric endolymphatic sac tumor

27-11-2019 – Kasey W. Rawlins, Kevin Y. Zhan, Jameson K. Mattingly, Alexander Z. Feldman, Oliver F. Adunka

Journal Article

A 15-year-old female presented for evaluation of progressive hearing loss over a year. Computed tomographic imaging revealed a 11 x 6 × 6 mm osseous lesion with groundglass appearance within the left posterior petrous bone lateral to vestibular aqueduct suspicious for an endolymphatic sac tumor. Surgical excision revealed fibrous dysplasia on histological analysis. Fibrous dysplasia of the temporal bone is not uncommon, but few cases of extension causing sensorineural hearing loss exist in the literature. We describe the first reported case of fibrous dysplasia mimicking an endolymphatic sac tumor; interestingly, the patient also showed hearing improvement immediately following removal.

Grisel syndrome, sigmoid sinus thrombosis and rheumatic carditis: Case report of a rare association

18-11-2019 – Maria Elena Cucuzza, Angela DAmbra, Francesco Roberto Evola, Filippo Greco, Pierluigi Smilari

Journal Article

Grisels syndrome (GS) is a non traumatic atlanto-axial rotatory subluxation of C1–C2 joint. A six year old girl, 20 days after an episode of fever, developed a torticollis and a 3/6 heart murmur. The echocardiography showed a Rheumatic Carditis. The Brain and cervical spine Magnetic resonance imaging (MRI) and the Computerized Tomography (CT) showed rotary dislocation of C1–C2 vertebrae, compatible with GS, and cerebral venous thrombosis (CVT). An antibiotic therapy, Prednisone and a low molecular weight heparin for 7 days was prescribedfollowed by an oral anticoagulant for 6 months. After a month the MRI showed a reduction of the dislocation and cerebral venous recanalization.

Bevacizumab as adjuvant therapy for recurrent respiratory papillomatosis in an infant

11-11-2019 – Christopher Gates, Patrick Tomboc, Andrew Allison, Michele Carr

Journal Article

Recurrent Respiratory Papillomatosis (RRP) is a benign disease of the airway that can result in symptoms ranging from mild dysphonia to respiratory distress to respiratory failure related to colonization of the lung parenchyma. It is a disease that typically begins in childhood and can require treatment indefinitely, though may remit in adolescence. Although treatment includes surgical management, certain cases require adjuvant therapy. Many therapies have been tried over the years with varying levels of success. Our patient presented as a 7 month old male in respiratory distress, who was subsequently diagnosed with RRP. He has required a tracheostomy, ventilation, and frequent surgical debridement for the last 20 months. Due to the aggressiveness of his disease, he was started on systemic bevacizumab (Avastin) in an effort to stabilize his lesions. Though early effect seemed apparent based on Derkay scores, systemic bevacizumab has failed to elicit clinical improvement in our patient. He remains with a tracheostomy receiving bevacizumab 10 mg/kg as an intermittent infusion approximately every 4 weeks along with surgical debridement with little to no clinical improvement thus far. In light of the recent successes with bevacizumab therapy, this case highlights the difficulty in finding new treatment for this disease.

Congenital meningocele associated with corpus callosum agenesis and midline superior labial cleft due to Tessier 0–14 facial fissure: A case report

11-11-2019 – Alejandro Fandiño, Alfredo J. Herrera, Oscar E. Feo, Angela M. Ronderos, Juan C. Ospina, Oscar De León

Journal Article

A case of a 31-day-old infant patient with a Tessier 0–14 deformity related to multiple midline deformities is presented. Although Transnasal endoscopic surgery is the mainstay for the treatment of anterior and middle skull base meningoceles, there are complex cases in which a combined and multidisciplinary approach is necessary.

The surgical decisions and techniques are described. To date, this is the first patient reported with Tessier 0–14 deformity treated with a combined endoscopic and external surgical approach.

Surgical mapping of middle ear cholesteatoma with fusion of computed tomography and diffusion-weighted magnetic resonance images: Diagnostic performance and interobserver agreement

28-11-2019 – Sunil Dutt Sharma, Andrew Hall, Anthony C. Bartley, Paul Bassett, Arvind Singh, Ravi K. Lingam

Journal Article

Objective

To assess the diagnostic performance in detecting primary cholesteatoma at various anatomical subsites using Computed Tomography (CT), Diffusion-weighted Magnetic Resonance Imaging (DWMRI) and Fusion of CT and DWMRI (Fusion CT-MRI) images.

Study design

A retrospective study of 22 children identified from a prospective database of surgically treated cholesteatoma cases over a five year period. All cases underwent pre-operative CT, non-echo planar DWMRI and Fusion CT-DWMRI, and with clearly documented surgical findings. For each imaging modality, two radiologists scored for the presence or absence of cholesteatoma with confidence levels at different anatomical subsites. The radiologists were blinded to the surgical findings to which their findings were compared.

Setting

Large Teaching Hospital in London.

Patients

22 children with cholesteatoma confirmed surgically.

Intervention

CT, DWMRI imaging and fusion CT-MRI.

Main outcome measure

Diagnostic performance of subsite localisation of cholesteatoma by CT, DWMRI and fusion CT-MRI imaging with intra-operative findings.

Results

Twenty-two patients were included (12 women and 10 men). The median age of patients was 11 years. When considering all subsites combined, the result for all imaging methods suggested ‘good’ agreement between both observers. When all subsites were examined together, all methods had relatively high sensitivity values (87% for CT vs 84% for DWMRI vs 85% for fusion CT-DWMRI). Specificity was highest with fusion CT-DWMRI (46% for CT vs 76% for DWMRI vs 97% for fusion CT-DWMRI), as was accuracy (66% for CT vs 80% for DWMRI vs 91% for fusion).

Conclusions

Our study has demonstrated that fusion CT-DWMRI is superior to DWMRI or CT separately in localizing cholesteatoma at various middle ear cleft subsites and bony relations, making it a valuable tool for surgical planning.

Relation of public health staffing to follow-up after newborn hearing screening in three health districts in Georgia, 2009–2015

25-11-2019 – Kareem Al-Mulki, N. Wendell Todd

Journal Article

Objective

To describe the association, or non-association, of public health district staffing (specifically, Early Hearing Detection and Intervention EHDI coordinator/navigator) and loss to follow-up in newborns who did not pass hearing screening in selected public health districts in Georgia, USA.

Methods

By Freedom of Information request, data regarding newborn hearing screening and loss to follow-up for diagnostic testing and staffing were acquired for three districts in Georgia for six years. The districts were chosen because their coordinator/navigator positions were unfilled at times.

Results

Lapses in staffing of the district EHDI coordinator/navigator position aligned temporally with decreased follow-up. Aggregate three district data showed that follow-up rates in quarter-years with a fulltime navigator were higher than quarter-years without a full-time navigator (p < .001).

Conclusion

Lapses in staffing dedicated to EHDI navigation-coordination correlated with poorer follow-up after not passing newborn hearing screening.

Video head impulse test in children with otitis media with effusion and dizziness

25-11-2019 – Mesut Tozar, Ela Cömert, Ziya Şencan, Gökçe Şimşek, Nuray Bayar Muluk, Rahmi Kılıç

Journal Article

Objective

The objectives of the study were to evaluate the vestibular impairment in children with otitis media with effusion (OME) and dizziness by using v
HIT test, and to compare their results with healthy children.

Methods

The study population consisted of 30 pediatric patients with OME and dizziness and 30 healthy children, age between 4 and 15. Otoscopic and tympanometric examination and v
HIT testing were performed to all subjects. v
HIT test parameters were compared between pediatric patients with OME and dizziness and healthy children. Additionally, the differences of the mean v
HIT gains between tympanogram types, otoscopic findings and the presence of saccades were analyzed.

