Children who are deaf or hard of hearing (D/HH) are at high risk for permanent deficits in language acquisition and downstream effects such as poor academic performance, personal-social maladjustments, and emotional difficulties. Identification of children born D/HH through newborn hearing screening and subsequent timely early intervention can prevent or reduce many of these adverse consequences. Ongoing surveillance for changes in hearing thresholds after infancy is also important and should be accomplished by subjective assessment for signs of atypical hearing and with objective screening tests. Scheduled hearing screening may take place in the primary care setting, or via referral to an audiologist according to the Bright Futures/American Academy of Pediatrics "Recommendations for Preventive Pediatric Health Care" (also known as the periodicity schedule). This report covers hearing assessment beyond the newborn period, reviews risk factors for hearing level change, and provides guidance for providers of pediatric primary care on the assessment and care of children who are D/HH.

Background PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome is diagnosed clinically. Adult-onset PFAPA syndrome is rare and often has a more diverse clinical presentation that its childhood counterpart. This is the first reported case of adult-onset PFAPA syndrome with complete response to lingual tonsillectomy. Case Summary A 41-year-old man was evaluated for periodic fevers associated with uvulitis, cervical lymphadenitis, pharyngitis, and lower extremity rash. He had a variable response to steroids and was intolerant of colchicine. Laboratory workup revealed intermittent elevation of erythrocyte sedimentation rate and C-reactive protein level. Computed tomography neck and laryngoscopy confirmed adenoidal and lingual tonsillar hypertrophy. He underwent adenoidectomy and lingual tonsillectomy with resolution of symptoms. Conclusions Hypertrophy of the remaining lymphoid structures within Waldeyer's ring may be associated with remote recurrence of PFAPA syndrome after tonsillectomy. Lingual tonsillectomy may be an alternative treatment strategy in select patients with PFAPA, prominent lingual hypertrophy, and incomplete response to steroids.

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.

Objective: To review histopathologic diagnoses from tonsillectomy specimens and determine whether routine pathologic exam is necessary. Methods: Pathology reports of patients undergoing tonsillectomy from 2005 to 2014 at our pediatric tertiary care hospital were reviewed. Histopathologic diagnoses were recorded with special attention to identification of malignancy. Results: A total of 8807 paired tonsil specimens were sent to pathology over a 10-year course. Gross analysis was performed on all. Microscopic histopathologic analysis was performed on 612 (6.95%) specimens with all but one demonstrating strictly reactive lymphoid hyperplasia. The single specimen (0.16%) demonstrated follicular hyperplasia with focal necrotizing granulomatous lymphadenitis without organisms identified on special staining. The surgeon requested pathologic diagnosis to rule out lymphoma in 4 of 8087 (0.05%) of the specimens. No malignancies were identified. The approximate charges for gross examination of a paired tonsillectomy specimen and microscopic examination were $136.10 and $294.54, respectively. Over the 10 year period of the study, total charges were estimated at $1,115,340 (gross) and $180,258 (microscopic). Discussion: Microscopic analysis of tonsil specimens is unlikely to identify abnormal pathology that changes patient management. This study suggests that neither gross nor microscopic pathologic examination of tonsillectomy specimens is necessary on a routine basis. Histologic analysis of tonsils should be requested only on a case by case basis when clinical suspicion for malignancy is high. Avoiding routine pathologic exam of tonsils may be cost effective and medically safe. (C) 2017 Elsevier B.V. All rights reserved.

STUDY OBJECTIVES:To describe characteristics and surgical and clinical outcomes of obese children with obstructive sleep apnea (OSA). METHODS:At our institution from 2000 to 2010, 143 obese children with an overnight polysomnography (OPSG) diagnosis of OSA, excluding children with comorbidities, were identified. Relationships between demographics, clinical findings, and the severity of OSA were assessed. Presurgery and postsurgery OPSG indices were compared. We defined cure as an apneahypopnea index (AHI) < 1.5/h on the postsurgery OPSG, and we compared the cure rates of different surgeries. RESULTS:A total of 143 children, median age 12.4 y (interquartile range [IQR] 9.6-14.9) and BMI z-scores 2.8 (IQR 2.6-2.9), were included. Seventy-eight (55%) (Median age 12 y [IQR 9-15]) underwent surgery: 1 had tonsillectomy; 1 tonsillectomy + uvulopharyngopalatoplasty (UPPP); 23 adenotonsillectomy (AT); 27 AT + UPPP; 11 adenoidectomy + UPPP; 8 UPPP; and 7 AT + turbinate trim ± tongue base suspension. Overall, surgery cured 19 children (26%), but AHI improved in the majority of children (p = 0.001). Similarly, the arousal index, PETCO2, and SpO2 nadir improved significantly (p < 0.002, p = 0.019, p < 0.001, respectively). AHI improved significantly in children with mild-to-moderate OSA in comparison to severe OSA (p < 0.001). Children with enlarged tonsils and no history of prior surgery benefitted more often from surgery (p < 0.004 and p = 0.002, respectively). AT was the only surgery reducing the AHI significantly (p = 0.008). Children did not lose weight despite intervention. Adherence with PAP was poor. CONCLUSIONS:Surgery improved OPSG indices in the majority of obese children with OSA.

