Rate and management of tympanic membrane perforations in children with Down syndrome and middle ear disorder.

OBJECTIVE: To characterize the management and outcomes of observation versus surgical intervention of tympanic membrane (TM) perforations in children with Down syndrome (DS). In addition, to estimate the prevalence of TM perforations in children with DS. METHODS: Retrospective case review analysis of TM perforation rate in children with DS with history of tympanostomy tube (TT) insertion at a tertiary pediatric referral center. Patients were divided into observation or surgical intervention groups and then further evaluated for the type of intervention, the number of required procedures, and success rate of hearing improvement. Risk factors contributing to perforations were analyzed, including TT type, number of TT surgeries, and perforation size. RESULTS: The TM perforation rate in children with DS with TT history was 7.0 %. Tympanoplasty was performed in 41.5 % of perforated ears with a success rate of 53.1 %. There was no statistical difference between the surgical intervention and observation groups regarding perforation characteristics or TT number and type, but the surgical intervention cohort was older. Hearing improvement based on postoperative pure tone average (PTA) threshold was noted in the successful surgical intervention group. CONCLUSION: The rate of TM perforations in children with DS after TTs is comparable to the general population. Improved PTA thresholds were noted in the surgical success group influencing speech development. The overall lower success rate of tympanoplasty in patients with DS emphasizes the need to factor in the timing of surgical intervention based on the predicted age of Eustachian tube maturation.

The Effect of Developmental Delay and Autism Spectrum Disorder on External Auditory Canal Foreign Body Extraction.

OBJECTIVES: To determine the effect of developmental delay (DD) and autism spectrum disorder (ASD) on pediatric external auditory canal foreign body (EAC FB) retrieval outcomes. METHODS: A retrospective chart review of children presenting with EAC FB at a tertiary children's hospital was performed between January 2018 and December 2019. Charts were reviewed for demographics, presence of otalgia, complications, number of EAC FB episodes, indications for operating room removal, DD, and ASD status. RESULTS: A total of 1467 patients underwent EAC FB removal. One hundred thirty-seven children (9.3%) had DD, and, of those with DD, 63 (46%) had ASD. Children with DD were 1.76 years older compared with children with non-DD (NDD) ( P < 0.0001) at the time of presentation, whereas children with ASD were 1.45 years older than children with NDD ( P = 0.0023). Children with DD and ASD were more likely to require removal of FB in the operating room (OR) compared with the NDD group (36.5% vs 16.7%, P = 0.0001). This was not true for children with DD without ASD. Patients with DD reported significantly less otalgia when compared with NDD patients (26.3% vs 37.4%, P = 0.0097). A similar trend, although not statistically significant, was observed when comparing children with ASD with NDD patients. The NDD patients (1.1) had fewer EAC FB episodes than patients with DD (1.6, P < 0.0001) and ASD (1.8, P < 0.0016). Hazard ratios for multiple episodes of FB were 4.5 (95% confidence interval, 2.9-6.8) for DD, and 5.6 for ASD (95% confidence interval, 3.2-9.9). The complication rate for all groups was low. CONCLUSIONS: Due to the different ways that children with DD and ASD present compared with NDD children, physicians should be vigilant when evaluating symptoms and conducting physical examinations for EAC FB in those patients. A lower threshold for referral to otolaryngologists may result in more favorable outcomes.

Anosmia: Brighton Collaboration case definition and guidelines for data collection, analysis, and presentation of immunization safety data.

This is a Brighton Collaboration case definition of anosmia to be used in the evaluation of adverse events following immunization, and for epidemiologic studies for the assessment of background incidence or hypothesis testing. The case definition was developed by a group of experts convened by the Coalition for Epidemic Preparedness Innovations (CEPI) in the context of active development of SARS-CoV-2 vaccines. The case definition format of the Brighton Collaboration was followed to develop a consensus definition and defined levels of certainty, after an exhaustive review of the literature and expert consultation. The document underwent peer review by the Brighton Collaboration Network and by two expert reviewers prior to submission.