Results

The mean v
HIT gains and gain asymmetry values of patients with OME and dizziness and healthy children were comparable. No significant difference was observed between the mean v
HIT gains of patients with type B and type C2 tympanogram. Covert saccades were observed in 57% of the patients with OME and dizziness. None of the patients had over saccades and none of the healthy children had saccades.

Conclusion

Our study is a preliminary study analyzing the vestibular impairment in children with OME and dizziness using v
HIT test. Based on our results, it can be assumed that the children with OME and dizziness usually dont have a great vestibular impairment that can be detected with v
HIT test. The covert saccades detected in this patient group are accepted as a sign of slight vestibular impairment.

Publishing trends in velopharyngeal insufficiency

25-11-2019 – Charles Poff, Joshua Horton, Ryan Boerner, Alexander P. Marston, Shaun A. Nguyen, David R. White

Journal Article

Objective

This study seeks to describe publishing trends for VPI over a 33-year span with regard to treating specialty, methods of assessment, related diagnoses, and methods of treatment for each specialty.

Methods

A PubMed search was performed on “velopharyngeal insufficiency” using medical subject headings terms from 1985 to 2017. Publisher specialty, method(s) of VPI assessment, associated diagnosis/diagnoses, and method(s) of VPI treatment per specialty and combined across specialties were analyzed. Respective publications were totaled in 11-year intervals and two-way analysis of variance was used to compare change over time within specialties and across specialties.

Results

763 publications were included for analysis. The total number of publications on VPI increased from a total of 6 in 1985 to a peak of 67 in 2015. The specialties that showed the largest increase in relative frequency of publication were Otolaryngology (p < 0.001), Plastic Surgery (p < 0.001), and Multidisciplinary (p < 0.001). Publications on endoscopic (p < 0.001) evaluation of VPI have significantly increased over time relative to magnetic resonance imaging and lateral cephalometry. Across all specialties, publications that feature pharyngoplasty (p < 0.001), palatoplasty (p < 0.001), and pharyngeal flap (p < 0.001) as methods of VPI treatment have significantly increased over time.

Conclusion

There is a trend towards endoscopy for diagnostics and a multidisciplinary approach when managing patients with VPI. The specialty that showed the largest increase in the relative frequency of publication was Otolaryngology. Surgical methods of treatment continue to be described at increasing frequency relative to more conservative treatments.

Dysphonia screening in vocally trained and untrained children

24-11-2019 – R. Pribuisiene, A. Pasvenskaite, K. Pribuisis, T. Balsevicius, V. Liutkevicius, V. Uloza

Journal Article

Aim

To evaluate dysphonia rate and impact of vocal training in vocally trained (VT) and vocally untrained (VUT) children using the Lithuanian version of Glottal Function Index (GFI-LT) questionnaire.

Methods

262 children (age range from 6 to 16 years) were included into prospective study. There were 115 consecutive children from four Music Schools in the VT group and 147 healthy children from four Secondary Schools in the VUT group. Dysphonia was suspected when the GFI-LT score was >3.0 points. The rate of singing activity and vocal training type (KLASAK) were evaluated. The means GFI-LT and dysphonia rate were compared between VT and VUT groups. Correlations between the GFI-LT and the degree of KLASAK were evaluated to assess the impact of vocal training on dysphonia rate.

Results

28 (10.6%) children from the VT and 15 (5.7%) from VUT group did not pass the screening (p < 0.05). The mean GFI-LT scores in VT (2.4 ± 3.1) and VUT (1.1 ± 3.1) groups revealed a statistically significant difference (p < 0.001). The rate of dysphonia in the VT group (5.2%) was significantly higher (χ2 = 25.6, p < 0.05) than in the VUT group (2.1%). Significant correlations were found both between the GFI-LT scores and duration of attendance of Music School (r = 0.39, p < 0.001) as well as between dysphonia rate and voice load (KLASAK) (r = 0.34, p < 0.001).

Conclusions

Vocal training could predispose to pediatric dysphonia. The greatest attention should be paid to children who engage in the extensive singing activities. GFI is a valid and reliable test to screen possible voice disorder early.

Point of care, clinician-performed laryngeal ultrasound and pediatric vocal fold movement impairment

04-12-2019 – Wynne Q. Zhang, Elton M. Lambert, Julina Ongkasuwan

Journal Article

Introduction

Vocal fold movement impairment (VFMI) is a well-known sequela of cervical and thoracic procedures performed in the vicinity of the recurrent laryngeal nerve. Interpretation of flexible nasolaryngoscopy (FNL) can be difficult in young children due to crying, secretions, and obstructing supraglottic structures. We have previously published on the use of radiologist performed and interpreted, laryngeal ultrasound (LUS) to evaluate vocal fold mobility with substantial agreement with FNL in infants in the cardiovascular intensive care unit. The purpose of this study was to evaluate point of care, clinician performed, LUS for vocal fold mobility in a pediatric voice clinic.

Methods

LUS and FNL were performed and recorded on 30 consecutive patients (11 with a clinical diagnosis of VFMI and 19 with clinically normal mobility) in a pediatric voice clinic. All LUS was performed by a single clinician (reviewer 1) with a GE logiq P9 and 12 MHz linear probe. Deidentified recordings of the LUS and FNL (without sound) were reviewed in random order by 2 fellowship trained pediatric otolaryngologists who were blinded to the vocal fold mobility. Cohens kappa was used to determine agreement.

Results

There was substantial agreement (<math xmlns:mml="http://www.w3.org/1998/Math/Math
ML” altimg=”si1.svg” class=”math”>κ  = 0.7) between the reviewers regarding interpretation of LUS as well as regarding interpretation of FNL <math xmlns:mml="http://www.w3.org/1998/Math/Math
ML” altimg=”si1.svg” class=”math”>κ  = 0.7802. In addition, each reviewer had near perfect to substantial agreement between their interpretation of the LUS and FNL (reviewer 1 <math xmlns:mml="http://www.w3.org/1998/Math/Math
ML” altimg=”si1.svg” class=”math”>κ  = 0.9294 and reviewer 2 <math xmlns:mml="http://www.w3.org/1998/Math/Math
ML” altimg=”si1.svg” class=”math”>κ  = 0.8413).

Conclusion

Point of care, clinician performed, LUS can be used for the identification of VFMI with substantial agreement with FNL with good inter-rater reliability. This provides clinicians with another tool in their armamentarium for the evaluation of challenging larynges.

Endoscopic approach to the round window through posterior tympanotomy for cochlear implantation in children: A study on feasibility

23-11-2019 – Nader Nassif, Luca Oscar Redaelli de Zinis

Journal Article

Objective

To demonstrate the feasibility of rigid endoscopy through posterior tympanotomy, which provides both a view of the round window and direction of the scala tympani in children.

Methods

After a standard mini-invasive surgical approach with postauricular access and transmastoid posterior tympanotomy of 2 mm, a 0°, 1.9 mm diameter and 11 cm long endoscope is positioned in proximity of the upper part of the posterior tympanotomy to obtain a panoramic view of the inferior part of the medial wall of the tympanic cavity. Surgical complications and changes in hearing threshold were analyzed.

Results

Eight children were submitted to cochlear implantation with endoscopic assistance through posterior tympanotomy. Complete visualization of the round window niche was possible in every ear. No complications related to the procedure were observed. Preoperative threshold was preserved in 9 of 10 ears.

Conclusions

Direct endoscopic view through the posterior tympanotomy allows visualization of the entire round window niche as well as the angle of introduction of the multi-electrode array along the direction of the scala tympani.