Infant hearing loss is common. Screening is performed in more than 98% of US infants. Otolaryngologists play an important role in identification and management of infants and children who are deaf and hard of hearing. Otolaryngologists should routinely assess for hearing screening results and intervene for screens not passed. Long-term follow-up and reassessment of patients with hearing loss is an ongoing component of otolaryngology practice. This article reviews the otolaryngologist's role in the management of infants and children who are deaf or hard of hearing from screening to intervention and management.

IMPORTANCE:Supraglottoplasty (SGP) failure is frequently attributed to coexistent medical comorbidities, but studies specifically evaluating outcomes in these populations are lacking. OBJECTIVE:To assess SGP outcomes in patients with neurologic and syndromic comorbidities and severe laryngomalacia (LM). DESIGN, SETTING, AND PARTICIPANTS:Case series with retrospective review of medical records of 54 patients with neurologic and/or syndromic comorbidity and severe LM who underwent SGP between 2004 and 2012 at a tertiary care pediatric institution. INTERVENTIONS:Patients presented with severe LM that required SGP. Supraglottoplasty failure necessitated revision SGP, tracheostomy, or gastrostomy tube insertion, or LM and obstructive sleep apnea that required assisted ventilation (continuous positive airway pressure and bilevel positive airway pressure). MAIN OUTCOMES AND MEASURES:Medical records were reviewed with a focus on patient factors, surgical timing, complications, and surgical and dysphagia outcomes. Patients were grouped based on their age at the time of SGP as infants (aged ≤12 months) and children (aged >12 months). Statistical comparisons were performed with SGP outcomes of infants with LM and no comorbidities. RESULTS:Fifty-four patients met the inclusion criteria. Thirty-one (13 infants, 18 children) had a neurologic condition and 23 (15 infants, 8 children) had syndromes. The overall success rate of SGP was 67% (36 of 54) in these populations. Neurologic (P = .003) and syndromic (P < .001) comorbidities were associated with significant reduction in SGP success rates vs no comorbidities. Among SGP failures (18 of 54 [33%]), 13% (7 of 54) required tracheostomy, 9% (5 of 54) needed assisted ventilation, 7% (4 of 54) required a postoperative gastrostomy tube, and 4% (2 of 54) required revision SGP. In the neurologic comorbidities group, patients with cerebral palsy had significantly higher tracheostomy rates compared with those who had other neurologic pathologies constituting comorbidities (2 of 11 [18%] vs 0 of 20; P = .049). In infants, acute airway obstruction was the most common indication for SGP in the neurologically comorbidity and syndrome populations (success rates, 69% and 67%, respectively). In children, obstructive sleep apnea was the most common indication for SGP in the neurologic comorbidity and syndrome populations (success rates, 78% and 50%, respectively). CONCLUSIONS AND RELEVANCE:Supraglottoplasty remains useful and outcomes were better in patients with neurologic comorbidity than in patients with syndromic comorbidity.

Objectives: Disturbances in breathing or feeding may profoundly affect parental perceptions of an infant's health. Previous research into quality of life (QOL) for patients with laryngomalacia is limited to retrospective analysis. The purpose of this study is to prospectively evaluate the QOL of children with laryngomalacia and their parents and the impact of surgical and non‐surgical treatments. Methods: Pilot prospective analysis using the Laryngomalacia QOL Survey in patients with newly diagnosed laryngomalacia under age one year. A 29‐question survey regarding severity of symptoms related to overall health, airway, and swallowing is completed at initial and post‐treatment visits. Responses are quantified over a range from 1 to 5 (5 always or severe). Results: Twenty‐seven patients were enrolled in the study. Twelve were managed medically, and 15 underwent supraglottoplasty. The overall mean QOL score for patients treated medically was 2.57 (standard error SE 0.16) on initial visit and 1.67 (SE 0.16) post‐treatment (mean 3.9 months). Patients undergoing supraglottoplasty had an overall mean QOL score of 3.59 (SE 0.14) on initial visit and 2.22 (SE 0.22) post‐treatment (mean 3.5 months). Analysis of variance and post‐hoc testing revealed significant improvement between initial and follow‐up visits in both treatment groups ( P < 0.01). Patients who underwent supraglottoplasty had significantly higher scores at initial visit ( P < 0.01). No statistically significant difference was noted between patient groups post‐treatment ( P > 0.05). Conclusions: Prospective QOL assessment of children with laryngomalacia and their families reveals a significant burden of disease. Quality of life improves in all patients but may improve more significantly in patients managed surgically.

