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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. This enables titratable gene delivery with unprecedently low charge transfer. The clinic-ready bionics-derived CC-GET device achieved neurotrophin-encoding miniplasmid DNA delivery to the cochlea to promote auditory nerve regeneration; validated in deafened guinea pig and cat models, leading to improved central auditory tuning with bionics-based hearing. The performance of CC-GET is evaluated in the brain, an organ problematic for pulsed electric field-based plasmid DNA delivery, due to high required currents causing Joule-heating and damaging electroporation. Here CC-GET enables safe precision targeting of gene expression. In the guinea pig, reporter expression is enabled in physiologically critical brainstem regions, and in the striatum (globus pallidus region) delivery of a red-shifted channelrhodopsin and a genetically-encoded Ca 2+  sensor, achieved photoactivated neuromodulation relevant to the treatment of Parkinson's Disease and other focal brain disorders., (© 2024 The Author(s). Advanced Science published by Wiley‐VCH GmbH.)

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. Safety data, audiological thresholds, speech recognition thresholds in noise, and patient-reported outcomes were collected and evaluated. In addition, pre-and 6-month post-implantation data were collected retrospectively for this recipient cohort enrolled into the earlier study (ClinicalTrials.gov NCT04041700)., Results: Between 6- and 24-month follow-up, there was no statistically significant change in free-field hearing thresholds or speech reception thresholds in noise (p =  > 0.05), indicating that aided improvements were maintained up to 24 months of follow-up. Furthermore, improvements in health-related quality of life and daily hearing ability, as well as clinical and subjective measures of hearing benefit remained stable over the 24-month period. No serious adverse events were reported during extended follow-up., Conclusions: These study results provide further evidence to support the longer term clinical safety, hearing performance, and patient-related benefits of the Osia 2 System in patients with either a conductive hearing loss, mixed hearing loss, or single-sided sensorineural deafness., Trial Registration: ClinicalTrials.gov Identifier: NCT04754477. First posted: February 15, 2021., (© 2023. The Author(s).)

Objective: To investigate ictal nystagmus and audiovestibular characteristics in episodic spontaneous vertigo after cochlear implantation (CI)., Study Design: Retrospective and prospective case series., Patients: Twenty-one CI patients with episodic spontaneous vertigo after implantation were recruited., Interventions: Patient-initiated home video-oculography recordings were performed during one or more attacks of vertigo, using miniature portable home video-glasses. To assess canal and otolith function, video head-impulse tests (vHITs) and vestibular-evoked myogenic potential tests were conducted., Main Outcome Measures: Nystagmus slow-phase velocities (SPVs), the presence of horizontal direction-changing nystagmus, and post-CI audiovestibular tests., Results: Main final diagnoses were post-CI secondary endolymphatic hydrops (48%) and exacerbation of existing Ménière's disease (29%). Symptomatic patients demonstrated high-velocity horizontal ictal-nystagmus (SPV, 44.2°/s and 68.2°/s in post-CI secondary endolymphatic hydrop and Ménière's disease). Direction-changing nystagmus was observed in 80 and 75%. Two were diagnosed with presumed autoimmune inner ear disease (SPV, 6.6°/s and 172.9°/s). One patient was diagnosed with probable vestibular migraine (15.1°/s).VHIT gains were 0.80 ± 0.20 (lateral), 0.70 ± 0.17 (anterior), and 0.62 ± 0.27 (posterior) in the implanted ear, with abnormal values in 33, 35, and 35% of each canal. Bone-conducted cervical and ocular vestibular-evoked myogenic potentials were asymmetric in 52 and 29% of patients (all lateralized to the implanted ear) with mean asymmetry ratios of 51.2 and 35.7%. Reversible reduction in vHIT gain was recorded in three acutely symptomatic patients., Conclusion: High-velocity, direction-changing nystagmus time-locked with vertigo attacks may be observed in post-CI implant vertigo and may indicate endolymphatic hydrops. Fluctuating vHIT gain may be an additional marker of a recurrent peripheral vestibulopathy., Competing Interests: Sources of support and disclosure of funding: A.S.Y. received scholarship funding from the University of Sydney and reports no conflict of interest. B.Y.C.K. was a previous employee of Cochlear Ltd before commencing this study and holds an insignificant (<100) amount of Cochlear Ltd shares. M.S.W. receives funding from the Garnett Passe and Rodney Williams Memorial Foundation, and the National Health and Medical Research Council of Australia and reports no conflict of interest. The remaining authors declare no conflict of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

