ORCID Profile
0000-0002-6323-773X
Current Organisations
Children's Hospital at Westmead
,
Macquarie University
,
Royal Institute for Deaf and Blind Children
,
Royal Prince Alfred Hospital
,
University of Sydney
Does something not look right? The information on this page has been harvested from data sources that may not be up to date. We continue to work with information providers to improve coverage and quality. To report an issue, use the Feedback Form.
Related Links
Publications
Impact of Cochlear Implantation on Canal and Otolith Function
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 20-01-2022
DOI: 10.1097/MAO.0000000000003459
Abstract: To quantify the impact of cochlear implantation (CI) on all five vestibular end-organs and on subjective ratings of post-CI dizziness. 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. 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 (χ 2 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 litudes 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 litude, and AC oVEMP litude 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). 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.
A comparative study of audiologic outcomes for two transcutaneous bone-anchored hearing devices
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 09-2015
Auditory neuropathy in Brown–Vialetto–Van Laere syndrome due to riboflavin transporter RFVT2 deficiency
Publisher: Wiley
Date: 25-02-2016
DOI: 10.1111/DMCN.13084
Abstract: 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 in iduals with the phenotype of RFVT2 deficiency. Understanding the characteristics of hearing loss in riboflavin transporter deficiency would enable early diagnosis and therapy. We performed hearing assessments in seven children (from four families) with RFVT2 deficiency and reviewed results from previous assessments. Assessments were repeated after 12 months and 24 months of riboflavin therapy and after cochlear implantation in one in idual. Hearing loss in these in iduals was due to auditory neuropathy spectrum disorder (ANSD). Hearing loss was identified between 3 years and 8 years of age and progressed rapidly. Hearing aids were not beneficial. Riboflavin therapy resulted in improvement of hearing thresholds during the first year of treatment in those with recent-onset hearing loss. Cochlear implantation resulted in a significant improvement in speech perception in one in idual. Riboflavin transporter deficiency should be considered in all children presenting with an auditory neuropathy. Speech perception in children with ANSD due to RFVT2 deficiency may be significantly improved by cochlear implantation.
Pediatric cochlear implantation: Associated with minimal postoperative pain and dizziness
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 02-2015
Bilateral duplication of the internal auditory canals and bilateral cochlear implant outcomes and review
Publisher: Elsevier BV
Date: 04-2019
DOI: 10.1016/J.IJPORL.2019.01.015
Abstract: 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. 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. 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. 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.
Feasibility and Process Evaluation of a Need-Supportive Physical Activity Program in Aged Care Workers: The Activity for Well-Being Project
Publisher: Frontiers Media SA
Date: 30-09-2020
Neurotrophin gene augmentation by electrotransfer to improve cochlear implant hearing outcomes
Publisher: Elsevier BV
Date: 09-2019
DOI: 10.1016/J.HEARES.2019.06.002
Abstract: 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.
Impedance, neural response telemetry, and speech perception outcomes after reimplantation of cochlear implants in children
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 09-2014
Monitoring Cochlear Health With Intracochlear Electrocochleography During Cochlear Implantation: Findings From an International Clinical Investigation
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 08-11-2022
DOI: 10.1097/AUD.0000000000001288
