Results and patient satisfaction from an early access infant hearing detection clinic.

INTRODUCTION: National recommendations in the United States specify that all infants with hearing impairment should be identified by 3 months of age. Infants who fail universal newborn hearing screening (UNHS) require follow up testing after hospital discharge. Follow up testing may be difficult to obtain in some communities within the ideal time frame. A rapid access multidisciplinary clinic was established for failed UNHS. The objective of this study is to report outcomes and patient satisfaction from an early access hearing detection clinic. METHODS: Infants that failed UNHS were seen in the multidisciplinary clinic between 1/1/19 and 2/28/22. Patients underwent automated auditory brainstem response (ABR) and distortion product otoacoustic emissions testing and consulted with an otolaryngology nurse practitioner. Failed results were followed by diagnostic ABR. Surveys were administered at the beginning and end of the appointment. RESULTS: In total, 169 infants were seen at a mean age of 8.4 weeks (95%CI 7.5, 9.4). Repeat testing was abnormal in 38 (22.4%). Diagnostic ABR was performed at an average age of 13.7 weeks (n = 34, 95% CI: 10.8, 16.6) and led to a diagnosis of hearing loss in 18 infants. Twenty-seven parents completed surveys at the initial visit. Anxiety level among patients with normal repeat testing (n = 20) decreased from 1.9 to 1.2 (p = .002), while anxiety level among those with abnormal repeat testing (n = 7) was not statistically different before and after (2.1 vs 2.7, p = .2). Satisfaction level was 3.7 ± 0.7 (scored 1-4). All parents reported having a better understanding of their child's hearing problem after the visit. DISCUSSION: This novel nurse practitioner-led early hearing detection clinic enabled timely diagnosis of hearing loss and reassurance to families without hearing loss. Age at hearing loss diagnosis compares favorably to published cohorts.

Detection and Quantification of Calcitonin Gene-Related Peptide (CGRP) in Human Plasma Using a Modified Enzyme-Linked Immunosorbent Assay.

Calcitonin gene-related peptide (CGRP) is a vasoactive neuropeptide that plays a putative role in the pathophysiology of migraine headaches and may be a candidate for biomarker status. CGRP is released from neuronal fibers upon activation and induces sterile neurogenic inflammation and arterial vasodilation in the vasculature that receives trigeminal efferent innervation. The presence of CGRP in the peripheral vasculature has spurred investigations to detect and quantify this neuropeptide in human plasma using proteomic assays, such as the enzyme-linked immunosorbent assay (ELISA). However, its half-life of 6.9 min and the variability in technical details of assay protocols, which are often not fully described, have yielded inconsistent CGRP ELISA data in the literature. Here, a modified ELISA protocol for the purification and quantification of CGRP in human plasma is presented. The procedural steps involve sample collection and preparation, extraction using a polar sorbent as a means of purification, additional steps to block non-specific binding, and quantification via ELISA. Further, the protocol has been validated with spike and recovery and linearity of dilution experiments. This validated protocol can theoretically be used to quantify CGRP concentrations in the plasma of individuals not only with migraine, but also with other diseases in which CGRP may play a role.

Trends in Timing and Provision of Pediatric Cochlear Implant Care During COVID-19.

Objectives: To identify trends in timing of pediatric cochlear implant (CI) care during COVID-19. Study Design: Retrospective cohort. Setting: Tertiary care center. Methods: Patients under 18 years of age who underwent CI between 1/1/2016 and 2/29/2020 were included in the pre-COVID-19 group, and patients implanted between 3/1/2020 and 12/31/2021 comprised the COVID-19 group. Revision and sequential surgeries were excluded. Time intervals between care milestones including severe-to-profound hearing loss diagnosis, initial CI candidacy evaluation, and surgery were compared among groups, as were the number and type of postoperative visits. Results: A total of 98 patients met criteria; 70 were implanted pre-COVID-19 and 28 during COVID-19. A significant increase in the interval between CI candidacy evaluation and surgery was seen among patients with prelingual deafness during COVID-19 compared with pre-COVID-19 (µ = 47.3 weeks, 95% confidence interval [CI]: 34.8-59.9 vs µ = 20.5 weeks, 95% CI: 13.1-27.9; p < .001). Patients in the COVID-19 group attended fewer in-person rehabilitation visits in the 12 months after surgery (µ = 14.9 visits, 95% CI: 9.7-20.1 vs µ = 20.9, 95% CI: 18.1-23.7; p = .04). Average age at implantation in the COVID-19 group was 5.7 years (95% CI: 4.0-7.5) versus 3.7 years in the pre-COVID-19 group (95% CI: 2.9-4.6; p = .05). The time interval between hearing loss confirmation and CI surgery was on average 99.7 weeks for patients implanted during COVID-19 (95% CI: 48.8-150) versus 54.2 weeks for patients implanted pre-COVID (95% CI: 39.6-68.8), which was not a statistically significant difference (p = .1). Conclusion: During the COVID-19 pandemic patients with prelingual deafness experienced delays in care relative to patients implanted before the pandemic.

