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.

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.

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.

Novel treatment of a septal ulceration using an extracellular matrix scaffold (septal ulceration treatment using ECM).

BACKGROUND: Septal ulceration is a mucositis involving the mucous membranes of the nasal septum. Patients often complain of nasal irritation, crusting, and epistaxis. Presently, there is no gold standard for the treatment of septal ulcerations. Currently described therapies include local debridement, septal dermoplasty, septal flap reconstruction, and cadaveric dermal graft repair; however, no therapy has demonstrated a consistent improvement of symptoms. We present a novel approach for the treatment of chronic septal ulceration, using an extracellular matrix scaffold (MatriStem® Wound Care Matrix, ACell, Inc.) to repair unilateral partial septal mucosal defects. METHODS: This is a retrospective chart review of three patients with age range from 42 to 74years. All three patients underwent several years of unsuccessful conservative medical management and two patients had prior unsuccessful septoplasty and septal ulcer debridement procedure. There are no complications noted in the post-operative period. RESULT: All three patients had complete symptom relief on post-operative visit after chronic septal ulceration repair using an extracellular matrix scaffold mechanism. Patients were able to manage with conservative nasal regiment after surgery with significant improvement on quality of life. CONCLUSION: The use of extracellular matrix scaffolding provides the nasal septum with a framework for the in-growth of healthy mucosa over ulcerated areas. We propose this as a new treatment approach for patients who failed conservative medical management. Chronic septal ulcerations can be healed to provide improved quality of life to patients.

A nasal mucocele originating from complex facial fractures.

Mucoceles are benign, epithelial-lined mucous cysts. Commonly mucoceles form secondary to obstruction of a sinus outflow tract or from mucosal gland entrapment from chronic infection, inflammation, iatrogenic trauma, external trauma, or neoplasm. We present a rare case of a nasal mucocele in a 37-year old male arising from a remote history of maxillofacial trauma. To our knowledge, mucoceles associated with nasal bone fractures have not been reported in the literature.

Lateral arm microvascular free tissue reconstruction of a large neck keloid.

Keloid scars pose a surgical challenge in the region of the head and neck. We present a rare case of a large infected and recurrent neck keloid that failed multiple prior reconstructive attempts. Ultimately, the tissue was resected and the surgical bed was repaired with a lateral arm microvascular free flap. This repair emphasizes the need for adequate coverage of the defect while maintaining neck mobility, ease of swallowing, and cosmesis. This is the second report in the literature of free tissue transfer as treatment for a large keloid scar, and the first to show the feasibility of early post operative low dose irradiation of the surgical bed.

Hearing and vertigo outcomes after congenital labyrinthine cholesteatoma resection.

Congenital labyrinthine cholesteatoma can cause varying degrees of hearing loss and vertigo. Hearing preservation is not always possible when treatment involves total labyrinthectomy. Since 1969, there have been several case reports and case series documenting hearing preservation following surgical treatment of congenital labyrinthine cholesteatoma. However, none of the case reports or case series documents the patients' vestibular complaints after surgery. Herein, we report a case series of four patients who were treated with the goal of hearing preservation and resolution of vertigo after partial or complete removal of the bony labyrinth for congenital inner ear cholesteatoma. We will also discuss possible explanations for the mechanisms of preservation of hearing and vestibular function with a review of the current literature.

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.

Adenocarcinoma of the anterior tongue: a case report.

Adenocarcinoma of the minor salivary gland more commonly involves the palate and base of tongue but rarely presents in the anterior tongue. We report a rare case of adenocarcinoma of the minor salivary gland located in the anterior togue of a 74-year-old man. Furthermore, we discuss the histopathological features of this neoplasm, the treatment plan, and a literature review of the current standard of care.

Foreign body impaction of the vertebral canal.

Foreign body impactions in the aerodigestive tract are common, but have the potential for serious complications. A foreign body may disrupt the mucosal lining and migrate regionally thereby risking impingement or injury to critical neurovascular structures in the cervical region. It is important to recognize potential complications that may arise from luminal compromise. In such cases, expeditious surgical treatment is warranted.

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.

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.

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.

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.

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.

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.

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.