Abstract: 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 litude tends to rise or may plateau as the electrode is inserted. The aim of this study was to explore whether compromise of the ECochG signal, defined as a fall in its litude of 30% or more during insertion, whether transient or permanent, is associated with poorer postoperative acoustic hearing, and to examine how preoperative hearing levels may influence the ability to record ECochG. The specific hypotheses tested were threefold: (a) deterioration in the pure-tone average of low-frequency hearing at the first postoperative follow-up interval (follow-up visit 1 [FUV1], 4 to 6 weeks) will be associated with compromise of the cochlear microphonic (CM) litude during electrode insertion (primary hypothesis) (b) an association is observed at the second postoperative follow-up interval (FUV2, 3 months) (secondary hypothesis 1) and (c) the CM response will be recorded earlier during electrode array insertion when the preoperative high-frequency hearing is better (secondary hypothesis 2). International, multi-site prospective, observational, between groups design, targeting 41 adult participants in each of two groups, (compromised CM versus preserved CM). Adult CI candidates who were scheduled to receive a Cochlear Nucleus CI with a Slim Straight or a Slim Modiolar electrode array and had a preoperative audiometric low-frequency average thresholds of ≤80 dB HL at 500, 750, and 1000 Hz in the ear to be implanted, were recruited from eight international implant sites. Pure tone audiometry was measured preoperatively and at postoperative visits (FUV1 and follow-up visit 2 [FUV2]). ECochG was measured during and immediately after the implantation of the array. From a total of 78 enrolled in iduals (80 ears), 77 participants (79 ears) underwent surgery. Due to protocol deviations, 18 ears (23%) were excluded. Of the 61 ears with ECochG responses, litudes were 1 µV throughout implantation for 18 ears (23%) and deemed “unclear” for classification. EcochG responses µV in 43 ears (55%) were stable throughout implantation for 8 ears and compromised in 35 ears. For the primary endpoint at FUV1, 7/41 ears (17%) with preserved CM had a median hearing loss of 12.6 dB versus 34/41 ears (83%) with compromised CM and a median hearing loss of 26.9 dB ( p 0.014). In assessing the practicalities of measuring intraoperative ECochG, the presence of a measurable CM ( µV) during implantation was dependent on preoperative, low-frequency thresholds, particularly at the stimulus frequency (0.5 kHz). High-frequency, preoperative thresholds were also associated with a measurable CM 1 µV during surgery. Our data shows that CM drops occurring during electrode insertion were correlated with significantly poorer hearing preservation postoperatively compared to CMs that remained stable throughout the electrode insertion. The practicality of measuring ECochG in a large cohort is discussed, regarding the suggested optimal preoperative low-frequency hearing levels ( 80 dB HL) considered necessary to obtain a CM signal µV.
Management of otitis media with effusion in children with primary ciliary dyskinesia: A literature review
Publisher: Elsevier BV
Date: 12-2009
DOI: 10.1016/J.IJPORL.2009.08.024
Abstract: 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. 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. 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. 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.
Large vestibular aqueduct syndrome: Impedance changes over time with different cochlear implant electrode arrays
Publisher: Informa UK Limited
Date: 22-06-2015
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
Publisher: American Society of Neuroradiology (ASNR)
Date: 14-05-2020
DOI: 10.3174/AJNR.A6541
Clinical Performance, Safety, and Patient-Reported Outcomes of an Active Osseointegrated Steady-State Implant System
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 08-2022
DOI: 10.1097/MAO.0000000000003590
Abstract: To investigate the clinical performance, safety, and patient-reported outcomes of an active osseointegrated steady-state implant system that uses piezoelectric technology. A prospective, multicenter, open-label, single-arm, within-subject clinical investigation. Three tertiary referral clinical centers located in Melbourne, Sydney, and Hong Kong. Twenty-nine adult subjects, 24 with mixed hearing loss or conductive hearing loss and 5 with single-sided sensorineural deafness. Implantation with the Cochlear Osia 2 System. Audiological threshold evaluation and speech recognition in quiet and in noise. Patient satisfaction and safety. 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. 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.
The impact of Primary Ciliary Dyskinesia on the upper respiratory tract
Publisher: Elsevier BV
Date: 03-2016
DOI: 10.1016/J.PRRV.2015.09.006
Abstract: 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.
Mastoid Langerhans cell histiocytosis: classic imaging findings with variable clinical presentations
Publisher: Wiley
Date: 15-04-2019
DOI: 10.1111/ANS.15136
Hearing loss associated with large internal auditory meatus: A report of five paediatric cases
Publisher: Cambridge University Press (CUP)
Date: 11-1999
DOI: 10.1017/S0022215100145864
Abstract: 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 iding 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.
