International Pediatric Otolaryngology Group (IPOG): Juvenile-onset recurrent respiratory papillomatosis consensus recommendations.

OBJECTIVES: To develop consensus recommendations for the evaluation and management of juvenile-onset recurrent respiratory papillomatosis (JORRP) in pediatric patients. METHODS: Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). The mission of the IPOG is to develop expertise-based consensus recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care. The consensus recommendations herein represent the first publication by the group. RESULTS: Consensus recommendations including diagnostic considerations, surgical management, systemic adjuvant therapies, postoperative management, surveillance, and voice evaluation. These recommendations are based on the collective opinion of the IPOG members and are targeted for otolaryngologists, primary care providers, pulmonologists, infectious disease specialists, and any other health care providers that manage patients with JORRP. CONCLUSIONS: Pediatric JORRP consensus recommendations are aimed at improving care and outcomes in this patient population.

Design and Implementation of an Interactive Website for Pediatric Voice Therapy-The Concept of In-Between Care: A Telehealth Model.

Purpose: This article describes the design and implementation of a web-based portal developed to provide supported home practice between weekly voice therapy sessions delivered through telehealth to children with voice disorders. This in-between care consisted of supported home practice that was remotely monitored by speech-language pathologists (SLPs). Methods: A web-based voice therapy portal (VTP) was developed as a platform so participants could complete voice therapy home practice by an interdisciplinary team of SLPs (specialized in pediatric voice therapy), telehealth specialists, biomedical informaticians, and interface designers. The VTP was subsequently field tested in a group of children with voice disorders, participating in a larger telehealth study. Results: Building the VTP for supported home practice for pediatric voice therapy was challenging, but successful. Key interactive features of the final site included 11 vocal hygiene questions, traditional voice therapy exercises grouped into levels, audio/visual voice therapy demonstrations, a store-and-retrieval system for voice samples, message/chat function, written guidelines for weekly therapy exercises, and questionnaires for parents to complete after each therapy session. Ten participants (9-14 years of age) diagnosed with a voice disorder were enrolled for eight weekly telehealth voice therapy sessions with follow-up in-between care provided using the VTP. Conclusion: The development and implementation of the VTP as a novel platform for the delivery of voice therapy home practice sessions were effective. We found that a versatile individual, who can work with all project staff (speak the language of both SLPs and information technologists), is essential to the development process. Once the website was established, participants and SLPs effectively utilized the web-based VTP. They found it feasible and useful for needed in-between care and reinforcement of therapeutic exercises.

Structure and Functions of Pediatric Aerodigestive Programs: A Consensus Statement.

Aerodigestive programs provide coordinated interdisciplinary care to pediatric patients with complex congenital or acquired conditions affecting breathing, swallowing, and growth. Although there has been a proliferation of programs, as well as national meetings, interest groups and early research activity, there is, as of yet, no consensus definition of an aerodigestive patient, standardized structure, and functions of an aerodigestive program or a blueprint for research prioritization. The Delphi method was used by a multidisciplinary and multi-institutional panel of aerodigestive providers to obtain consensus on 4 broad content areas related to aerodigestive care: (1) definition of an aerodigestive patient, (2) essential construct and functions of an aerodigestive program, (3) identification of aerodigestive research priorities, and (4) evaluation and recognition of aerodigestive programs and future directions. After 3 iterations of survey, consensus was obtained by either a supermajority of 75% or stability in median ranking on 33 of 36 items. This included a standard definition of an aerodigestive patient, level of participation of specific pediatric disciplines in a program, essential components of the care cycle and functions of the program, feeding and swallowing assessment and therapy, procedural scope and volume, research priorities and outcome measures, certification, coding, and funding. We propose the first consensus definition of the aerodigestive care model with specific recommendations regarding associated personnel, infrastructure, research, and outcome measures. We hope that this may provide an initial framework to further standardize care, develop clinical guidelines, and improve outcomes for aerodigestive patients.

Significance of unilateral enlarged vestibular aqueduct.

