Effect of Hearing Device Use on Speech-in-Noise Performance in Children with Severe-to-Profound Unilateral Hearing Loss.

OBJECTIVES: Limited evidence exists for the use of rerouting devices in children with severe-to-profound unilateral sensorineural hearing loss. Many laboratory studies to date have evaluated hearing-in-noise performance in specific target-masker spatial configurations within a small group of participants and with only a subset of available hearing devices. In the present study, the efficacy of all major types of nonsurgical devices was evaluated within a larger group of pediatric subjects on a challenging speech-in-noise recognition task. DESIGN: Children (7-18 years) with unaided severe-to-profound unilateral hearing loss (UHL' n = 36) or bilateral normal hearing (NH, n = 36) participated in the present study. The signal-to-noise ratio (SNR) required for 50% speech understanding (SNR-50) was measured using BKB sentences in the presence of proprietary restaurant noise (R-SPACE BSIN-R) in the R-SPACE Sound System. Subjects listened under 2 target/masker spatial configurations. The target signal was directed toward subjects' NH or hearing-impaired ear (45º azimuth), while the interfering restaurant noise masker was presented from the remaining 7 loudspeakers encircling the subject, spaced every 45º. Head position was fixed during testing. The presentation level of target sentences and masking noise varied over time to estimate the SNR-50 (dB). The following devices were tested in all participants with severe-to-profound UHL: air conduction (AC) contralateral routing of signal (CROS), bone conduction (BC) CROS fitted on a headband with and without the use of remote microphone (RM), and an ear-level RM hearing assistance technology (HAT) system. RESULTS: As a group, participants with severe-to-profound UHL performed best when the target signal was directed toward their NH ear. Across listening conditions, there was an average 8.5 dB improvement in SNR-50 by simply orienting the NH ear toward the target signal. When unaided, participants with severe-to-profound UHL performed as well as participants with NH when the target signal was directed toward the NH ear. Performance was negatively affected by AC CROS when the target signal was directed toward the NH ear, whereas no statistically significant change in performance was observed when using BC CROS. When the target signal was directed toward participants' hearing-impaired ear, all tested devices improved SNR-50 compared with the unaided condition, with small improvements (1-2 dB) observed with CROS devices and the largest improvement (9 dB) gained with the personal ear-level RM HAT system. No added benefit nor decrement was observed when RM was added to BC CROS using a 50/50 mixing ratio when the target was directed toward the impaired ear. CONCLUSIONS: In a challenging listening environment with diffuse restaurant noise, SNR-50 was most improved in the study sample when using a personal ear-level RM HAT system. Although tested rerouting devices offered measurable improvement in performance (1-2 dB in SNR-50) when the target was directed to the impaired ear, benefit may be offset by a detriment in performance in the opposing condition. Findings continue to support use of RM HAT for children with severe-to-profound UHL in adverse listening environments, when there is one primary talker of interest, to ensure advantageous SNRs.

Common genetic susceptibility loci link PFAPA syndrome, Behçet's disease, and recurrent aphthous stomatitis.

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. The disease appears to cluster in families, but the pathogenesis is unknown. We queried two European-American cohorts and one Turkish cohort (total n = 231) of individuals with PFAPA for common variants previously associated with two other oropharyngeal ulcerative disorders, Behçet's disease and recurrent aphthous stomatitis. In a metaanalysis, we found that a variant upstream of IL12A (rs17753641) is strongly associated with PFAPA (OR 2.13, P = 6 × 10-9). We demonstrated that monocytes from individuals who are heterozygous or homozygous for this risk allele produce significantly higher levels of IL-12p70 upon IFN-γ and LPS stimulation than those from individuals without the risk allele. We also found that variants near STAT4, IL10, and CCR1-CCR3 were significant susceptibility loci for PFAPA, suggesting that the pathogenesis of PFAPA involves abnormal antigen-presenting cell function and T cell activity and polarization, thereby implicating both innate and adaptive immune responses at the oropharyngeal mucosa. Our results illustrate genetic similarities among recurrent aphthous stomatitis, PFAPA, and Behçet's disease, placing these disorders on a common spectrum, with recurrent aphthous stomatitis on the mild end, Behçet's disease on the severe end, and PFAPA intermediate. We propose naming these disorders Behçet's spectrum disorders to highlight their relationship. HLA alleles may be factors that influence phenotypes along this spectrum as we found new class I and II HLA associations for PFAPA distinct from Behçet's disease and recurrent aphthous stomatitis.

