Predictors of successful natural sleep MRI for sensorineural hearing loss in infants.

OBJECTIVES: Cochlear implantation (CI) in children with sensorineural hearing loss (SNHL) before 12 months of age (mo) improves language outcomes. MRI is important to assess CI candidacy. Anesthesia before 3 years old may increase risk of neurocognitive delay. Natural sleep MRI (NS-MRI) is an emerging technique to avoid anesthesia in infants, but relies on successful sleep for adequate imaging. Our multidisciplinary team hypothesized the following predictors of successful NS-MRI for CI evaluation: age, distance travelled, comorbidities, primary language, insurance type, HL characteristics, time and duration of MRI. METHODS: We performed retrospective review of children 0-12mo who attempted NS-MRI. The NS-MRI was successful if imaging was sufficient for definitive clinical management per the managing otolaryngologist. RESULTS: Among 26 patients (29 scans), the median age was 3.2mo (range: 1.2-6.8mo), distance travelled was 16.3 miles (range: 0.9 to 365 miles), 12 (46%) children had medical comorbidities. 8 (31%) had public insurance. 10 (38%) had bilateral HL. 52% (15/29) of scans were successful. Patients with comorbidities had significantly lower odds of successful NS-MRI (OR 0.09; 95% CI 0.01-0.54). Success was not associated with age, distance travelled, insurance type, primary language, HL characteristics, time or duration of MRI on univariable analysis. All 11 children who failed NS-MRI underwent hearing-aid fitting and/or imaging with sedation and CI as clinically indicated before 12mo. CONCLUSION: NS-MRI was successful in 52% of infants, regardless of age, demographics, HL or MRI characteristics. Unsuccessful NS-MRI did not result in delayed intervention. NS-MRI is an effective consideration for a broad range of infants with SNHL.

Early surgical and audiologic outcomes of active, transcutaneous, osseointegrated bone-conduction hearing device (Osia 2® system) placement.

OBJECTIVE: To assess the outcomes of pediatric Osia 2® System placements. METHODS: We performed a retrospective chart review of primary and revision Osia 2® System surgical cases at two tertiary academic children's hospitals. Operative details and post-operative surgical and audiologic outcomes were recorded. RESULTS: 18 cases were performed on 14 children (mean age: 11.5 years, range 7-16) and included 9 primary surgeries and 9 revisions from BAHA Attract®, Connect® and Sophono® implants. Surgical planning for revision surgeries was complex due to prior incisions, implants, and bony contour. Post-operative aided audiograms revealed pure tone average (0.5-4 kHz) of 26.2 ± 2.5 dB HL (mean ± SD), with no high frequency roll-off (8 kHz aided threshold: 23.8 ± 7.5 dB HL). Two minor post-operative complications were identified. CONCLUSION: Bone-conduction hearing devices (BCHDs) are used to improve access to sound for children with conductive hearing loss, single-sided deafness, and aural atresia. Traditional passive, percutaneous abutment-based and transcutaneous magnet-based surgical BCHDs can be limited by skin complications and high-frequency acoustic attenuation. Recent availability of active, transcutaneous osseointegrated BCHD systems presents potential for improvement on both of these traditional limitations. Initial experience with the Osia 2® System demonstrates overall successful, uncomplicated placement with excellent audiologic outcomes. Revision cases require careful surgical planning. Further follow-up and comparative studies with other BCHDs are necessary to fully evaluate the effectiveness of the Osia 2® System.

Racial and ethnic disparities in diagnostic efficacy of comprehensive genetic testing for sensorineural hearing loss.