General anesthesia risk across pediatric surgical specialties: Low in otolaryngology

23-11-2019 – Caroline M. Kolb, Dee Tinley-Strong, Rajiv Rangarajan, Tetsu Uejima, Udayan K. Shah

Journal Article

Purpose

To determine the relevance of the Food and Drug Administration (FDA) warning regarding general anesthesia (GA) in children under 3 years of age for procedures lasting longer than 3 h, by surgical specialty and for otolaryngology specifically.

Methods

A one-year retrospective review was conducted at a tertiary-care medical center for all children younger than 3 years undergoing surgical procedures with durations greater than 3 h. De-identified data related to age, surgical service, procedure types, American Society of Anesthesiologists (ASA) physical status classification, and general anesthesia time were collected and examined.

Results

During 2017, 430 of 11,757 patients (3.7%) met the age and duration of anesthesia criteria. Procedures performed by the cardiothoracic surgery service were mostly likely to result in duration of surgery greater than 3 h (46.6%), followed by neurosurgery (12.9%), cardiology (9.3%), plastic surgery (7.1%), general surgery (6.6%), and urology (5.1%). Less than 2% of patients undergoing ophthalmology (1.9%), orthopedic surgery (1.7%), and otolaryngology (0.5%) procedures required anesthesia greater than 3 h.

Conclusion

Less than 4% of patients younger than 3 years undergoing surgery required general anesthesia for longer than 3 h. The theoretical risks of general anesthesia per the FDA warning are discussed and must be balanced against the known functional and neurodevelopmental consequences of not performing critical and time-sensitive surgery on children in this age group. A strategy for addressing parental and provider concerns is discussed.

Childhood nontuberculous mycobacterial lymphadenitis-observation alone is a good alternative to surgery

23-11-2019 – Annina Lyly, Antti Kontturi, Eeva Salo, Tea Nieminen, Johanna Nokso-Koivisto

Journal Article

Objective

Cervicofacial lymphadenitis caused by nontuberculous mycobacteria (NTM) is commonly treated with surgery or antimicrobial therapy. The aim of this study was to analyze the utility of our new blood-based diagnostic method and the treatment protocol, surgery or observation alone, in NTM lymphadenitis in children.

Methods

All patients under 16 years of age with cervicofacial NTM lymphadenitis diagnosed and treated at Childrens Hospital or at the Department of Otorhinolaryngology, Helsinki University Hospital (Helsinki, Finland) in 2007–2017 were retrospectively reviewed.

Results

Fifty-two patients, 33 (63%) of whom were girls, were included in the study. The median age at initial presentation of the NTM lymphadenitis was 2.9 years. The novel blood-test had been performed on 49 (94%) of the patients and in all of them it was indicative of NTM infection. A sample for mycobacterial culture was available from 34 patients, and Mycobacterium avium was the most common species detected. Most patients (n = 33, 63%) were treated conservatively with observation alone. Of these, nine patients (27%) did not develop a skin fistula, and the lymphadenitis resolved without drainage.

Conclusions

The novel blood test is clinically feasible method for diagnosing childhood cervicofacial NTM lymphadenitis noninvasively. Observation alone is a good alternative to surgery, without the risk of complications.

Ten years of paediatric airway foreign bodies in Western Australia

22-11-2019 – Allison Reid, Anton Hinton-Bayre, Shyan Vijayasekaran, Hayley Herbert

Journal Article

Background

Whenever a paediatric airway foreign body (PAFB) is suspected, decisions may be difficult without a clearly defined or accepted treatment algorithm. History and examination are commonly non-diagnostic and the risks associated with either watchful waiting or proceeding to MLB are significant. This paper reviews a 10 year cohort of suspected PAFBs for the predictive utility of history, examination and investigations and subsequent positive findings at MLB.

Objectives

1.

To review cases of suspected PAFB undergoing MLB in Perth, Western Australia over 10 years

2.

To examine the relative strengths of history, examination and imaging for predicting the presence of a foreign body, and their utility in treatment decision making

3.

To characterise the epidemiology of our cohort and to Evaluate our complication rate

4.

To consider the limitations and benefits of the use of CT imaging in such cases

Study design

The medical records of 127 children who underwent MLB for suspected PAFB between 2007 and 2016 were examined. The data was retrospectively reviewed for epidemiological details, history, examination, radiological findings and MLB outcomes.

Results

Sensitivity for PAFB on MLB with all three; history, examination and imaging (x-ray) positive for PAFB was 87.7%. Of the patients who were both symptom and sign positive (n = 96), chest x-ray findings did not significantly alter the chance of finding a PAFB. Chest x-ray had a low specificity (17%) in symptom and sign positive patients. Conversely, sensitivity of chest x-ray was high (88%), for symptom and sign positive patients.

Conclusions

For a child with both signs and symptoms, xray is unlikely to assist in decision making around suspected PAFB. When only sign or symptom is present, positive imaging may significantly increase the chance that PAFB is the cause.
For patients with a low suspicion of PAFB, consideration of a CT can be a helpful means of excluding a PAFB and avoiding an unnecessary general anaesthetic in this potentially high-risk group.
A greater level of public awareness is needed with regards to appropriate food types for children and the importance of eating seated and supervised in order to reduce the risk of PAFB.

Development of auditory perception in preschool children

23-11-2019 – Nadia Vilela, Seisse Gabriela Gandolfi Sanches, Renata Mota Mamede Carvallo

Journal Article

Objective

The aim of this study was to determine whether the auditory skills presented by five-year-old children can predict their performance in auditory processing (AP) tests at seven years old.

Design

Thirty-five children were evaluated for AP at two different times. At evaluation 1, the children were between 5 years 2 months and 6 years 1 month of age, and at evaluation 2, they were between 7 years 1 month and 7 years 8 months of age. The interval between the evaluations ranged from 18 to 23 months. After evaluation 2, the 7-year-olds were classified according to their performance on central AP tests. The children were divided into three groups: GI included nine children with at least two AP tests that scored two standard deviations below the mean and the presence of a speech complaint; GII included 18 children with at least two AP tests that scored two standard deviations below the mean and no speech complaints; and GIII included eight children with no more than one test scored two standard deviations below the mean and no speech disorders complaint. The analysis was performed considering each test individually and as a battery of applied tests. From the results obtained, a discriminant analysis was performed to assess the differences in test performance between the groups when the children were 5 years old.

Results

The discriminant analysis showed that with the results obtained during evaluation 1, it was possible to predict which group 74.3% of the children would be classified into after evaluation 2. The percentage of correct classifications for each group was 77.8% for GI, 66.7% for GII and 87.5% for GIII. That is, 87.5% of the children who were classified as GIII after evaluation 2 had already demonstrated good auditory performance in the tests applied at 5 years of age.

Conclusions

Children who exhibited lower scores on AP tests at 7 years of age had demonstrated poor auditory perception at 5 years of age. This finding is relevant because it offers the possibility of stimulating or training these auditory skills in preschoolers to foster their development.

