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.

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.

Clinical, oculographic and vestibular test characteristics of Ménière's disease.

Seventy Ménière's disease (MD) patients with spontaneous vertigo (100%), unilateral aural fullness (57.1%), tinnitus (78.6%), and subjective hearing loss (75.

What Influences Decision-Making for Cochlear Implantation in Adults? Exploring Barriers and Drivers From a Multistakeholder Perspective.

Objectives: To explore the factors influencing the uptake of cochlear implants in adults, determine the impact of each factor, and to conceptualize the journey to implantation from a multistakeholder perspective.

Design: Concept mapping was used to integrate input from multiple stakeholders, each with their own experience and expertise. This mixed participatory method collects qualitative and quantitative data collection and enables further quantitative analysis.

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.

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.

Bilateral auditory ossicular expansions in a child with beta-thalassemia major: Case report and literature review.

Marrow proliferation of the ossicular chain is a rare phenomenon. To date, only two other cases have described this rarity. We report a third paediatric case from Australia.

The Clinical Uses of Electrocochleography.

The clinical uses of electrocochleography are reviewed with some technical notes on the apparatus needed to get clear recordings under different conditions. Electrocochleography can be used to estimate auditory thresholds in difficult to test children and a golf club electrode is described. The same electrode can be used to obtain electrical auditory brainstem responses (EABR).

Cochlear Implantation in Ménière's Disease With and Without Labyrinthectomy.

Objective: To investigate outcomes of cochlear implantation (CI) in patients with Ménière's disease (MD) with and without surgical labyrinthectomy.

Study Design: Retrospective study.

Setting: Multiple tertiary referral centers.

The Role of the World Health Organization's International Classification of Functioning, Health and Disability in Models of Infant Cochlear Implant Management.

Newborn hearing screening has led to the early diagnosis of hearing loss in neonates and early device fitting is common, based primarily on electrophysiologic and radiologic information, with some supplementary behavioral measures. Such early fitting of hearing devices, in particular cochlear implants (CIs), has been beneficial to the majority of children implanted under the age of 12 months who meet the cochlear implant candidacy criteria. Comorbidities are common in children with hearing loss, although they may not be evident in neonates and may not emerge until later in infants.

Cochlear Implant Outcomes in Cochlea Nerve Aplasia and Hypoplasia.

Objective: To assess cochlear implant (CI) outcomes, and factors affecting outcomes, for children with aplasia/ hypoplasia of the cochlea nerve. We also developed a new grading system for the nerves of the internal auditory meatus (IAM) and cochlea nerve classification.

Study Design: Retrospective patient review.

Music appreciation and music listening in prelingual and postlingually deaf adult cochlear implant recipients.

Objective: To explore the music appreciation of prelingually deaf adults using cochlear implants (CIs).

Design: Cohort study. Adult CI recipients were recruited based on hearing history and asked to complete the University of Canterbury Music Listening Questionnaire (UCMLQ) to assess each individual's music listening and appreciation.

Utility of bilateral acoustic hearing in combination with electrical stimulation provided by the cochlear implant.

Objective: The aim of the study was to quantify the benefit provided by having access to amplified acoustic hearing in the implanted ear for use in combination with contralateral acoustic hearing and the electrical stimulation provided by the cochlear implant.

Design: Measures of spatial and non-spatial hearing abilities were obtained to compare performance obtained with different configurations of acoustic hearing in combination with electrical stimulation. In the combined listening condition participants had access to bilateral acoustic hearing whereas the bimodal condition used acoustic hearing contralateral to the implanted ear only.

Auditory neuropathy in Brown-Vialetto-Van Laere syndrome due to riboflavin transporter RFVT2 deficiency.

Aim: Mutations in the genes encoding the riboflavin transporters RFVT2 and RFVT3 have been identified in Brown-Vialetto-Van Laere syndrome, a neurodegenerative disorder characterized by hearing loss and pontobulbar palsy. Treatment with riboflavin has been shown to benefit individuals with the phenotype of RFVT2 deficiency. Understanding the characteristics of hearing loss in riboflavin transporter deficiency would enable early diagnosis and therapy.

An epidemiological study to investigate the relationship between Meniere's disease and migraine.

Objectives: A relationship between Meniere's disease and migraine has been postulated previously. This study investigates this relationship further and determines the most influential factors for developing Meniere's disease.

Design: Epidemiological study.

A Comparative Study of Audiologic Outcomes for Two Transcutaneous Bone-Anchored Hearing Devices.

Objective: In 1977, Tjellstrom et al. used the concept of osseointegration and implanted patients with a titanium screw as part of the first bone-anchored hearing solution. Despite good hearing outcomes with the percutaneous device, there has been a drive for the development of transcutaneous bone conduction systems because of soft tissue complications, aesthetics, and loss or failure of the abutment.

CHARGE syndrome and Cochlear implantation: difficulties and outcomes in the paediatric population.

Objectives: CHARGE syndrome is a complex cluster of congenital abnormalities, these children may have absent or hypoplastic auditory nerves. Our objective was to assess preoperative factors and outcomes for paediatric cochlear implant recipients with CHARGE syndrome, to enable better surgical preparation and family counselling.

Methods: The Sydney Cochlear Implant Centre database was searched for children with CHARGE syndrome who had received a cochlear implant at ages 16 and less.

Unilateral auditory neuropathy spectrum disorder: retrocochlear lesion in disguise?

Objective: To investigate whether the aetiology for hearing impairment in neonates with unilateral auditory neuropathy spectrum disorder could be explained by structural abnormalities such as cochlear nerve aplasia, a cerebellopontine angle tumour or another identifiable lesion.

Methods: In this prospective case series, 17 neonates were diagnosed with unilateral auditory neuropathy spectrum disorder on electrophysiological testing. Diagnostic audiology testing, including auditory brainstem response testing, was supplemented with computed tomography and/or magnetic resonance imaging.

A global patient outcomes registry: Cochlear paediatric implanted recipient observational study (Cochlear(™) P-IROS).

Background: Currently, there is a paucity of data concerning the long-term outcomes, educational placement and quality of life of children implanted with hearing devices from large and representative samples of the population. To address this concern, a large, prospective, multicentre, multinational patient-outcomes registry for paediatric recipients of implantable hearing devices was developed. The benefits of this registry, its approach and methodology are described.