Evaluation of Parental Perspectives and Concerns about Pediatric Cochlear Implantation: A Social Media Analysis.

OBJECTIVE: Parents often experience uncertainty during decision-making for their child's cochlear implantation (CI) surgery, and online forums provide insight on parental opinions that might not be expressed in clinic. This study aims to evaluate parental perspectives and concerns about pediatric CI using social media analysis. STUDY DESIGN: Qualitative study. SETTING: Three online forums involving parental posts about pediatric CI. INTERVENTION/METHODS: Forums were queried using keywords (e.g., "cochlear implant") to gather all U.S. parent-initiated posts about pediatric CI from 2006 to 2021. Thematic content analysis was performed to classify posts by overarching domain, themes, and subthemes. Posts were reviewed for thematic synthesis and double coded. Descriptive statistics were calculated for each theme by unique users. RESULTS: A total of 79 posts by 41 unique users were analyzed. Themes relating to decision for CI included facilitators, inhibitors, resources, and feelings. Parents posted about lack of benefit from hearing aids promoting decision for CI and high cost as an inhibitor. Some expressed concern about making a major decision for a minor. Parents often mentioned their child's healthcare providers and social media as resources. CONCLUSION: Through social media posts, parental priorities and concerns for decision-making of CI surgery were identified. Findings may guide discussions between physicians and parents and facilitate shared decision-making about CI.

Delays in Infant Hearing Detection and Intervention During the COVID-19 Pandemic: Commentary.

Early detection and intervention for congenital hearing loss are critical for speech and language development. Newborns should receive hearing screening, diagnosis, and intervention by 1, 3, and 6 months, respectively. The COVID-19 pandemic has caused delays in each step of this process. Increased out-of-hospital births and shortages of essential health care services likely reduced the proportion of newborns completing screening. Additional factors have contributed to delayed diagnosis. We estimate that up to 50% of infants born with hearing loss in Maryland in 2021 may be delayed in diagnosis. Hearing loss interventions have been affected due to delayed initiation, reduced availability, and lack of in-person services. Delayed diagnosis and treatment of congenital hearing loss are likely to have significant effects on individual patients and public health, the full magnitude of which will not be known for years. Opportunities exist for providers to mitigate the negative effects of COVID-19 on pediatric hearing health care.

Cochlear Implantation Can Improve Auditory Skills, Language and Social Engagement of Children With Autism Spectrum Disorder.

OBJECTIVE: To review outcomes of cochlear implantation (CI) in children diagnosed with autism spectrum disorder (ASD). STUDY DESIGN: Retrospective case review and parent survey. SETTING: Tertiary care children's hospital. PATIENTS: Thirty children with ASD who underwent CI between 1991 and 2018. Mean age at CI = 3.5 years (0.8-11.8), mean age at diagnosis of ASD = 5.1 years (2.0-15.0) (22/30 diagnosed after CI), mean follow-up = 10.5 years (1.4-21.6). Parents of 7 children returned a survey. INTERVENTION: Unilateral or bilateral cochlear implantation. MAIN OUTCOME MEASURES: Speech perception; expressive communication mode; educational placement; social engagement; consistency of CI use; parent survey of child behavior change. RESULTS: Thirty-three percent of all and 45% of the 22 consistent device users developed measurable open-set speech perception by an average of 4.5 years of device use. Educational placement at last follow-up included 13% mainstreamed without interpreter, 50% Special Education programs, 10% therapeutic residential or day programs, 23% total communication programs, and one home schooled. Spoken language alone was used by 31% and spoken plus sign by 14%, with the remainder using sign alone, augmentative communication devices or no mode of communication. By parent report, 86% showed improvement in social engagement compared to pre-CI. Survey results showed the behaviors most frequently ranked as most affected by CI were communication and attention, while awareness of environment had the lowest (most affected) mean ranking. CONCLUSIONS: Findings support a growing body of literature that cochlear implantation has the potential to improve auditory skills, language, and enhance social engagement in some deaf children with autism spectrum disorder.