Linguistic adaptation and validation of Italian version of OSA-18, a quality of life questionnaire for evaluation of children with obstructive sleep apnea-hypopnea syndrome (OSAS)

18-11-2019 – Elena Arezzo, Paola Festa, Vincenzo DAntò, Ambra Michelotti, Giovanni Carlo De Vincentiis, Emanuela Sitzia, Monica Giuliani, Simone Piga, Angela Galeotti

Journal Article

ATP6V1B1 recurrent mutations in Algerian deaf patients associated with renal tubular acidosis

17-11-2019 – Malika Dahmani, Sonia Talbi, Fatima Ammar-Khodja, Sofiane Ouhab, Farid Boudjenah, Merieme Djebbar, Crystel Bonnet, Christine Petit

Journal Article

Hereditary distal renal tubular acidosis (d
RTA) is a rare disorder characterized by metabolic acidosis due to impaired renal acid excretion. To date, three genes (ATP6V1B1, ATP6V0A4 and SLC4A1) have been reported to be responsible for this genetic disorder. Notably, mutations of ATP6V1B1 gene, which encode B1-subunit of H + -ATPase pump cause distal renal tubular acidosis often, associated with sensorineural hearing loss (SNHL). Furthermore, enlarged vestibular aqueduct (EVA) was also described in some patients with ATP6V1B1 mutations. Four Algerian unrelated patients presented with d
RTA and SNHL were recruited. The ATP6V1B1 gene was preferentially analyzed in all these patients by Sanger sequencing.

We identified two previously reported variants in ATP6V1B1 gene: a frameshift mutation (c.1155dup
C: p.(Ile386Hisfs*56) in exon 12 and a splicing mutation in intron 2 (c.175-1G > C: p?). Both mutations were homozygous in affected members. Interestingly, one patient with p.(Ile386Hisfs*56) mutation presented profound SNHL and bilateral enlarged vestibular aqueduct (EVA).

Our study indicates the importance contribution of ATP6V1B1 gene mutations to the pathogenesis of the d
RTA in the Algerian population and will contribute to introducing principles to predict the characteristics of the d
RTA in patients. Thus, screening for this gene could allow rapid patient management and provide adequate genetic counseling.

Advanced practice providers and children’s hospital-based pediatric otolarynology practices

17-11-2019 – Kenny H. Chan, Jordyn K. Dinwiddie, Gurpreet S. Ahuja, Erica C. Bennett, Matthew T. Brigger, David H. Chi, Daniel I. Choo, Michael J. Cunningham, Ravindhra G. Elluru, Carla M. Giannoni, Steven L. Goudy, Jeffrey A. Koempel, Carol J. MacArthur, Barbara Malone, Anna H. Messner, Ron B. Mitchell, Albert H. Park, Gresham T. Richter, Kristina W. Rosbe, Udayan K. Shah

Journal Article

Introduction

Advanced practice providers (APPs), including nurse practitioners and physician assistants, have been deployed in childrens hospital-based academic pediatric otolaryngology practices for many years. However, this relationship in terms of prevalence, roles, financial consequences and satisfaction has not been examined. The objective of this study is to explore how APPs impact healthcare delivery in this setting.

Methods

Pediatric otolaryngology chiefs of all academic childrens hospitals in the US were electronically surveyed about the ways APPs intersected clinically and financially in their respective practice.

Results

A total of 29 of 36 childrens hospital-based pediatric otolaryngology practices completed the survey, of which 26 practices (90%) utilized APP. There were large variances within the APP practice cohort in faculty size (mean/median/range = 9.4/8.5/3–29); annual patient visits (mean/median = 18,373/17,600); number of practice site (mean/median/range = 4.3/4/2–9) and number of outpatient APP (mean/median/range = 6.3/5/1–30). No factors (faculty size, annual visits and number of practice sites) differentiated between the APP and non-APP practices. Among APP practices, significant correlation (p<.00001) was observed between size of APP cohort to faculty size and annual visits. 69% of the practices did not differentiate job functions of nurse practitioners and physician assistants. 85% of the practices utilized APPs in all practice sites and 19% utilized APPs in the operating room. 77% of APPs billed independently and 46% had on-site supervision. The most prevalent APP salary bracket based on 0–5, 6–10 and > 11 years of tenure were $76–100K (65%), $100–150K (77%) and $100–150K (86%), respectively. In 46% of the practices, APPs were able to generate enough revenue to cover more than 75% of their salary and 23% of practices generated a profit. 81% of the chiefs ranked the effectiveness of APPs as high (4 and 5) on a 5-point Likert scale.

Discussion

The majority of academic pediatric otolaryngology practices employed APPs. Despite the diversity seen in practice complexity, APP functionality and financial impact, most found the APP model to be beneficial in improving patient care, patient access and faculty productivity.

A retrospective study of patients with Robin sequence: Patient characteristics and their impact on clinical outcomes

18-11-2019 – Stefan Hamilton, Agnieszka Dzioba, Murad Husein

Journal Article

Introduction

Robin sequence (RS) is a congenital set of abnormalities of the head and neck, consisting of a hypoplastic mandible (micrognathia), a tongue that is displaced posteriorly (glossoptosis), and obstruction of the airway. A clear set of diagnostic criteria for this complex condition has recently been established, but there is still no consensus in the literature with respect to managing the associated airway, feeding, and hearing difficulties. The objectives of the study are: 1) to describe the management of airway and feeding issues in children with RS; and 2) to evaluate the impact of airway and feeding strategies on important clinical outcomes.

Methods

A retrospective chart review was conducted of all pediatric patients diagnosed with RS at London Health Sciences Centre from January 1995 until September 2017. The frequencies of all airway and feeding interventions were collected. Data were collected on several clinical outcomes including initial admission length, enteral feeding duration, tympanostomy tube insertion frequency, and hearing thresholds. Statistical analyses to evaluate the impact of airway and feeding strategies on clinical outcomes were carried out using independent samples t-tests and Chi-square tests, where appropriate.

Results

Twenty-four patients were identified. Five patients (20.8%) required airway surgery and 18 patients (75.0%) required enteral feeding. Airway surgery was significantly associated with a longer ICU admission (15.8 vs. 4.3 days, p < 0.05), a longer overall hospital admission (73.0 vs. 25.2 days, p < 0.05), a delay in introducing oral feeds (222.8 vs. 11.5 days, p < 0.05), and a higher frequency of tympanostomy tube insertions (80% vs. 23.5% requiring ≥2 insertions, p < 0.05). Enteral feeding was significantly associated with a longer ICU admission (8.8 vs. 0 days, p < 0.05) and a longer overall hospital admission (43.9 vs. 5.6 days, p < 0.05).

Conclusions

This study demonstrates the substantial impact that airway and feeding difficulties have on RS patient morbidity, particularly with respect to length of hospital and ICU admission. This information has prognostic value and may be helpful in generating a management algorithm for this complex patient population.

“Sjögrens syndrome in children with recurrent parotitis”

16-11-2019 – Breanne L. Schiffer, Sara M Stern, Albert H. Park

Journal Article

Objective

Optimize the diagnosis of pediatric Sjögrens syndrome in children who present with parotitis.

Methods

Twenty children presented to a pediatric otolaryngology or rheumatology clinic with recurrent parotitis. Presenting symptoms, serologies, sialendoscopy findings, and minor salivary gland biopsy pathology results were reviewed.

Results

Twenty patients aged 3–17 years presented with recurrent parotitis. Ten percent of this cohort met the American-European Consensus Group adult diagnostic criteria for Sjögrens syndrome. Forty percent of this cohort met diagnosis of Sjögrens syndrome when utilizing Bartunkovas proposed pediatric criteria for diagnosis of Sjögrens syndrome.

Conclusion

Sjögrens syndrome is surprisingly common in pediatric patients who present with recurrent parotitis. Otolaryngologists who treat pediatric parotitis should have a high index of suspicion for Sjögrens syndrome.

Level of Evidence

4.

Effect of congenital toxoplasmosis on the encoding of speech in infants

15-11-2019 – Laís Ferreira, Maria Clara da Silva Valadão, Piotr H. Skarzynski, Milaine Dominici Sanfins, Eliara Pinto Vieira Biaggio

Journal Article

Objective

To investigate the effect of congenital toxoplasmosis (CTP) on the Frequency-Following Response (FFR) in infants.

