Binaural Processing Deficits in a Child with Chiari Malformation (Type 1).

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.

Age-Related Hearing Loss: A cross-sectional study of healthy older Australians.

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.

Auditory and vestibular function in mitochondrial patients harbouring the m.3243A>G variant.

Hearing impairment is a frequent clinical feature in patients with mitochondrial disease harbouring the pathogenic variant, m.3243A>G. However, auditory neural dysfunction, its perceptual consequences and implications for patient management are not established.

Early cochlear implantation: exploring electrophysiological thresholds and their role in pre-behavioural recommendations.

Background And Aims: Studies investigating the relationship between initial electrophysiological thresholds and subsequent behavioural hearing thresholds have demonstrated uncertainty as to whether initial results can provide an accurate estimation of an infant's functional hearing. This estimation must be accurate if a cochlear implant (CI) is to be considered before behavioural audiometry.This study aims to explore the variance between initial electrophysiological thresholds and subsequent behavioural hearing thresholds in infants to determine under which conditions electrophysiological results can be used to support a CI recommendation before behavioural audiology.

Vestibular Function and Postural Control in Children with Autism Spectrum Disorder.

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.

Effectiveness of Noise Cancelling Earbuds in Reducing Hearing and Auditory Attention Deficits in Children with Autism.

: 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.

Diffusion-Weighted Magnetic Resonance Imaging (dMRI) and Cochlear Implant Outcomes in Axonal Auditory Neuropathy: A Case Report.

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.

Evaluating benefits of remote microphone technology for adults with hearing loss using behavioural and predictive metrics.

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.

Tone-burst elicited auditory brainstem responses in full-term and pre-term infants.

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.

Remediation of Perceptual Deficits in Progressive Auditory Neuropathy: A Case Study.

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.

The reliability of remote diagnostic hearing assessment in school-entry-aged children.

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.

Vestibular function and balance performance in children with sensorineural hearing loss.

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.

Interviews with musicians with hearing aids.

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.

Auditory neuropathy in mice and humans with Friedreich ataxia.

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.

The effect of classroom environment on literacy development.

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.

Acoustic change complex for assessing speech discrimination in normal-hearing and hearing-impaired infants.

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.

The Assistive Benefits of Remote Microphone Technology for Normal Hearing Children With Listening Difficulties.

Objectives: Children often present with listening difficulties (LiD) but with normal sound detection thresholds. These children are susceptible to learning challenges, and struggle with the suboptimal acoustics of standard classrooms. Remote microphone technology (RMT) is one way to improve the listening environment.

Increased rate of listening difficulties in autistic children.

Introduction: Auditory challenges are both common and disruptive for autistic children and evidence suggests that listening difficulties may be linked to academic underachievement (Ashburner, Ziviani & Rodger, 2008). Such deficits may also contribute to issues with attention, behavior, and communication (Ashburner et al., 2008; Riccio, Cohen, Garrison & Smith, 2005).

A randomized controlled trial of remote microphone listening devices to treat auditory deficits in children with neurofibromatosis type 1.

Background: A high proportion of patients with neurofibromatosis type 1 (NF1) present with functional hearing deficiency as a result of neural abnormality in the late auditory brainstem.

Methods: In this randomized, two-period crossover study, we investigated the hypothesis that remote-microphone listening devices can ameliorate hearing and communication deficits in affected school-aged children (7-17 years). Speech perception ability in background noise was evaluated in device-active and inactive conditions using the CNC-word test.

Evaluation of a game-based hearing screening program for identifying hearing loss in primary school-aged children.

Objectives: To evaluate a tablet-based hearing screening game in primary school aged children. To examine the prevalence of middle/outer ear pathology, hearing loss and spatial processing disorder in primary school aged children.

Design: The automated hearing test Sound Scouts was used as a screening tool, which measures hearing abnormalities through tests of speech-in-quiet/noise and tone-in-noise.

Auditory Dysfunction Among Individuals With Neurofibromatosis Type 1.

Importance: Neurofibromatosis type 1 (NF1) affects hearing through disruption of central auditory processing. The mechanisms, functional severity, and management implications are unclear.

Objective: To investigate auditory neural dysfunction and its perceptual consequences in individuals with NF1.

Fiber-Specific Changes in White Matter Microstructure in Individuals With X-Linked Auditory Neuropathy.

Objectives: Auditory neuropathy (AN) is the term used to describe a group of hearing disorders, in which the hearing impairment occurs as a result of abnormal auditory nerve function. While our understanding of this condition has advanced significantly over recent years, the ability to determine the site of lesion and the extent of dysfunction in affected individuals remains a challenge. To this end, we investigated potential axonal degeneration in the white matter tracts of the brainstem in individuals with X-linked AN.