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., Setting: A large cochlear implant program., Patients: Cochlear implant adult recipients younger than 65 years and 65 years and older at the time of surgery., Interventions: Therapeutic-cochlear implant., Main Outcome Measures: Speech perception outcomes, using City University of New York (CUNY) sentences and Consonant-Nucleus-Consonant (CNC) words. Outcomes were measured preoperatively and postoperatively at 3, 6and 12 months for cohorts younger than 65 years and 65 years and older., Results: Adult recipients younger than 65 years compared with those 65 years and older had comparable outcomes for CUNY sentence scores outcomes ( p = 0.11) and CNC word scores ( p = 0.69). The preoperative four-frequency average severe hearing loss (HL) cohort was significantly better compared with the profound HL cohort, for both the CUNY sentence scores ( p < 0.001) and CNC word scores ( p < 0.0001). The four-frequency average severe HL cohort had better outcomes irrespective of age., Conclusions: Senior citizens have similarly good speech perception outcomes as adults younger than 65 years. Those with preoperative severe HL have better outcomes than profound loss. These finds are reassuring and can be used when counseling older cochlear implant candidates., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

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.

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., Patients: All patients undergoing CI surgery in a 2-year period between March 2018 and February 2020., Interventions: Diagnosis of hearing loss, CI surgery, and subsequent habilitation and mapping., Main Outcome Measures: Distance traveled by patients to their audiological diagnostic, CI surgery hospital, and habilitation sites; subjects' index of relative socioeconomic advantage and disadvantage (IRSAD) and index of education and occupation (IEO)., Results: n = 201 children and n = 623 adults. There was a significant difference across IRSAD domains for children (p < 0.0001) and adults (p < 0.0001), and IEO in children (p = 0.015) and adults (p < 0.0001) when tested for equal proportions. The median driving distance from home to the diagnostic audiological site for children was 20 km (mean, 69 km; range, 1-1184 km; upper quartile, 79 km; lower quartile, 8 km). There was no significant difference between the driving distances from home to the CI surgery hospital site, or the mapping/habilitation sites between children and adults. There was no correlation for age at first surgery and either IRSAD/IEO., Conclusions: The burden of distance for access to CI in Australia is significant for the upper quartile who may not live within the large city centers. Greater consideration needs to be given regarding barriers to CI for those in lower socioeconomic and educational groups to ensure equity of access across different socioeconomic and educational level backgrounds., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

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. Socioeconomic disadvantage may impact on a patient's family's capacity to participate in necessary intervention and follow-up., Methods: Infants with severe-profound cuSNHL referred to a large Australian quaternary pediatric center between October 2004 and December 2020 were retrospectively included. Audiometric and clinical data, and the presence of hearing loss risk factors were obtained from a prospectively collated database. In Australia MRI scans are provided free-of-charge to citizens and residents. MRI scans were reviewed to determine the status of the nerves within the internal acoustic meatus (IAM grade) along with attendance rates. Travel distance to the hospital was also calculated. Reasons for non-attendance at MRI were obtained from patient medical records and correspondence. Socioeconomic, educational, and occupational indices, and travel distances were obtained using patient residential postcodes with reference to Australian Bureau of Statistics data., Results: A total of 98 patients were reviewed, 64.3% (n = 63) of whom underwent MRI. The median age at diagnosis was 40 days (IQR 27). The prevalence of CND was 75% (n = 47). Importantly, there was no significant difference in the degree of hearing loss between IAM grades (F(4,57) = 1.029, p = 0.405). Socioeconomic indices were significantly lower in patients not attending MRI investigations compared with patients who did attend. Travel distance was not significantly different between the two groups., Conclusion: CND is a prominent cause of cuSNHL in Australian infants. MRI at a young age allows parent education regarding management options and timely intervention where indicated. Socioeconomic disadvantage significantly impacts on participation in further routine assessment of cuSNHL, potentially limiting management options for these children long term., Competing Interests: Declaration of competing interest The authors have no competing interests to declare., (Copyright © 2023 Elsevier B.V. All rights reserved.)