Transcanal Endoscopic Ear Surgery for Congenital Cholesteatoma: A Multi-institutional Series.

OBJECTIVE: To assess outcomes of transcanal endoscopic ear surgery (TEES) for congenital cholesteatoma. STUDY DESIGN: Case series with chart review of children who underwent TEES for congenital cholesteatoma over a 10-year period. SETTING: Three tertiary referral centers. METHODS: Cholesteatoma extent was classified according to Potsic stage; cases with mastoid extension (Potsic IV) were excluded. Disease characteristics, surgical approach, and outcomes were compared among stages. Outcomes measures included residual or recurrent cholesteatoma and audiometric data. RESULTS: Sixty-five cases of congenital cholesteatoma were included. The mean age was 6.5 years (range, 1.2-16), and the mean follow-up was 3.9 years (range, 0.75-9.1). There were 19 cases (29%) of Potsic stage I disease, 10 (15%) stage II, and 36 (55%) stage III. Overall, 24 (37%) patients underwent a second-stage procedure, including 1 with Potsic stage II disease (10%) and 21 (58%) with Potsic stage III disease. Eight cases (12%) of residual cholesteatoma occurred. One patient (2%) developed retraction-type ("recurrent") cholesteatoma. Recidivism occurred only among Potsic stage III cases. Postoperative air conduction hearing thresholds were normal (<25 dB HL) in 93% of Potsic stage I, 88% of stage II, and 36% of stage III cases. CONCLUSION: TEES is feasible and effective for removal of congenital cholesteatoma not extending into the mastoid. Recidivism rates were lower with the TEES approach in this large series than in previously reported studies. Advanced-stage disease was the primary risk factor for recidivism and worse hearing result. As minimally invasive TEES is possible in the youngest cases, children benefit from early identification and intervention.

Early Identification and Management of Congenital Cytomegalovirus.

Congenital cytomegalovirus (cCMV) is the most common nongenetic cause of sensorineural hearing loss. Despite its prevalence, universal screening for cCMV is not currently performed. Hearing loss caused by cCMV is most often severe to profound, often bilateral, and may be fluctuating or progressive. Infants with hearing loss at birth and confirmed cCMV might benefit from antiviral therapy. Roughly half of hearing loss cases owing to cCMV are delayed in onset, and consequently, these children pass newborn hearing screening. Children with cCMV require close audiologic monitoring, require appropriate management with hearing aids, and should be monitored for cochlear implant candidacy.

Congenital Cytomegalovirus Infection: Epidemiology, Timely Diagnosis, and Management.

Congenital cytomegalovirus (cCMV) infection is common because of the ubiquitous nature of the virus and the lack of an effective prevention strategy during pregnancy. Most infants with cCMV are asymptomatic, although a notable subset can have sequelae including, most commonly, sensorineural hearing loss and neurodevelopmental disability, which may not be present at birth. Timely screening for cytomegalovirus in the first weeks after birth is critical to appropriately diagnose congenital infection, evaluate affected infants, and determine the treatment course. Antiviral therapy with valganciclovir can optimize end hearing and neurodevelopmental outcomes in symptomatic infants. This review discusses the epidemiology and clinical manifestations of cCMV, targeted and universal screening approaches, and treatment and monitoring of infants with cCMV.

Prospective Determination of Airway Response to Upper Airway Stimulation: A New Opportunity for Advanced Device Titration.