Study design

11 infants diagnosed with CTP and 12 healthy infants with no risk indicators for hearing impairment, aged 29–90 days old. All infants underwent an FFR neurophysiological assessment. The test stimulus was the syllable da, 40 ms in duration, which was monaurally presented to the right ear at an intensity of 80 d
Bn
HL. Absolute latencies and amplitudes of the V, A, C, D, E, F, and O waves, the slope (μV/ms) and measure between onset (A) and offset (O), were compared between the two groups.

Results

Infants with CTP had increased latency of FFR waves V, A, E, F, and O, and decreased amplitude for waves A and F. They also showed a reduction in A–O slope and a higher latency difference between onset (A) and offset (O).

Conclusion

The neurophysiological responses of Frequency-Following Response can be influenced by congenital toxoplasmosis. Since, the CTP showed prolongation of the V, A, E, F and O waves and decrease of the amplitude for waves A and F.

Non-surgical correction of cryptotia and the analysis of treatment time and other influence factors

16-11-2019 – Di Wang, Haiyue Jiang, Qinghua Yang, Xia Liu, Leren He, Bo Pan, Lin Lin

Journal Article

Objective

To explore correlations between post-treatment outcomes of non-surgical correction for cryptotia and treatment time and other influence factors.

Methods

Forty-seven consecutive patients with 64 cryptotias were treated with the adjusted external stretching device and followed up over 12 months. A subjective evaluation scale was designed for patients to collect clinical data. Pretreatment and posttreatment evaluation were conducted by two blinded investigators. The correlations between influence factors and outcomes were explored through fractional polynomial method, multiple logistic regression, and robust linear regression methods.

Results

Thirty-five patients with 49 cryptotias were included. Twenty-nine cryptotias (23 patients) have been successfully managed. Two of 17 unilateral cryptotias achieved nearly complete symmetry. The final optimal cutoff value for initiating treatment time is 6 months and for the duration of treatment per day is 5 h per day. Positive relationships between initiating treatment time >6 months and onset time, type II and onset time, initiating treatment time >6 months and effect stabilization time were observed.

Conclusions

Initiating treatment time <6 months and duration of treatment per day >5 h benefit for the posttreatment outcomes. Patients wearing the device under 6 months old would have earlier onset time and effect stabilization time. It is hard to achieve complete bilateral symmetry in unilateral patients. The adjustable devices can used for the auricles with different sizes and removed and equipped conveniently.

Contrasting esophageal coin removal in countries with different sized coins in circulation

16-11-2019 – Ceazon T. Edwards, Hossam S. Alslaim, Mohanad M. Alebbini, Moses O. Evbuomwan, Jonathan C. Chan, Shadi Hamouri, Nathan M. Novotny

Journal Article

Is nurse-led telephone follow-up for post-operative obstructive sleep apnoea patients effective? A prospective observational study at a paediatric tertiary centre

14-11-2019 – Hussein Walijee, Salil Sood, Anne Markey, Madhankumar Krishnan, Anita Lee, Sujata De

Journal Article

Objectives

To assess the effectiveness and patient satisfaction of a nurse-led telephone follow-up service in children following surgery for sleep disordered breathing (SDB)/obstructive sleep apnoea (OSA) in a tertiary paediatric hospital.

Design

Prospective observational uncontrolled study.

Participants

Children under the age of 16 undergoing adenoid and/or tonsil surgery between June 2015 and June 2018 for SDB or OSA. Parents were contacted by telephone six weeks post-operatively by an ENT nurse specialist. The T-14 questionnaire was utilised to assess post-operative outcomes. Parents were subsequently asked to evaluate their experience of this nurse-led telephone consultation service between June 2016 and April 2017.

Results

535 patients were included with an average post-operative T-14 score of 2.13 (95% CI 1.7–2.5). 430 patients were discharged following the nurse-led telephone consultation with a mean post-operative T-14 score 1.0 (95% CI 0.8–1.2). 105 patients were subsequently reviewed in clinic with an average T-14 score of 6.88 (95% CI 5.25–8.51). 36 (6.7%) patients had ongoing symptoms of SDB or OSA. 55 parents were invited to provide an evaluation of the nurse-led telephone FU clinic, which showed a 100% satisfaction rate with the service.

Conclusion

A nurse-led telephone follow-up service is efficient and safe with high levels of parental satisfaction. It reduces unnecessary follow-up of uncomplicated patients whilst providing a robust safety net for those with ongoing problems.

The spectrum of obstructive sleep apnea in infants and children with Down Syndrome

11-11-2019 – Karen A. Waters, Chenda Castro, Jasneek Chawla

Journal Article

Objective

Describe the spectrum of OSA across time in infants and children with Down syndrome.

Methods

Retrospective records review of children who underwent formal polysomnography (PSG) in one of two Australian tertiary sleep centres over selected 3.5-year periods. 152 children were identified, then all sleep study and treatment records were retrieved for the lifetime of the child through 2018.

Results

3.8 ± 3.2 studies (range 1–17) were retrieved per child and 38.2% had mild disease at worst. Children having only 1 study were more likely to have a normal or mild result than those having ≥2 (chi-square 11.25, p-value 0.0008) Studies were more often severe in children age <2 compared to those ≥2 years, (chi-square 12.87, p = 0.005). After age 2 years, OSA severity increased with age. Amongst 91 (56.4%) children with ≥2 studies, 71 (78.0%) had moderate or severe disease at some time. Studies evaluating the effects of surgery (most often adenotonsillectomy) showed resolution of disease to mild or normal in 53.3%. Where ≥2 studies were evaluated, the last study polarised towards normal or mild disease 40 (44.0%), or treatment titrations 34 (37.4%) with moderate or severe disease in 17 (18.7%).

Conclusions

In a tertiary sleep unit, a full spectrum of sleep disordered breathing in Down syndrome was seen from infancy onwards. Children having only one study were more likely to have normal results. Children with multiple studies reflected disease surveillance, including follow-up after treatment interventions.

A retrospective review of Paparella Type 1 tympanostomy tubes

11-11-2019 – Devika D. Sparks, Danielle Blake, Bridgette Carter, Scott Schoem

Journal Article

Objective

This study aims to provide data on ear tube extrusion and complication rates for patients who have Paparella Type 1 tympanostomy tube (TT) placement.

Methods

Retrospective chart review of patients 6 months to 12 years old who underwent insertion of Paparella Type 1 TT by a single surgeon.

Results

Of 197 tubes evaluated, 3% were plugged between 1 and 3 months after surgery. Of the 144 tubes evaluated long-term, all tubes extruded within 4 years. There were no tympanic membrane perforations.

Conclusions

This chart review showed expected rate of initial ear tube plugging. The rate of tympanic membrane perforation was lower than expected.

Differences in laryngeal movements during exercise in healthy and dyspnoeic adolescents

11-11-2019 – Katarina Norlander, Henrik Johansson, Margareta Emtner, Christer Janson, Lennart Nordvall, Leif Nordang

Journal Article

Objectives

To compare glottic and supraglottic movements in healthy adolescents and adolescents experiencing dyspnoea during strenuous exercise.

Methods

Using the continuous laryngoscopy exercise (CLE)-test laryngeal movements during exercise were analysed in healthy controls (n = 28) and compared to subjects with exercise induced bronchoconstriction (EIB) (n = 10), exercise induced laryngeal obstruction (EILO) (n = 10) and subjects experiencing exercise-induced dyspnoea without having any of these diagnoses (n = 31). Images from the video recordings were assessed regarding glottic angle, glottic area and supraglottic area using the software measuring tool EILOMEA.