OBJECTIVES/HYPOTHESIS: To describe the clinical phenotype of pediatric patients with unilateral enlarged vestibular aqueduct (EVA) and then to compare the findings to two clinically related phenotypes: bilateral EVA and unilateral hearing loss without EVA. In view of clinical observations and previously published data, we hypothesized that patients with unilateral EVA would have a much higher rate of contralateral hearing loss than patients with unilateral hearing loss without EVA. STUDY DESIGN: Retrospective cohort study. METHODS: Patients with unilateral or bilateral EVA were identified from a database of children with sensorineural hearing loss who were seen at a tertiary care institution between 1998 and 2010. Those with imaging findings consistent with well-established EVA criteria were identified. A comparative group of patients with unilateral hearing loss without EVA was also identified. The following specific outcome measurements were analyzed: 1) hearing loss phenotype, 2) laterality of EVA and hearing loss, 3) midpoint and operculum vestibular aqueduct measurements, and 4) genetic test results. RESULTS: Of the 144 patients who met our inclusion criteria, 74 (51.4%) had unilateral EVA. There was a strong correlation between the presence of hearing loss and ears with EVA. Fifty-five percent of patients with unilateral EVA had hearing loss in the contralateral ear; in most of these patients, the hearing loss was bilateral. Contralateral hearing loss occurred in only 6% of patients with unilateral hearing loss without EVA. No significant differences were found in temporal bone measurements between the ears of patients with unilateral EVA and ipsilateral hearing loss and all ears with EVA and normal hearing (P = .4). There was no difference in the rate of hearing loss progression in patients with unilateral EVA between ears with or without EVA (16 of 48 [33.3%] vs. 9 of 27 [33.3%], respectively; P = 1.0). There was no difference in the rate of hearing loss progression in patients with bilateral and unilateral EVA (41 of 89 ears [46.1%] vs. 25 of 75 ears [33.3%], respectively; P = .1); however, both EVA groups had higher rates of progression compared to patients with unilateral hearing loss without EVA. There was a strong correlation between the presence of hearing loss at 250 Hz and the risk of more severe hearing loss and progressive hearing loss. Patients with bilateral EVA and SLC26A4 mutations had a higher rate of progression than patients who had no mutations (P = .02). No patients with unilateral EVA had Pendred syndrome. CONCLUSIONS: Children with unilateral EVA have a significant risk of hearing loss progression. Hearing loss in the ear contralateral to the EVA is common, suggesting that unilateral EVA is a bilateral process despite an initial unilateral imaging finding. In contrast to bilateral EVA, unilateral EVA is not associated with Pendred syndrome and may have a different etiology. Temporal bone measurements, hearing loss severity, and hearing loss at 250 Hz were all correlated with the risk of progressive hearing loss. Clinicians should become knowledgeable regarding the implications of this disease process so that families can be counseled appropriately.

Psychological Functioning of Children and Adolescents With Eosinophil-Associated Gastrointestinal Disorders.

This study examined health-related quality of life and adjustment among children with eosinophil- associated gastrointestinal disorders (EGID) compared with an age-matched sample without acute or chronic illness. Participants were youth ages 2 to 18 years. Children and caregivers completed measures of psychological symptoms and health-related quality of life (HRQOL). Significant group differences were found for child report of depressive, as well as anxiety symptoms. Significant group differences were also found for caregiver report of psychological symptoms and social skills. Finally, based on parent and youth report, HRQOL and greater school absenteeism were associated with EGID diagnosis.

Perceptual evaluation of severe pediatric voice disorders: rater reliability using the consensus auditory perceptual evaluation of voice.