Expert Consensus Statement: Management of Pediatric Persistent Obstructive Sleep Apnea After Adenotonsillectomy.

OBJECTIVE: To develop an expert consensus statement regarding persistent pediatric obstructive sleep apnea (OSA) focused on quality improvement and clarification of controversies. Persistent OSA was defined as OSA after adenotonsillectomy or OSA after tonsillectomy when adenoids are not enlarged. METHODS: An expert panel of clinicians, nominated by stakeholder organizations, used the published consensus statement methodology from the American Academy of Otolaryngology-Head and Neck Surgery to develop statements for a target population of children aged 2-18 years. A medical librarian systematically searched the literature used as a basis for the clinical statements. A modified Delphi method was used to distill expert opinion and compose statements that met a standardized definition of consensus. Duplicate statements were combined prior to the final Delphi survey. RESULTS: After 3 iterative Delphi surveys, 34 statements met the criteria for consensus, while 18 statements did not. The clinical statements were grouped into 7 categories: general, patient assessment, management of patients with obesity, medical management, drug-induced sleep endoscopy, surgical management, and postoperative care. CONCLUSION: The panel reached a consensus for 34 statements related to the assessment, management and postoperative care of children with persistent OSA. These statements can be used to establish care algorithms, improve clinical care, and identify areas that would benefit from future research.

Speech and Language outcomes in Auditory Neuropathy Spectrum Disorder (ANSD) children managed with amplification.

OBJECTIVE: To evaluate speech and language outcomes in children with Auditory Neuropathy Spectrum Disorder (ANSD) without significant comorbidities who received hearing rehabilitation in the form of hearing aids and/or cochlear implantation. METHODS: Retrospective chart review of pediatric ANSD patients at a large academic tertiary care institution from 2010 to 2019. Patients were included if they received a diagnosis of bilateral ANSD, had minimal to no comorbidities, and had speech and language testing (SLT) on at least two occasions. RESULTS: 51 patients were reviewed and 7 met inclusion criteria. Average age at ANSD diagnosis was 1 year and 11 months, and average age of hearing aid fitting was 3 years and 3 months. Hearing loss ranged from mild to profound, with four of the children wearing behind (BTE) hearing aids and three eventually receiving cochlear implants. Four of five patients who received hearing aids prior to their first speech and language evaluation demonstrated a delay at their initial evaluation, and all five patients continued to demonstrate a delay at their most recent evaluation, despite appropriate audiologic management and speech and language therapy. There were two patients who were unaided at the time of their initial and latest evaluations; one patient showed a delay at both timepoints, and one patient showed no speech delay at either timepoint. CONCLUSIONS: Pediatric ANSD patients, who are otherwise typically developing and received hearing rehabilitation and speech and language therapy, continue to show a speech and language delay (SLD). This outcome underscores the importance of close monitoring of speech and language development, providing early amplification and/or cochlear implantation, and promoting additional education and psychosocial support.

Congenital cholesteatoma in identical twins.

Congenital cholesteatoma in identical twins has only been described once in Otolaryngology literature thus far. This report describes a case of monozygotic twins with a history of recurrent acute otitis media and bilateral middle ear effusions without tympanic membrane perforation. Upon myringotomy with pressure equalization tube insertion, both were found to have right-sided cholesteatoma with nearly identical location and pattern of progression. In the context of previous case series demonstrating familial clustering and reports of possible genetic associations of this condition, the authors present an important addition to the current understanding of congenital cholesteatoma disorder.

Prevalence and surgical management of cholesteatoma in Down Syndrome children.