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., Patients: Twenty-nine adult subjects, 24 with mixed hearing loss or conductive hearing loss and 5 with single-sided sensorineural deafness., Intervention: Implantation with the Cochlear Osia 2 System., Main Outcome Measures: Audiological threshold evaluation and speech recognition in quiet and in noise. Patient satisfaction and safety., Results: At 6-month follow-up after surgery, a mean improvement in pure-tone average of 26.0 dB hearing level and a mean improvement of 8.8 dB signal-to-noise ratio in speech reception threshold in noise was achieved with the investigational device as compared with the unaided situation. Usability of the investigational device was rated 71.4/100 mm for sound processor retention and 81.4/100 mm for overall comfort using a visual analog scale., Conclusion: These outcomes confirm the clinical safety, performance, and benefit of an innovative active transcutaneous bone conduction implant using a piezoelectric transducer design in subjects with conductive hearing loss, mixed hearing loss, or single-sided sensorineural deafness., (Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Otology & Neurotology, Inc.)

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., Competing Interests: The authors disclose no conflicts of interests., (Copyright © 2022, Otology & Neurotology, Inc.)

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. Retrospective impedance thresholds were analysed for one surgeon's cases between January 2018 and December 2019., Results: There were 235 cochlear implants performed for 217 patients (5,020 electrode contacts) analysed. Thirty-three electrodes had abnormal impedance thresholds on first intraoperative cycle of testing, 76% resolving with the second testing cycle electrode contacts that demonstrated abnormal impedance during both intraoperative test cycles were 16.54 times (95%CI 2.55-107.13, p = 0.003) more likely to be abnormal at three months. Fifty percent resolved by switch on. The intraoperative abnormalities made up 26% of electrode abnormalities seen at 3 months postoperatively., Discussion: This study demonstrates the utility of 2 cycles of intraoperative impedance testing, with persistently abnormal electrodes having 16 times the likelihood of persistent abnormalities of impedance, and 50% resolution. These persistent intra-operative abnormal electrodes are responsibly for 26% of electrode abnormalities at 3 months. This information is useful for the surgeon when considering use of the backup cochlear implant device., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

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., Results: Nineteen percent of patients reported new or worsened dizziness postsurgery. Post-CI abnormalities (new lesions and significant deteriorations) were seen in the AC cVEMP (48%), AC oVEMP (34%), BC cVEMP (10%), and BC oVEMP (7%); and lateral (L) (17%), posterior (P) (10%), and anterior (A) (13%) SC vHITs. CI surgery was more likely to affect the AC cVEMP compared with the other tests (χ2 test, p < 0.05). Fifty percent of patients reported no dizziness pre- and postsurgery. In the implanted ear, normal pre-CI vHIT gain was preserved in lateral semicircular canal (LSC) (69%), anterior semicircular canal (ASC) (74%), and posterior semicircular canal (PSC) (67%), and normal reflex amplitudes were found in AC cVEMP (25%), AC oVEMP (20%), BC cVEMP (59%), and BC oVEMP (74%). Statistically significant decreases were observed in LSC vHIT gain, AC cVEMP amplitude, and AC oVEMP amplitude postsurgery (p < 0.05). There was a significant moderate positive correlation between change in DHI scores and the summed vestibular deficit postsurgery (r(51) = 0.38, p < 0.05)., Conclusion: CI can impact tests that assess all five vestibular end-organs and subjective ratings of dizziness. These results support pre and post-surgical vestibular testing and assist preoperative counseling and candidate selection., Competing Interests: Conflict of Interest and Source of Funding: A.Y. received scholarship funding from the University of Sydney and receives research funding from the Garnett Passe and Rodney Williams Memorial Foundation and reports no conflict of interest. B.K. was a previous employee of Cochlear Ltd. prior to commencing this study and reports no conflict of interest. M.W. receives funding from the Garnett Passe and Rodney Williams Memorial Foundation, and the National Health and Medical Research Council of Australia, and reports no conflict of interest. The remaining authors declare no conflict of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

