Two Cases of External Auditory Canal Osteonecrosis in Patients on Antiresorptive Therapy for Osteoporosis.

Bisphosphonates and denosumab have demonstrated overwhelmingly favorable skeletal benefit/risk profile in managing postmenopausal osteoporosis. External auditory canal osteonecrosis is a rare skeletal complication of antiresorptives previously described in 11 patients with bisphosphonate exposure and 1 bisphosphonate-naïve patient on denosumab. We present 2 patients who developed external auditory canal osteonecrosis while taking antiresorptives for postmenopausal osteoporosis; a 79-year-old asymptomatic bisphosphonate-naïve woman with 2-year exposure to denosumab, and a 64-year-old woman with otalgia after 5 years of risedronate and 5 years of denosumab treatment. Neither patient had previous exposure to glucocorticoids or local radiotherapy. Otoscopy performed by an ear/nose/throat (ENT) surgeon revealed exposed areas of bone in external auditory canal in both patients. Computed tomography of temporal bones found no evidence of bone erosion. Bone turnover markers were suppressed. Both patients ceased denosumab and were managed conservatively, with stable external auditory canal findings after 12 months. Although external auditory canal osteonecrosis is a rare skeletal complication of antiresorptive use, development of localizing symptoms in the ear should alert physicians to this rare clinical entity and prompt ENT surgical referral for early diagnosis and initiation of management.

Management of Recurrent and Delayed Post-Tonsillectomy and Adenoidectomy Hemorrhage in Children.

OBJECTIVE: To review our experience on post-tonsillectomy and/or adenoidectomy hemorrhage (PTAH) at a tertiary pediatric referral hospital and to evaluate the management and risk factors for recurrent postoperative hemorrhage and for delayed bleeding after day 14. METHODS: A retrospective chart review was performed for all pediatric patients admitted to The Children's Hospital at Westmead for PTAH between July 01, 2014, and June 30, 2019. Patients with recurrent hemorrhage and those with bleeding after day 14 were selected for subanalysis. RESULTS: Of the 291 patients admitted for PTAH, 31 (11%) patients had recurrent postoperative hemorrhage, and 11 (4%) patients had delayed bleeding after day 14. Surgical intervention for cessation of hemorrhage was required in 88 (30%) patients, including 2 patients who required return to the theater more than once. Nine (3%) patients received blood transfusions. The average number of days between bleeding episodes was 4 days. Recurrent postoperative hemorrhage occurred in 8.5% of patients who were managed operatively at their first presentation compared to 11.4% of patients who were managed nonoperatively (odds ratio: 1.1; 95% confidence interval 0.43-2.8). No association was found between abnormal coagulation profile, surgical indication, and risk of delayed postoperative hemorrhage. CONCLUSIONS: Recurrent or delayed postoperative hemorrhage represents a small proportion of children with postoperative bleeding and cannot be reliably predicted. Management of first presentations with either a conservative or a surgical approach is reasonable since the risk of recurrent of PTAH may be unrelated to the choice of management at initial presentation. Careful preoperative counseling of patients and their families is important to help set expectations in the event of PTAH.

Yolk sac tumours of the orbit and sinonasal tract.

PURPOSE: The aim of this study is to report two cases of paediatric Yolk sac tumours (YST) of the orbit and sinonasal tract, with a major review on the subject. METHODS: Two case reports along with a comprehensive retrospective literature review of all English language publications between 1974 and 2021 is presented. Literature review examined the demographics, clinical presentation and diagnostic and prognostic factors of extragonadal YSTs of the orbit and sinonasal tract. RESULTS: Orbit and sinuses are rare sites for YST, with only 25 paediatric cases reported in the literature. Extragonadal yolk sac tumours carry a significantly worse outcome than those localised to the gonads, with the 5-year survival of 66% and 81-89%, respectively. Our review found the median age of presentation to be 18 months (18 months for males and 24 months for females), and females are more commonly affected. The most common presentations were proptosis, facial swelling and ophthalmoplegia. Treatments and therefore outcomes varied in the cases due to the large time period. Of the cases reported in the last 10 years, all patients with data provided were alive and disease-free at follow-up. CONCLUSION: Sino-orbital yolk sac tumours are rare and have variable presentations, dependent on the extent of local invasion. Early diagnosis and treatment with multimodal therapy are paramount in having improved overall survival.

Misplaced Cochlear Implant Electrodes Outside the Cochlea: A Literature Review and Presentation of Radiological and Electrophysiological Findings.