OBJECTIVE: The objective was to report the outcomes of surgical management of cholesteatoma in Down Syndrome (DS) children and to determine the prevalence of cholesteatoma in DS children at three academic tertiary care centers. METHODS: A retrospective chart review was performed of all DS children diagnosed with cholesteatoma from three tertiary care academic centers. Demographic data, comorbidities, imaging findings, surgical management, and pre- and postoperative pure tone averages were collected. RESULTS: The prevalence of cholesteatoma in DS patients at these three institutions was calculated to be 8/802 (0.998%), 4/448 (0.893%), and 4/1016 (0.394%), with an average prevalence of 0.762%. Of the 16 patients with cholesteatoma identified, the average age of diagnosis was 10.8 years, and 5/16 (31%) had at least three sets of pressure equalizing tubes (PETs) placed. Of the 13 patients with preoperative CTs, 4/13 (30.8%) had blunting of the scutum, 4/13 (30.8%) had tegmen involvement, 1/13 (7.7%) had erosion of the semicircular canal, 2/13 had facial nerve dehiscence, and 7/13 (53.8%) had ossicular chain erosion. For surgical management, disease control was attained in 4/16 children with tympanoplasty only and canal wall up (CWU) tympanomastoidectomy in 11/16 patients. Available pre- and postoperative audiograms were evaluated; however, there was insufficient audiological data to contribute to outcomes. CONCLUSION: Cholesteatoma occurs in nearly 1% of DS patients, and the disease tends to be advanced with tegmen involvement in nearly a third of patients, and ossicular erosion seen in more than half the patients. A CWU procedure can be successful in managing DS patients with cholesteatoma when consistent long term follow-up is planned. Close monitoring and a high index of suspicion for cholesteatoma are essential to prevent delays in diagnosis and treatment of DS patients with a history of multiple sets of PET placement.

Safety of Cochlear Implantation in Children 12 Months or Younger: Systematic Review and Meta-analysis.

OBJECTIVE: To systematically review the literature to determine safety of cochlear implantation in pediatric patients 12 months and younger. DATA SOURCE: Ovid MEDLINE, EMBASE, CINAHL, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from inception to March 20, 2021. REVIEW METHODS: Studies that involved patients 12 months and younger with report of intraoperative or postoperative complication outcomes were included. Studies selected were reviewed for complications, explants, readmissions, and prolonged hospitalizations. Two independent reviewers screened all studies that were selected for the systematic review and meta-analysis. All studies included were assessed for quality and risk of bias. RESULTS: The literature search yielded 269 studies, of which 53 studies underwent full-text screening, and 18 studies were selected for the systematic review and meta-analysis. A total of 449 patients and 625 cochlear implants were assessed. Across all included studies, major complications were noted in 3.1% of patients (95% CI, 0.8-7.1) and 2.3% of cochlear implantations (95% CI, 0.6-5.2), whereas minor complications were noted in 2.4% of patients (95% CI, 0.4-6.0) and 1.8% of cochlear implantations (95% CI, 0.4-4.3). There were no anesthetic complications reported across all included studies. CONCLUSION: The results of this systematic review and meta-analysis suggest that cochlear implantation in patients 12 months and younger is safe with similar rates of complications to older cohorts.

Effects of Age on Delayed Facial Palsy After Otologic Surgery: A Systematic Review and Meta-Analysis.

OBJECTIVES: To report 4 cases of delayed facial palsy (DFP) after pediatric middle ear (ME) surgery and systematically review and analyze the associated literature to evaluate the effects of age on DFP etiology, management, and prognosis. METHODS: Systematic review of PubMed, Cochrane Library, and Embase for articles related to DFP after cochlear implantation (CI) was performed. These articles were assessed for level of evidence, methodological limitations, and number of cases. Meta-analysis was performed to assess the effects of age on DFP incidence. Furthermore, a comprehensive list of all pediatric DFP cases after otologic surgery was assembled through a multi-institutional retrospective review and systematic review of the literature. RESULTS: Twenty-nine articles fit the criteria for inclusion in the meta-analysis. The incidence of DFP after CI was 0.23% and 1.01% for pediatric and adult cases, respectively. This difference was statistically significant (P < .001, odds ratio 4.36). Twenty-three cases, adding to the 4 presented herein, were suitable for a comprehensive list. The mean age was 6.9 years. Average postoperative day of paresis onset was 5.4, with an average maximum House-Brackmann grade of 3.5. All patients obtained full facial recovery after an average of 23.5 days. CONCLUSIONS: The systematic review demonstrates that DFP after pediatric CI is rare and occurs at a significantly lower rate than in adults, further supporting the viral reactivation hypothesis of DFP. The prognosis for pediatric DFP after otologic surgery is excellent, with a high rate of full recovery in a short time frame. However, steroid administration can be considered. LEVEL OF EVIDENCE: IIa.