Results

No significant differences were detected between controls, the dyspnoea group without a diagnosis of EIB or EILO and the EIB group regarding glottis angle, glottis area or supraglottic area at maximum effort. All three parameters differed significantly in the EILO group compared to the other groups (p=<0.001). In the group with EILO all but one had supraglottic obstruction (corresponding to a CLE-test score ≥2). Movement of the laryngeal structures, corresponding to a CLE-test score of 1, at glottic and/or supraglottic level was seen in 26 of 35 (74%) of controls, 34 out of 41 (83%) of patients in the dyspnoea group, and in 25 of 38 (66%) of EIB-subjects.

Conclusion

Minor movements at both glottic and supraglottic level are equally common in healthy controls as among adolescents with exercise induced dyspnoea without EIB or EILO and adolescents with EIB. Adolescents with EILO had a statistically significant more pronounced supraglottic obstruction than the other groups.

Pediatric flexible laryngoscopy: Trends in diagnostic abilities throughout training

11-11-2019 – Sarah E. Maurrasse, Carol Li, Vikash K. Modi

Journal Article

Objectives

Our objectives were to evaluate the ability of residents to diagnose pathology of the pediatric larynx on laryngoscopy, to trend this ability throughout training, to compare their skills to pediatric otolaryngologists, and to determine whether reviewing digitally captured videos in slow motion, as opposed to a live exam, enhanced diagnostic abilities. In addition, we identified pathologies and anatomical sub-sites that posed diagnostic challenges.

Methods

Qualtrics was used to design and distribute an online test, which included 15 pediatric laryngoscopy videos. Participants selected a diagnosis for each video after (1) watching it once at full speed and (2) watching the video multiple times in slow motion. Anonymous responses were exported into excel for statistical analysis, including T-test, ANOVA, and descriptive statistics.

Results

There were 21 total participants. Median scores for full speed versus slow motion video review were 47% and 60% respectively. When analyzed by training level, there was no significant difference in scores for full speed videos, but there was a significant difference across groups for slow motion review (p = 0.04). Post Graduate Year (PGY) 4 residents and pediatric otolaryngologists performed best with an average of 69% and 77% respectively. Base of tongue, subglottic, and laryngeal cleft lesions were the most difficult to diagnose. Motor, laryngeal, and vallecular pathologies were more accurately identified.

Conclusions

The ability to diagnose pathology on pediatric laryngoscopy tends to improve throughout residency training. Slow motion review enhances diagnostic skills. Laryngeal and vallecular pathologies are more easily diagnosed than base of tongue and subglottic lesions.

Outcomes intensive care unit placement following pediatric adenotonsillectomy

11-11-2019 – David Z. Allen, Noah Worobetz, Jordan Lukens, Cameron Sheehan, Amanda Onwuka, Ryan M. Dopirak, Tendy Chiang, Charles Elmaraghy

Journal Article

Introduction

Adenotonsillectomy (AT) is the most common surgical procedure for the treatment of sleep related breathing issues in children. While overnight observation in the hospital setting is utilized frequently in children after a AT, ICU setting is commonly used for patients with sleep apnea. This objective of this study is to examine factors associated with the preoperative decision to admit patients to PICU following AT as well as co-morbidities that may justify necessity for higher level of care.

Methods

This is a retrospective chart review from the years of 2009–2016. All patients who underwent AT for known sleep-related breathing issues at Nationwide Childrens Hospital were eligible for inclusion. A complication was defined as an adverse event such as pulmonary edema, re-intubation, or a bleeding event. Respiratory support was defined as utilizing supplementary oxygen for more than one day, positive pressure ventilation, or intubation. Proportions and medians were used to describe the overall rate of complications/complexities in care, and bivariate statistics were used to evaluate the relationship between patient characteristics and outcomes. Similar methods were used to evaluate factors associated with preoperative referral to the PICU.

Results

There were 180 patients admitted to hospital in non-ICU setting and 158 patients with a planned PICU stay. The patients with planned PICU stays had higher rates of technological dependence (13% vs. 3%; p = 0.0006), perioperative sleep studies (80% vs. 29%; p < 0.0001), and more severe classifications of OSA (p < 0.0001). Patients with planned ICU placement also had higher rates of apneas, hypopneas, respiratory disturbance indexes, apnea hypopnea indexes, lower oxygen saturation nadirs, and a longer time spent below 90% oxygenation in sleep studies (p < 0.0001). Nearly 45% of the patients with planned ICU stays required respiratory support compared to just 8% of non-PICU patients. Additionally, 32% of the patients with planned ICU stays experienced complications compared to just 8% of the floor population. Complications were associated with younger ages, gastrointestinal comorbidities, technological dependence, viral infections, and a history of reflux. Interestingly, there were no differences in the complication rate by sleep studies findings. Similarly, there were no population level differences between patients who required respiratory support in the ICU and those that did not. Unplanned PICU placement was a rare but significant adverse event (n = 24). None of the hypothesized risk factors were associated with unplanned PICU placement.

Conclusions

This study suggest that while our pre-operative referral program for PICU placement is effective in identifying patients needing higher levels of care, the program places many patients in the PICU who did not utilize respiratory support or suffer from complications. We observed some misalignment between characteristics associated with planned ICU stays and actual complications. This suggests that patients with specific clinical histories, not findings on their sleep studies, should be prepared to receive higher levels of care.

Sedation withdrawal following single stage laryngotracheal reconstruction: Does dexmedetomidine help?

11-11-2019 – Lilun Li, David Strum, Sophie R. Pestieau, George Zalzal, Diego Preciado

Journal Article

Objectives

Single-stage laryngotracheal reconstruction (SS-LTR) requires a period of post-operative intubation, during which time adequate sedation is needed to ensure graft healing. Commonly used agents include benzodiazepines, opioids, and more recently, dexmedetomidine, a centrally-acting α2 adrenoreceptor. This study aims to compare withdrawal outcomes between various sedation regimens following SS-LTR.

Methods

Retrospective chart review of 56 patients who underwent SS-LTR between 2008 and 2018 at a tertiary free-standing childrens hospital was performed. Of 47 patients with complete records, 18 patients received dexmedetomidine for >75% of their intubation period with midazolam (Dex
WM), 9 received dexmedetomidine for >75% without midazolam (Dex
WOM), and 20 received dexmedetomidine for <75% with midazolam (no
Dex). Results: There was no significant difference in length of PICU or hospital stay between the groups. The no
Dex group trended toward a higher re-intubation rate of 25%, as compared with 11% of Dex
WOM and 5.6% of Dex
WM (p = 0.21). There was no significant difference in days of oral sedation taper required or Withdrawal Assessment Tool (WAT-1) score for post-extubation days 1 and 3. By post-extubation day 5, 100% of the Dex
WM group had WAT-1 scores <3 as compared with 71.4% of the no
Dex group (p = 0.037). Notably, lower average daily doses of dexmedetomidine and midazolam were used in the Dex
WM group, as compared with the Dex
WOM and no
Dex groups, respectively. Conclusion: Dexmedetomidine as a primary sedation agent with midazolam allows for adequate sedation following SS-LTR. The combination of the two drugs in the Dex
WM group not only reduced the dosage of each drug needed, but also significantly improved WAT-1 scores by post-extubation day 5, as compared with the alternative sedation regimens.

Outcomes of audiometric testing in children with auditory neuropathy spectrum disorder

11-11-2019 – Joseph B. Meleca, Gina Stillitano, Maxwell Y. Lee, Whitney Lyle, Yi-Chun Carol Liu, Samantha Anne

Journal Article

Introduction

Auditory Neuropathy Spectrum Disorder (ANSD) is characterized by hearing loss ranging from normal to profound. Additionally, results are confounded by commonly fluctuating hearing thresholds in ANSD. As such, we sought to evaluate results of audiometric testing on children with ANSD and the impact of age and time on testing results.