Ear Abnormalities among Children with Fetal Alcohol Spectrum Disorder: A Systematic Review and Meta-Analysis
Publisher: Elsevier BV
Date: 03-2021
DOI: 10.1016/J.JPEDS.2021.11.016
Abstract: 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). 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. Studies reporting on functional and/or structural ear abnormalities among children (<18 years) with prenatal alcohol exposure and/or FASD were eligible. Data extraction and quality assessment were performed by one reviewer and independently checked by another. A random effects meta-analysis was conducted. A total of 31 studies met the inclusion criteria and 25 were included in the meta-analyses, representing a total of 843 children with prenatal alcohol exposure and 1653 children with FASD. Functional ear abnormalities with the highest pooled prevalence were chronic serous otitis media (88.5% 95% CI, 70.4%-99.3%), abnormal auditory filtering (80.1% 95% CI, 76.5%-84.3%), and unspecified conductive hearing loss (68.0% 95% CI, 51.9%-82.2%). Structural ear abnormalities with the highest pooled prevalence were microtia (42.9% 95% CI, 26.8%-59.7%), railroad track ear (16.8% 95% CI, 8.1%-27.7%), and misplaced ear (12.3% 95% CI, 7.6%-17.9%). Our findings highlight the importance of examining the ears during assessment for FASD, and the need for public health messaging regarding the harms of prenatal alcohol exposure.
Distance and socieoeconomic status as barriers to cochlear implantation
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 29-11-2023
DOI: 10.1097/MAO.0000000000003765
Abstract: 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. Retrospective case review. A CI services provider operating across multiple centers. All patients undergoing CI surgery in a 2-year period between March 2018 and February 2020. Diagnosis of hearing loss, CI surgery, and subsequent habilitation and mapping. 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). 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. 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.
Misplaced cochlear implant electrodes outside the cochlea
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 08-03-2022
DOI: 10.1097/MAO.0000000000003523
Abstract: 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. 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. Systematic literature review on cochlear implant misplacements and malpositions and a retrospective review of our program's cases in over 6,000 CI procedures. 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. 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.
Cochlear implant outcomes in large vestibular aqueduct syndrome
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 09-2019
DOI: 10.1097/MAO.0000000000002314
Abstract: Examine postoperative speech perception outcomes in a large vestibular aqueduct syndrome (LVAS) patients at a major cochlear implantation center. Retrospective analysis of the Sydney Cochlear Implant Centre (SCIC) database and medical records from January 1994 to December 2015 was performed. Tertiary referral center. 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. Therapeutic. : Postoperative speech perception scores. 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. 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.
Cochlear implant adult speech perception outcomes
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 07-03-2023
DOI: 10.1097/MAO.0000000000003846
Abstract: 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. Retrospective case review of 785 patients, between 2009 and 2016. A large cochlear implant program. Cochlear implant adult recipients younger than 65 years and 65 years and older at the time of surgery. Therapeutic—cochlear implant. 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. 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. 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.
Cochlear implant outcomes in cochlea nerve aplasia and hypoplasia
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 06-2016
Obstructive sleep apnoea in a paediatric patient with laryngeal Crohn's disease
Publisher: Wiley
Date: 06-11-2018
DOI: 10.1111/JPC.13753
Pediatric cochlear implants: Additional disabilities prevalence, risk factors, and effect on language outcomes
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 10-2012
Comparison of electrode impedance measures between a dexamethasone-eluting and standard Cochlear™ Contour Advance® electrode in adult cochlear implant recipients.
Publisher: Elsevier BV
Date: 05-2020
To Use the Back-up Cochlear Implant or Not? Using Intra-operative Impedance to Guide Your Decisions
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 02-03-2022
DOI: 10.1097/MAO.0000000000003483
Abstract: 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. 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. 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. 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.
Intraoperative cochlear implant reinsertion effects evaluated by electrode impedance
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 07-2020
DOI: 10.1097/MAO.0000000000002650
Abstract: To assess the effect on impedance levels of intraoperative reinsertion of a cochlear implant (CI) array compared with matched controls. Retrospective patient review. Cochlear implant center. 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. Cochlear implantation. 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. 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). 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.