HYPOTHESIS: It is possible to detect when misplacement and malposition of the cochlear implant (CI) electrode array has occurred intraoperatively through different investigations. We aim to explore the literature surrounding cochlear implant misplacements and share our personal experience with such cases to formulate a quick-reference guide that may be able to help cochlear implant teams detect misplacements early. BACKGROUND: Misplacement and malposition of a cochlear implant array can lead to poor hearing outcomes. Where misplacements go undetected during the primary surgery, patients may undergo further surgery to replace the implant array into the correct intracochlear position. METHODS: Systematic literature review on cochlear implant misplacements and malpositions and a retrospective review of our program's cases in over 6,000 CI procedures. RESULTS: Twenty-nine cases of CI misplacements are reported in the English literature. Sixteen cases of cochlear implant misplacements are reported from our institution with a rate of 0.28%. A further 12 cases of intracochlear malpositions are presented. The electrophysiological (CI electrically evoked auditory brainstem response, transimpedance matrix) and radiological (X-ray and computed tomography scan) findings from our experience are displayed in a tabulated quick-reference guide to show the possible characteristics of misplaced and malpositioned cochlear implant electrode arrays. CONCLUSION: Both intraoperative electrophysiological and radiological tests can show when the array has been misplaced or if there is an intracochlear malposition, to prompt timely intra-operative reinsertion to yield better outcomes for patients.

International Pediatric Otolaryngology Group (IPOG) Consensus Recommendations: Congenital Cholesteatoma.

OBJECTIVE: To provide recommendations to otolaryngologists and allied physicians for the comprehensive management of children who present with signs and symptoms of congenital cholesteatoma. METHODS: A two-iterative Delphi method questionnaire was used to establish expert recommendations by the members of the International Pediatric Otolaryngology Group, on the preoperative work-up, the perioperative considerations, and follow-up. RESULTS: Twenty-two members completed the survey, in 14 tertiary-care center departments representing 5 countries. The main consensual recommendations were: a precise otoscopic description of the quadrants involved, extensive audiological workup (bilateral tonal, vocal audiometry, and BERA), and a CT scan are required. Facial nerve monitoring and a combination of microscope and telescope are recommended for surgical removal. Clinical and audiological follow-up should be pursued yearly for at least 5 years. First MRI follow-up should be done at 18 months postoperatively if the removal violated the matrix. MRI follow-up duration depends on the initial extent of the cholesteatoma. CONCLUSION: The goal of preoperative and follow-up consensus from International Pediatric Otolaryngology Group participants is to help manage infants and children with congenital cholesteatoma. The operative techniques may vary, and experienced surgeons must perform these procedures.

MRI without magnet removal in neurofibromatosis type 2 patients with cochlear and auditory brainstem implants.

OBJECTIVE: To assess the impact on image quality of MRI without magnet removal in cochlear implant (CI) and auditory brainstem implant (ABI) users with neurofibromatosis type 2 (NF2). STUDY DESIGN: Prospective cohort. SETTING: Tertiary center for cochlear and auditory brainstem implantation. PATIENTS: Thirteen patients (10 ABI, 3CI) with NF2 underwent a total of 76 MRI scans. INTERVENTIONS: MRI without magnet removal. MAIN OUTCOME MEASURE: Ability to visualize the ipsilateral and contralateral cerebellopontine angles (CPAs) and internal auditory meati (IAM) with head MRI. RESULTS: Of the 76 scans, 40 were of the head, 28 of the spine and 8 of other regions. Scanning was performed with a tight head bandage and plastic card. There were no cases of altered implant function or demagnetization of the device magnet.A grading system was used to assess the view of the ipsilateral IAM-CPA. In 85% of head scans, the view was unimpaired (Grade 0). In 13%, there was distortion (Grade 1). In 2% (1 case), the view was entirely obscured by artifact (Grade 2). Views of the contralateral CPA and IAM were unimpaired in all cases. The best 3 sequences for the depiction of the ipsilateral IAM-CPA (percent graded as 0) were as follows: axial 3D inversion recovery prepared fast spoiled gradient echo (100%), 2 mm coronal T1W of the IAM-CPA (88.9%), and 2 mm axial T1W of the IAM-CPA (76.9%). CONCLUSION: MRI scanning without magnet removal is safe and well tolerated in NF2 patients with auditory implants. With appropriate MRI sequences, the image quality is not significantly impaired.

Systematic review of randomized controlled trials comparing intracapsular tonsillectomy with total tonsillectomy in a pediatric population.

OBJECTIVE: To perform a systematic literature review and data synthesis of level-1 evidence comparing recovery-related outcomes after intracapsular tonsillectomy (IT) (any technique) with those of total tonsillectomy (TT) (any technique) in a pediatric population. DATA SOURCES: Two independent reviewers searched the following databases: Ovid MEDLINE, including old MEDLINE and pre-MEDLINE, EBM reviews, Books@Ovid and Journals@Ovid, the Web of Science with Conference Proceedings, and references from indexed articles. STUDY SELECTION: Inclusion criteria were randomized controlled trials conducted on a pediatric population comparing IT performed by any technique of dissection with TT, also performed by any technique of dissection. Two independent reviewers determined included trials with difference of opinion resolved by a third reviewer. DATA EXTRACTION: Independent data extraction by 2 reviewers on the following outcomes: postoperative pain, analgesic use, recovery time, diet, bleeding rate, infection, and regrowth rate requiring further surgical intervention. DATA SYNTHESIS: Heterogeneity of outcome measures and lack of reporting of raw data precluded formal meta-analysis. For quantitative data that could be extracted, pooled data analysis was performed using nonparametric tests. CONCLUSION: Recovery-related outcomes for IT were superior to TT (secondary hemorrhage rate, number of days until pain free) in a pediatric population with obstructive symptoms (level-1 evidence).