Methods

Retrospective chart review on children <18 years of age diagnosed with ANSD at two tertiary care academic institutions. Data analyzed included initial audiogram with speech detection thresholds (SDT) and pure tone averages (PTA) as well as most recent unaided audiogram SDT and PTA.

Results

75 ANSD patients were analyzed, of which 32 (42.7%) were female. Bilateral and unilateral ANSD was seen in 55 (73.3%) and 20 (26.7%), respectively. A total of 130 ears with ANSD were assessed with 80 (61.5%) meeting inclusion criteria. Of these patients, the median age in years at first audiogram and most recent audiogram were 1.94 (0.45–13.68) and 4.22 (0.97–14.61), respectively. The median ages at which an SDT and PTA could first be acquired on the audiogram in ANSD patients were 1.94 (0.50–13.68) and 2.86 (0.45–13.68), respectively. The average SDT/PTA at the initial and most recent audiogram were 47.5/45.7 and 49.4/53.0 d
B, respectively. SDT to PTA within the same audiogram exhibited strong correlation (r = 0.82, p < 0.001). Similarly, comparison of initial SDT to SDT at a later time interval showed strong correlation (r = 0.73, p < 0.001). SDT and PTA at initial audiogram and PTA at later time demonstrated lower correlation but was still statistically significant (r = 0.49, p < 0.009 and r = 0.51, p < 0.044, respectively). Individual PTA was associated with age (r = −0.56, p < 0.001).

Conclusions

SDT and PTA within the same audiogram and initial SDT to SDT acquired at a later time correlate strongly in audiometric testing in children with ANSD. Although not as strong, initial SDT and PTA still correlate with PTA at a later time interval. These findings suggest that audiometric results yielding a reliable SDT and frequency specific information necessary to calculate the PTA is not typically obtained until 2–3 years old. However, once this information is obtained, the childs hearing is fairly stable but may fluctuate over time.

Allergic rhinitis and asthma assessment of risk factors in pediatric patients: A systematic review

18-11-2019 – Domenico Testa, Matteo DI Bari, Michele Nunziata, Generoso DE. Cristofaro, Giuseppe Massaro, Giuseppina Marcuccio, Gaetano Motta

Journal Article

Allergic rhinitis (AR) is the most prevalent allergic disease in children and can be associated with asthma (A); this association can have significant effect on childs quality of life. The objective of this work was to systematically review existing literature on the risk factors of AR and A in children to better understand the link between these two diseases. We performed a literature search over the last 25 years in PubMed and Medline. Inclusion criteria comprised English language papers containing original human data with greater than 30 subjects and papers that statistically analyze the relationship between AR and A and the risk factor(s), in children population. A statistically significant correlation was found between children with AR and A and ambient Polycyclic Aromatic Hydrocarbons exposure, live in an industrialized city with elevated traffic, dampness and moulds exposure, electric cooking, male gender, single nucleotide polymorphisms in PTNP22 gene and CTLA-4 gene, fast food and margarine products consumption, use of paracetamol in last year, history of tuberculosis, parental atopy, high total serum Ig
E, antibiotics in uterus and infections in uterus exposure, history of formula feeding and caesarian section. A strong and complex link between AR and A was accounted: A represents a major risk factor for the onset of AR, that correlates with more severe asthmatic symptoms. Even the onset of A in a child with AR worsen it. The interaction of genetic and environmental risk factors and the consequent epigenetic, microbiota and immunological changes, were found to led to the development of AR and A in children, with both atopic and non-atopic pathways. Close monitoring of evidenced risk factors may help with an early recognize and treat A in patients with AR.

Audiological benefit and subjective satisfaction of children with the ADHEAR audio processor and adhesive adapter

07-11-2019 – Amelia Favoreel, Emilie Heuninck, Anne-Laure Mansbach

Journal Article

Objectives

The main objective of this study was to evaluate the audiological benefit of the ADHEAR system in a group of children with a uni- or bilateral conductive hearing loss (CHL) during a short-term exposure of three weeks, and to compare it to a conventional bone conduction hearing aid (BCHA) on a softband. The secondary aim was to assess the quality of life improvement and patient satisfaction with the ADHEAR system.

Methods and materials

The study was designed as a prospective study with repeated measures, where each subject served as his/her own control. Ten children (4–17 y/o) with a uni- or bilateral congenital or acquired CHL were included in this study. Pure tone audiometry and speech audiometry in quiet, both unaided and aided, were performed initially with the ADHEAR system and a BCHA on a softband, and after three weeks with the ADHEAR alone. Furthermore, patient satisfaction and quality of life were assessed using the SSQ12 and the ADHEAR questionnaire.

Results

The mean unaided free field hearing threshold of 50 d
B HL (with 95%CI between 41.7 and 57.5 d
B HL) expressed in ‘Bureau International d’Audiophonologie’ (BIAP), improved significantly by 22 d
B (13.0–29.9) with the ADHEAR and by 23 d
B (13.6–32.9) with the BCHA (p < 0.001). Furthermore, the mean unaided speech recognition threshold (SRT) in quiet improved significantly by 19 d
B (10.3–28.1) with the ADHEAR and by 21 d
B (12.6–29.4) with the BCHA (p < 0.001). For both audiological tests, there were no significant differences between the ADHEAR and the BCHA.

After three weeks of use, the mean pure tone threshold of 28 d
B HL (20.0–36.5) and the mean SRT of 47 d
B SPL (41.9–51.5) with the ADHEAR system were comparable and not significantly different than the outcomes during the first visit.

Speech understanding in noise and in multiple streams, sound localization and sound quality were rated significantly better with the ADHEAR, compared to the ratings without the ADHEAR system (p < 0.001). None of the children reported skin irritations or pain.

Conclusions

The children included in our study had significantly improved hearing thresholds, speech perception in quiet and quality of life with the ADHEAR. The device can be an effective treatment method and a valuable alternative to other BCHA for children with a CHL, although the subjective experience of each child has to be taken into account.

Clinical trial registration number

NCT03327194.

Factors associated with all-cause mortality in pediatric invasive fungal rhinosinusitis

05-11-2019 – Azamar Alejandro, Carlos de la Torre González, Mantilla Edgar, Villamor Perla

Journal Article

Introduction

Pediatric invasive fungal rhinosinusitis (PIFR) is a potentially lethal infection seen in immunocompromised pediatric patients. Even with timely treatments, mortality ranges between 18 and 80% of the cases.

Objective

To analyze the factors associated with all-cause mortality in pediatric patients with acute invasive fungal rhinosinusitis.

Setting

Tertiary pediatric referral center.

Results

A total of 18 patients were included, 12 male and 6 female. The average age at diagnosis was 8.7 years (range 4 months–17 years), with 56% overall mortality and 44% survival after 60 months. The most common cause of immunosuppression was acute lymphoblastic leukemia. The only factor found affecting mortality was a time between diagnosis and surgery greater than 7 days.

Conclusion

PIFR is an aggressive entity with high mortality. An appropriate diagnosis with an opportune surgical debridement followed by systemic antifungal therapy is essential to improve survival. Delay in surgical treatment is associated with higher mortality.

The impact of persistent pediatric obstructive sleep apnea on the Quality of Life of Patients’ families

05-11-2019 – Mathieu Bergeron, Angela L. Duggins, Aliza P. Cohen, Brittany A. Leader, Stacey L. Ishman

Journal Article

Objectives

To (1) quantify the quality of life (QOL) for families of children affected by persistent obstructive sleep apnea (OSA), and (2) assess factors contributing to the negative impact of OSA on families.

