Improving Real-Time Feedback During Cochlear Implantation: The Auditory Nerve Neurophonic/Cochlear Microphonic Ratio.

Objectives: Real-time monitoring of cochlear function to predict the loss of residual hearing after cochlear implantation is now possible. Current approaches monitor the cochlear microphonic (CM) during implantation from the electrode at the tip of the implant. A drop in CM response of >30% is associated with poorer hearing outcomes.

Disparities among Different Countries in Access for Diagnosis and Treatment of Pediatric Sensorineural Hearing Loss.

Objective: Timely diagnosis and management of pediatric sensorineural hearing loss (SNHL) is essential for best outcomes. This study evaluated the differences of health access among cochlear implant (CI) centers of countries with different health care systems.

Study Design: Retrospective.

Gene Electrotransfer via Conductivity-Clamped Electric Field Focusing Pivots Sensori-Motor DNA Therapeutics: "A Spoonful of Sugar Helps the Medicine Go Down".

Viral vectors and lipofection-based gene therapies have dispersion-dependent transduction/transfection profiles that thwart precise targeting. The study describes the development of focused close-field gene electrotransfer (GET) technology, refining spatial control of gene expression. Integration of fluidics for precise delivery of "naked" plasmid deoxyribonucleic acid (DNA) in sucrose carrier within the focused electric field enables negative biasing of near-field conductivity ("conductivity-clamping"-CC), increasing the efficiency of plasma membrane molecular translocation.

Innominate Artery Pseudoaneurysm Requiring Cartilage Tracheal Repair in a Child.

Mycotic aneurysms are rare but potentially catastrophic. We report a case of an innominate artery pseudoaneurysm in a 4-year-old patient that caused a tracheoinnominate fistula requiring tracheoplasty with a costal cartilage graft and a homograft iliac artery replacement of the diseased innominate artery, with a successful outcome.

Clinical performance, safety, and patient-reported outcomes of an active osseointegrated bone-conduction hearing implant system at 24-month follow-up.

Purpose: To investigate 2-year post-operative hearing performance, safety, and patient-reported outcomes of hearing-impaired adults treated with the Osia 2 System, an active osseointegrated bone-conduction hearing implant that uses piezoelectric technology.

Methods: A prospective, multicenter, open-label, single-arm, within-subject clinical study conducted at three tertiary referral clinical centers located in Melbourne, Sydney and Hong Kong. Twenty adult recipients of the Osia 2 System were enrolled and followed up between 12 and 24 months post-implantation: 17 with mixed or conductive hearing loss and 3 with single-sided sensorineural deafness.

Cochlear Implant Adult Speech Perception Outcomes: Seniors Have Similar Good Outcomes.

Objective: The primary aim was to analyze the speech perception outcomes of patients with cochlear implants 65 years and older, compared with those younger than 65 years. The secondary aim was to analyze if preoperative hearing levels, severe compared with profound, had an effect on speech perception outcomes in senior citizens.

Study Design: Retrospective case review of 785 patients, between 2009 and 2016.

Cochlear nerve dysplasia in unilateral severe to profound congenital sensorineural hearing loss - Prevalence in Australian children and the impact of socioeconomic disadvantage on its management.

Introduction: Congenital unilateral sensorineural hearing loss (cuSNHL) carries potentially significant social, educational, and developmental consequences. Early diagnosis enables investigation, and consideration of options for management and early intervention, helping to mitigate the effects of hearing loss. Cochlear nerve dysplasia (CND) is a prominent cause of cuSNHL and may affect candidacy for cochlear implantation.

Distance and Socieoeconomic Status as Barriers to Cochlear Implantation.

Objective: To assess the distance burden for access to cochlear implant (CI)-related services and to assess whether socioeconomic disadvantage or level of education and occupation influenced uptake of CIs.

Study Design: Retrospective case review.

Setting: A CI services provider operating across multiple centers.

Monitoring Cochlear Health With Intracochlear Electrocochleography During Cochlear Implantation: Findings From an International Clinical Investigation.

Objectives: Electrocochleography (ECochG) is emerging as a tool for monitoring cochlear function during cochlear implant (CI) surgery. ECochG may be recorded directly from electrodes on the implant array intraoperatively. For low-frequency stimulation, its amplitude tends to rise or may plateau as the electrode is inserted.

Clinical Performance, Safety, and Patient-Reported Outcomes of an Active Osseointegrated Steady-State Implant System.

Objective: To investigate the clinical performance, safety, and patient-reported outcomes of an active osseointegrated steady-state implant system that uses piezoelectric technology.

Study Design: A prospective, multicenter, open-label, single-arm, within-subject clinical investigation.

Setting: Three tertiary referral clinical centers located in Melbourne, Sydney, and Hong Kong.

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.

To Use the Back-up Cochlear Implant or Not? Using Intra-operative Impedance to Guide Your Decisions.