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., Patients: CI recipients in the Sydney Cochlear Implant Centre (SCIC) database who required intraoperative array reinsertion and matched controls. Exclusion criteria; known preceding meningitis or labyrinthitis ossificans; electrode array buckling; incomplete "final" insertion., Intervention: Cochlear implantation., Main Outcome Measures: Impedance values measured intraoperatively, at switch on, 3 months, 6 months, and 12 months postoperatively were analyzed. The Generalized Estimating Equation (GEE) Model was used to compare cases with controls for each device, at each time point, and for each channel., Results: Thirty-one reinsertion cases identified; six CI 422 arrays; 14 CI 24RE (ST) arrays, and 11 CI 512 arrays. No increase in impedance levels was found in the reinsertion cases when compared with their matched controls. The only statistical difference in impedance was seen in the CI 422 cohort at switch on with the reinsertion cases having lower impedances (p = 0.03)., Conclusion: This is the first study to examine impedance values in patients who underwent intraoperative CI array reinsertion and to compare them with the impedances of matched controls. No significant increase found in impedances between our reinsertion cases and matched controls, suggesting the reinsertion did not result in any additional trauma or inflammation. This has implications for surgery both in routine cases such as a faulty electrode and also for future design of mechanisms for delivery of intracochlear therapies.

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., Patients: Cochlear implant adult recipients (16 yr and above) having surgery between 2008 and 2015 with speech perception results and four frequency averaged severe (70-89 dBHL) or profound (90 dBHL and above) hearing loss. Prelingual deaf adults were included in the data., Intervention: Cochlear implant., Main Outcome Measures: Speech perception scores with CUNY sentences and monosyllabic (CNC/CVC) word scores at preoperative and 3, 6, and 12 months postoperatively testing. Mann-Whitney U test was performed to compare outcomes of the two groups. Interquartile comparisons were also made., Results: The severe group had significantly better speech perception than the profound hearing loss group for CUNY sentences and CNC/CVC word scores preoperatively (p < 0.001) (p < 0.001), at 6 months (p < 0.001) (p < 0.001), and at 12 months (p < 0.01) (p < 0.001), respectively. At 3 months there was no significant difference. The number of patients in each severe or profound group at the different time points ranged from 92 to 367 patients for CUNY sentences and from 52 to 216 patients for the word scores. The 12 months' lower quartile score for CUNY sentences for severe and profound groups was 83% and 75% respectively. The lower quartile score for words was 32% and 26% respectively., Conclusion: Adult CI recipients showed marked improvements in speech perception with a CI. Those with severe hearing loss have significantly better outcomes compared with profound hearing loss patients. These outcomes can inform CI candidacy evaluation criteria.

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. Mesenchymal cells which line the cochlear perilymphatic compartment can be selectively transfected with (naked) plasmid DNA using array - based gene electrotransfer, termed 'close-field electroporation'. This technology is able to drive expression of brain derived neurotrophic factor (BDNF) in the deafened guinea pig model, causing re-growth of the spiral ganglion peripheral neurites towards the mesenchymla cells, and hence into close proximity with cochlear implant electrodes within scala tympani. This was associated with functional enhancement of the cochlear implant neural interface (lower neural recruitment thresholds and expanded dynamic range, measured using electrically - evoked auditory brainstem responses). The basis for the efficiency of close-field electroporation arises from the compression of the electric field in proximity to the ganged cochlear implant electrodes. The regions close to the array with highest field strength corresponded closely to the distribution of bioreporter cells (adherent human embryonic kidney (HEK293)) expressing green fluorescent reporter protein (GFP) following gene electrotransfer. The optimization of the gene electrotransfer parameters using this cell-based model correlated closely with in vitro and in vivo cochlear gene delivery outcomes. The migration of the cochlear implant electrode array-based gene electrotransfer platform towards a clinical trial for neurotrophin-based enhancement of cochlear implants is supported by availability of a novel regulatory compliant mini-plasmid DNA backbone (pFAR4; plasmid Free of Antibiotic Resistance v.4) which could be used to package a 'humanized' neurotrophin expression cassette. A reporter cassette packaged into pFAR4 produced prominent GFP expression in the guinea pig basal turn perilymphatic scalae. More broadly, close-field gene electrotransfer may lend itself to a spectrum of potential DNA therapeutics applications benefitting from titratable, localised, delivery of naked DNA, for gene augmentation, targeted gene regulation, or gene substitution strategies., (Copyright © 2019 Elsevier B.V. All rights reserved.)