Understanding racial and ethnic disparities in diagnostic rates of genetic testing is critical for health equity. We sought to understand the extent and cause of racial and ethnic disparities in diagnostic efficacy of comprehensive genetic testing (CGT) for sensorineural hearing loss (SNHL). We performed a retrospective cohort study at two tertiary children's hospitals on a diverse cohort of 240 consecutive pediatric patients (76% publicly insured, 82% non-White) with SNHL of unknown etiology who underwent CGT. Definite and possible genetic diagnoses were assigned for each patient, representing the likelihood of a genetic cause of hearing loss. Associations between diagnostic rates were examined. 3.8 ± 2.1 variants were detected per patient; this frequency did not vary between White/Asian and Hispanic/Black cohorts. Overall, 82% of variants were variants of uncertain significance (VUS). Compared with White and Asian subjects, variants identified among Hispanic and Black children were less likely to be classified as pathogenic/likely pathogenic (15% vs. 24%, p < 0.001), and Hispanic and Black children were less likely to have a definite genetic diagnosis (10% vs. 37%, p < 0.001). The adjusted odds ratio for definite genetic diagnosis in Black and Hispanic children compared with White and Asian children was 0.19. Expanding genetic diagnostic criteria to include predicted deleterious VUSs reduced these disparities between White/Asian and Hispanic/Black children, with comparable molecular diagnostic rates (41% vs. 38%, p = 0.72). However, in silico predictions are insufficiently valid for clinical use. Increased inclusion of underrepresented groups in genetic hearing-loss studies to clinically validate these variants is necessary to reduce racial and ethnic disparities in diagnostic efficacy of comprehensive genetic testing.

Efficacy of endoscopic sinus surgery alone versus in combination with neurosurgical intervention for the treatment of pediatric subdural empyema.

OBJECTIVES: To compare treatment outcomes for sinogenic subdural empyema (SE) between those managed with initial endoscopic sinus surgery (ESS) alone versus those treated with a combination of ESS and craniotomy over the last decade at our institution. To better characterize subdural empyema with regard to presentation, causative pathogens, and treatment course. METHODS: Retrospective single-center chart review to identify and evaluate pediatric SE patients between 2009 and 2019. Patients meeting inclusion criteria were classified in one of two groups: those who initially underwent ESS or frontal trephination without concurrent neurosurgical procedure and those who underwent craniotomy or burr hole in addition to a sinus procedure. Presenting characteristics and treatment outcomes were compared between the two groups. RESULTS: Eighteen patients met inclusion criteria. The ESS alone and the ESS + craniotomy subgroups each had 9 patients with similar baseline characteristics. The ESS + craniotomy group was more likely to present with neurological symptoms (p = 0.039) and have multiple intracranial fluid collections (p = 0.046). 74.1% of patients presented to the Emergency Department (ED) or to their primary medical doctor and were treated with outpatient management prior to hospitalization with definitive surgical management. The most common presenting symptoms were fever, headache and nausea/vomiting. There were no differences between treatment groups in rate of return to the operating room (OR) (p = 1.00), length of stay (LOS) (p = 0.553), or adverse neurological outcomes (p = 0.456). 44.4% of patients in the ESS alone group eventually required neurosurgical intervention. CONCLUSIONS: Surgical SE patients often present to medical professionals in the primary care setting or ED and are managed with outpatient treatment before admission with definitive treatment. In this small retrospective cohort patients who underwent sinus intervention alone had similar rates of return to OR, LOS and adverse neurological outcomes use as those who underwent a sinus procedure in coordination with a neurosurgical intervention. There may be a group of patients with SE who may be managed with endoscopic procedures alone and further studies should seek to determine the characteristics of this population.

Impact of neck CT on the management of suspected pediatric deep neck space infection.