Electrostatic Discharge (ESD) as a Cause of Facial Nerve Stimulation After Cochlear Implantation: A Case Report.

OBJECTIVE: To discuss persistent facial nerve stimulation (FNS) related to repeated electrostatic discharge (ESD) shock following cochlear implantation. METHODS: Single case report with literature review. RESULTS: FNS is a feared complication after cochlear implantation, occurring in approximately 7% of cases, with most patients having anatomic abnormalities. The presented case has no anatomical abnormalities but reported frequent environmental static shock. FNS during the first 1 to 3 seconds of processor attachment caused a significant decrease in the patient's quality of life, requiring subsequent re-implantation with full resolution. CONCLUSIONS: FNS is a complication of cochlear implantation that can cause a great deal of distress and discomfort. Frequent electrostatic discharge (ESD) contributed to device malfunctioning and FNS in a patient with otherwise normal anatomy and should be avoided if possible.

Utilization of Alexandrite Laser for Hair Removal in Pediatric Auricular Reconstruction.

Auricular reconstruction with autologous rib cartilage involves using a soft tissue envelope to cover the cartilage framework. In patients with a low hairline, hair-bearing skin may be incorporated on the reconstructed ear, creating a difficult and conspicuous aesthetic problem. A retrospective chart review was conducted to summarize and share the experience of using the Candela GentleMax Pro 755-nm alexandrite laser system (Candela Corp) in children following auricular reconstruction. Nine patients received laser hair removal via the alexandrite laser system with good results. The number of completed sessions ranged from 1 to 5. The procedures were completed without the need for premedication or procedural sedation. Laser hair removal with an alexandrite laser system was safe, fast, and effective. With multistage auricular reconstruction, it was feasible to incorporate laser hair removal between the stages.

Fabrication of 3D Models for Microtia Reconstruction Using Smartphone-Based Technology.

OBJECTIVE: Microtia reconstruction is technically challenging due to the intricate contours of the ear. It is common practice to use a two-dimensional tracing of the patient's normal ear as a template for the reconstruction of the affected side. Recent advances in three-dimensional (3D) surface scanning and printing have expanded the ability to create surgical models preoperatively. This study aims to describe a simple and affordable process to fabricate patient-specific 3D ear models for use in the operating room. STUDY DESIGN: Applied basic research on a novel 3D optical scanning and fabrication pathway for microtia reconstruction. SETTING: Tertiary care university hospital. METHODS: Optical surface scanning of the patient's normal ear was completed using a smartphone with facial recognition capability. The Heges application used the phone's camera to capture the 3D image. The 3D model was digitally isolated and mirrored using the Meshmixer software and printed with a 3D printer (MonopriceTM Select Mini V2) using polylactic acid filaments. RESULTS: The 3D model of the ear served as a helpful intraoperative reference and an adjunct to the traditional 2D template. Collectively, time for imaging acquisition, editing, and fabrication was approximately 3.5 hours. The upfront cost was around $210, and the recurring cost was approximately $0.35 per ear model. CONCLUSION: A novel, low-cost approach to fabricate customized 3D models of the ear is introduced. It is feasible to create individualized 3D models using currently available consumer technology. The low barrier to entry raises the possibility for clinicians to incorporate 3D printing into various clinical applications.

Diagnosis of Auditory Neuropathy Spectrum Disorder in the Neonatal Intensive Care Unit Population.