Incidence, Time Course, and Implications of Electrode Abnormalities in Pediatric Cochlear Implant Recipients.

OBJECTIVES: To identify the incidence of specific abnormal impedance patterns or electrode faults, and determine their implication and significance, in a pediatric population of cochlear implant recipients. DESIGN: Nine hundred fifty-six cochlear implant devices (621 recipients) were included in this retrospective study. Devices were included if the implantation surgery was performed at our tertiary care hospital, and the recipient was 21 years of age or younger at the time the device was implanted. Device models incapable of producing impedance measures by telemetry were excluded from the study. Individual devices with abnormal impedance measures indicating an open circuit (OC), short circuit (SC), or partial short circuit (partial SC) were included in the study, unless these abnormalities occurred only in the OR and not postoperatively. Device and patient characteristics were examined to determine their relationship to increased incidence of electrode faults or atypical patterns. RESULTS: The incidence of software-identified electrode faults in our exclusively pediatric population was similar to that reported in the literature containing mixed-age cohorts. Nine percent of devices experienced at least one OC or one pair of SCs. Although higher incidence of these faults was seen in some specific device models, the long-term average of these faults was equivalent across manufacturers. No factors examined in this study increased the likelihood of experiencing a software-identified electrode fault. Within the study period under examination (October 1997 to March 2018), partial SCs (presenting as zig-zag or low-flat impedance patterns) were only observed in Cochlear devices. While the incidence of these partial SC abnormalities (non-software-identified faults) was 6% across all models of Cochlear devices, the CI24RCS experienced the highest incidence of partial SCs. The incidence of this pattern was lower in models manufactured after CI24RCS. CONCLUSIONS: This study provides incidence of various cochlear implant electrode impedance abnormalities across a large cohort of pediatric recipients. The incidence of all electrode abnormalities was relatively low, particularly partial SCs, which are less well recognized and not currently identified by clinician-accessible software. Incidence of software-identified electrode faults (i.e., SCs and OCs) in our pediatric-only study is similar to the incidence reported in other mixed-population and adult-only studies. These common electrode faults generally are not associated with device failure, and clinicians should feel comfortable reassuring families that an individual electrode fault does not imply an impending device failure. Conversely, those atypical impedance patterns not currently flagged by the programming software as abnormal, but visible to the clinician's eye (i.e., partial SCs in zig-zag or low-flat patterns), have a higher likelihood of device damage and failure. Performance in patients with electrode arrays exhibiting these atypical patterns should be closely monitored for any functional decrement, and proactively managed to maintain performance whenever possible.

Incidence of Hearing Loss in Patients With Neurofibromatosis Type 1 at a Tertiary Care Pediatric Hospital.

BACKGROUND: Hearing loss has not been thoroughly investigated as a comorbidity in larger cohorts with neurofibromatosis type 1 (NF1). METHODS: Available audiometric data were reviewed from patients with NF1 seen at a tertiary pediatric hospital to assess prevalence and risk factors for hearing loss. RESULTS: Of 1172 patients with NF1 seen between 2010 and 2022, 90 had available audiometric data and 48 of 90 patients (53%) had one or more audiogram revealing hearing loss. Those not referred to audiology were presumed to have normal hearing, resulting in a conservative hearing loss estimate of 4% for children and young adults with NF1. Of 90 patients with audiograms, 29 (32%) had conductive loss (CHL), 15 (17%) had sensorineural loss (SNHL), and 3 (3%) had mixed hearing loss. Hearing loss type was undetermined for one patient. For children with CHL, six had permanent CHL secondary to plexiform neurofibroma, 19 CHL were transient due to active middle ear dysfunction, and four CHL cases were indeterminate in etiology. For three children with SNHL or mixed hearing loss, etiology included history of ototoxic chemotherapy and/or family history of SNHL. In the 16 patients with SNHL or mixed hearing loss with more than one audiogram over time, progressive hearing decline was noted in eight of 16, and 26 of 178 hearing thresholds (15%) progressed. CONCLUSIONS: Our findings suggest that audiometric evaluations should be considered for at least a subset of children with NF1, given the higher-than-expected rate of hearing loss in patients with NF1 compared with the general population.

