Development of an interdisciplinary microtia-atresia care model: A single-center 20-year experience.

Objectives: Microtia and aural atresia are congenital ear anomalies with a wide-ranging spectrum of phenotypes and varied functional and psychosocial consequences for patients. This study seeks to analyze the management of microtia-atresia patients at our center over a 20-year period and to propose recommendations for advancing microtia-atresia care at a national level. Methods: We performed a retrospective analysis of data from patients presenting to the Massachusetts Eye and Ear (Boston, MA) for initial otolaryngology consultation for congenital microtia and/or aural atresia between 1999 and 2018. Results: Over the 20-year study period, 229 patients presented to our microtia-atresia center at a median age of 7 years. The severity of microtia was most commonly classified as grade III (n = 87, 38%), 61% (n = 140) of patients had complete atresia, the median Jahrsdoerfer grading scale score was 6 (range 0-10), and 81 patients (35%) underwent surgery for microtia repair. For hearing rehabilitation, 30 patients (64%) underwent bone conduction device implantation and 17 patients (36%) underwent atresiaplasty. The implementation of an interdisciplinary, longitudinal care model resulted in an increase in patient (r = 0.819, p < .001) and surgical volume (microtia surgeries, r = 0.521, p = .019; otologic surgeries, r = 0.767, p < .001) at our center over time. Conclusion: An interdisciplinary team approach to microtia-atresia patient care may result in increased patient volume, which could improve aesthetic and hearing outcomes over time by concentrating care and surgical expertise. Future work should aim to establish standardized clinical consensus recommendations to guide the creation of high-quality microtia-atresia care programs. Level of Evidence: 4.

Congenital Cytomegalovirus Targeted Screening Implementation and Outcomes: A Retrospective Chart Review.

OBJECTIVE: To assess the effectiveness and outcomes of a targeted cytomegalovirus (CMV) testing protocol. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care institution. METHODS: Targeted screening for CMV in children who did not pass the newborn hearing screening (NHS) was introduced in July 2015 for the level 2 and 3 nurseries at our hospital. In January 2016, this policy was extended to include all nurseries. Retrospective chart review was performed for all newborns who did not pass their NHS between 2013 and 2020. CMV testing rates and related outcomes were compared before and after rollout. RESULTS: A total of 891 charts were reviewed for infants who did not pass their NHS: 530 (60%) had CMV testing, of which 8 (1.5%) tested positive. Three cases were detected prior to and 5 occurred after initiation of targeted screening. Six CMV+ infants demonstrated hearing loss on confirmatory auditory brainstem response, all of whom began treatment with oral valganciclovir. Hearing remained stable in 3 patients, progressed in 2, and improved in 1. The rate of CMV testing in children who did not pass their NHS increased from 14% to 88% after full implementation of targeted screening (P < .001). The average age at initial infectious disease consultation was significantly younger for infants born after targeted screening (P < .001). CONCLUSION: Targeted screening is a feasible and effective method to identify CMV+ infants early in life. Implementation of a targeted screening program for CMV in children who do not pass the NHS resulted in significantly higher rates of CMV testing and earlier referral to infectious disease.

Hearing Aid Use in Children With Unilateral Hearing Loss: A Randomized Crossover Clinical Trial.

