Identification And Characterisation Of Mouse Models For Recessively Inherited Deafness.
Funder
National Health and Medical Research Council
Funding Amount
$691,893.00
Summary
Hearing loss affects 10% of Australians. Approximately 1 in 1000 children are born deaf. A progressive hearing impairment occurs with age so that more than 50% of people over the age of 75 have a substantial hearing loss. The financial, social and personal costs of deafness are significant. Deafness is caused by environmental and-or inherited factors. In the majority of children and young people with a hearing impairment the underlying cause is genetic. It is also known that genetic predispositi ....Hearing loss affects 10% of Australians. Approximately 1 in 1000 children are born deaf. A progressive hearing impairment occurs with age so that more than 50% of people over the age of 75 have a substantial hearing loss. The financial, social and personal costs of deafness are significant. Deafness is caused by environmental and-or inherited factors. In the majority of children and young people with a hearing impairment the underlying cause is genetic. It is also known that genetic predisposition frequently contributes to the time of onset and the severity of age-related hearing loss, as well as susceptibility to noise and ototoxic drugs. It has proven difficult to identify the genes causing deafness, especially those genes associated with age-related hearing loss and susceptibility to noise and ototoxic drugs. The mouse ear is very similar to the human ear and therefore well suited to genetic and molecular studies of human deafness. Australia has a unique resource of mutagenised mice that are being bred to uncover recessive deafness, the most common type of inherited hearing loss. We have so far identified 10 mouse strains with recessive hearing impairment. 5 of the strains have an age-related hearing loss. We have identified the genetic mutation in 3 of the strains, including a mutation in a novel deafness gene. Another strain has a mutation in a novel deafness gene yet to be identified. We propose to continue the studies of these and additional mice. We will investigate why changes in these genes cause hearing loss and determine in detail how genetic and environmental factors lead to hearing loss in young and old. The results will allow us to offer earlier diagnosis and better counselling to affected families, and in the longer term we believe our research will enable us to develop improved or novel treatments to delay or prevent deafness.Read moreRead less
The research will investigate the mechanisms by which our brains are able to listen selectively to sounds of interest in competing background noise. This will be investigated in normal hearing subjects, those with partial deafness and in profoundly deaf patients who use a cochlear implant. If deaf patients can learn to use cues to enhance detection of sounds of interest this could have an impact on the effectiveness of hearing aids and cochlear implants in noisy listening situations
Improving Music Appreciation For People With Prosthetic Hearing Devices By Enhancing Auditory Stream Segregation
Funder
National Health and Medical Research Council
Funding Amount
$266,560.00
Summary
Music perception is one of the most often-cited problems for people with hearing aids or cochlear implants. Part of the problem is related to the reduced ability to hear different instruments or melodic lines separately. This ability is based on perceptual differences between auditory streams. Psychophysics experiments will be performed to understand the effect of different acoustic parameters on auditory streaming. An innovative approach to restore music appreciation will be tested on people wi ....Music perception is one of the most often-cited problems for people with hearing aids or cochlear implants. Part of the problem is related to the reduced ability to hear different instruments or melodic lines separately. This ability is based on perceptual differences between auditory streams. Psychophysics experiments will be performed to understand the effect of different acoustic parameters on auditory streaming. An innovative approach to restore music appreciation will be tested on people with impaired hearing.Read moreRead less
Efferent Control Circuitry Of The Auditory Brainstem
Funder
National Health and Medical Research Council
Funding Amount
$406,306.00
Summary
Detection of important sounds within a noisy background is a crucial function of the mammalian hearing system and defects in this function impair social interaction, learning and development. In addition, activity in the brain needs to be carefully regulated by intrinsic circuitry in order to prevent excessive activity responsible for conditions such as tinnitus. The mechanisms by which the brain achieves this are poorly understood and this project aims to improve our understanding of some of th ....Detection of important sounds within a noisy background is a crucial function of the mammalian hearing system and defects in this function impair social interaction, learning and development. In addition, activity in the brain needs to be carefully regulated by intrinsic circuitry in order to prevent excessive activity responsible for conditions such as tinnitus. The mechanisms by which the brain achieves this are poorly understood and this project aims to improve our understanding of some of the brain circuits involved.Read moreRead less
Signals And Noise: A Study Of The Neurocognitive Mechanisms Underpinning Habituation To Noise In Normal And Damaged Hearing
Funder
National Health and Medical Research Council
Funding Amount
$408,938.00
Summary
McLachlan and Wilson recently published the first model of hearing that combines brain structure with function. This model postulates that recognition mechanisms initiate first, and then regulate the processing of other features. This project will investigate whether recognition mechanisms enable the auditory system to adapt to repetitive (background) noise by predicting and inhibiting responses to these sounds, and any changes in these mechanisms that may accompany hearing damage and tinnitus.
Early Indicators Of Noise Injury: Are Decreased Auditory Processing Skills Evident In Noise-exposed Adults Prior To Diagnosis Of Hearing Loss?
Funder
National Health and Medical Research Council
Funding Amount
$367,605.00
Summary
Recent research indicates that noise-exposed individuals with similar hearing thresholds to non-noise exposed counterparts are more likely to have diminished temporal and spectral auditory processing abilities. This research aims to determine the relationship between noise exposure levels and auditory processing difficulties; the influence of musical training in ameliorating these difficulties; and a neurological model of causation, operation and possible remediation of these difficulties.
Cochlear Type II Neurons In Contralateral Suppression
Funder
National Health and Medical Research Council
Funding Amount
$459,434.00
Summary
Sound in one ear affects hearing in the other ear. This contralateral suppression is important for hearing attention and protection from noise damage. We will test the hypothesis that cochlear type II sensory neurons provide the sensory input for this process using models where neuronal development is altered, or the neurons are removed. The study addresses hearing disability in society, facilitating cochlear prosthesis development and the understanding of hearing loss.
Improving Treatment And Outcomes Of Congenital Hearing Loss At The Population Level
Funder
National Health and Medical Research Council
Funding Amount
$265,138.00
Summary
Around 600 Australian babies are born with congenital hearing loss each year. Despite great advances in early diagnosis and treatment, affected children have language and other skills well below what they can potentially achieve. My research will a) examine trends in congenital hearing loss outcomes, b) enrich existing data with novel genetic and clinical data, to enable early prediction of future outcomes to direct management, and c) discover ways to prevent hearing loss progression.