Publications by authors named "Rance G"

Chiari malformation is a condition involving caudal descent of the hindbrain which herniates the cerebellar tonsils through the foramen magnum. The purpose of this study was to quantify auditory deficits in an affected individual and to explore the hypothesis that cerebellar malformation specifically disrupts binaural processing. We present audiometric, electrophysiologic, imaging and auditory perceptual findings for a 17-year-old female with Chiari 1 malformation and for a cohort of 35 hearing- and age-matched controls.

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Introduction: Hearing loss is common in ageing populations, but thorough investigation of factors associated with objective hearing loss in otherwise healthy, community-dwelling older individuals is rare. We examined prevalence of age-related hearing loss (ARHL) in healthy, community-dwelling older adults, and determined whether sociodemographic, lifestyle, or health factors associate with hearing thresholds. Audiometry assessment was investigated with self-reports of hearing loss and hearing handicap.

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  • Hearing impairment is common in patients with the m.3243A>G mitochondrial disease variant, but the related auditory and vestibular dysfunctions aren't well-studied, prompting this case-control research.
  • In a study comparing 12 adults with m.3243A>G to matched controls with cochlear hearing loss, significant auditory neural abnormalities and perceptual deficits were found, including issues with rapid sound encoding and speech perception in noise.
  • Furthermore, participants with the mitochondrial disease exhibited greater vestibular dysfunction, increased dizziness, and poorer balance, suggesting the need for comprehensive auditory and vestibular evaluations in these patients.
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  • The study investigates the relationship between initial electrophysiological thresholds and later behavioral hearing thresholds in infants to clarify if initial tests can guide cochlear implant (CI) recommendations before behavioral assessments.
  • It involves a cohort of 63 infants suspected of severe to profound hearing loss and uses various auditory tests to evaluate their hearing capabilities.
  • Findings indicate that, in most cases, initial electrophysiological thresholds accurately predict later behavioral thresholds, suggesting a new pathway for CI evaluation in certain infants to expedite implantation and enhance language development.
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This study reports the development of the first copolymer material that (i) is resistant to fungal attachment and hence biofilm formation, (ii) operates via a nonkilling mechanism, i.e., avoids the use of antifungal actives and the emergence of fungal resistance, (iii) exhibits sufficient elasticity for use in flexible medical devices, and (iv) is suitable for 3D printing (3DP), enabling the production of safer, personalized medical devices.

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  • It involves a case-control design with 10 individuals affected by AN, 7 with neurofibromatosis type 1 (NF1), and 37 control participants, comparing white matter fiber density across different auditory pathways.
  • Results show that the presence and pattern of white matter abnormalities differ based on the underlying cause of AN, and these abnormalities have correlations with certain auditory functional measures like speech perception in noise.
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Postural control deficits have been documented in children with autism spectrum disorder (ASD), yet vestibular system contributions to postural control have not been widely considered. The purpose of this study is to explore the relationship between functional balance, postural sway, and vestibular function in children with ASD. Ten children with a confirmed diagnosis of ASD according to DSM-V guidelines along with ten children with no known neurodevelopmental or motor delays participated in the study.

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: Autism spectrum disorder (ASD) is a lifelong neurodevelopmental condition characterised by impairments in social communication, sensory abnormalities, and attentional deficits. Children with ASD often face significant challenges with speech perception and auditory attention, particularly in noisy environments. This study aimed to assess the effectiveness of noise cancelling Bluetooth earbuds (Nuheara IQbuds Boost) in improving speech perception and auditory attention in children with ASD.

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  • Age-related hearing loss is common in older adults, negatively impacting communication and quality of life, and may be worsened by cochlear degeneration and inflammation.
  • The study aimed to assess whether daily low-dose aspirin affects the progression of hearing loss in healthy individuals aged 70 and older compared to a placebo over 3 years.
  • Results showed that aspirin did not significantly alter hearing loss progression compared to placebo, with a similar percentage of participants in both groups reporting hearing loss over the study period.
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  • Ritlecitinib, a drug being tested for alopecia areata, caused reversible axonal swelling and auditory potential changes in dogs at doses higher than what's approved for humans.
  • * A phase 2a study evaluated the effects of ritlecitinib on auditory and nerve fiber changes in 71 patients, comparing results with a placebo group over 9 months.
  • * Findings showed no significant differences in auditory or nerve fiber measures between the treatment and placebo groups, suggesting that dog toxicity findings may not apply to human patients.
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Background: Progressive auditory dysfunction is common in patients with generalized neurodegenerative conditions, but clinicians currently lack the diagnostic tools to determine the location/degree of the pathology and, hence, to provide appropriate intervention. In this study, we present the white-matter microstructure measurements derived from a novel diffusion-weighted magnetic resonance imaging (dMRI) technique in a patient with axonal auditory neuropathy and consider the findings in relation to the auditory intervention outcomes.

Methods: We tracked the hearing changes in an adolescent with Riboflavin Transporter Deficiency (Type 2), evaluating the sound detection/discrimination, auditory evoked potentials, and both structural- and diffusion-weighted MRI findings over a 3-year period.

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Objective: To investigate the benefit of remote-microphone (RM) systems for adults with sensory hearing loss.

Design: Speech recognition in quiet and in background noise was assessed. Participants with hearing loss underwent testing in two device conditions: hearing aids (HAs) alone and HAs with a RM.

