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Objective: Telemedicine, particularly real time video-otoscopy in rural and remote Australia holds great potential in assessing and managing otology conditions. There is good evidence of store and forward images for assessment, however limited evidence exists for the use of real-time video-otoscopy. The objective of this study was to assess the validity of using real time video-otoscopy, compared to standard store and forward still image otoscopy, in a paediatric population., Method: Fifty-two paediatric tympanic membranes in 27 patients were examined and photographed by a telehealth facilitator with prior otoscope training. This occurred at two rural Western Australian health centre sites. These images were stored and forwarded to a tertiary paediatric hospital for otolaryngology department assessment on the day of real-time video-otoscopy consultation. During this consultation the same twenty-seven patients underwent real-time video-otoscopy assessment, which was recorded. Across six domains including, image quality, focus, light, cerumen amount, field of view and tympanic membrane landmarks, real-time video-otoscopy was compared against still image capture. The recording of each real-time video-otoscopy and still image tympanic membrane was assessed by two otology specialists for the ability to diagnose each as either normal or abnormal. An inter-rater reliability agreement was then calculated., Results: There was greater image adequacy across five of the six domains for real time video-otoscopy compared to standard store and forward otoscopy images. Substantial agreement in diagnosing each tympanic membrane as either normal or abnormal between each rater was evident., Conclusion: This study supports the use of real time video-otoscopy during telemedicine consultation. With greater image quality, focus, light, field of view and identification of tympanic membrane landmarks video-otoscopy compared to still images has broad clinical applications. This includes primary assessment of the tympanic membrane and post operative follow-up clinical settings. Video-otoscopy offers a promising new way to over-come barriers in delivering ear health care in rural populations., Competing Interests: Declaration of competing interest No potential conflict of interest was reported by the author(s)., (Crown Copyright © 2024. Published by Elsevier B.V. All rights reserved.)

Intracochlear schwannomas (ICS) are very rare benign tumours of the inner ear. We present histopathological proof of the extremely rare bilateral occurrence of intracochlear schwannomas with negative blood genetic testing for neurofibromatosis type 2 (NF2). Bilateral schwannomas are typically associated with the condition NF2 and this case is presumed to have either mosaicism for NF2 or sporadic development of bilateral tumours. For progressive bilateral tumour growth and associated profound hearing loss, surgical intervention via partial cochleoectomy, tumour removal, preservation of the modiolus, and simultaneous cochlear implantation with lateral wall electrode carrier with basal double electrode contacts was performed. The right side was operated on first with a 14-month gap between each side. The hearing in aided speech recognition for consonant-nucleus-consonant (CNC) phonemes in quiet improved from 57% to 83% 12 months after bilateral cochlear implantation (CI). Bilateral intracochlear schwannomas in non-NF2 patients are extremely rare but should be considered in cases of progressive bilateral hearing loss. Successful tumour removal and cochlear implantation utilizing a lateral wall electrode is possible and can achieve good hearing outcomes., (© 2023. The Author(s).)

Objective: To analyze the outcomes of the endoscopic transcanal approach for removal of early stage middle ear paraganglioma tumors (MEPT)., Study Design: Cases series with chart review., Setting: Two tertiary Australian Otology centers., Patients: Adult patients with middle ear paraganglioma tumors treated with transcanal endoscopic approach from 2/2016 to 12/2019. Tumor staging was described using the Modified Fisch-Mattox (MFM). Inclusion criteria included patients with an MFM Class A or B. Exclusion criteria included higher staged or syndromic disease., Intervention: All tumors were managed with transcanal endoscopic approach., Main Outcome Measures: Primary outcome measures included disease clearance and hearing measured according to the AAO-HNS guidelines. Secondary outcomes included complications, duration of surgery, and length of stay., Results: Ten patients underwent totally endoscopic transcanal resection of MEPT (9 female, mean age of 45.5 years, 70% were left sided). Mean tumor size was 6.1 mm (SD 3.4 mm). Five cases (50%) were classified using the MFM system as class A1, two cases were class A2, and three cases were class B1. Three cases required canalplasty for access but were completed entirely endoscopically. Nine of the 10 cases had complete audiometric data. Pre- and postoperative mean air conduction remained stable with a decrease in mean air-bone gap of 2.84 dB. Postoperative complications include one pinhole perforation. There were no facial nerve complications. Mean follow-up period was 10 months (range 4-25 mo) with all cases having resolution of pulsatile tinnitus and no tumor recurrence., Conclusion: The transcanal endoscopic approach for early stage MEPT offers excellent visualization and permits safe and effective removal of disease with the advantages of a minimally invasive technique for patient recovery., Competing Interests: The authors disclose no funding and conflicts of interest., (Copyright © 2021, Otology & Neurotology, Inc.)