OBJECTIVES/HYPOTHESIS: Response to upper airway stimulation (UAS) is associated with the degree of airway opening during stimulation. UAS programming may affect this opening. The objective of this study was to examine airway changes in response to five different electrode configurations programmable within the Inspire UAS system. STUDY DESIGN: Prospective single-arm cohort study. METHODS: Subjects who underwent UAS implantation were recruited for a prospective single-arm cohort study during UAS device activation. Functional thresholds were recorded for all settings. Awake nasopharyngoscopy was performed to examine the retropalatal (RP) and retroglossal (RG) regions at rest and during activation with all settings at their functional thresholds. Cross-sectional measurements were made by two blinded reviewers and reported as percent change in airway size. RESULTS: Sixteen patients were included. The standard setting (+-+) resulted in the greatest change in RP area in 43.8% of patients. An alternative setting resulted in greatest change in 56.2% of patients (--- and o-o in 18.8% each, -o- in 12.5%, and -+- in 6.3% of patients). Average response to all five settings was utilized to classify degree of palatoglossal coupling. Most patients had some enlargement (20%-70% change in RP area, 43.8%) or no enlargement (<20% change, 43.8%), whereas a minority of patients (12.5%) had marked enlargement (>70% change). RP and RG expansion were not correlated. CONCLUSION: Degree of RP expansion varied among patients and settings. Although the standard setting resulted in greatest RP change in a plurality of patients, over half had a greater response to an alternative setting. Future studies should address whether choice of setting based on RP expansion results in improved outcomes. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:218-223, 2021.

Development of a 3-Dimensional Middle Ear Model to Teach Anatomy and Endoscopic Ear Surgical Skills.

Mastery of ear anatomy and otologic surgical skills is challenging for trainees, and educational resources are limited. Advancements in 3-dimensional (3D) printing have enabled the construction of complex microscopic models. Otoendoscopy provides excellent visualization and has been shown to enhance anatomic learning. Our aim was to develop a 3D model of the middle ear and external auditory canal using computed tomography images of a pediatric temporal bone for use with otoendoscopes. Resulting models are life sized, anatomically accurate, and allow for identification of relevant middle ear structures. Forty-six trainees were recruited for a pilot study and randomized to study using the model or standard resources. There were no differences in pretest, posttest, or 1-week posttest performance between groups; however, trainees assigned to the model reported higher prospective interest, satisfaction, and subjective improvement. This model may be used with otoendoscopes for anatomic and surgical training and represents an advancement in otologic surgical simulation.

Comparison of Traditional Upper Airway Surgery and Upper Airway Stimulation for Obstructive Sleep Apnea.

OBJECTIVE: To compare patients with moderate-severe obstructive sleep apnea (OSA) undergoing traditional single and multilevel sleep surgery to those undergoing upper airway stimulation (UAS). STUDY DESIGN: Case control study comparing retrospective cohort of patients undergoing traditional sleep surgery to patients undergoing UAS enrolled in the ADHERE registry. SETTING: 8 multinational academic medical centers. SUBJECTS AND METHODS: 233 patients undergoing prior single or multilevel traditional sleep surgery and meeting study inclusion criteria were compared to 465 patients from the ADHERE registry who underwent UAS. We compared preoperative and postoperative demographic, quality of life, and polysomnographic data. We also evaluated treatment response rates. RESULTS: The pre and postoperative apnea hypopnea index (AHI) was 33.5 and 15 in the traditional sleep surgery group and 32 and 10 in the UAS group. The postoperative AHI in the UAS group was significantly lower. The pre and postoperative Epworth sleepiness scores (ESS) were 12 and 6 in both the traditional sleep surgery and UAS groups. Subgroup analysis evaluated those patients undergoing single level palate and multilevel palate and tongue base traditional sleep surgeries. The UAS group had a significantly lower postoperive AHI than both traditional sleep surgery subgroups. The UAS group had a higher percentage of patients reaching surgical success, defined as a postoperative AHI <20 with a 50% reduction from preoperative severity. CONCLUSION: UAS offers significantly better control of AHI severity than traditional sleep surgery. Quality life improvements were similar between groups.

Thyroid Lobectomy for T1 Papillary Thyroid Carcinoma in Pediatric Patients.