Methods

Prospective case series in a multidisciplinary upper airway center at a tertiary pediatric institution. Our study included patients with persistent OSA referred to our clinic from 2014 to 2016. Both patients and their families completed validated questionnaires assessing QOL and OSA symptoms, including the Family Impact Questionnaire (FIQ), Pediatric Sleep Questionnaire, the Pediatric Quality of Life Inventory, the OSA-18, and the Epworth Sleepiness Scale.

Results

Families of 67 patients were included. The mean patient age was 12.5 (95%CI 11.9–13.1); 23 (34.3%) were female, and the mean BMI percentile was 80.2 (95%CI 73.6–86.8). The single most common comorbidity was Down syndrome (45.6%). The mean obstructive apnea-hypopnea index was 9.7 ± 10.3 events/hour. A significant association exists between OSA severity and 18 FIQ negative subscore (P < 0.001). Financial impact was the primary negative concern for parents of patients with OSA compared to those without OSA (P = 0.03). There were no other significant differences between those with and without OSA.

Conclusions

There was a significant correlation between persistent OSA severity and the FIQ negative impact of disease score on patients’ families. Concern regarding financial burden was more common for families of children with OSA than for those without OSA. This suggests that targeted interventions, particularly regarding the financial burden of persistent OSA diagnosis and management, may provide some relief to families.

The conchal butterfly graft in secondary reconstruction of the bilateral cleft lip nasal deformity

05-11-2019 – Oksana Jackson, Nicholas Wingate, Alfred Lee, Alison E. Kaye

Journal Article

Objectives

The bilateral cleft nasal deformity is characterized by a short columella and a broad, flattened nasal tip. Secondary correction is challenging and often complicated by skin envelope scarring and hypoplastic alar cartilages. Adequate and durable tip projection requires a procedure that adequately augments the nasal tip and maximizes tip support. We describe a novel technique for secondary correction of this deformity in the immature pediatric population.

Methods

The conchal butterfly graft technique utilizes a folded cartilage construct to simultaneously increase tip projection and provide tip support. A retrospective review of 19 consecutive patients with bilateral cleft lip who underwent secondary tip rhinoplasty utilizing our technique over a 7-year period was conducted. Charts were reviewed for age at operation, length of follow-up and secondary revision procedures. Pre- and post-operative photographs were compared using two different methods to assess the degree of change and overall aesthetic result. Three plastic surgeons who previously were unfamiliar with the technique independently rated seven nasal tip characteristics. Nasal tip projection (NTP) was then measured objectively by two widely utilized methods: the Goode method and the Baum method-modified by Powell.

Results

Long lasting correction of the bilateral cleft nasal deformity was achieved utilizing this technique in 19 patients over a 7-year period with a mean length of follow-up of 5.4 years. We demonstrate augmented nasal tip projection that approaches ideal relationships from both relative measurement and survey data.

Conclusion

The conchal butterfly graft technique is effective for reconstruction of the bilateral cleft nasal deformity, and in our experience, has resulted in long lasting nasal projection and overall improved facial balance.

Hearing loss and speech understanding in noise in Aboriginal and Torres Strait Islander children from locations varying in remoteness and socio-educational advantage

05-11-2019 – Kiri Mealings, Samantha Harkus, Jennifer Hwang, Jacinto Fragoso, King Chung, Harvey Dillon

Journal Article

Objective

Otitis media resulting in conductive hearing loss is a major health issue for Aboriginal and Torres Strait Islander children, which can also lead to the child developing spatial processing disorder (SPD). This study examined the prevalence of hearing loss and deficits in speech understanding in noise, including SPD, in Aboriginal and Torres Strait Islander children from schools varying in remoteness and socio-educational advantage.

Method

288 Aboriginal and Torres Strait Islander children aged 4–14 years from three schools varying in remoteness and socio-educational advantage completed audiological assessment and the Listening in Spatialized Noise – Sentences test to assess for hearing loss and SPD. Children also completed Sound Scouts, a self-administered tablet-based hearing test which screens for these deficits. The prevalence of hearing issues was compared to what is expected from a typical population.

Results

The proportion of children with hearing problems was related to the schools socio-educational advantage, with higher proportions in schools with a lower socio-educational advantage. Proportions of children with speech-in-noise deficits (including SPD) was related to the remoteness of the school, with higher proportions in schools that were more remote.

Conclusions

The prevalence of hearing loss and SPD is much higher in Aboriginal and Torres Strait Islander children than described for non-Aboriginal populations, and is related to the socio-educational advantage or remoteness of the school. Resources are needed to reduce the incidence of hearing loss and health disparity in Aboriginal communities, especially those in remote areas with lower socio-educational advantages.

Nasal glial heterotopia in children: Two case reports and literature review

19-11-2019 – Yang-yan Yan, Zhi-ying Zhou, Jing Bi, Yong Fu

Journal Article, Review

Nasal glial heterotopia is a rare developmental anomaly. We present two cases of nasal glial heterotopia. The presenting symptoms, imaging findings, treatment, histological appearance, and follow-up are described in detail. We additionally reviewed all pediatric cases of nasal glial heterotopia published between 1980 and 2018 in the PubMed and Baidu Scholar databases. We identified 60 pediatric patients (36 60% boys). The main symptoms were nasal congestion and open-mouth breathing. Treatment was successful in all patients, and the recurrence rate was low (3/60, 5%). Nasal glial heterotopia requires multidisciplinary management but has a good prognosis.

Next generation sequencing and genetics of hereditary hearing loss in the iranian population: New insights from a systematic review

11-11-2019 – Mahbobeh koohiyan

Journal Article, Review

Background

Hereditary hearing loss (HL) as a common disorder is genetically heterogeneous, which poses a challenge for clinical and molecular diagnosis. Next-generation sequencing (NGS) technologies have proven to be the best solution for mutational screening, even though it is not always conclusive. Here, we have reviewed the results of previously published data on HL mutations identified with NGS, as well as the efficiency of this technology in detecting HL in Iran.

Methods

A systematic literature review of the PubMed, Google Scholar, Web of Science, and Science Direct databases were conducted for articles published before May 2019. The primary data of these studies, including the number of samples, mutation frequency and so on were extracted.

Results

Seventy-five articles were reviewed, and 10 met our inclusion criteria. Totally 432 unrelated families were included and analyzed for the type and prevalence of the gene mutations and pathogenic variants were discovered in 34 non-syndromic HL (NSHL) genes. Altogether 237 different genetic mutations were detected. However, p. Gln1576Stop in PCDH15 was the most common mutation accounting for 1% of the populations studied. NGS platforms have yielded only a 47.1% molecular diagnosis rate for NSHL etiologies in the Iranian population, which is significantly lower than that identified in the other part of the Middle East.

Conclusion

The results showed that NGS platforms can greatly assist and enhance HL diagnosis and improve molecular diagnostic outcome. However, researchers were unable to provide 53% of their Iranian cohort with a molecular diagnosis for their HL. It seems that many rare genes are responsible for the majority of HL in the Iranian cohort. Future explorative investigations utilizing NGS technologies, such as WES, into the Iranian population are warranted.

G130V de novo mutation in case with nonsyndromic hearing loss without palmoplantar keratoderma

02-12-2019 – Beuy Joob, Viroj Wiwanitkit

Letter

Statistical analysis on “Early auditory skills development in Mandarin speaking children after bilateral cochlear implantation”

20-08-2019 – Mohammad Soleimani, Sepehr Eslami, Reza Amani-Beni, Sadra Ansaripour

Letter