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., Patients: Patients with a diagnosis of LVAS who received a cochlear implant (CI). Only those with speech perception outcomes recorded at least 12 months post implant were included in our analysis., Intervention(s): Therapeutic., Main Outcome Measure(s): Postoperative speech perception scores., Results: Between 1994 and 2015, 176 adult and pediatric patients with a diagnosis of LVAS underwent cochlear implantation at SCIC. Postoperative Bamford-Kowal Bench (BKB) sentence test scores were obtained for 97 patients. The postoperative median BKB score was 93% with a lower quartile score of 85% and an upper quartile score of 98%. Smaller numbers were available for post-CI City University of New York (CUNY) and Consonant-Nucleus-Consonant (CNC) word scores yet similar excellent results were seen., Conclusions: Our study results suggest the CI should be considered when BKB scores have dropped to 85%. We suggest that rather than LVAS cases representing a challenge to cochlear implantation, they are amongst the best candidates for surgery, and should receive a CI at an earlier stage in hearing loss, when they have better speech perception. This allows stable hearing to be established earlier along with excellent speech perception outcomes.

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. Data was collected regarding clinical history, MRI and CT findings, auditory brainstem responses (ABR), tympanometry and otoacoustic emissions (OAE), visually reinforced orientation audiometry, auditory brainstem response, electrocochleography (ECochG), transtympanic electrical auditory brainstem response (ABR), aided cortical evoked potentials (CAEP) and intraoperative neural response telemetry (NRT) and CI evoked electrical auditory brainstem testing., Results: two children with bilateral duplication of the IAC were identified who successfully underwent bilateral cochlear implantation. Audiological development was monitored for 2 and 3 years respectively, both children could spontaneously verbalise and displayed Categories of Auditory Performance (CAP) score of 5 and 6 respectively., Conclusion: Children with duplication of the IAC, with accompanying cochlear nerve dysplasia (CND) can benefit from CI surgery, and verbal receptive and expressive language is possible., (Copyright © 2019. Published by Elsevier B.V.)

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. A seven-year-old with thalassemia major demonstrated conductive impairment during surveillance for Deferasirox ototoxicity. Otitis media was assumed, however, CT scan of the petrous temporal bone revealed extramedullary haematopoiesis causing bilateral ossicular expansions and fixed conductive deficit. Reports of hearing loss in the thalassemia population focus on sensorineural impairment from iron chelation therapies. Clinicians should suspect ossicular deformation where treatment has been delayed, poorly controlled or conductive deficit persists without effusion., (Crown Copyright © 2018. Published by Elsevier B.V. All rights reserved.)

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., Setting: Tertiary referral hospital and cochlear implant program., Patients: Children 0 to 16 years inclusive with a CI who had absent/hypoplastic cochlea nerve on magnetic resonance imaging (MRI)., Intervention: Cochlear implant., Main Outcome Measures: MRI, trans-tympanic electrical auditory brainstem response, intraoperative electrical auditory brainstem response, Neural Response Telemetry, Categories of Auditory Perception score, Main mode of communication., Results: Fifty CI recipients (26 males and 24 females) were identified, 21 had bilateral CIs, 27 had developmental delay. MRI showed cochlea nerve aplasia in 64 ears, hypoplasia in 25 ears, and a normal nerve in 11 ears. Main mode of communication was analyzed for 41 children: 21 (51%) used verbal language (15 speech alone, 5 speech plus some sign, 1 bilingual in speech and sign), and 20 (49%) used sign language (10 sign alone, 9 sign plus some speech, 1 tactile sign). Seventy-three percent of children used some verbal language. Cochlea nerve aplasia/ hypoplasia and developmental delay were both significant factors affecting main mode of communication. Categories of Auditory Performance scores were available for 59 CI ears; 47% with CN Aplasia (IAM nerve grades 0-III) and 89% with CN hypoplasia (IAM nerve grade IV) achieved Categories of Auditory Performance scores of 5 to 7 (some verbal understanding) (p = 0.003)., Conclusion: Our results are encouraging and useful when counselling families regarding the likelihood of language outcomes and auditory understanding.

Primary Ciliary Dyskinesia (PCD) is an autosomal recessive genetic condition affecting the function of motile cilia. The upper respiratory tract is lined with ciliated epithelium and hence a hallmark of PCD is the development, from the neonatal period onwards, of persisting secretion retention and suppurative infection in the middle ear, nose and facial sinuses [1]. This review aims to remind the clinician involved in the care of a patient with PCD of the complexities of making the diagnosis of chronic rhinosinusitis (CRS) and chronic otitis media with effusion (ChOME), the morbidity associated with CRS and ChOME and of current evidence of best practice for the management of these conditions., (Crown Copyright © 2015. Published by Elsevier Ltd. All rights reserved.)