Vestibular Preservation in Pediatric Cochlear Implantation.

OBJECTIVES: Evaluate the rate of preserved vestibular function in pediatric cochlear implant surgery. STUDY DESIGN: Retrospective case review. METHODS: Pre- and post-operative vestibular tests were compared in children who underwent cochlear implantation at a tertiary level pediatric hospital over a 4-year period. RESULTS: Data from 59 implanted ears in 44 children was included. Median age was 2.8 years at initial testing (range 7 months - 21 years) with 1:1 male/female ratio. Implant surgeries were 26 unilateral, 13 bilateral simultaneous, and 5 bilateral sequential. The majority were implanted with slim, non-styletted electrodes (86.4%) via a round window approach (91.5%). Normal pre-operative results were preserved post-operatively on rotary chair testing in 75% (21/28) of patients, cervical vestibular evoked myogenic potential testing in (75%) 30/40 of ears tested, ocular vestibular evoked myogenic potential testing in 85.7% (6/7) of ears tested, video head impulse testing in 100% (9/9) of ears tested, and computerized dynamic posturography in 100% (5/5) of patients tested. Overall, 62.5% of patients had no new deficits on any vestibular test performed post-operatively. CONCLUSIONS: Preservation rates of vestibular function following cochlear implant surgery were higher in this cohort than what has been reported in many earlier studies. Contemporary, less traumatic electrodes and insertion techniques may be a significant factor. The risk of causing a new, severe bilateral vestibular loss with long-term functional impacts appears to be low. Further study is warranted on the impacts of different cochlear implant electrode designs and insertion approaches on post-operative vestibular preservation. LEVEL OF EVIDENCE: 4, Case Series Laryngoscope, 134:1913-1918, 2024.

Prognosis tool based on a modified children's implant profile for use in pediatric cochlear implant candidacy evaluation.

OBJECTIVES: We developed a prediction tool to assist in evaluation of pediatric candidates for cochlear implantation (CI) and to help plan for preoperative and postoperative support. METHODS: Between 1995 and 2005, 277 patients underwent CI at Children's Hospital Boston. Of these 277 patients, 250 had at least 2 years of post-CI follow-up and adequate pre-CI information for rating by our prediction tool. Of the 250, 106 were randomly selected for inclusion. The patients were divided into group A (auditory/oral communicator); group B (auditory/oral communicator with visual assistance), group C (visual/manual communicator with auditory/oral skills assistance), and group D (will not derive communicative benefit from implant). Predictions were performed with clinical assessment and two statistical techniques: regression modeling and classification and regression tree (CART) analysis. RESULTS: Among patients who became auditory/oral communicators (group A), clinical assessment predicted that outcome accurately 65% of the time, CART analysis had intermediate sensitivity (79%), and regression modeling was the most sensitive (95%). Groups B through D were predicted 45% of the time by regression modeling, 90% of the time by clinical assessment, and 100% of the time by CART analysis. CONCLUSIONS: A combination of speech-language, medical, and educational constructs can provide a reliable prediction of the communication outcome. Our goal for the prognosis tool is to make it part of the overall candidacy process in supporting decision-making about CI and planning for post-CI therapy.

Pain at the Cochlear Implant Site Requiring Device Removal in Pediatric Patients.