New!Keep up with current practice guidelines and policies with the latest, most up-to-date edition of this clinical reference classic. This evidence-based decision-making tool for managing common pediatric conditions has been revised and updated for 2014, with the latest clinical practice guidelines for more than 30 conditions, plus every AAP policy statement, clinical report, and technical report through December 2013.Updated and expanded for 2014 including:- Three new AAP clinical practice guidelines- Management of Newly Diagnosed Type 2 Diabetes Mellitus- Diagnosis and Management of Acute Bacterial Sinusitis in Children Aged 1 to 18 Years- Diagnosis and Management of Acute Otitis Media- New Periodicity Schedule- Full text of more than 60 new or revised AAP policies- CD-ROM includes the full text of more than 400 AAP clinical practice guidelines, policy statements, clinical reports, and technical reports.- More than 30 clinical practice guidelines including Sleep Apnea, ADHD, bronchiolitis, dysplasia of the hip, gastroenteritis, otitis media, urinary tract infection and more.- 2014 immunization schedule.

BACKGROUND:Tracheostomy wounds are commonly encountered in children but rarely reported. Relatively few treatments are available or have been investigated to manage this problem. Healing times for pediatric tracheostomy wounds are often unpredictable and protracted. Recent use of maltodextrin gel (MD) and a silver alginate sponge (AG) at our institution has demonstrated expedited healing and interest in this novel treatment for tracheostomy wounds. METHODS:We conducted an 11-month retrospective review of children with wound complications following tracheostomy placement at a tertiary care facility. Wounds were evaluated and rated based upon the National Pressure Ulcer Advisory Panel staging system. Subjects identified with stage 2 or greater tracheostomy-related ulcers treated with MD and/or AG were included. Subject characteristics and wound healing rates were tabulated in a database that included age, wound site, initial and final wound stage, type of treatment, length of treatment, and complications. Tracheostomy wounds treated as an out-patient were excluded from the study. RESULTS:Eighteen subjects, which included both in-patients and out-patients, were treated with AG and/or MD during the study period for tracheostomy-related wounds. Of the 26 subjects with tracheostomies performed during the study period, 10 (38.5%) were treated for postoperative wounds. A total of 11 subjects completed their in-patient wound treatment and were thus included in the study. Average subject age was 5.3 y (range 0.25-15.6 y). Wound locations were as follows: infrastomal region (n = 8), stoma (n = 2), and lateral neck (n = 1). Six subjects had stage 2 wounds, 4 had stage 3 wounds, and 1 had a stage 4 ulcer. All wounds achieved complete epithelialization following treatment with MD and/or AG. The average length of treatment was 12.8 d (range 6-28 d). No adverse effects were identified. CONCLUSIONS:Postoperative tracheostomy wounds are common. The use of MD and AG provides an effective and safe treatment for tracheostomy-related ulcers.

Purpose Prematurity is thought to be to be an independent risk factor for supraglottoplasty (SGP) failure. The purpose of this study was to compare the outcomes of supraglottoplasty in premature infants with congenital laryngomalacia (LM) with that of term infants. Study Design Case series with chart review. Setting Tertiary-care pediatric institution. Methods Analysis was performed on 325 consecutive patients undergoing SGP between 2004 and 2012. Patients older than 12 months age or with syndrome, neurologic, or cardiac comorbidities were excluded. Resolution of airway symptoms after SGP was considered successful while revision SGP and tracheostomy were considered failures. The rates of secondary airway lesions (SAL), dysphagia, and gastrostomy tube (GT) placement were also compared. Results A total of 176 infants (136 term, 40 preterm) were identified. SGP was successful in 92.7% term and 90% preterm infants with no significant difference (P = .5865). Incidence of revision SGP and tracheostomy was similar among the groups. The outcomes of SGP were the same in all groups stratified by age. Incidence of SAL was significantly higher in preterm (72.5%) compared to term infants (34.6%, P = .0002). Dysphagia rates in the follow-up were higher in preterm (32.5%) versus term infants (6.6%, P < .001). The rate of GT insertion was significantly greater (P = .003) in preterm (27.5%) than term infants (6.6%). The preoperative dysphagia, persistent dysphagia, and GT in follow-up was significantly higher when gestation age <32 weeks (25%, P = .0168). Conclusion Supraglottoplasty outcomes in term and preterm infants were similar irrespective of gestation age. Higher rates of dysphagia in follow-up are seen when gestation age <32 weeks.