OBJECTIVES/HYPOTHESIS: In children with mild to moderately severe unilateral hearing loss (UHL), assess whether subject-reported quality of life (QOL) and teacher- and parent-reported perception of listening difficulty are affected by use of a hearing aid (HA) with baseline accommodations, compared to children receiving only baseline accommodations. STUDY DESIGN: Randomized crossover clinical trial. METHODS: Thirty-seven children 6-12 years of age with mild to moderately severe UHL and ≥80% word recognition scores in the poorer hearing ear were randomized into arm 1, using baseline accommodations (frequency-modulated system and strategic seating) for 12 weeks, followed by addition of a HA for 12 weeks. The other participants were randomized into the reverse methodology: arm 2, using a HA in addition to baseline accommodations for 12 weeks, followed by baseline accommodations alone. Surveys of QOL (Hearing Environments and Reflection on Quality of Life) and listening difficulties or challenges with hearing amplification (CHILD and LIFE-R questionnaires) were administered at 6-week intervals. Differences in mean survey scores, percent change, and improvement over time were computed between the two arms and inter-arm intervals. Per-protocol analysis was used. RESULTS: Of the 37 children enrolled, 34 children underwent the study interventions and were included in the analysis, (arm 1 = 20, arm 2 = 14) (mean [standard deviation] age = 8 [1.5] years; 21 boys [61.8%]). Survey scores averaged across both arms during the HA interval (77.79 [15.13]) were significantly higher than during the baseline-only interval (69.67 [14.69], P = .036). There was no significant difference between trial arms in mean scores between the two HA intervals (P = .450) and two baseline-only intervals (P = .539). CONCLUSIONS: Hearing-related QOL and listening ability improved in children who met eligibility criteria with mild to moderately severe UHL with HA use compared with baseline accommodations alone. TRIAL REGISTRATION: ClinicalTrials.gov identifier: NCT02269124. LEVEL OF EVIDENCE: 1 Laryngoscope, 132:881-888, 2022.

Otoendoscopes to Enhance Telemedicine During the COVID-19 Pandemic.

OBJECTIVES: As telemedicine has become increasingly utilized during the COVID-19 pandemic, portable otoendoscopy offers a method to perform an ear examination at home. The objective of this pilot study was to assess the quality of otoendoscopic images obtained by non-medical individuals and to determine the effect of a simple training protocol on image quality. METHODS: Non-medical participants were recruited and asked to capture images of the tympanic membrane before and after completion of a training module, as well as complete a survey about their experience using the otoendoscope. Images were de-identified, randomized, and evaluated by 6 otolaryngologists who were blinded as to whether training had been performed prior to the image capture. Images were rated using a 5-point Likert scale. RESULTS: Completion of a training module resulted in a significantly higher percentage of tympanic membrane visible on otoendoscopic images, as well as increased physician confidence in identifying middle ear effusion/infection, cholesteatoma, and deferring an in-person otoscopy (P < .0001). However, even with improved image quality, in most cases, physicians reported that they would not feel comfortable using the images to for diagnosis or to defer an in-person examination. Most participants reported that the otoendoscope was simple to use and that they would feel comfortable paying for the device. CONCLUSIONS: At-home otoendoscopes can offer a sufficient view of the tympanic membrane in select cases. The use of a simple training tool can significantly improve image quality, though often not enough to replace an in-person otoscopic exam.

Cochlear Implantation in Children With Single-Sided Deafness: A Systematic Review and Meta-analysis.