Objective: To analyze the outcomes of an open anterior cervical approach and tospecifically describe a novel extended tracheotomy incision ("Key-hole technique") torepair H-type and other challenging tracheoesophageal fistulae (TOF) at a singletertiary pediatric center., Method: A retrospective chart analysis of pediatric patients (0-18 years old) who had undergone repair of TOF's between January 2006 and March 2020 were reviewed. A case series of patients who had undergone open cervical utilizing three different techniques were included. Patient demographics, surgical management and post-operative surgical outcomes including complications were evaluated., Results: During the study period, 117 pediatric patients were diagnosed and anaged with TOFs with or without oesophageal atresia. Within this group, 12 patients (10%) had anterior open cervical repair of congenital or persisting TOFs (6 males and 6 females). Eight cases (7%) had congenital Type E (known as H-type), two had type D, one type B and one type C TOF. Median gestational age was 37 weeks (range 28-41 weeks), age of presentation ranged from 1 day old to 3 years old with 67% being diagnosed within the first month of life. At the time of definitive surgery all patients had a bronchoscopy and oesophagoscopy to confirm the diagnosis, identify the level of the fistula and place a catheter through the fistula. This cases series of open anterior cervical repair of TOFs comprised of seven (58%) patients who had primary extraluminal tracheal approach, four (33%) with extended tracheotomy incision ('Key-hole' technique) and one (9%) patient with slide tracheoplasty for recurrent type C TOF in the presence of subglottic stenosis. Eleven of the twelve patients had successful open anterior cervical repair of TOF. One patient who had primary open anterior cervical repair with the 'Key-hole' technique had recurrence managed successfully with slide tracheoplasty. There were no cases of recurrent laryngeal nerve injury., Conclusion: This series demonstrates that open anterior cervical approach to correct TOFs is an effective and safe method in the majority of cases of congenital and acquired fistulae where there is no oesophageal atresia or the atresia is corrected (in the case of recurrent or second fistulae). We also present the outcomes of a novel surgical "Keyhole" technique to manage TOF fistulas via an extended-tracheotomy incision. We also found that slide tracheoplasty is an effective salvage operation in the case of complex recurrent fistulae., (Crown Copyright © 2020. Published by Elsevier B.V. All rights reserved.)

Objective: To assess long-term hearing outcomes following stapedectomy using a self-crimping shape memory nitinol prosthesis. The results were compared with those of a group of patients who received a conventional prosthesis., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: All patients who underwent stapedectomy for otosclerosis between July 2001 and November 2008 with a minimum dataset of preoperative, early postoperative (≤2 yr), and late postoperative (≥6 yr) audiometry were included. Fifty-six patients with a nitinol prosthesis and 27 patients with a titanium prosthesis met the inclusion criteria., Intervention: Stapedectomy using a nitinol or conventional prosthesis., Main Outcome Measure(s): Hearing outcomes by audiological assessment., Results: Mean duration of follow up was 9.5 (standard deviation [SD] 1.4) years in the nitinol group and 12.6 (SD 2.1) years in the titanium group. The early and late mean postoperative air-bone gaps (ABGs) were 9.7 and 9.8 dB in the nitinol group and 11.0 and 12.6 dB in the titanium group, respectively. The proportion of patients achieving an ABG less than or equal to 20 dB at early and late follow up was 96% and 96% in the nitinol group and 92% and 86% in the titanium group respectively., Conclusions: The excellent closure of the ABG achieved at early follow up remains remarkably stable up to 12 years using a self-crimping shape memory nitinol prosthesis. There is no evidence that firm fixation of the hook around the long process of incus has a detrimental effect in the long-term.

Background: The use of dual growing rods is a fusionless surgical approach to the treatment of early onset scoliosis which aims to harness potential growth and correct spinal deformity. The purpose of this study was to compare the in-vitro biomechanical response of two different dual rod designs under axial rotation loading., Methods: Six porcine spines were dissected into seven level thoracolumbar multi-segment units. Each specimen was mounted and tested in a biaxial Instron machine, undergoing nondestructive left and right axial rotation to peak moments of 4 Nm at a constant rotation rate of 8 deg. s(-1). A motion tracking system (Optotrak) measured 3D displacements of individual vertebrae. Each spine was tested in an un-instrumented state first and then with appropriately sized semi-constrained and 'rigid' growing rods in alternating sequence. The range of motion, neutral zone size and stiffness were calculated from the moment-rotation curves and intervertebral range of motion was calculated from Optotrak data., Findings: Irrespective of test sequence, rigid rods showed a significant reduction of total rotation across all instrumented levels (with increased stiffness) whilst semi-constrained rods exhibited similar rotational behavior to the un-instrumented spines (P<0.05). An 11.1% and 8.0% increase in stiffness for left and right axial rotation respectively and 14.9% reduction in total range of motion were recorded with dual rigid rods compared with semi-constrained rods., Interpretation: Based on these findings, the Semi-constrained growing rods were shown to not increase axial rotation stiffness compared with un-instrumented spines. This is thought to provide a more physiological environment for the growing spine compared to dual rigid rod constructs., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Pex11p (formerly Pmp27) has been implicated in peroxisomal proliferation (Erdmann, R., and G. Blobel. 1995. J. Cell Biol. 128; 509-523; Marshall, P.A., Y.I. Krimkevich, R.H. Lark, J.M. Dyer, M. Veenhuis, and J.M. Goodman, 1995. J. Cell Biol. 129; 345-355). In its absence, peroxisomes in Saccharomyces cerevisiae fail to proliferate in response to oleic acid; instead, one or two large peroxisomes are formed. Conversely, overproduction of Pex11p causes an increase in peroxisomal number. In this report, we confirm the function of Pex11p in organelle proliferation by demonstrating that this protein can cause fragmentation in vivo of large peroxisomes into smaller organelles. Pex11p is on the inner surface of the peroxisomal membrane. It can form homodimers, and this species is more abundant in mature peroxisomes than in proliferating organelles. Removing one of the three cysteines in the protein inhibits homodimerization. This cysteine 3-->alanine mutation leads to an increase in number and a decrease in peroxisomal density, compared with the wild-type protein, in response to oleic acid. We propose that the active species is the "monomeric" form, and that the increasing oxidative metabolism within maturing peroxisomes causes dimer formation and inhibition of further organelle division.