Objectives/HypothesisFirst branchial cleft anomalies are rare and their various anatomical relationships to the facial nerve have been described. We encountered a 15-year-old female with a type II first branchial cleft cyst presenting as a right neck mass that we found during surgical excision to transverse two main facial nerve trunks. To our knowledge, this is the first reported case of a first branchial cleft anomaly in conjunction with a duplicated facial nerve trunk.

BACKGROUND: Many pediatric patients need positive airway pressure (PAP) for treatment of obstructive sleep-disordered breathing. Adherence to PAP (defined as percent of nights with PAP use of > 4 h) is often poor and not sustained long-term. With any chronic disease, education has been shown to help with patient outcomes. Education of patients and parents regarding PAP can be provided by different healthcare professionals. There is no published literature assessing the role of respiratory therapists (RTs) in improving adherence to PAP in children. We hypothesized that the addition of RT visits to a PAP clinic would improve PAP adherence. METHODS: RT services for PAP patients were introduced in a multidisciplinary pediatric sleep clinic in May 2006. We identified children who had been followed in clinic, and had adherence download information before and after introduction of RT services. We collected demographic, polysomnography, and CPAP adherence data at clinic visits. RESULTS: Forty-six subjects met criteria for inclusion. The mean ± SD age was 14.9 ± 6 y. The mean ± SD apnea-hypopnea index was 26.7 ± 30 events/h. Other than the addition of the RT intervention, all subjects continued to receive the same clinical services as before. Subjects were divided into 3 groups, based on baseline adherence: 0% use, use for 1–50% of nights, and use for > 50% of nights. There was a statistically significant improvement in PAP adherence in the subjects with baseline use of 0% and 1–50%, but no improvement in those with > 50% use at baseline. There was no significant change in PAP use at subsequent RT visits. CONCLUSIONS: Utilization at clinic visits of an RT trained in the use of PAP improved adherence in pediatric subjects with obstructive sleep-disordered breathing when their baseline PAP adherence was < 50%.

Objective: Children with cerebral palsy (CP) are commonly affected by obstructive sleep apnea (USA). This study examines the efficacy of combined surgical techniques for USA including tongue base suspension (TBS), using perioperative polysomnograms (PSG) in pediatric patients with CP. Study design: Case series with outcome analysis. Setting: University based tertiary care children's hospital.Methods: A 7-year retrospective chart review of children with CP who underwent surgical management for OSA. Surgical procedures, postoperative complications, and perioperative PSG data were examined. Only patients with both preoperative and postoperative PSG results were included in the study. Based upon procedures performed patients fell into 2 equal groups for analysis.Results: Fourteen children were identified. Seven patients (mean age = 6.0 years) underwent combined adenotonsillectomy (T&A), uvulopalatopharyngoplasty (UPPP), and tongue base suspension (TBS). Another 7 patients (mean age = 63 years) underwent T&A and UPPP alone. Those who received TBS had a mean preoperative AHI of 272 compared to 6.8 in the group that did not have TBS. The AHI decreased by a mean of 16.5 in the TBS group and 5.0 in the non-TBS group. The mean oxygen saturation nadir improved in both the TBS (74.0-84.0) and non-TBS (64.8-84.6) groups. The arousal index also improved in the TBS (33.1-20.7) and non-TBS (11.0-5.8) groups. No surgical complications occurred.Conclusion: This study suggests that concomitant surgical approaches for USA in children with CP are effective. Moderate to severe USA in this population may safely benefit from the added technique of tongue base suspension. Published by Elsevier Ireland Ltd.