Importance: The current recommendation for pediatric patients with papillary thyroid cancer (PTC) is a total thyroidectomy. This recommendation applies to all stages of PTC, including papillary thyroid microcarcinoma (≤1 cm, T1a) tumors. Objective: To evaluate the characteristics of American Joint Committee on Cancer T1 PTC tumors in a large pediatric population and to identify a subgroup of patients who may benefit from a thyroid lobectomy instead of a total thyroidectomy. Design, Setting, and Participants: This retrospective cohort study was conducted from January 1, 2009, to May 31, 2020. The study took place at a tertiary care medical center and included 102 patients who were surgically treated for T1 PTC: 52 with stage T1a (≤1 cm) tumors and 50 with stage T1b (>1 cm but ≤2 cm) tumors. Main Outcomes and Measures: Primary outcomes included the presence of bilateral disease and lymph node metastasis. Results: A total of 102 patients (mean age, 15.3 years [range, 9.7-18.9 years]; 84 girls [82.4%]) were included in the analysis. Among 52 patients with T1a tumors, 10 (19.2%) had bilateral disease, and 15 (28.8%) had central neck lymph node (N1a) metastasis. Among 50 patients with T1b tumors, 10 (20%) had bilateral and 13 (26%) had N1a disease. Of those with T1a, unilateral multifocality was associated with bilateral disease (odds ratio [OR], 2.1; 95% CI, 1.3-3.4) and N1a disease (OR, 5.1; 95% CI, 1.5-17.6). Both N1a disease (OR, 20.0; 95% CI, 3.5-115.0) and ≥4 positive lymph nodes (OR, 8.6; 95% CI, 1.2-60.9) were associated with bilateral disease. In patients with no pathologic evidence of lymph node metastasis (N0), there was a 95% rate of unilateral PTC. In patients with T1b tumors, unilateral multifocality was also associated with bilateral disease (OR, 1.8; 95% CI, 1.3-2.7). Patients with T1b tumors had an increased risk of lateral (N1b) neck lymph node metastasis when compared with those with T1a tumors (OR, 3.7; 95% CI, 1.0-14.5). Conclusions and Relevance: The findings of this cohort study suggest that, in patients with unifocal T1a PTC without clinically evident nodal disease on preoperative ultrasonography, a thyroid lobectomy and central neck dissection may be considered. If there is no evidence of unilateral multifocality or if there are fewer than 4 positive lymph nodes on postoperative pathology, then close observation may be considered. These findings have substantial clinical implications and may result in practice changes regarding the extent of thyroid surgery on low-stage pediatric PTC.

Management of the difficult airway in the COVID-19 pandemic: Illustrative complex head and neck cancer scenario.

BACKGROUND: This case highlights challenges in the assessment and management of the "difficult airway" patient in the SARS-CoV-2 (COVID-19) pandemic era. METHODS: A 60-year-old male with history of recent transoral robotic surgery resection, free flap reconstruction, and tracheostomy for p16+ squamous cell carcinoma presented with stridor and dyspnea 1 month after decannulation. Careful planning by a multidisciplinary team allowed for appropriate staffing and personal protective equipment, preparations for emergency airway management, evaluation via nasopharyngolaryngoscopy, and COVID testing. The patient was found to be COVID negative and underwent imaging which revealed new pulmonary nodules and a tracheal lesion. RESULTS: The patient was safely transorally intubated in the operating room. The tracheal lesion was removed endoscopically and tracheostomy was avoided. CONCLUSIONS: This case highlights the importance of careful and collaborative decision making for the management of head and neck cancer and other "difficult airway" patients during the COVID-19 epidemic.

Bilateral papillary thyroid cancer in children: Risk factors and frequency of postoperative diagnosis.

