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.

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.

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.

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.

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.

Fixel-based Analysis of Diffusion MRI: Methods, Applications, Challenges and Opportunities.

Diffusion MRI has provided the neuroimaging community with a powerful tool to acquire in-vivo data sensitive to microstructural features of white matter, up to 3 orders of magnitude smaller than typical voxel sizes. The key to extracting such valuable information lies in complex modelling techniques, which form the link between the rich diffusion MRI data and various metrics related to the microstructural organization. Over time, increasingly advanced techniques have been developed, up to the point where some diffusion MRI models can now provide access to properties specific to individual fibre populations in each voxel in the presence of multiple "crossing" fibre pathways.

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.

Review: Using diffusion-weighted magnetic resonance imaging techniques to explore the microstructure and connectivity of subcortical white matter tracts in the human auditory system.

Since its inception 30 years ago, diffusion-weighted magnetic resonance imaging (dMRI) has advanced to become a common component of routine clinical MRI examinations. Diffusion-weighted magnetic resonance offers a way to measure anisotropic diffusion in-vivo, which has led to the development of techniques capable of characterising the orientation of diffusion within living tissue. These modelling techniques can be used to investigate the microstructure and connectivity of white matter tracts within the human brain.

Functional hearing in the classroom: assistive listening devices for students with hearing impairment in a mainstream school setting.

Objective: To assess the benefit of assistive listening devices (ALDs) for students with hearing impairment in mainstream schools.

Design: Speech recognition (CNC words) in background noise was assessed in a typical classroom. Participants underwent testing using four device configurations: (1) HA(s)/CI(s) alone, (2) soundfield amplification, (3) remote microphone (Roger Pen) on desk and (4) remote microphone at the loudspeaker.