OBJECTIVES: Idiopathic pain at the cochlear implant (CI) site outside of the immediate postoperative period is an uncommon occurrence but may necessitate device explantation. Our objective was to describe the clinical course for pediatric patients with CI site pain who ultimately required device explantation. STUDY DESIGN: Retrospective chart review. METHODS: We performed a retrospective database review of CIs performed at a tertiary referral center for pediatric cochlear implantation. We specifically evaluated pediatric patients who presented with pain at or near the CI device site and ultimately required explantation. RESULTS: Fifteen patients (16 CIs) had pain at or near the CI site requiring device explantation. Cultures taken during site exploration or device explantation identified bacteria in 86% and 81% of procedures, respectively. Propionibacterium acnes and Staphylococcus non-aureus were the most commonly identified organisms. CONCLUSIONS: The majority of patients with idiopathic pain in this cohort ultimately requiring CI explantation had chronic bacterial colonization. LEVEL OF EVIDENCE: 4 (Case series) Laryngoscope, 132:2044-2049, 2022.

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: A review.

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most common periodic fever condition in children, with most cases appearing by the age of 5. Although PFAPA is generally a self-limited condition, it can have a major impact on a child's quality of life, as well as that of their family. Recent research has continued to shed light on the genetic and immunologic factors that play a role in the pathogenesis of PFAPA. There also exists significant heterogeneity in treatment strategies, and progress has been made to develop evidence-based management strategies and establish a standard of care. This review will outline current knowledge regarding the pathogenesis of PFAPA, as well as treatment strategies and our clinical experience.

Adverse events in pediatric cochlear implant patients undergoing magnetic resonance imaging.

OBJECTIVE: To investigate the prevalence and nature of adverse events in magnetic resonance imaging (MRI) of pediatric cochlear implant (CI) patients. METHODS: Retrospective chart review at a tertiary pediatric hospital. CI patients who underwent MRI from 2004 through 2019 were identified via our internal radiology database. Comorbidities, CI model, age at MRI, number of MRIs, type of MRIs, indication for MRIs, precautions taken for MRIs, quality of MRIs, anesthesia during MRIs, patient language abilities, and adverse events were recorded from the electronic medical record. The literature was reviewed, and our results were compared to those of previous similar series. RESULTS: From 2004 to 2019, 12 pediatric patients (17 ears) with CIs underwent 22 MRIs. 12 MRIs were performed in CI patients with retained internal magnet. 4/22 MRIs resulted in morbidity; 2 patients experienced pain requiring MRI abortion, 1 experienced magnet rotation requiring surgical replacement, and 1 underwent operative removal of the magnet prior to the scan with surgical replacement thereafter. 19/22 MRIs were performed to evaluate the brain; 17/22 of the radiologic reports noted limitation of evaluation due to artifact. 18/22 MRIs required the administration of anesthesia. 9 of the 22 MRI events involved 2 patients whose CIs had been without internal magnet in anticipation of future MRI requirement. CONCLUSIONS: Adverse events affecting pediatric patients with CI can occur as a result of MRI, despite appropriate precautions. Safety requires consideration of factors unique to a pediatric hearing-impaired population. Clinicians must remain informed on best practices and manufacturer recommendations.

Obesity as a Possible Risk Factor for Pediatric Sensorineural Hearing Loss.