Introduction: Intra-operative electrophysiological testing is being increasingly used to determine device functionality. Impedance abnormalities (open or short circuits) measured at time of surgery pose a dilemma: is it likely to resolve or is it a permanent fault? There is little in the literature on how to manage these intraoperative finding and if, at time of surgery, the back-up device should be used.

Methods: We routinely undertake impedance testing twice intraoperatively, as well as at switch on, 1 and 3 months postoperatively.

Impact of Cochlear Implantation on Canal and Otolith Function.

Objective: To quantify the impact of cochlear implantation (CI) on all five vestibular end-organs and on subjective ratings of post-CI dizziness.

Methods: Seventy-two patients undergoing unilateral CI were recruited for the study. All participants completed pre- and post-CI three-dimensional video head-impulse tests (3D vHITs) to assess semicircular-canal (SC) function, air- and bone-conducted (AC and BC) cervical and ocular vestibular-evoked myogenic potentials (cVEMPs and oVEMPs) to assess otolith-function and the dizziness handicap inventory (DHI) to measure self-perceived disability.

Ear Abnormalities Among Children with Fetal Alcohol Spectrum Disorder: A Systematic Review and Meta-Analysis.

Objective: To systematically review and conduct meta-analysis on studies that report the type and prevalence of functional and structural ear abnormalities among children with prenatal alcohol exposure and/or fetal alcohol spectrum disorder (FASD).

Study Design: MEDLINE, PubMed, Embase, Web of Science, PsycINFO, ERIC, CINAHL, and Maternity and Infant Care were searched from 1806 through March 2021. Reference lists of relevant articles were manually searched.

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.

Intraoperative Cochlear Implant Reinsertion Effects Evaluated by Electrode Impedance.

Objectives: To assess the effect on impedance levels of intraoperative reinsertion of a cochlear implant (CI) array compared with matched controls.

Study Design: Retrospective patient review.

Setting: Cochlear implant center.

A Case Series of X-Linked Deafness-2 with Sensorineural Hearing Loss, Stapes Fixation, and Perilymphatic Gusher: MR Imaging and Clinical Features of Hypothalamic Malformations.

X-linked deafness-2 (DFNX2) is an X-linked recessive disorder characterized by profound sensorineural hearing loss and a pathognomonic temporal bone deformity. Because hypothalamic malformations associated with DFNX2 have been rarely described, we aimed to further describe these lesions and compare them with features of a nonaffected population. All patients diagnosed with DFNX2 between 2006 and 2019 were included and compared with age-matched patients with normal MR imaging findings and without hypothalamic dysfunction.

Cochlear Implant Outcomes in Patients With Severe Compared With Profound Hearing Loss.

Objective: To compare the speech perception outcomes for patients with preoperative severe versus profound hearing loss with a cochlear implant (CI).

Study Design: Retrospective patient review.

Setting: Cochlear implant program.

Comparison of electrode impedance measures between a dexamethasone-eluting and standard Cochlear™ Contour Advance® electrode in adult cochlear implant recipients.

Objective: To compare the difference in electrode impedance across discrete time points to 24 months post-activation for two groups of adult cochlear implant recipients, one using an investigational perimodiolar (Contour Advance®) array augmented with 40% concentration weight per weight (w/w) dexamethasone (the Drug Eluting Electrode, 'DEE' Group), and the other the commercially available Contour Advance ('Control' Group).

Design: Ten adult subjects were implanted with the DEE and fourteen with the Control. Electrode impedances were measured intra-operatively, one-week post-surgery, at initial activation (approximately two-weeks post-surgery), and at approximately one, three, six, 12 and 24 months post-activation.

Cochlear Implant Outcomes in Large Vestibular Aqueduct Syndrome-Should We Provide Cochlear Implants Earlier?

Objective: Examine postoperative speech perception outcomes in a large vestibular aqueduct syndrome (LVAS) patients at a major cochlear implantation center.

Study Design: Retrospective analysis of the Sydney Cochlear Implant Centre (SCIC) database and medical records from January 1994 to December 2015 was performed.

Setting: Tertiary referral center.

Neurotrophin gene augmentation by electrotransfer to improve cochlear implant hearing outcomes.

This Review outlines the development of DNA-based therapeutics for treatment of hearing loss, and in particular, considers the potential to utilize the properties of recombinant neurotrophins to improve cochlear auditory (spiral ganglion) neuron survival and repair. This potential to reduce spiral ganglion neuron death and indeed re-grow the auditory nerve fibres has been the subject of considerable pre-clinical evaluation over decades with the view of improving the neural interface with cochlear implants. This provides the context for discussion about the development of a novel means of using cochlear implant electrode arrays for gene electrotransfer.

Bilateral duplication of the internal auditory canals and bilateral cochlear implant outcomes and review.

Objectives: Bilateral duplication of the internal auditory canal (IAC) is rare and is associated with profound sensorineural hearing loss. The present study aims to review our experience with bilateral cochlear implantation (CI) in children with a duplication of the IAC and to review the literature.

Methods: The Sydney Cochlear Implant Centre database was searched for children with duplication of the internal auditory canal.