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. This study compares audiologic and quality of life questionnaire outcomes (author-designed questionnaire) for two transcutaneous bone conduction implants., Study Design: Cross-sectional cohort study., Setting: Tertiary referral hospital and national audiology service., Patients: Twelve patients, 10 children and two adults. Six recipients of each device., Main Outcome Measures: Pure-tone audiometry, aided thresholds, speech discrimination scores, and quality of life questionnaire results., Results: The unaided four-frequency average air conduction for affected ears was 60.8 dB HL for the Attract group and 57.8 dBHL for the Sophono group; these improved to mean aided thresholds of 30.6 dB HL and 29.8 dB HL, respectively. Therewas no statistical difference between the speech discrimination scores for the two devices in quiet at 55 dB SPL (p = 0.33) orin noise (p = 0.87)., Conclusion: Both systems provide audiologic benefit compared with the unaided situation. Comparing small numbers of patients, there was no significant difference in aided thresholds or speech discrimination scores between these two transcutaneous bone-anchored implants. All Attract and Sophono users reported improvement in quality of life and would recommend their device to others in a similar situation.

Aim: Primary ciliary dyskinesia (PCD) is a rare (1:15,000) condition resulting in recurrent suppurative respiratory tract infections, progressive lung damage and hearing impairment. As the diagnosis is often delayed for years, the purpose of this study was to review the presenting features of children with PCD attending Australia's initial diagnostic PCD service over a 30-year period., Method: A retrospective review of the symptoms of children diagnosed with PCD at Concord Hospital between 1982 and 2012 was undertaken., Results: One thousand thirty-seven paediatric patients were referred for assessment and underwent nasal ciliary brushing. Eighty-four (8.1%) had PCD based on microscopic analysis of nasal cilia. This included 81 with ciliary ultrastructural abnormalities demonstrated on electron microscopy and 3 with a suggestive phenotype, reduced ciliary beat frequency and a family history of PCD. The median age at diagnosis was 6.4 years (range 0.1 to 18.2 years). Forty-six per cent had situs abnormalities and 31% had a family member with PCD. Recurrent cough (81%), rhinosinusitis (71%), recurrent otitis media (49%) and neonatal respiratory distress (57%) were reported. Bronchiectasis at presentation was documented in 32%. Situs abnormalities and neonatal respiratory distress were present together in 26%., Conclusion: PCD remains under-recognised by health-care workers. The combination of neonatal respiratory distress, chronic suppurative cough and rhinosinusitis was the most common documented symptom cluster at presentation in cases of PCD. A heightened awareness of the clinical features of the disease may help to lower the age at diagnosis, facilitate appropriate treatment and improve long-term outcomes., (© 2014 The Authors. Journal of Paediatrics and Child Health © 2014 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

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. Data were collected regarding clinical history; hearing assessments; MRI and CT scan findings; preoperative transtympanic electrical Auditory Brainstem Response (ABR); intraoperative findings and intraoperative electrical ABR and Neural Response Telemetry; and language outcomes in terms of main language used and Categories of Auditory Performance scores (0-7 ranking)., Results: Ten children were identified. All seven prelingual profoundly deaf children with CHARGE syndrome had hypoplastic or absent auditory nerves bilaterally on MRI scans. Middle ear anatomy was often abnormal, affecting surgical landmarks and making identification of the cochlea very difficult in some cases. Three cases required repeated surgery to obtain successful cochlear implant insertion, one under CT scan image guided technique. All seven children used sign language, or simpler gestures, as their main mode of communication. Two children of of these children, who were implanted early, also attained some spoken language. CAP scores ranged from 0 to 6. The three children with CHARGE syndrome and progressive sensorineural hearing loss had a normal auditory nerve in at least one ear on MRI scans. All had preoperative verbal language, with CAP scores of 6, and continued with CAP scores of 6 following receipt of the cochlear implant., Conclusion: Children with CHARGE and congenital profound hearing loss all had hypoplasia or absent auditory nerves, affecting their outcomes with cochlear implants. They often had markedly abnormal middle ear anatomy and CT image guided surgery can be helpful. These children should be offered a bilingual early intervention approach, using sign language and verbal language, to ensure best language outcomes. Children with CHARGE syndrome and progressive profound hearing loss did well with cochlear implants and continue to be able to use verbal language., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Objective: To prospectively document the surgical pain, assessing analgesia use as a proxy, and postoperative dizziness in children over the first week after cochlear implantation., Study Design: Prospective., Setting: Tertiary referral hospital and cochlear implant program., Patients: Children aged 0 to 16 years inclusive undergoing cochlear implant surgery, who returned to see the primary surgeon for the postoperative 1-week follow-up appointment., Interventions: One-week postoperative collection of data via direct questioning of parents and children., Main Outcome Measures: Analgesia use, duration of analgesia use and dizziness (nil, slight, or moderate), type of surgery, and radiologic findings., Results: Data were available for 61 of 98 children aged 5 months to 15 years. Children underwent first side implant (n = 27), sequential second side implant (n = 15), bilateral simultaneous (n = 16), and explant reimplantation (n = 3). On average, children used paracetamol for only 1.9 days after discharge from the hospital. Longer average paracetamol use was associated with bilateral simultaneous surgery (3.3 d after discharge from the hospital) and also the younger age group of 0 to 12 months (3.2 d). Slight dizziness was reported by 8% of all children at 1 week postsurgery.No child had marked dizziness or unsteadiness. Four children had large vestibular aqueducts on radiology scans, two (50%) of these children has slight unsteadiness at 1 week postoperatively., Conclusion: Our study shows cochlear implant surgery is well tolerated by children. This information enables better counseling of families and children considering cochlear implantation.