OBJECTIVE: To identify clinical and radiographic factors that predict successful medical management in children with suspected deep neck space infection. METHODS: Retrospective chart review of 313 immunocompetent children admitted to a tertiary care children's hospital after undergoing a CT scan for a suspected neck abscess. RESULTS: A rim-enhancing hypodensity, suggestive of an abscess, was identified in 131/313 patients (42%). Medical management with IV antibiotics was utilized for more than 24 h in 57/131 (43%) of children with radiographic findings suggestive of a coalescent or evolving abscess. Medical management was successful in 36/57 (63%) of patients with a rim-enhancing collection. Children who underwent incision and drainage within 24 h had a larger fluid collection than those who underwent initial medical management (mean diameter 30 mm vs 20.5 mm; p < 0.0001), however, there was no difference between the size of abscesses that failed medical management compared to those successfully managed with IV antibiotics (22.9 mm vs 19.1 mm; p = 0.07). Clinical factors and white blood cell count were not predictive of response to antibiosis. CONCLUSION: The majority of children with suspected DNSI were successfully managed with IV antibiotics alone, however, clinical and radiographic factors are not reliable predictors of successful medical management. In children who are hemodynamically stable without airway symptoms, one should consider a trial of IV antibiotics prior to requesting a CT scan.

The Prevalence of Ultrarapid Metabolizers of Codeine in a Diverse Urban Population.

OBJECTIVE: To examine the prevalence of ultrarapid metabolizers of codeine among children in an ethnically diverse urban community. STUDY DESIGN: Cross-sectional study. SETTING: A tertiary care academic children's hospital in the Bronx, New York. SUBJECTS AND METHODS: In total, 256 children with nonsyndromic congenital sensorineural hearing loss were analyzed. DNA was assessed for 63 previously described single-nucleotide polymorphisms (SNPs) and copy number variants (CNVs) known to alter the function and expression of the CYP2D6 gene primarily responsible for codeine metabolism. The rate of CYP2D6 metabolism was predicted based on participants' haplotype. RESULTS: Ethnic distribution in the study subjects paralleled recent local census data, with the largest portion (115 children, 45.8%) identified as Hispanic or Latino. A total of 154 children (80.6%) had a haplotype that corresponds to extensive codeine metabolism, 18 children (9.42%) were identified as ultrarapid metabolizers (UMs), and 16 children (8.37%) were intermediate metabolizers. Only 3 children in our cohort (1.57%) were poor metabolizers. Patients identifying as Caucasian or Hispanic had an elevated incidence of UMs (11.3% and 11.2%, respectively) with extensive variability within subpopulations. CONCLUSIONS: The clinically significant rate of ultrarapid metabolizers reinforces safety concerns regarding the use of codeine and related opiates. A patient-targeted approach using pharmacogenomics may mitigate adverse effects by individualizing the selection and dosing of these analgesics.

Posterior cricoid-graft mismatch in laryngotracheal reconstruction.

INTRODUCTION/OBJECTIVE: In laryngotracheal reconstruction (LTR) with sutureless posterior cricoid grafting (PCG), the inset graft's thickness may not precisely approximate the anterior-posterior depth of the divided posterior cricoid plate. This case series highlights this phenomenon and describes the clinical profiles and airway characteristics of children with similar subglottic stenosis grades undergoing LTR with PCG with varying degrees of graft-cricoid mismatch. METHODS: This is a case series with retrospective chart review of children who underwent LTR with PCG by a single surgeon at a tertiary care urban children's hospital from 2008 to 2014. RESULTS: Twenty-one patients with twenty-two operations were identified. Varying degrees of graft-cricoid mismatch were present. Of the 22 operations, 15 were classified as grade I (flush), 5 grade II (<3 mm), and 2 grade III (>3 mm). Median preoperative grade of stenosis was III in all groups. Mean one month granulation grade was 1.84, compared to 1.92 in grade I, 1.6 in grade II mismatch, 2 in grade III mismatch, and 1.7 in any mismatch (p = 0.65). Mean follow up was 24 months. CONCLUSION: Graft-cricoid mismatch may occur at variable degrees during laryngotracheal reconstruction utilizing sutureless posterior cricoid grafting. Our preliminary data suggest several millimeters of graft-cricoid mismatch can be tolerated without significantly affecting postoperative granulation formation or adding to postoperative morbidity.

Severe Obstructive Sleep Apnea in a Child With Goldenhar Syndrome and Nasal Obstruction.