OBJECTIVE: To determine the incidence of auditory neuropathy spectrum disorder (ANSD) and its risk factors among the neonatal intensive care unit (NICU) population from 2009 to 2018 in the Pediatric Health Information System database. STUDY DESIGN: Retrospective national database review. SETTING: Population-based study. METHODS: The Pediatric Health Information System database was queried to identify patients ≤18 years old with NICU admission and ANSD diagnosis. Patient demographics, jaundice diagnosis, use of mechanical ventilation, extracorporeal membrane oxygenation, furosemide, and/or aminoglycosides were extracted. Multivariable linear regression was used to assess trends in incidence. Chi-square analysis was used to assess differences between patients with and without ANSD. Logistic regression was used to assess factors associated with ANSD. RESULTS: From 2009 to 2018, there was an increase in (1) NICU admissions from 14,079 to 24,851 (P < .001), (2) total ANSD diagnoses from 92 to 1847 (P = .001), and (3) annual total number of patients with ANSD and NICU admission increased from 4 to 16 (P = .005). There was strong correlation between the increases in total number of NICU admissions and total ANSD diagnoses over time (R = 0.76). The average ANSD incidence was 0.052% with no statistically significant change over 10 years. When compared with all NICU admissions, children with ANSD had a higher association with use of furosemide (P < .001) and ventilator (P < .001). CONCLUSION: Despite a statistically significant increase in NICU admissions and total ANSD diagnosis, the incidence of ANSD in the NICU population has not increased from 2009 to 2018. Furosemide and mechanical ventilator use were associated with increased likelihood of ANSD.

Early Outcomes of a New Active Transcutaneous Bone Conduction Implant in Pediatric Patients.

OBJECTIVE: To describe the early surgical and audiometric outcomes in pediatric patients implanted with a new active transcutaneous bone conduction implant system. STUDY DESIGN: Retrospective case review. SETTING: Tertiary pediatric hospital. PATIENTS: Pediatric patients (18 or younger) with conductive or mixed hearing loss that completed postoperative aided testing following implantation with the Cochlear Osia system from December 2019 to December 2020. INTERVENTION: Rehabilitative. MAIN OUTCOME MEASURE: Preoperative air conduction (AC), preoperative bone conduction (BC), and postoperative aided thresholds were compared. Pure-tone averages (PTA), air-bone gap (ABG), and functional gain were calculated. Surgical complications and patient satisfaction were summarized from the chart review. RESULTS: Sixteen patients (20 implants) met the inclusion criteria. The average age at the time of implantation was 12.9 ±â€Š2.4 years. The preoperative AC and BC thresholds were 64.4 dB (±11.9 dB) and 7.9 dB (±4.90 dB), respectively, with an average ABG of 56.5 dB (±12.8 dB). The average postoperative aided threshold was 21.2 dB (± 4.25 dB) with a mean functional gain of 43.1 dB (±10.2 dB). One patient developed seroma postoperatively, which was treated conservatively. No other complications were reported over a mean follow-up time of 7.1 ±â€Š4 months. For 13 patients with previous passive bone conduction implants or devices, the Osia system was universally favored. CONCLUSIONS: The new active transcutaneous bone conduction system showed favorable early clinical and audiometric outcomes. Repeated processor connectivity issues represent a potential area for future device development. This is the largest pediatric case series to date.Level of Evidence: Level 4-Retrospective Review.

Integrated microtia and aural atresia management.

Objectives: To present recommendations for the coordinated evaluation and management of the hearing and reconstructive needs of patients with microtia and aural atresia. Methods: A national working group of 9 experts on microtia and atresia evaluated a working document on the evaluation and treatment of patients. Treatment options for auricular reconstruction and hearing habilitation were reviewed and integrated into a coordinated care timeline. Results: Recommendations were created for children with microtia and atresia, including diagnostic considerations, surgical and non-surgical options for hearing management and auricular reconstruction, and the treatment timeline for each option. These recommendations are based on the collective opinion of the group and are intended for otolaryngologists, audiologists, plastic surgeons, anaplastologists, and any provider caring for a patient with microtia and ear canal atresia. Close communication between atresia/hearing reconstruction surgeon and microtia repair surgeon is strongly recommended.

Pediatric Aural Foreign Body Extraction: Comparison of Efficacies Among Clinical Settings and Retrieval Methods.