OBJECTIVES/HYPOTHESIS: Childhood hearing loss impacts linguistic, academic, social, and psychologic development, and may have lasting implications for future workforce performance. Current evidence for obesity as a pediatric sensorineural hearing loss (SNHL) risk factor is intriguing but equivocal. We hypothesized that obesity is associated with a higher risk of SNHL. We additionally examined whether underweight is associated with a higher risk of SNHL. STUDY DESIGN: Retrospective database review. METHODS: A single-institution audiologic database from 2015 to 2020 was queried for audiograms with type-A tympanograms from children aged 5 to 18 years old. Comorbidities known to be associated with hearing loss were excluded. We then examined both for sub-clinical (≥15 dB) high- or low-frequency hearing loss, and for clinical (≥21 dB) hearing loss, with the aim of examining the association between obesity and SNHL. Multivariable logistic regression was performed to adjust for age, gender, diabetes mellitus, attention deficit hyperactivity disorder, and autism. RESULTS: A total of 3,142 children were included. Obesity was not associated with risk of SNHL (adjusted OR 0.82; 95% CI: 0.60, 1.12). Underweight children had a higher risk of SNHL than normal weight children (adjusted OR 1.78; 95% CI: 1.08, 2.95). Autism was significantly associated with increased risk of sub-clinical SNHL only (adjusted OR 2.00; 95% CI 1.34, 2.98). CONCLUSIONS: No association was found between obesity and pediatric SNHL. Underweight children may represent a higher-risk population for SNHL. There appears to be an increasing risk of SNHL as children approach adolescence. Further study of systemic risk factors for SNHL is indicated. LEVEL OF EVIDENCE: 3 Laryngoscope, 131:1416-1419, 2021.

Peripheral Vestibular Dysfunction Is a Common Occurrence in Children With Non-syndromic and Syndromic Genetic Hearing Loss.

Hearing loss (HL) is the most common sensory deficit in humans and is frequently accompanied by peripheral vestibular loss (PVL). While often overlooked, PVL is an important sensory dysfunction that may impair development of motor milestones in children and can have a significant negative impact on quality of life. In addition, many animal and in vitro models of deafness use vestibular hair cells as a proxy to study cochlear hair cells. The extent of vestibular end organ dysfunction associated with genetic pediatric hearing loss is not well-understood. We studied children with a known genetic cause of hearing loss who underwent routine preoperative vestibular testing prior to cochlear implantation between June 2014 and July 2020. Vestibular testing included videonystagmography, rotary chair, video head impulse testing, and/or vestibular evoked myogenic potentials. Etiology of HL was determined through history, physical examination, imaging, laboratory testing, and/or genetic testing. Forty-four children (21 female/23 male) met inclusion criteria; 24 had genetic non-syndromic and 20 had genetic syndromic forms of HL. Mean age at the time of testing was 2.8 ± 3.8 years (range 7 months-17 years). The most common cause of non-syndromic HL was due to mutations in GJB2 (n = 13) followed by MYO15A (3), MYO6 (2), POU3F4 (2), TMPRSS3 (1), CDH23 (1), TMC1 (1), and ESRRB (1). The most common forms of syndromic HL were Usher syndrome (4) and Waardenburg (4), followed by SCID/reticular dysgenesis (3), CHARGE (2), CAPOS (1), Coffin-Siris (1), Jervell and Lange-Nielsen (1), Noonan (1), peroxisome biogenesis disorder (1), Perrault (1), and Trisomy 21 (1). Overall, 23 patients (52%) had PVL. A larger proportion of children with syndromic forms of HL had PVL (12/20, 60%) compared with children with genetic non-syndromic HL (11/24, 46%), though without statistical significant (p = 0.3). The occurrence of PVL varied by affected gene. In conclusion, PVL is a common finding in children with syndromic and non-syndromic genetic HL undergoing vestibular evaluation prior to cochlear implantation. Improved understanding of the molecular physiology of vestibular hair cell dysfunction is important for clinical care as well as research involving vestibular hair cells in model organisms and in vitro models.

Systemic Bevacizumab for Treatment of Respiratory Papillomatosis: International Consensus Statement.