BACKGROUND: The recommendation for children with papillary thyroid cancer (PTC) is total thyroidectomy (TT) based on the incidence of bilateral disease. Evaluating this assumption, we reviewed the characteristics of bilateral PTC in a large cohort of children. METHODS: A retrospective chart review for patients surgically treated for PTC from 2009 to 2018 analyzing preoperative risk factors, ultrasound findings, and pathology results was performed. Bilateral disease was defined as pathologic PTC in the contralateral lobe, including microscopic disease. RESULTS: Of the 172 patients included, 38.4% had bilateral disease with 23% diagnosed postoperatively. Multifocal disease on ultrasound was associated with bilateral disease (OR 2.9, 95% CI 1.5-5.9, p = 0.002). Nodule dimension >2 cm was associated with increased risk for postoperative bilateral disease (OR 3.5, 95% CI 1.6-7.4, p = 0.001). Patients with bilateral disease were more likely to have extrathyroidal extension, lymphovascular invasion, positive central lymph nodes, and extranodal extension (p < 0.001 for all). Diffuse-sclerosing variant PTC was also associated with bilateral disease. CONCLUSION: Thirty-eight percent of children were diagnosed with PTC demonstrate bilateral disease. Nearly one in four have occult bilateral disease. The features that predicted bilateral disease were multifocality, widely invasive PTC on ultrasound, and the presence of lymphadenopathy. Thus, TT is the appropriate surgical approach for pediatric patients with PTC. TYPE OF STUDY: Clinical Research, Retrospective Review. LEVEL OF EVIDENCE: Level IV.

Tracheostomy in the Extremely Premature Neonate: A Multi-Institutional Study.

OBJECTIVE: (1) To describe characteristics associated with tracheostomy placement and (2) to describe associated in-hospital morbidity in extremely premature infants. STUDY DESIGN: Pooled retrospective analysis of charts. SETTING: Academic children's hospitals. SUBJECTS AND METHODS: The patient records of premature infants (23-28 weeks gestational age) who underwent tracheostomy between January 1, 2012, and December 31, 2017, were reviewed from 4 academic children's hospitals. Demographics, procedural morbidity, feeding, respiratory, and neurodevelopmental outcomes at the time of transfer from the neonatal intensive care unit (NICU) were obtained. The contribution of baseline characteristics to mortality, neurodevelopmental, and feeding outcomes was also assessed. RESULTS: The charts of 119 infants were included. The mean gestational age was 25.5 (95% confidence interval, 25.2-25.7) weeks. The mean birth weight was 712 (671-752) g. Approximately 50% was African American. The principal comorbidity was chronic lung disease (92.4%). Overall, 60.5% of the infants had at least 1 complication. At the time of transfer, most remained mechanically ventilated (94%) and dependent on a feeding tube (90%). Necrotizing enterocolitis increased the risk of feeding impairment (P = .002) and death (P = .03). CONCLUSIONS: Tracheostomy in the extremely premature neonate is primarily performed for chronic lung disease. Complications occur frequently, with skin breakdown being the most common. Placement of a tracheostomy does not seem to mitigate the systemic morbidity associated with extreme prematurity.

Mental health among otolaryngology resident and attending physicians during the COVID-19 pandemic: National study.

BACKGROUND: Otolaryngologists are among the highest risk for COVID-19 exposure. METHODS: This is a cross-sectional, survey-based, national study evaluating academic otolaryngologists. Burnout, anxiety, distress, and depression were assessed by the single-item Mini-Z Burnout Assessment, 7-item Generalized Anxiety Disorder Scale, 15-item Impact of Event Scale, and 2-item Patient Health Questionnaire, respectively. RESULTS: A total of 349 physicians completed the survey. Of them, 165 (47.3%) were residents and 212 (60.7%) were males. Anxiety, distress, burnout, and depression were reported in 167 (47.9%), 210 (60.2%), 76 (21.8%), and 37 (10.6%) physicians, respectively. Attendings had decreased burnout relative to residents (odds ratio [OR] 0.28, confidence interval [CI] [0.11-0.68]; P = .005). Females had increased burnout (OR 1.93, CI [1.12.-3.32]; P = .018), anxiety (OR 2.53, CI [1.59-4.02]; P < .005), and distress (OR 2.68, CI [1.64-4.37]; P < .005). Physicians in states with greater than 20 000 positive cases had increased distress (OR 2.01, CI [1.22-3.31]; P = .006). CONCLUSION: During the COVID-19 pandemic, the prevalence of burnout, anxiety, and distress is high among academic otolaryngologists.

Concurrent Pregnancies and Parental Leaves Among Trainees in a Residency Program.

This Viewpoint shares the experience of a single Otolaryngology−Head & Neck Surgery residency program that faced the scheduling challenges of having nearly a quarter of its residents expecting a child and on parental leave.