OBJECTIVES: To compare the efficacy of aural foreign body (FB) extractions among medical care settings and determine if certain methods of extraction resulted in higher failure rates and procedural complications. STUDY DESIGN: Case series with chart review. SETTING: Emergency departments (EDs), urgent cares (UCs), pediatric primary care providers (PCPs), and otolaryngologists (ENTs). METHODS: A total of 366 pediatric patients with EAC foreign bodies at a tertiary children's hospital and associated satellite clinics. RESULTS: The ED/UCs (17.46%), PCPs (75.22%), and ENTs (94.54%) exhibited highly variable success rates for pediatric aural FB extractions. Complications were considerably higher for attempts by ED/UCs (22.22%) as compared with PCPs (2.61%) and those of unclear etiology (2.73% of all attempts). Use of balloon-tipped catheters and adhesive-tipped probes (eg, cyanoacrylate/superglue) for FB extraction exhibited 0% success rates with high severity of complications. CONCLUSION: Our statistics provide an example in which ED/UCs had higher failure rates at pediatric aural FB removals when compared with PCPs and ENTs. If a single provider (PCP or ED/UC) fails to retrieve an aural FB or if the provider is not comfortable conducting the procedure, then referral to an ENT is preferable to another PCP or ED/UC. Our evidence reveals that balloon-tipped catheters and adhesive-tipped probes for FB removal in the pediatric population are prone to failure and higher rates/severity of complications.

Lingual Hamartomas: Clinical Characteristics, Diagnostic Evaluation, Treatment, and Outcomes.

OBJECTIVES/HYPOTHESIS: To further clinically characterize lingual hamartomas, and to consolidate literature by analyzing clinical presentation, diagnostic evaluation, treatment, and outcomes. STUDY DESIGN: Retrospective chart review and literature review. METHODS: Case series from 1989 to 2020 at a tertiary pediatric center; follow-up ranging from 2 months to 12 years. Patient demographics, clinical presentations, physical examination findings, surgical pathology, operative methods, pre-operative imaging, and follow-up. A review of English-language literature from 1945 to 2020 was conducted. RESULTS: Seven patients (four male, three female) with nine lingual hamartomas were identified. Average age at surgical excision was 7.9 months (SD 3.5 months, range 3 to 14 months). Follow-up ranged from 2 months to 12 years, with all patients having no recurrence. Physical examination identified five pedunculated hamartomas and two sessile hamartomas. One patient had a neck CT, one had neck ultrasound, and two had facial MRIs. CT and ultrasounds confirmed presence of cervical thyroid. MRI suggested no muscular invasion for one patient, whereas motion artifact obscured findings for the other patient. Most patients who presented with dysphagia or poor weight gain achieved post-surgical improvement. The present cases combined with the literature review identified 57 patients with 63 lingual hamartomas. CONCLUSIONS: Lingual hamartomas appear as hypovascular pedunculated masses near the foramen cecum. There is no demographic predisposition, and most are identified during infancy. Imaging is not necessary, unless there is concern for lingual thyroid, in which case thyroid ultrasound should be performed. Surgical excision is curative, with dysphagia resolution and low likelihood for recurrence. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2080-E2088, 2021.

Bone conduction hearing device adherence in relationship to age in pediatric unilateral congenital aural atresia.

OBJECTIVE: To characterize the adherence of bone conduction hearing devices (BCHDs) for hearing management in children with unilateral congenital aural atresia (UCAA) in relation to the age of offer and fitting. BCHD Soft Bands help predict amplification benefits before surgery can be performed beginning around five years when both hearing and parental compliance reach stability. We hypothesized device compliance might decrease with age of fitting from lack of early acclimatization. METHODS: Retrospective case series of patients less than five years old at a tertiary pediatric center's microtia clinic database, born between 2014 and 2018 with UCAA. Adherence was assessed through electronic health record note documentation at less than 1 year, 1-2 years, and greater than 2 years from fitting. The ages at offer and fitting, along with the time from offer to fitting, were recorded. RESULTS: One hundred and eight patients with UCAA were identified, including 46 patients fit with a BCHD used for further analysis. Adherence rates at 1 year, 1-2 years, and greater than 2 years were 47.8%, 30%, and 43.5%, respectively. However, there was no significant association between age offered, age fit, or time from offer to fit and adherence at all time points. Also, there was no significant difference between ages at the time of BCHD offer for those who chose not to proceed with fitting (20.9 months) compared to the age of offer in patients that were subsequently fit (13.9 months). CONCLUSION: BCHD adherence in patients less than five years old may not be affected by the age offered or fit. The time between offer and fitting was also not associated with usage. BCHDs should be offered to UCAA patients regardless of age. Further investigation in this younger age group would help expand these findings.