Objectives:Prematurity is thought to be to be an independent risk factor for supraglottoplasty (SGP) failure. This study aimed to compare the outcomes of SGP in premature (<36 weeks) versus term infants.Methods:Retrospective analysis was performed on 325 patients undergoing SGP between 2004 and 2012. Patients >12 months age, syndromic, or with neurological or cardiac comorbidities were excluded. Resolution of airway symptoms after SGP was considered a success while revision SGP and tracheostomy were considered failures. The rates of secondary airway lesions (SAL), dysphagia, and gastrostomy tube (GT) placement were also compared.Results:A total of 178 infants (138 term, 40 premature) were identified for the analysis. SGP was successful in 91% (126/138) term and 90% (36/40) premature infants with no significant difference (P = 0.8) in success rates. Twelve term infants and 3 preterm infants required revision SGP. One premature infant required tracheostomy. Incidence of SAL was significantly higher in premature (72.5%) compared to term infants (34.97%) (P = 0.0002). Intubation rates were significantly higher in premature infants. This did not affect the higher SAL in premature infants compared to term infants. Dysphagia rates were also higher in premature (13/40, 32.5%) versus term infants (10/138, 7.25%, P < 0.001) and lasted longer on follow‐up. The rate of GT insertion was greater in premature (27.5%) compared to term infants (7.25%) (P = 0.003).Conclusions:Supraglottoplasty outcomes in term and premature infants were similar. However, premature infants experience significantly more SAL and dysphagia.

PURPOSE:Prematurity is thought to be to be an independent risk factor for supraglottoplasty (SGP) failure. The purpose of this study was to compare the outcomes of supraglottoplasty in premature infants with congenital laryngomalacia (LM) with that of term infants.STUDY DESIGN:Case series with chart review.SETTING:Tertiary-care pediatric institution.METHODS:Analysis was performed on 325 consecutive patients undergoing SGP between 2004 and 2012. Patients older than 12 months age or with syndrome, neurologic, or cardiac comorbidities were excluded. Resolution of airway symptoms after SGP was considered successful while revision SGP and tracheostomy were considered failures. The rates of secondary airway lesions (SAL), dysphagia, and gastrostomy tube (GT) placement were also compared.RESULTS:A total of 176 infants (136 term, 40 preterm) were identified. SGP was successful in 92.7% term and 90% preterm infants with no significant difference (P = .5865). Incidence of revision SGP and tracheostomy was similar among the groups. The outcomes of SGP were the same in all groups stratified by age. Incidence of SAL was significantly higher in preterm (72.5%) compared to term infants (34.6%, P = .0002). Dysphagia rates in the follow-up were higher in preterm (32.5%) versus term infants (6.6%, P < .001). The rate of GT insertion was significantly greater (P = .003) in preterm (27.5%) than term infants (6.6%). The preoperative dysphagia, persistent dysphagia, and GT in follow-up was significantly higher when gestation age <32 weeks (25%, P = .0168).CONCLUSION:Supraglottoplasty outcomes in term and preterm infants were similar irrespective of gestation age. Higher rates of dysphagia in follow-up are seen when gestation age <32 weeks.

Objectives:Synchronous airway anomalies below the supraglottis are thought to compound the symptoms of laryngomalacia (LM). Upper aerodigestive obstructive lesions may also be present but have not been discussed. This study examines other sources of upper airway obstruction in infants with severe LM requiring supraglottoplasty (SGP).Methods:Data were compiled on infants who received SGP between 2004 and 2011. Those with neurological conditions, syndromes, or major comorbidities were excluded. The incidence, type, and timing of procedures to relieve upper airway obstruction above the larynx were examined in each infant within 4 years after SGP.Results:One hundred fifty‐six infants were included. Forty‐two (26.9%) had other sources of upper airway obstruction necessitating a surgical procedure beyond SGP. Thirty‐two patients required an adenoidectomy alone, either during or within 30 months of SGP. Nine patients required adenotonsillectomy. One patient required tonsillectomy alone. Among the patients who underwent adenoidectomy, tonsillectomy was required later in 6 patients. Age at the time of SGP significantly affected the incidence of additional procedures. Infants receiving SGP at an age greater than 6 months were more likely to require management of adenotonsillar hypertrophy (82.1%) than their younger counterparts (14.8%) (P < 0.05).Conclusions:Accessory upper airway obstruction due to adenoidal or adenotonsillar hypertrophy, both synchronous and non‐synchronous (occurring after) is more common in patients with severe laryngomalacia. Infants older than 6 months receiving SGP are more likely to require additional procedures to relieve upper airway symptoms. Management of accessory sources of upper airway obstruction should be explored in infants with laryngomalacia.