Importance: In 2019, the US Food and Drug Administration approved cochlear implantation for children with single-sided deafness (SSD). The absence of robust clinical data specific to pediatric patients to guide shared decision-making and to identify potential advantages is a challenge in family counseling. Objective: To evaluate the audiological and patient-reported outcomes in children who underwent cochlear implantation for SSD and to assess the association between time of implantation, subjective outcomes, and cochlear implant device use rates. Data Source: MEDLINE, Embase, Scopus, Cochrane, and PubMed were searched for English-language articles that were published in a peer-reviewed journal from database inception to February 18, 2020. Study Selection: Inclusion criteria were designed to capture studies that evaluated pediatric patients (1) younger than 18 years, (2) with a diagnosis of SSD for which they underwent a cochlear implantation, and (3) with at least 1 outcome of interest measured numerically: speech perception, sound localization, device use, and patient-reported outcomes. Of the 526 articles reviewed, 12 (2.3%) met the selection criteria. Data Extraction and Synthesis: The Meta-analyses Of Observational Studies in Epidemiology (MOOSE) reporting guidelines were followed. Data were pooled using fixed-effect and random-effect models. The following information was obtained from each article: study characteristics, patient characteristics, hearing loss and intervention characteristics, and outcomes. Main Outcomes and Measures: Outcomes were (1) postoperative changes in speech perception (in quiet was measured as a proportion of correct responses, and in noise was measured as decibel signal to noise ratio for speech reception threshold) and sound localization (measured in degree of localization error), (2) patient-reported audiological outcomes (measured by the speech, spatial, and qualities of hearing scale), and (3) device use rates among children who received cochlear implantation for SSD. Results: Twelve observational studies that evaluated 119 children (mean [SD] age, 6.6 [4.0] years) with SSD who received a cochlear implant were included. Most children showed clinically meaningful improvement in speech perception in noise (39 of 49 children [79.6%]) and in quiet (34 of 42 children [81.0%]). Long duration of deafness (>4 years in congenital SSD and >7 years in perilingual SSD) was the most commonly proposed reason for lack of improvement. Sound localization as measured by degrees of error from true location (mean difference [MD], -24.78°; 95% CI, -34.16° to -15.40°; I2 = 10%) improved statistically significantly after cochlear implantation. Patients with acquired SSD and shorter duration of deafness compared with those with congenital SSD reported greater improvements in speech (MD, 2.27; 95% CI, 1.89-2.65 vs 1.58; 95% CI, 1.00-2.16) and spatial (MD, 2.95; 95% CI, 2.66-3.24 vs 1.68; 95% CI, 0.96-2.39) hearing qualities. The duration of deafness among device nonusers was statistically significantly longer than the duration of deafness among regular device users (median difference, 6.84; 95% CI, 4.02-9.58). Conclusions and Relevance: This systematic review and meta-analysis found that cochlear implantation for children with SSD was associated with clinically meaningful improvements in audiological and patient-reported outcomes; shorter duration of deafness may lead to better outcomes. These findings can guide future research efforts, refine cochlear implantation candidacy criteria, and aid in family counseling and shared decision-making.

Systematic Review of Endoscopic Ear Surgery Outcomes for Pediatric Cholesteatoma.

OBJECTIVE: Endoscopic ear surgery (EES) has been increasingly adopted to enhance visualization during otologic surgery including pediatric cholesteatoma resection. The purpose of this study is to systematically review the current literature reporting rates of residual cholesteatoma to evaluate outcomes of EES in the pediatric population. DATA SOURCES: PubMed, Embase, Cochrane. STUDY SELECTION: Using the Preferred Reporting Items for Systematic Reviews and Meta-Analysis recommendations (PRISMA), we systematically reviewed studies that reported outcomes for cholesteatoma surgery in children using the endoscope. Studies were included if they described the use of the endoscope for middle ear surgery and measured outcomes following cholesteatoma resection in pediatric patients. DATA EXTRACTION: Identified articles were assessed for study design, sample size, inclusion of pediatric population, the presence of a comparison group, outcomes measured such as: residual disease rate, recurrent cholesteatoma, audiologic outcome, quality of life measures, and complications. DATA SYNTHESIS: Fourteen articles in the literature discussed cholesteatoma outcomes in children when the endoscope was employed. Residual disease rate ranged from 0 to 22% when endoscope was used for dissection compared with 11 to 55% when endoscope was used for inspection or not at all. A meta-analysis was conducted on 11 studies. There was a significant risk reduction in residual disease rates for endoscopic dissection when compared with microscope (RR = 0.48, 95% CI = 0.37-0.63, p < 0.001). CONCLUSION: Among the studies included in this review, EES was associated with lower rates of residual cholesteatoma compared with traditional microscopic techniques. Additional prospective studies are needed to further assess these findings.

Assessment of Pain and Analgesic Use in Children Following Otologic Surgery.