ABSTRACT: A 4-year-old boy with Goldenhar syndrome and severe obstructive sleep apnea does not tolerate nasal continuous positive airway pressure. Evaluation with imaging showed nasal obstruction with an inverted tooth. This case illustrates the importance of personalized evaluation and treatment plan in a child with Goldenhar syndrome.

Tonsillectomies and respiratory complications in children: A look at pre-op polysomnography risk factors and post-op admissions.

OBJECTIVE: To identify predictors of post-operative respiratory complications in children undergoing tonsillectomy. METHODS: Consecutive case series with chart review of children who underwent polysomnography (PSG) and subsequent tonsillectomy with or without adenoidectomy for obstructive sleep apnea (OSA). Patients with craniofacial anomalies or significant cardiopulmonary comorbidities were excluded. Rates of post-surgical respiratory complication were reviewed and compared to patient specific factors and PSG findings to identify possible risk factors. RESULTS: Eighty-six patients (mean age 5.3 ± 2.2 years) were included. There was a statistically significant (p = 0.03) relationship between an AHI ≥40 (AHI40) and post-operative respiratory complications. AHI40 also had the greatest magnitude of association with postoperative respiratory complications (OR = 5.313). An AHI ≥25 (AHI25) was marginally significant (p = 0.067). No significant difference in outcome occurrence was found when analyzing rates of complication in patients with BMI above and below 18 (p = 0.20) or oxygen (O2) nadir above and below 80% (p = 0.09). The AHI ranged from 0 to 112.2, and no postoperative respiratory complications were identified in children with an AHI less than 10. CONCLUSIONS: Our results indicate an association between an AHI ≥40 and respiratory complications following an adenotonsillectomy, but we were not able to observe any significant difference at a cutoff of 25. An association between BMI or O2 nadir and postoperative respiratory complication was not able to be identified. Our results support the importance of AHI as a predictor of postoperative respiratory complications in children undergoing tonsillectomy for OSA.

Injection laryngoplasty in children with cystic fibrosis and abnormal swallow.

OBJECTIVE: Children with cystic fibrosis (CF) are uniquely vulnerable to the pulmonary complications of chronic aspiration. We present a case series of children with CF and evidence of chronic aspiration who underwent injection laryngoplasty to improve the safety and efficacy of their swallow. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care children's hospital. METHODS/SUBJECTS: A chart review was performed on three consecutive cases of children with CF and evidence of aspiration on modified barium swallow (MBS) evaluated at a tertiary care, academic children's medical center. RESULTS: Three patients with CF underwent injection laryngoplasty for evidence of aspiration or laryngeal penetration on MBS evaluation. Normal laryngeal anatomy was identified intraoperatively in each case. At the time of the procedure, patients were 22 months, 70 months, and 24 months old, and follow-up information was available for 7 months, 11 months, and 12 months post-procedure, respectively. Presenting symptoms included chronic cough, cough with oral liquids, and recurrent pneumonia. Each patient underwent successful injection into the interarytenoid space. Post-operatively, modified barium swallow demonstrated resolution of aspiration or penetration in all patients. No procedure-related complications were encountered. CONCLUSION: Patients with CF are highly susceptible to pulmonary infections, and aggressive treatment of chronic aspiration is often necessary. Injection laryngoplasty may be effective in normalizing swallowing in these children. Future study will elucidate the duration of effect and if this technique improves long-term pulmonary outcomes in CF patients.

The role of tonsillectomy in the treatment of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS).