OBJECTIVES/HYPOTHESIS: The purpose of this study is to develop consensus on key points that would support the use of systemic bevacizumab for the treatment of recurrent respiratory papillomatosis (RRP), and to provide preliminary guidance surrounding the use of this treatment modality. STUDY DESIGN: Delphi method-based survey series. METHODS: A multidisciplinary, multi-institutional panel of physicians with experience using systemic bevacizumab for the treatment of RRP was established. The Delphi method was used to identify and obtain consensus on characteristics associated with systemic bevacizumab use across five domains: 1) patient characteristics; 2) disease characteristics; 3) treating center characteristics; 4) prior treatment characteristics; and 5) prior work-up. RESULTS: The international panel was composed of 70 experts from 12 countries, representing pediatric and adult otolaryngology, hematology/oncology, infectious diseases, pediatric surgery, family medicine, and epidemiology. A total of 189 items were identified, of which consensus was achieved on Patient Characteristics (9), Disease Characteristics (10), Treatment Center Characteristics (22), and Prior Workup Characteristics (18). CONCLUSION: This consensus statement provides a useful starting point for clinicians and centers hoping to offer systemic bevacizumab for RRP and may serve as a framework to assess the components of practices and centers currently using this therapy. We hope to provide a strategy to offer the treatment and also to provide a springboard for bevacizumab's use in combination with other RRP treatment protocols. Standardized delivery systems may facilitate research efforts and provide dosing regimens to help shape best-practice applications of systemic bevacizumab for patients with early-onset or less-severe disease phenotypes. LEVEL OF EVIDENCE: 5 Laryngoscope, 131:E1941-E1949, 2021.

Association of Blood Type With Postsurgical Mucosal Bleeding in Pediatric Patients Undergoing Tonsillectomy With or Without Adenoidectomy.

Importance: Blood type (BT) O has been identified as a risk factor for bleeding complications, while non-O BTs may increase risk for thromboembolic events. Limited data are available in children undergoing tonsillectomy with or without adenoidectomy. Objective: To determine whether BT O is associated with hemorrhage after tonsillectomy with or without adenoidectomy. Design, Setting, and Participants: Retrospective cohort study of patients younger than 22 years who underwent tonsillectomy with or without adenoidectomy at a single institution between January 1, 2008, and August 7, 2017. Statistical analysis was performed from November 2017 to January 2019. Main Outcomes and Measures: Prevalence of hemorrhage following surgery was defined as any bleeding requiring cauterization up to 1 month after the procedure. Data on sex, age, von Willebrand disease (VWD) status, BT, white blood cell counts, and platelet counts closest to date of surgery were collected from an electronic medical record system, and the association of these factors with hemorrhage following surgery was investigated. Results: A total of 14 951 pediatric patients (median [range] age, 5.6 [0.8-21.9] years; 6956 [46.5%] female) underwent tonsillectomy with or without adenoidectomy. Prevalence of hemorrhage following the procedure was 3.9% (578 patients) for the full cohort and 2.8% (362 of 13 065) for patients with no BT identified or preprocedure VWD panel results at baseline. Children who had a BT identified and/or a VWD panel before surgery had higher bleeding rates (BT only, 14.9% [172 of 1156]; preprocedure VWD panel only, 4.6% [28 of 607]; and BT and preprocedure VWD panel, 13.0% [16 of 123]), all of which were significantly different from the baseline bleeding rate (P < .001). While the bleeding rates in children with BT O were not statistically different from those with non-O BT (14.8% and 14.6%, respectively; P > .99), mean von Willebrand factor values were statistically different (mean [SD] von Willebrand factor antigen level in O group, 86.9 [42.4] IU/dL in the O group vs 118.0 [53.8] IU/dL in the non-O group; P = .002; and mean [SD] von Willebrand factor ristocetin-cofactor in the O group, 72.2 [44.3] IU/dL vs 112.6 [68.0] IU/dL in the non-O group; P = .001). In addition, children older than 12 years had increased bleeding rates in the full cohort (8.3% vs 3.2%), in the testing-naive cohort (6.5% vs 2.3%), and in those with a preprocedure VWD panel only (13.5% vs 3.1%) compared with children aged 12 years or younger. Conclusions and Relevance: Type O blood was not a risk factor associated with hemorrhage after tonsillectomy with or without adenoidectomy despite lower baseline von Willebrand factor antigen and von Willebrand factor ristocetin-cofactor values in children with BT O vs those with non-O BT in our study cohort. No association was found between VWD status and bleeding, and there was no difference in VWD panel values in those who experienced hemorrhage vs those who did not within BT groups. Further studies elucidating the utility of von Willebrand factor values for children undergoing tonsillectomy with or without adenoidectomy are needed.