The effects of a theory‐informed intervention on physical activity behaviour, motivation and well‐being of frontline aged care workers: A pilot study with 6‐month follow‐up
Publisher: Wiley
Date: 09-05-2023
DOI: 10.1002/HPJA.740
Abstract: Due to the nature of their jobs, frontline aged care workers may be a population at risk of poor health and lifestyle habits. Support of their well‐being through the workplace is likely to be complex. The objective of this study was to assess the effectiveness of a need‐supportive program for changing physical activity and psychological well‐being via the motivational processes of behavioural regulations and perceived need satisfaction. Frontline aged care workers ( n = 25) participated in a single cohort, pre–post pilot trial. The program included a Motivational Interviewing style appointment, education on goal setting and self‐management, the use of affect, exertion and self‐pacing for regulating physical activity intensity and practical support activities. Outcomes (7‐day accelerometery, 6‐min walk, K10 and AQoL‐8D), and motivational processes (BREQ‐3 and PNSE) were measured at baseline, 3 and 9 months, and analysed using linear mixed models for repeated measures. There were significant increases in perceived autonomy at 3 months (Δ .43 ± SE: .20 p = .03) and 6‐min walk distance at 9 months (Δ 29.11 m ± SE: 13.75 p = .04), which appeared to be driven by the relative autonomy index (behavioural regulations in exercise questionnaire [BREQ‐3]). Amotivation increased at 3 months (Δ .23 ± SE:.12 p = .05) which may have been due to low scores at baseline. No other changes were demonstrated at any timepoint. Participants demonstrated positive changes in motivational processes and physical function, however, due to the low levels of participation in the program, the program had a negligible impact at the organisational level. Future researchers and aged care organisations should aim to address factors impacting participation in well‐being initiatives.
Cochlear implant outcomes in patients with severe compared with profound hearing loss
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 04-2020
DOI: 10.1097/MAO.0000000000002574
Abstract: To compare the speech perception outcomes for patients with preoperative severe versus profound hearing loss with a cochlear implant (CI). Retrospective patient review. Cochlear implant program. 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. Cochlear implant. 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. 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. 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.
Childhood Hearing Australasian Medical Professionals network: Consensus guidelines on investigation and clinical management of childhood hearing loss
Publisher: Wiley
Date: 09-2019
DOI: 10.1111/JPC.14508
What to do with the other ear after cochlear implantation
Publisher: Maney Publishing
Date: 03-2009
DOI: 10.1002/CII.373
Abstract: Unilateral cochlear implantation has become a widely accepted surgical intervention for both deaf children and adults. It is a reliable and effective method to rehabilitate profound deafness. Recently the benefits of the use of a contralateral hearing aid (bimodal stimulation) with a cochlear implant became clear. Bilateral cochlear implantation benefits bilateral input into the auditory system for adults and children. To provide the binaural advantages experienced by normal hearing subjects bilateral cochlear implantation or bimodal stimulation is probably indicated. Whether to choose between both possibilities depends on many factors. Cortical auditory evoked potential (CAEP) measurements can be an important tool to decide bilateral implantation in young children. Enough residual hearing in the non-implanted ear might benefit from bimodal stimulation. New protocols are needed for the audiological management for recipients of cochlear implants.
Primary ciliary dyskinesia: Overlooked and undertreated in children
Publisher: Wiley
Date: 18-06-2014
DOI: 10.1111/JPC.12628
Abstract: 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.
Emergency EXIT: an urgent ex utero intrapartum tracheostomy for giant fetal neck mass.
Publisher: Wiley
Date: 09-2013
DOI: 10.1111/ANS.12316
CHARGE syndrome and Cochlear implantation: Difficulties and outcomes in the paediatric population
Publisher: Elsevier BV
Date: 04-2015
DOI: 10.1016/J.IJPORL.2015.01.004
Abstract: 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. 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). 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. 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.
Clinical performance, safety, and patient-reported outcomes of an active osseointegrated bone-conduction hearing implant system at 24-month follow-up
Publisher: Springer Science and Business Media LLC
Date: 08-08-2023
DOI: 10.1007/S00405-023-08133-3
Abstract: 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. 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). 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. 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. ClinicalTrials.gov Identifier: NCT04754477. First posted: February 15, 2021.
Management of recurrent and delayed post-tonsillectomy and adenoidectomy hemorrhage in children
Publisher: SAGE Publications
Date: 09-03-2023
Abstract: 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. 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. 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. 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.
Pinna perichondritis in a rural setting: a 10-year review of cases and an introduction to the PinCH as a novel technique for improving cosmetic outcomes following pinna abscess
Publisher: AME Publishing Company
Date: 04-2023
DOI: 10.21037/AJO-22-41
Presentation of primary ciliary dyskinesia in children: 30 years' experience
Publisher: Wiley
Date: 15-12-2015
DOI: 10.1111/JPC.12791
Abstract: 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. A retrospective review of the symptoms of children diagnosed with PCD at Concord Hospital between 1982 and 2012 was undertaken. 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%. 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.