The purpose of this nonrandomized prospective study was to quantify the inter- and intrarater reliability of experienced speech-language pathologist's perceptual ratings of voice in pediatric patients post-laryngotracheal reconstruction (LTR). Moderate to severe dysphonia is common in this population. Using the sentence portion of the Consensus Auditory Perceptual Evaluation-Voice (CAPE-V) rating scale, three experienced speech-language pathologists independently rated randomized voice samples of 50 participants ages 4-20 years, who had acquired or congenital airway conditions requiring at least one LTR on the six salient perceptual vocal attributes. Data collection and listening conditions were carefully controlled. Seventeen (34%) of the samples were randomly selected for rerating at a later time. Estimates of interrater reliability were strongest for perceptual ratings of breathiness (intraclass correlation coefficient [ICC]=71%), roughness (ICC=68%), pitch (ICC=68%), and overall severity (ICC=67%). Reliability was lower for ratings of loudness (ICC=57%) and strain (ICC=35%). For each rater, the intrarater reliability on all but one parameter (strain) was moderate to strong (ICC=63-93%). There was a strong interrater eliability for four of six vocal parameters rated using the CAPE-V in a population of children and adolescents with marked dysphonia. The parameter of strain, when rated by auditory sample alone and apart from the clinical context, was difficult to rate.

Hearing loss progression and contralateral involvement in children with unilateral sensorineural hearing loss.

OBJECTIVES: We undertook this study to determine the rate of hearing loss progression in the affected ear of children with unilateral sensorineural hearing loss and without an enlarged vestibular aqueduct, and the rate of new-onset hearing loss in the contralateral ear. METHODS: We searched the database at our pediatric tertiary care center to identify patients who met the inclusion criteria, examining demographic variables, audiometric data, and presumptive causes. RESULTS: We identified 198 patients. At presentation, they showed slight left-sided and male predominances. Of 142 patients who had sufficient audiometric follow-up for us to evaluate progression, 21% showed ipsilateral progression and 10.6% developed new-onset hearing loss in the contralateral ear. Isolated high-frequency loss was identified in 11 patients (5.6%), 8 of whom had sufficient follow-up for us to identify progression. Two showed progression; 4 others with progression in the ipsilateral ear developed new-onset high-frequency loss in the contralateral ear. Temporal bone anomalies were identified in 26 children (13%), and these children were more likely to have profound hearing loss than were those without temporal bone anomalies (46% versus 23%). CONCLUSIONS: The findings suggest that unilateral sensorineural hearing loss may not always be a unilateral process, but that it may be the initial manifestation of bilateral auditory dysfunction.

Late-onset laryngomalacia: a variant of disease.

OBJECTIVE: To identify and describe the features of laryngomalacia (LM) in a cohort of older children, with the goal of providing an approach to diagnosis and management of these patients. Laryngomalacia is a common congenital disorder characterized by decreased laryngeal tone, supraglottic collapse, and stridor during inspiration and is rarely seen in older children. However, the presence of LM in this population may be obscured by related but uncommon clinical features. DESIGN: Prospective collection and retrospective evaluation of older children with evidence of LM from 1998 to 2005. SETTING: Two tertiary pediatric institutions. PATIENTS: Prospective data collection of 239 patients with LM, including 222 with congenital LM and 17 diagnosed as having LM when they were older than 2 years and without a medical history of prior disease or symptoms-late-onset LM (mean age at onset, 6.6 years). INTERVENTION: Supraglottoplasty. MAIN OUTCOME MEASURE: Symptom improvement. RESULTS: Patients with late-onset LM were classified into 3 categories according to their symptom complex; those presenting with feeding-disordered LM (n = 7; mean age at onset, 3.3 years), sleep-disordered LM (n = 7; mean age at onset, 6.3 years), and exercise-induced LM (n = 3; mean age at onset, 15 years). Stridor was rarely present except in patients with exercise-induced LM during strenuous activity. Profound arytenoid redundancy and prolapse was discovered in all patients during nasolaryngeal endoscopy. Typical anatomic features of congenital LM (shortened aryepiglottic folds or retroflexed epiglottis) were not discovered. No patient had a history of neuromuscular disease. Supra-arytenoid reduction (supraglottoplasty) led to clinical cure in all patients (mean duration of follow-up, 7.4 months). Prior adenotonsillectomies were performed in 5 patients with sleep-disordered LM. This did not improve symptoms, and these patients displayed evidence of LM as the source of obstruction. CONCLUSIONS: Physicians should consider late-onset LM as a potential cause of feeding difficulties in toddlers, sleep apnea in children, and exercise intolerance in teenagers. As in infants with LM, supraglottoplasty improves late-onset disease.