Objectives: The aim of this study was to assess if large vestibular aqueduct syndrome (LVAS), with the increase in perilymphatic pressure, affects impedance changes over time with different types of Cochlear(®) implant electrode arrays Contour, Straight, and CI 422. To report speech perception outcomes for these cochlear implant recipients., Methods: Retrospective case review of impedance levels and categories of auditory performance. Impedance data were collected at switch on, 1 month, 3, 6, 12, and 24 months after cochlear implantation and compared with control (non-LVAS cochlear implant recipient) data for each array type. Forty-seven patients with exclusive LVAS and no other vestibulocochlear abnormalities or other identifiable cause of deafness were eligible for inclusion in the study., Results: In LVAS patients, there was a significant difference in impedance between the three types of device (P < 0.0001). Time since switch on was associated with a decrease in impedance for all three devices (P < 0.0001). The mean impedance reduced between switch on and 1 month and remained relatively constant thereafter. Sound variation with softening of sounds was seen in four CI 422 (Straight Research Array) recipients due to ongoing fluctuations in electrode compliance., Discussion: For all three array types, there was no significant difference in the mean impedance between the LVAS patients and controls over the first 12 months. In keeping with previous studies cochlear implant recipients with LVAS hear very well through the cochlear implant.

Primary ciliary dyskinesia (PCD) is a multi-organ disorder associated with chronic oto-sino-pulmonary disease, neonatal respiratory distress, situs abnormalities and reduced fertility. Repeated respiratory tract infections leads to the almost universal development of bronchiectasis. These clinical manifestations are a consequence of poorly functioning motile cilia. However, confirming the diagnosis is quite difficult and is often delayed, so the true incidence of PCD may be significantly higher than current estimates. Nasal nitric oxide has been earmarked as a useful screening tool for identifying patients, but its use is limited in pre-school-aged children. Due to the rarity of PCD, the evidence base for management is somewhat limited, and treatment regimens are extrapolated from other suppurative lung disorders, like cystic fibrosis., (© 2014 The Authors. Journal of Paediatrics and Child Health © 2014 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

Objective: To compare mean impedance levels, neural response telemetry (NRT), and auditory perception after initial and explant-reimplant pediatric cochlear implants., Study Design: Retrospective case review., Setting: Tertiary referral hospital and cochlear implant program., Patients: Children 0 to 16 years inclusive who have undergone explant-reimplant of their cochlear implant., Intervention: Impedance levels, NRT, and speech perception performance., Main Outcome Measures: Impedance, NRT, and auditory perception at switch on, 3 months, 12 months, 3 years, and 5 years after initial cochlear implant and reimplantation., Results: The explant-reimplant group receiving Cochlear contour array had significantly (p < 0.001) raised impedance at switch on, 3 months, 12 months, and 3 years, compared with their initial implant. The explant-reimplant group receiving Cochlear straight array had marginally significant (p = 0.045) raised impedance at switch on, 3 months, 12 months, and 3 and 5 years. Infection was associated with greater increases in impedance in the reimplant Contour group. NRT was increased in the explant-reimplant group but not significantly (p = 0.06). Auditory perception returned to preexplant levels within 6 months in 61% of children., Conclusion: Impedance is higher after explant-reimplant and remains increased for years after explant-reimplant with Cochlear contour and to a lesser degree the straight array device.