Torticollis in children with enlarged vestibular aqueducts.

OBJECTIVES: To evaluate the association between torticollis and enlarged vestibular aqueduct (EVA). METHODS: An online/phone survey was administered to parents of 133 children diagnosed with the following disorders: EVA, GJB2 (Connexin 26) mutations associated congenital hearing loss and epistaxis (control). The survey included questions regarding symptoms of torticollis, vertigo, and hearing loss. RESULTS: Patients with EVA had a 10-fold greater odds of having torticollis than controls (31% vs. 4%; OR = 10.6; 95% CI: 2.9, 39.2). No patients with GJB2 had a reported history of torticollis. Torticollis preceded the diagnosis of hearing loss in most (87%) patients with EVA who had a reported history of torticollis. EVA patients were more likely to have reported motor delay than controls (40% vs. 15%; p = 0.002). EVA patients with prior torticollis (80%; 12/15) were more likely to have balance impairment than EVA patients without prior torticollis (12%; 4/33; p < 0.001). Twelve patients had a reported history of paroxysmal torticollis, all of whom had EVA. CONCLUSION: Torticollis in infants may be a marker of EVA. Infants with torticollis should be monitored closely for hearing loss and motor delay, especially when the torticollis is paroxysmal.

Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA): a framework to evaluate treatment responses from the childhood arthritis and rheumatology research alliance (CARRA) PFAPA work group.

BACKGROUND: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. There is considerable heterogeneity in management strategies and a lack of evidence-based treatment guidelines. Consensus treatment plans (CTPs) are standardized treatment regimens that are derived based upon best available evidence and current treatment practices that are a way to enable comparative effectiveness studies to identify optimal therapy and are less costly to execute than randomized, double blind placebo controlled trials. The purpose of this project was to develop CTPs and response criteria for PFAPA. METHODS: The CARRA PFAPA Working Group is composed of pediatric rheumatologists, infectious disease specialists, allergists/immunologists and otolaryngologists. An extensive literature review was conducted followed by a survey to assess physician practice patterns. This was followed by virtual and in-person meetings between 2014 and 2018. Nominal group technique (NGT) was employed to develop CTPs, as well as inclusion criteria for entry into future treatment studies, and response criteria. Consensus required 80% agreement. RESULTS: The PFAPA working group developed CTPs resulting in 4 different treatment arms: 1. Antipyretic, 2. Abortive (corticosteroids), 3. Prophylaxis (colchicine or cimetidine) and 4. Surgical (tonsillectomy). Consensus was obtained among CARRA members for those defining patient characteristics who qualify for participation in the CTP PFAPA study. CONCLUSION: The goal is for the CTPs developed by our group to lead to future comparative effectiveness studies that will generate evidence-driven therapeutic guidelines for this periodic inflammatory disease.

International Pediatric Otolaryngology Group (IPOG) Consensus Recommendations: Congenital Cholesteatoma.

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

Sensorineural hearing loss in patients with cystic fibrosis.

OBJECTIVE: To determine the prevalence of sensorineural hearing loss (SNHL) in cystic fibrosis (CF) patients and its relationship to antibiotic use. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care pediatric hospital. SUBJECTS AND METHODS: We reviewed the medical records of CF patients seen in our children's hospital between March 1994 and December 2007. Data collected included patient demographics, audiograms, tympanograms, genotype, and use of potentially ototoxic antibiotics. RESULTS: Seven of 50 (14%) patients had SNHL. Three percent of patients who received 10 courses (P<0.01). No patients who received five or fewer courses of nasal irrigation with aminoglycosides had SNHL versus 23 percent of those who received more than five courses (P<0.05). Nine percent of patients who received five or fewer courses of macrolides had SNHL versus 60 percent of those who received more than five courses (P=0.079). CONCLUSION: CF patients receiving aminoglycosides are at high risk for developing SNHL.