IMPORTANCE: Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) in children describes neuropsychiatric symptom exacerbations that relate temporally to streptococcal infections. Recent case reports suggest tonsillectomy may effectively reduce these symptoms; however, no consensus treatment guidelines exist. This study examines whether tonsillectomy improves neuropsychiatric symptoms in children with PANDAS who have incomplete response to antibiotic therapy. OBSERVATIONS: Ten patients met strict diagnostic criteria for PANDAS. Comparisons were made between parental reports of symptom severity at diagnosis, after antibiotic treatment (in 10 patients), and after tonsillectomy (in 9). From a baseline severity score of 10, antibiotics alone improved symptoms to a median (interquartile range [IQR]) score of 8 (6.5-10.0) (P = .03). Nine children who subsequently underwent tonsillectomy reported symptom improvement in comparison with treatment with antibiotics alone, including those with no response to antibiotics. Symptom severity improved at all periods after tonsillectomy compared with antibiotics alone. The median score [IQR] 3 months postoperatively was 3 (0.0-6.5) (P = .01); 6 months postoperatively, 3 (0.0-5.0) (P = .02); 1 year postoperatively, 3 (0.0-5.0) (P = .02); and 3 years postoperatively, 0.5 (0.0-2.3) (P = .03). Four of the 9 had complete resolution after tonsillectomy. CONCLUSIONS AND RELEVANCE: This PANDAS cohort whose neuropsychiatric symptoms did not respond sufficiently to antibiotics may have gained benefit from tonsillectomy.

Vascular Anomalies in Otolaryngology-Head and Neck Surgery Resident Education.

The evaluation and treatment of vascular anomalies is rapidly evolving. In recent years, improved imaging, medical therapies, interventional radiology procedures, and technical advances have led to improved functional and aesthetic outcomes with reduced morbidity. With management of vascular anomalies becoming increasingly complex, we wanted to assess the opinions of otolaryngology-head and neck surgery resident trainees regarding education in this evolving subspecialty. The results of our survey show that a significant majority of trainees feel that vascular anomalies are best managed by a multidisciplinary team, consistent with practice in large vascular anomalies centers. While training in this area does not seem to be deficient, it may be helpful to identify those otolaryngology residents who are interested in gaining exposure to patients with vascular anomalies, so that they may seek additional subspecialty experiences to complement their otolaryngology training.

Laryngotracheal reconstruction outcomes in hypotonic children.

IMPORTANCE: Children with poor muscle tone may demonstrate upper airway obstruction due to several mechanisms including obstructive sleep apnea, laryngopharyngeal reflux, and laryngomalacia. Though hypotonia has been shown to compromise the pediatric airway, and some authors suggest that neurologic deficits can compromise the success of laryngotracheal reconstruction (LTR), to our knowledge no studies have evaluated the effect of neurologic diagnoses or hypotonia on outcomes in LTR. OBJECTIVE: To determine whether hypotonic children with subglottic stenosis have lower rates of successful decannulation after LTR compared with children without neurologic deficit. DESIGN, SETTING, AND PARTICIPANTS: A retrospective medical chart review was conducted for 27 children aged 0 to 6 years, who underwent LTR for subglottic stenosis between December 2007 and December 2012 at a tertiary care children's hospital. Children were classified based on documented neurologic findings. Group 1 comprised those children without neurologic impairment (n = 16). Group 2 included those children with a documented neurocognitive or neuromuscular diagnosis but without evidence of hypotonia (n = 7). Group 3 comprised hypotonic children (n = 4). INTERVENTIONS Laryngotracheal reconstruction. MAIN OUTCOMES AND MEASURES: The number of procedures performed after LTR to optimize the airway and whether the child was successfully decannulated. RESULTS: All 16 of the neurologically intact patients (100%) were decannulated. Among children with a neurologic deficit, 5 of 7 (71%) were ultimately decannulated. No hypotonic children 0 of 4 were decannulated. The difference in rates of decannulation between unaffected and normotonic children with a neurologic deficit was not statistically significant (P = .08). However, the difference in outcomes between hypotonic children and neurologically intact patients was statistically significant (P < .001). CONCLUSIONS AND RELEVANCE: Findings from this study suggest that hypotonic children may experience poorer rates of post-LTR decannulation compared with children without neurologic deficit. Dynamic upper airway obstruction may be unappreciated in hypotonic children. Future research may be directed at the appropriate evaluation and treatment of children with poor muscle tone and subglottic stenosis.

