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.

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.

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.

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.

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.

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.

Revision cochlear implant surgery in children: Surgical and audiological outcomes.

OBJECTIVES/HYPOTHESIS: To determine the incidence of cochlear implant failure and to examine surgical and audiological outcomes. STUDY DESIGN: Retrospective review, case series. METHODS: This study sought indications for revision surgery, surgical findings, and outcomes, and audiological outcomes in pediatric cochlear implant patients. Pre- and postcochlear reimplantation word recognition performance was analyzed using a modified version of the Pediatric Ranked Order Speech Perception (PROSPER) score. RESULTS: Over a 20-year period, a total of 868 cochlear implants were performed in 578 patients. The overall institutional reimplant rate was 5.9%. The indications for explantation were hard failure (30), soft failure (23), and medical/surgical indication (13). A significant portion of devices belonged to vendor recalled batches (15) or were damaged by head trauma (eight). Full electrode insertion was achieved in all 62 reimplantations. Post-reimplantation Boston Children's Hospital modified PROSER scores were either stable or improved compared to pre-reimplantation scores. CONCLUSIONS: The need for cochlear implant revision/reimplantation is infrequent, but the rate is not inconsequential. Hard and soft device failures account for the majority of reimplants. Surgical complications during reimplantation is low, and post-reimplantation audiological performance is excellent. LEVEL OF EVIDENCE: 4. Laryngoscope, 2619-2624, 2018.

Comparison of postoperative pain in pediatric patients undergoing coblation tonsillectomy versus cautery tonsillectomy.

OBJECTIVE: To compare postoperative pain scores between monopolar electrocautery and coblation subcapsular tonsillectomy. STUDY DESIGN: Prospective double-blind randomized study. SETTING: Tertiary care children's hospital. SUBJECTS AND METHODS: Between December 2004 and April 2008, 61 children, ages 4 to 20 years (mean age, 10 years; SD, 4 years), were randomized to have one tonsil removed by electrocautery and the other tonsil removed by coblation. Subjects used the FACES scale to rate pain on each side immediately postoperatively, 2 days postoperatively, and 2 weeks postoperatively. Postoperative hemorrhage was also tracked. RESULTS: Coblation tonsillectomy resulted in statistically less pain than electrocautery immediately after surgery, but this difference was not clinically significant. CONCLUSIONS: Pediatric pain is similar following monopolar electrocautery or coblation subcapsular tonsillectomy.

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.

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.

Bilateral congenital vocal cord paralysis: a 16-year institutional review.

OBJECTIVE: To review the management and outcome of bilateral congenital true vocal cord paralysis in 22 patients treated over a 16-year period and to review the role of tracheostomy in these patients. DESIGN: Retrospective chart review. SETTING: Pediatric tertiary hospital. PATIENTS: Twenty-two pediatric patients diagnosed with bilateral congenital true vocal cord paralysis. INTERVENTIONS: Flexible or rigid diagnostic evaluation, tracheostomy, and vocal cord lateralization procedures. MAIN OUTCOMES MEASURES: Vocal cord recovery and decannulation. RESULTS: With a mean follow up of 50 months, 15 of 22 patients (68%) with bilateral vocal cord paralysis required tracheostomy for airway securement. Of the 15 tracheotomized patients, 10 were successfully decannulated (8 had spontaneous recovery, whereas 2 required lateralization procedures). Eleven of these patients with tracheostomy had comorbid factors, including neurologic abnormalities (midbrain/brainstem dysgenesis, Arnold-Chiari malformation, global hypotonia, and developmental delay). Of the 7 patients not requiring tracheostomy, 6 recovered vocal cord function (86%). CONCLUSION: In our series of 22 patients with bilateral vocal cord paralysis, 14 had spontaneous recovery of function. Patients managed with tracheostomy were noted to have a high incidence of comorbid factors. In this series, recovery rates were found to be higher in nontracheostomized patients than in tracheostomized patients. Patients can be carefully selected for observation versus tracheostomy at the time of diagnosis based on underlying medical conditions.

The role of chromosomal translocation (15;19) in the carcinoma of the upper aerodigestive tract in children.

OBJECTIVE: To further evaluate the role of chromosomal translocation (15;19) in the presentation of the carcinoma (CA) of the upper aerodigestive tract. STUDY DESIGN AND SETTING: A retrospective study at a tertiary care pediatric medical center. RESULTS: Seven patients with a mean age of 12 years presented with CA of nasopharynx (N = 2), sinonasal region (N = I), parotid gland (N = 2), or larynx (N = 2). Treatments included combinations of surgery (N = 5), chemotherapy (N = 5), and radiation therapy (N = 4). One patient with sinonasal CA and one patient with laryngeal CA had chromosomal translocation (15;19); these patients both died of their disease with a mean survival of 6 months. The 5 patients without translocation (15;19) responded well to treatment and are disease-free with a mean follow-up of 47 months. CONCLUSION: The preliminary results appear to indicate poor prognosis associated with the presentation of chromosomal translocation (15;19) despite aggressive multi-modality treatment. Further investigation is needed to better understand the cause and relationship of the translocation (15;19) and aggressive behavior of these tumors.