Objective: Few studies exist on children with common cavity, fewer still on their long-term audiological development after having received a cochlear implant. Our goal was to observe and report the long-term audiological progress of children with common cavity who were implanted with a custom-made electrode., Methods: In this longitudinal, multi-center study, 19 children were implanted with a MED-EL custom-made electrode via either single slit cochleostomy or double posterior labyrinthotomy. We observed their audiological development with a test battery consisting of Categories of Auditory Performance (CAP), Speech Intelligibility Rating (SIR), and Ling 6-Sounds tests. We tested the children 1 month prior to the surgery; at first fitting; at 1, 3, 6, 12, and 18 months post first-fitting; at 2 years after first-fitting; and, whenever possible, at 3, 4, and 5 years after first-fitting., Results: Children with common cavity tend to steadily and significantly improve their audiological skills over time. This development may, however, be highly individual; probably in part due to relatively high levels of additional needs. Parents should be counseled to establish realistic post-implantation expectations. Surgically, contrary to our expectations, we cannot confirm that double posterior labyrinthotomy reduces intracochlear electrode movement or that the MED-EL custom electrode leads to fewer incidences of intra- or post-implantation complications., Conclusions: Cochlear implantation is a safe and effective treatment option in children with common cavity. The majority of children with CC derive significant audiological benefit from implantation. Intra- and post-surgical complications, while serious, and be dealt with effectively in most cases., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Objective: Primary ciliary dyskinesia is an autosomal recessively inherited group of disorders of ciliary ultrastructure. Otolaryngologists are frequently involved in the management of some of the most common symptoms of primary ciliary dyskinesia including chronic rhinitis, sinusitis and otitis media with effusion. A dilemma for otorhinolaryngologists is whether ventilation tubes are of benefit in children with primary ciliary dyskinesia and otitis media with effusion and what effective alternatives exist. This paper aims to address this issue via a literature review and case presentation., Methods: An extensive review of the literature was undertaken and a discussion of the advantages and disadvantages of ventilation tubes in the management of otitis media with effusion in these children is presented and compared with that of the general population. We present a case of a 9 months old boy with Kartagener's Syndrome and chronic bilateral otitis media with effusion to illustrate our findings., Results: Eight papers were identified, all with small study numbers. The main outcome measures were hearing, otorrhoea and tympanic membrane structural changes. The natural history of otitis media with effusion and hearing loss in primary ciliary dyskinesia appears to be fluctuant into adulthood. Therefore, otitis media with effusion in primary ciliary dyskinesia does not resolve by the age of 9 years, regardless of treatment, as previously assumed. Ventilation tube insertion improves hearing in primary ciliary dyskinesia, but may lead to a higher rate of otorrhoea when compared to the general population. Tympanic membrane changes were clinically insignificant. Our patient eventually underwent successful insertion of bilateral ventilation tubes with a marked improvement in hearing and language with minimal otorrhoea., Conclusion/discussion: The highest level of evidence found for the management of otitis media with effusion in children with primary ciliary dyskinesia was level IV. Currently, the evidence is inconclusive and conflicting. Whilst our results are promising, clearly higher quality research on a larger number of patients is required to definitively evaluate the management options for otitis media with effusion in these children.

This paper describes the abnormality of a large internal auditory meatus (LIAM). Computed tomography (CT) scans show the otic capsule to be affected by a widened, bulbar internal auditory meatus with loss of or reduction of the bony wall dividing the lateral fundus of the meatus from the cochlea. The vestibule is abnormally dilated. We report five cases of children with LIAM and profound hearing loss. Three of these children are girls and two children were boys. Three had congenital progressive hearing loss, one of these had an accompanying large vestibular aqueduct and dysplasia of the cochlea. Two patients had had meningitis resulting in profound loss.

Chronic stenosing external otitis is an indolent condition that is difficult to treat. Twelve cases that were operated on during a 3-year period were reviewed. The results of treatment are presented and the surgical methods are discussed. This involves excision of all fibrous tissue and if necessary the drum, a wide canalplasty, a meatoplasty, and then reconstruction with a fascial graft if necessary followed by split skin grafts, with emphasis on maintaining the anterior tympanomeatal angle.