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Objective: To investigate the reliability of tone-burst auditory brainstem response (TB-ABR) latencies and thresholds in pre-term and full-term infants.

Design: TB-ABRs to 500 Hz and 4000 Hz tone-burst stimuli were recorded at two-week intervals over the first six weeks of life in a group of full-term infants (40-46 weeks gestational age [GA]) and over ten weeks (36-46 weeks gestation) in a group of preterm infants. Linear mixed model analyses evaluated within-subject changes and the effects of the subject group, age at assessment, and stimulus frequency on ABR latency and threshold.

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Background: Auditory neuropathy (AN) is a hearing disorder that affects neural activity in the VIIIth cranial nerve and central auditory pathways. Progressive forms have been reported in a number of neurodegenerative diseases and may occur as a result of both the deafferentiation and desynchronisation of neuronal processes. The purpose of this study was to describe changes in auditory function over time in a patient with axonal neuropathy and to explore the effect of auditory intervention.

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Objective: To explore the feasibility of remote diagnostic hearing testing within schools, for school-entry aged children (4-7 years).

Design: A prospective cross-sectional comparative study design was used to establish the reliability of remote hearing assessment by comparing traditional face-to-face pure tone audiometry (PTA) with remote PTA, traditional otoscopy with remote video otoscopy and interpretation of tympanograms in-person with remote analysis.

Study Sample: 464 school-entry year level children from 18 schools.

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Room-temperature magnetically switchable materials play a vital role in current and upcoming quantum technologies, such as spintronics, molecular switches, and data storage devices. The increasing miniaturization of device architectures produces a need to develop analytical tools capable of precisely probing spin information at the single-particle level. In this work, we demonstrate a methodology using negatively charged nitrogen vacancies (NV) in fluorescent nanodiamond (FND) particles to probe the magnetic switching of a spin crossover (SCO) metal-organic framework (MOF), [Fe(1,6-naphthyridine)(Ag(CN))] material (), and a single-molecule photomagnet [X(18-crown-6)(HO)]Fe(CN)·2HO, where X = Eu and Dy (materials and , respectively), in response to heat, light, and electron beam exposure.

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Single-atom dynamics of noble-gas elements have been investigated using time-resolved transmission electron microscopy (TEM), with direct observation providing for a deeper understanding of chemical bonding, reactivity, and states of matter at the nanoscale. We report on a nanoscale system consisting of endohedral fullerenes encapsulated within single-walled carbon nanotubes ((Kr@C)@SWCNT), capable of the delivery and release of krypton atoms on-demand, via coalescence of host fullerene cages under the action of the electron beam () or heat (). The state and dynamics of Kr atoms were investigated by energy dispersive X-ray spectroscopy (EDS), electron energy loss spectroscopy (EELS), and X-ray photoelectron spectroscopy (XPS).

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Objective: Balance difficulties are common in children with sensorineural hearing loss (SNHL). For some of these children, concomitant vestibular deficits may impact postural control. This study aimed to explore vestibular function, functional balance and postural control, and the relationship between these measures in children with SNHL.

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Objectives: Musicians who wear hearing aids are a unique subset of hearing-impaired individuals. There are a number of issues musicians experience with hearing aids, making effective hearing rehabilitation a challenge. Research suggests hearing aid satisfaction in musicians is lower partly due to their advanced listening skills, however, qualitative research addressing musicians who wear hearing aids for music is scarce.

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The cardiac operating room (OR) is a high-risk, high-stakes environment inserted into a complex socio-technical healthcare system. During cardiopulmonary bypass (CPB), the most critical phase of cardiac surgery, the perfusionist has a crucial role within the interprofessional OR team, being responsible for optimizing patient perfusion while coordinating other tasks with the surgeon, anesthesiologist, and nurses. The aim of this study was to investigate objective digital biomarkers of perfusionists' workload and stress derived from heart rate variability (HRV) metrics captured via a wearable physiological sensor in a real cardiac OR.

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Magnetosomes are biologically-derived magnetic nanoparticles (MNPs) naturally produced by magnetotactic bacteria (MTB). Due to their distinctive characteristics, such as narrow size distribution and high biocompatibility, magnetosomes represent an attractive alternative to existing commercially-available chemically-synthesized MNPs. However, to extract magnetosomes from the bacteria, a cell disruption step is required.

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Objective: Recent studies have found that human Friedreich ataxia patients have dysfunction of transmission in the auditory neural pathways. Here, we characterize hearing deficits in a mouse model of Friedreich ataxia and compare these to a clinical population.

Methods: Sixteen mice with a C57BL/6 background were evaluated.

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The physical characteristics of a child's learning environment can affect health, wellbeing and educational progress. Here we investigate the effect of classroom setting on academic progress in 7-10-year-old students comparing reading development in "open-plan" (multiple class groups located within one physical space) and "enclosed-plan" (one class group per space) environments. All learning conditions (class group, teaching personnel, etc.

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Objective: This study examined (1) the utility of a clinical system to record acoustic change complex (ACC, an event-related potential recorded by electroencephalography) for assessing speech discrimination in infants, and (2) the relationship between ACC and functional performance in real life.

Methods: Participants included 115 infants (43 normal-hearing, 72 hearing-impaired), aged 3-12 months. ACCs were recorded using [szs], [uiu], and a spectral rippled noise high-pass filtered at 2 kHz as stimuli.

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