OBJECTIVE: To compare postoperative pain and analgesic use in children following transcanal endoscopic ear surgery (TEES) vs non-transcanal endoscopic ear surgery (non-TEES). STUDY DESIGN: Prospective case series. SETTING: Tertiary care center. METHODS: Surveys using the Wong-Baker FACES Pain Rating Scale and recording the frequency and dosage of consumed analgesics were administered prospectively to caregivers of children undergoing otologic surgery between May 2018 to February 2020. Pain intensity and medication use were recorded twice daily for 6 days, starting on postoperative day 0. Mean pain scores and mean number of consumed analgesic doses were compared between groups. RESULTS: Survey response rate was 57.9%. Among 53 patients who completed the survey, 35 (66.0%) underwent TEES and 18 (34.0%) underwent non-TEES. Mean pain ratings on postoperative days 0 and 1 were significantly lower among children undergoing TEES (2.2 and 2.1) vs non-TEES (4.0 and 4.1), P = .045 and P = .008, respectively (Mann-Whitney U test). The mean pain ratings across the 6 days were similar in TEES (1.7) and non-TEES (2.6) (P = .140, Mann-Whitney U test). The mean number of analgesic doses consumed per half-day over the 6 days was significantly lower among children undergoing TEES (0.3) vs non-TEES (0.6; P = .049, Mann-Whitney U test). CONCLUSION: Postoperative pain following TEES and non-TEES in children was overall low. Children undergoing TEES had a small but statistically significant decrease in pain on postoperative days 0 and 1 and decreased use of pain medications compared to non-TEES.

Audiologic testing in children with Down Syndrome: Are current guidelines optimal?

INTRODUCTION: Down Syndrome (DS) is a Tier 1 risk factor for hearing loss. Guidelines exist to ensure close monitoring of children with DS for hearing loss. It is important to consider the timing of testing in order to obtain meaningful audiologic data in this high-risk population. The purpose of this study is to present hearing outcomes for children with DS during the first 8 years of life and to assess these outcomes in the context of current screening guidelines. METHODS: Retrospective review of audiometric outcomes was conducted for children with DS age 8 or younger who presented to a multidisciplinary DS clinic between January 2014 to June 2017. Age at the time of testing, as well as test success rate and hearing loss type and severity were noted. RESULTS: 131 patients were included in the study, 52% of which were male. 36% of the patients failed their newborn hearing screening and only 9% of those subjects had normal hearing on subsequent testing. Most hearing loss identified was mild and conductive in nature. Inconclusive results were most likely to be obtained at 6-10 months of age. CONCLUSION: Hearing loss is common among children with DS. To optimize the quality of testing and avoid the need for sedation in followup testing, routine follow-up hearing screening should be performed either before 6 months of age or after 10 months of age.

Quality of Life in Children with Sensorineural Hearing Loss.

OBJECTIVE: To assess quality of life (QOL) in pediatric patients with sensorineural hearing loss (SNHL) with the Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0) and the Hearing Environments and Reflection on Quality of Life 26 (HEAR-QL-26) and HEAR-QL-28 surveys. STUDY DESIGN: Prospective longitudinal study. SETTING: Tertiary care center. SUBJECTS AND METHODS: Surveys were administered to patients with SNHL (ages 2-18 years) from July 2016 to December 2018 at a multidisciplinary hearing loss clinic. Patients aged >7 years completed the HEAR-QL-26, HEAR-QL-28, and PedsQL 4.0 self-report tool, while parents completed the PedsQL 4.0 parent proxy report for children aged ≤7 years. Previously published data from children with normal hearing were used for controls. The independent t test was used for analysis. RESULTS: In our cohort of 100 patients, the mean age was 7.7 years (SD, 4.5): 62 participants had bilateral SNHL; 63 had mild to moderate SNHL; and 37 had severe to profound SNHL. Sixty-eight patients used a hearing device. Mean (SD) total survey scores for the PedsQL 4.0 (ages 2-7 and 8-18 years), HEAR-QL-26 (ages 7-12 years), and HEAR-QL-28 (ages 13-18 years) were 83.9 (14.0), 79.2 (11.1), 81.2 (9.8), and 77.5 (11.3), respectively. Mean QOL scores for patients with SNHL were significantly lower than those for controls on the basis of previously published normative data (P < .0001). There was no significant difference in QOL between children with unilateral and bilateral SNHL or between children with SNHL who did and did not require a hearing device. Low statistical power due to small subgroup sizes limited our analysis. CONCLUSION: It is feasible to collect QOL data from children with SNHL in a hearing loss clinic. Children with SNHL had significantly lower scores on validated QOL instruments when compared with peers with normal hearing.