Effect of adenotonsillectomy in PFAPA syndrome.

OBJECTIVE: To assess the benefits of adenotonsillectomy in the treatment of pediatric patients with PFAPA (periodic fever, aphthous ulcers, pharyngitis, and adenitis) syndrome. DESIGN: Prospective case series. SETTING: Tertiary care pediatric hospital. PATIENTS: Pediatric patients meeting criteria for PFAPA syndrome. INTERVENTION: Tonsillectomy with or without adenoidectomy. MAIN OUTCOME MEASURE: Resolution of PFAPA symptoms. RESULTS: Twenty-seven (14 female, 13 male) children with PFAPA syndrome underwent tonsillectomy with or without adenoidectomy from 2004 through 2006. The length of follow-up for all patients ranged from 8 to 41 months. A total of 26 patients experienced a complete resolution of their symptoms. The 1 child who continued to have febrile episodes had fever cycles that were not regular in duration or interval and in hindsight was not likely a patient with PFAPA syndrome. CONCLUSIONS: Our findings showed complete resolution of symptoms in 26 of 27 patients with PFAPA syndrome treated surgically. Patients who meet clinical criteria for PFAPA syndrome should be considered for tonsillectomy and adenoidectomy if they do not respond to medical management.

Use of a preoperative bleeding questionnaire in pediatric patients who undergo adenotonsillectomy.

OBJECTIVE: To determine the efficacy of a preoperative bleeding questionnaire (POBQ) and coagulation screening in predicting hemorrhage associated with adenotonsillectomy. STUDY DESIGN: Retrospective study. SUBJECTS AND METHODS: Between January 1998 and December 2003, 7730 tonsillectomy and adenotonsillectomy patients were administered the POBQ preoperatively. Further coagulation screening was based on POBQ responses. RESULTS: A total of 232 (3.0%) of 7730 had postoperative bleeding; 184 (3.2%) of 5782 patients who had negative questionnaires bled postoperatively compared with 48 (2.5%) of 1948 patients with positive questionnaires (P = 0.126). Of 1948 patients with positive questionnaires, 141 (7.2%) had abnormal preoperative coagulation screens and 9 (6.4%) of 141 bled; of the 1807 (92.8%) with negative coagulation screens, 39 (2.2%) bled (P = 0.005). CONCLUSION: The POBQ is an effective tool for identifying patients who are at potential risk for post-tonsillectomy bleeding. Patients with both a positive POBQ and coagulation screen had a statistically higher likelihood of postoperative bleeding than other patients. The POBQ allowed the identification of individuals with bleeding disorders to be treated before surgery, likely decreasing the risk of bleeding in these patients.

Phosphorylcholine-coated antibiotic tympanostomy tubes: are post-tube placement complications reduced?

OBJECTIVE: To determine if a phosphorylcholine (PC) antibacterial coating on standard Armstrong beveled tympanostomy tubes (TT) reduced the incidence of post-tube placement complications. METHODS: A prospective cohort aged 8-51 months received bilateral TTs for otitis media with effusion between July 2002 and February 2004 at a tertiary care pediatric hospital. Seventy children were randomized to receive a PC-coated TT in one ear and an uncoated TT in the other. Otologic examinations at prescribed intervals over two years post-operatively ascertained the status of sequelae. We analyzed the incidence of TT complications: otorrhea, premature extrusion, persistent tympanic membrane perforations, granulation tissue, and ventilation tube lumen obstruction. RESULTS: There was no statistical difference in the incidence of any of these sequelae between standard and PC-coated tympanostomy tubes (p>0.05) during the 24-month-follow-up period. Results after 13 months of follow-up may have been affected by patients lost to follow-up and therefore a smaller sample size as the study continued. CONCLUSIONS: This study found that there is no statistically significant difference in the incidence of complications between uncoated and PC-coated fluoroplastic Armstrong beveled TTs.