Pectus excavatum in children with laryngomalacia.

BACKGROUND: Laryngomalacia is the most common congenital laryngeal anomaly and is associated with several disorders including gastric reflux, sleep apnea, hypotonia and failure to thrive. Pectus excavatum (PE) is the most common chest wall deformity affecting 1-300/1000 individuals. Though many authors presume a relationship between PE and laryngomalacia, there is no published data to establish this association. GOAL: To test the hypothesis that patients referred to our pediatric otolaryngology clinic for evaluation of laryngomalacia exhibit higher rates of PE than the general population. METHODS: Retrospective review of prospectively enrolled children who presented with laryngomalacia (January 2008-June 2012) to a tertiary care, hospital based, pediatric otolaryngology practice. Each chart was examined for a concurrent diagnosis of pectus deformity. RESULTS: Of the 137 laryngomalacia patients, 9 (6.6%) had documented PE. This represents a significantly increased rate of PE when compared to children without laryngomalacia (p = 0.001). Four of the 9 children with PE underwent supraglottoplasty for laryngomalacia, a significantly greater proportion than the 9/128 of the children with isolated laryngomalacia who underwent supraglottoplasty (p = 0.004). CONCLUSIONS: This study suggests an association between laryngomalacia and PE. Pediatric otolaryngologists should be cognizant of this relationship, though further studies are needed to elucidate the nature of this association.

Pediatric nasal lobular capillary hemangioma.

Background. LCH is a benign vascular growth of the skin and mucous membranes commonly affecting the head and neck. Since it was first described in the nineteenth century, this entity has been variously known as "human botryomycosis" and "pyogenic granuloma." The shifting nomenclature reflects an evolving understanding of the underlying pathogenesis. We review the histopathology of and current epidemiological data pertaining to LCH which suggests that the development of these lesions may involve a hyperactive inflammatory response influenced by endocrine factors. We report two new cases of pediatric lobular capillary hemangioma (LCH) of the nasal cavity and review current theories regarding the etiology, diagnosis, and treatment of nasal LCH. Methods. Retrospective case series. Case Series. Two adolescent females presented with symptoms of recurrent epistaxis, nasal obstruction, and epiphora. Both patients underwent computed tomography imaging and biopsy of their intranasal mass. The tumors were excised using image-guided transnasal endoscopic technique. Seven other cases of nasal LCH have been reported to date in the pediatric population. Conclusion. Nasal LCH is a rare cause of an intranasal mass and is associated with unilateral epistaxis, nasal obstruction, and epiphora. We advocate for image-guided endoscopic excision of LCH in the adolescent population.

Obstacles to communication in children with cri du chat syndrome.

BACKGROUND: Cri du chat syndrome (CCS) is a genetic disorder resulting from the deletion of the short arm of chromosome 5. Perhaps the most distinctive characteristic of this syndrome is the congenital high-pitched cry, which frequently brings these patients to the attention of an otolaryngologist. Speech and language development in children with CCS is notable for a reduced receptive vocabulary and a profound deficit in expressive language. Currently, no clear guidelines have been established for the treatment of the speech and language difficulties exhibited by these patients. In this article, we present a case report and discuss the current literature regarding the challenges to effective communication in CCS. METHODS: Case report. CASE: We present a 7-year-old girl with CCS who sought help to improve her ability to communicate. The patient presented with a persistent high-pitched voice unchanged since birth and a breathy dysphonia. Findings on examination were significant for an abnormally oriented larynx with atrophic vocal folds. She continues to undertake intensive speech therapy to assist in her language development. CONCLUSION: CCS is a genetic disorder that universally results in profound deficits in expressive speech. Although patients with CCS commonly present with a high-pitched voice and marked laryngeal abnormalities, they are unlikely to benefit from surgical intervention. Speech and language therapy, including augmentative communication devices, may enhance effective communication and improve the quality of life of these patients.