Pediatric Endoscopic Ossiculoplasty Following Surgery for Chronic Ear Disease.

OBJECTIVE: Rigid endoscopes can improve visualization of the tympanic space compared to traditional microscopic techniques. This study investigates whether use of transcanal endoscopic ossiculoplasty influences audiologic outcomes compared to microscopic ossiculoplasty following chronic ear surgery in children. STUDY DESIGN: Comparative cohort study at two tertiary care centers. METHODS: Retrospective review of pediatric chronic ear cases where ossiculoplasty was performed from February 2009 to March 2018. RESULTS: We identified 100 ears that underwent endoscopic ossiculoplasty and 100 ears that underwent microscopic ossiculoplasty. The mean age was 11 years (range, 4-18 years) with 63% males. There were no significant differences in these parameters between the two groups. Subjects underwent either primary ossiculoplasty or ossiculoplasty during second-look procedures. There was no significant difference in air conduction pure tone average (PTA) after microscopic cases compared to endoscopic cases (-12.5 dB vs. -10.5 dB, P = .40). These results were independent of prosthesis type. Microscopic ossiculoplasty was significantly more likely to use a post-auricular approach (P = .0001). There was no difference in complication rate between the two groups. The malleus was more likely to be absent or removed prior to endoscopic ossiculoplasty (P = .0004) with no significant difference in the change in PTA between groups. CONCLUSIONS: Transcanal endoscopic ossiculoplasty was found to have equivalent audiometric outcomes with significantly fewer post-auricular approaches and no increase in complications compared to microscopic ossiculoplasty. While the malleus was more likely to be absent in endoscopic cases, this did not appear to influence the change in PTA. LEVEL OF EVIDENCE: 4 Laryngoscope, 2020.

Utilization of diagnostic testing for pediatric sensorineural hearing loss.

BACKGROUND/OBJECTIVE: Sensorineural hearing loss is a common diagnosis among children. The diagnostic workup varies widely among practitioners. This study's aim was to assess the utilization of diagnostic testing for SNHL and determine the yield of each test. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care center. SUBJECTS: 827 patients with a diagnosis of SNHL from January 1, 2011 to January 1, 2015. RESULTS: 746 patients met inclusion criteria. Temporal bone imaging was performed on 561 (75%) of patients with 224 (40%) having positive results that explained the etiology of the SNHL. Congenital SNHL was more likely to be associated with abnormal imaging than acquired SNHL (109/299 versus 106/316 respectively) (p = 0.001). Unilateral SNHL was more likely to be associated imaging abnormalities than bilateral SNHL (101/221 and 123/340 respectively) (p = 0.028). Genetic testing was performed on 244 (33%) patients, of which 94 (39%) had abnormalities. Positive genetics results were more common with bilateral than unilateral SNHL (82/191 and 12/53 respectively) (p = 0.007). There was no statistically significant difference in the utility of genetic testing for congenital and acquired SNHL (p = 0.0836). Cytomegalovirus (CMV) testing was available for 104 (14%) of patients with 13 (12.5%) being positive and consistent with congenital CMV. Electrocardiogram, urinalysis, and Lyme titers were less useful. CONCLUSIONS: Imaging and genetic testing had the highest yield in the evaluation of children with SNHL and were the most commonly performed. CMV testing was valuable in neonates who failed newborn hearing screening.