Cochlear nerve dysplasia in unilateral severe to profound congenital sensorineural hearing loss
Publisher: Elsevier BV
Date: 02-2023
Acquired tracheo-esophageal fistula in the pediatric population
Publisher: Elsevier BV
Date: 07-1998
DOI: 10.1016/S0165-5876(98)00035-4
Abstract: Acquired tracheo-esophageal fistula (TEF) in the paediatric population is a rare entity, an acquired fistula can be due to tracheotomy tubes and tracheotomy cuffs. Patients with burns are at greater risk from these tracheotomy complications. Acquired TEF can also occur due to a foreign body impaction. Prevention and early diagnosis are important. In patients with possible airway burns, 'safe' intracuff pressures maybe too high. To avoid further damage of the mucosa, the patient should have a small air leak maintained if a cuff is used. Most acquired TEF do not close spontaneously and surgical closure is required. Our paper presents two cases of acquired TEF in the pediatric population and reviews the literature on this subject.
Efficacy of theory-informed workplace physical activity interventions: a systematic literature review with meta-analyses
Publisher: Informa UK Limited
Date: 29-01-2020
DOI: 10.1080/17437199.2020.1718528
Abstract: This review aimed to assess the efficacy of workplace physical activity interventions compare the efficacy of those that were and were not informed by behaviour change theory, and outline the effectiveness of different intervention components. A search was undertaken in Medline, Embase, PsycINFO, Ovid Emcare (previously CINAHL) and SportDiscus. Randomised, non-randomised and cluster-controlled trials with objectively measured physical activity and/or measured or predicted maximal oxygen uptake (VO
Common cavity and custom-made electrodes
Publisher: Elsevier BV
Date: 08-2013
DOI: 10.1016/J.IJPORL.2013.04.008
Abstract: 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. 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. Children with common cavity tend to steadily and significantly improve their audiological skills over time. This development may, however, be highly in idual 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. 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.
Candidacy considerations and other medical and surgical issues for cochlear implantation in children
Publisher: Springer Nature Singapore
Date: 2022
Novel variant in Sp7/Osx associated with recessive osteogenesis imperfecta with bone fragility and hearing impairment
Publisher: Elsevier BV
Date: 05-2018
DOI: 10.1016/J.BONE.2018.01.031
Abstract: Osteogenesis imperfecta (OI) is a connective tissue disorder characterized by low bone density and recurrent fractures with a wide genotypic and phenotypic spectrum. Common features include short stature, opalescent teeth, blue sclerae and hearing impairment. The majority (>90%) of patients with OI have autosomal dominant variants in COL1A1/COL1A2, which lead to defects in type 1 collagen. More recently, numerous recessive variants involving other genes have also been identified. Sp7/Osx gene, is a protein coding gene that encodes a zinc finger transcription factor, osterix, which is a member of the Sp subfamily of sequence-specific DNA-binding proteins. Osterix is expressed primarily by osteoblasts and has been shown to be vital for bone formation and bone homeostasis by promoting osteoblast differentiation and maturation. In animal models, Sp7/Osx has also been shown to regulate biomineralization of otoliths, calcium carbonate structures found in the inner ear of vertebrates. Until recently, only one report of a boy with an Sp7/Osx pathogenic variant presenting with bone fragility, limb deformities and normal hearing has been described in the literature. We have identified a novel Sp7/Osx variant in another sibship that presented with osteoporosis, low-trauma fractures and short stature. Progressive moderate-to-severe and severe-to-profound hearing loss secondary to otospongiosis and poor mineralization of ossicles and petrous temporal bone was also noted in two of the siblings. A homozygous pathogenic variant in exon 2 of the Sp7/Osx gene was found in all affected relatives c.946C>T (p.Arg316Cys). Bone biopsies in the proband and his male sibling revealed significant cortical porosity and high trabecular bone turnover. This is the second report to describe children with OI associated with an Sp7/Osx variant. However, it is the first to describe the bone histomorphometry associated with this disorder and identifies a significant hearing loss as a potential feature in this OI subtype. Early audiology screening in these children is therefore warranted.
Related Organisations
Related Funding Activities
No related grants have been discovered for Catherine Birman.