Image-guided surgical navigation for bone-conduction hearing device implant placement.

INTRODUCTION: For pediatric patients, bone-conduction hearing devices (BCHD) have demonstrated excellent outcomes. Unique to this population, BCHD implant surgeries can be technically challenging in children due to thinner, developing bone and syndromes with atypical anatomy. Image-guided surgical navigation (IGSN) clarifies underlying skull structure, potentially improving outcomes. IGSN is commonly used in otorhinolaryngologic surgeries, but current use in BCHD placement surgeries remains unprecedented. We report favorable results of IGSN in BCHD implantation for three children with complex otologic anatomy: two syndromic patients with variable temporal bone thickness, and one with prior mastoidectomies. The three patients each underwent a successful hearing implant surgery without significant intra- or post-surgical complications. All patients had good audiologic outcomes. METHODS AND MATERIALS: We report using IGSN to assist in BAHA or BONEBRIDGE™ implant surgery for three medically complicated patients. For stereotactic imaging, the patients each received pre-operative high-resolution CT scans using the paranasal sinus fusion protocol without contrast. The first patient was a 6-year-old male with CHARGE-associated abnormal temporal bone anatomy, atretic left auditory nerve, and bilateral chronic tympanic membrane perforation and otorrhea resulting in bilateral mixed conductive and sensorineural hearing loss. The patient thus was unable to consistently tolerate hearing aids. The second patient was an 18-year-old male with Rosai-Dorfman disease, history of bilateral chronic mastoiditis and middle ear infections, bilateral mastoidectomies, and bilateral malleus and incus removal resulting in mixed conductive and sensorineural hearing loss. The third patient was an 11-year-old male with Treacher Collins Syndrome, bilateral microtia, and bilateral atresia of the external auditory canals resulting in bilateral conductive hearing loss. RESULTS: The patients each underwent a successful hearing implant surgery without significant intra- or post-surgical complications. All patients had good audiologic outcomes. CONCLUSION: Intraoperative IGSN can be a beneficial adjunct to BCHD implant placement surgeries for pediatric patients with abnormal temporal bone anatomy. IGSN can help identify the optimal surgical implantation sites, thereby reducing the risk for major morbidities associated with BCHD implantations. Furthermore, our findings expand application of IGSN use to placement of both BAHA and BONEBRIDGE™.

A case of rare isolated agnathia and literature review.

Agnathia is a rare congenital malformation with unknown etiology characterized by absence of the mandible, microstomia, and tongue aplasia, often found to have other anomalies including holoprosencephaly. The purpose of this paper was to describe the symptoms and imaging of a case of isolated agnathia and to conduct a comprehensive literature review of reported patients with isolated agnathia. Case reports of isolated agnathia are very rare, with most infants as stillborn. We report a child's management of isolated agnathia with microstomia and tongue aplasia. A literature review was performed with focus on diagnosis, airway, and feeding management of isolated agnathia. Polyhydramnios was a common pregnancy complication reported in 25 out of the 39 patients in the case study. Five infants were stillborn, while 23 died within the neonatal period. Of the deceased infants within the neonatal period, 19 died within minutes to hours while four died within days to weeks. There are nine patients with agnathia that survived past infancy. The results of this study suggest that isolated agnathia is a rare malformation which requires a multi-disciplinary approach for airway and feeding management.