Prevention of Noise-induced Damage by Use of Antioxidants
Noise-induced TinnitusNoise-induced Hearing LossThe current study is a dubble-blinde placebo-controlled cross-over study verifying the preventive effect of antioxidants on noise-induced hearing loss (NIHL) and noise-induced tinnitus (NIT). The antioxidants comprise of a mixture of magnesium and n-acetylcystein which should be taken 1h before leisure noise above 100dB for at least 30 minutes.
Hearing Instruments in Alzheimer's Disease
Alzheimer DiseaseHearing LossThere is a strong connection between hearing loss and cognitive impairment, particularly dementia, in old age. Worldwide, dementia affects approximately 5% of persons over the age of 65 years. Hearing loss is even more prevalent in old age, affecting an estimated one third of persons over the age of 65 years. Thus, there is likely a large degree of overlap between the impairments. Indeed, this overlap may influence older adults' everyday functioning, communication, social engagement and quality of life, as well as influencing the well-being of their family caregivers. This project will examine whether patients with hearing loss and Alzheimer's disease, the most common form of dementia, derive benefit the from hearing aids prescribed and fit to them following current best practice procedures in a geriatric audiology clinic. For the first time, a formal evaluation of the potential benefits of hearing aids for the patients' family caregivers will also be conducted.
Prevalence of POU4F3 and SLC17A8 Mutations
Familial DeafnessThe study will allow to identify the prevalence of the SLC17A8 gene mutations in patients suffering from deafness. This phenotype also corresponds to DFNA15 deafness caused by POU4F3 : mutations of this gene will be screened as well.
Genetic Analysis of Human Hereditary Hearing Impairment
Partial Hearing LossThis studied is designed to discover the genes that cause hearing impairment. More precisely, this study aims to map and clone genes that are important for the development and maintenance of the anatomy and physiology related to hearing (auditory system). The study will begin by finding large families who have members with hearing impairment. Once families are found, members with and without hearing impairment will be evaluated by an audiologist and a clinician (doctor). An audiologist, is a person trained in evaluating, habilitating, and rehabilitating people with disorders of hearing function. The clinician's responsibility is to examine the patients and check for other signs and symptoms related to hearing. Finding the gene for hearing impairment requires: <TAB>DNA samples of hearing impaired family members, taken from standard blood samples. <TAB>DNA samples of members of the family without hearing impairment, taken from standard blood samples. <TAB>Results of hearing tests conducted by the audiologist for all participants. Once all members of the family are evaluated researchers can create a pedigree. A pedigree is like a family tree that charts members of a family with a genetic disorder, like hearing impairment. Pedigrees are used to determine the mode of inheritance of the gene responsible for a particular condition. Finally, researcher intend on using all the information gathered as well as methods for genetic analysis to map out the location of the gene. Patients participating in this study will not directly benefit from its research, but scientific understanding achieved may help researchers better understand the auditory system and someday prevent deafness.<TAB>...
Unilateral Blindness/ Unilateral Deafness-relation to Neck Pain
Neck PainUnilateral Blindness1 moreNeck pain is a common complaint in the general population affecting 10-15% of the general population. females more than men. Known risk factors are: Trauma, Repetitive lifting of heavy objects at work, driving vibrating vehicles, frequents diving from boards and smoking. No study dealt with the possible role of unilateral loss of vision or hearing as a risk factor for the development of neck pain. Intuitively, one might suspect that unilateral blindness or deafness will lead to a tilted position of the head, in order to cover a better visual/audio field. This position, in time, might lead to the development of neck pain. Our hypothesis is that patients with unilateral blindness/deafness will suffer from neck pain more frequently than the general population.
Tradeoff Between Spatial and Temporal Resolution
DeafnessIn this study, investigators will examine how temporal rate processing might be affected when the width of neural excitation narrows. The manipulation of neural excitation width will be achieved for example by changing electrode configurations or stimulating single versus multiple electrodes simultaneously. Investigators will then measure if the extent to which rate processing is affected by spatially restricted stimulation can predict a subject's overall speech recognition and predict also whether the subject is likely to benefit from a focused electrode configuration. Lastly, investigators will introduce and test a novel current focusing strategy where current focusing is applied in a channel specific manner and quantify if this intervention improves speech recognition. The primary endpoint of the study is speech recognition, and the secondary endpoint is psychophysical sensitivity to stimulation rate change.
Biomarker for Alport Syndrome (BioAlport)
NephritisHereditary1 moreInternational, multicenter, observational, longitudinal monitoring study to identify biomarker/s for Alport syndrome and to explore the clinical robustness, specificity, and long-term variability of these biomarker/s
Degenerative Consequences of Congenital Deafness
Congenital DeafnessIn this study, the investigators will study one of the basic biophysical properties of the auditory nerve, charge integration, behaviorally (detection threshold versus phase duration functions). The investigators will compare charge integration in two subject groups: congenitally deafened and deafened at a later age in life. The investigators will then examine if behaviorally estimated neural excitation patterns differ between short phase duration and long phase duration stimulation. Lastly, The investigators will measure if speech recognition improves with using long phase duration stimulation, relative to using the standard default short phase duration stimulation. The primary endpoint of the study is speech recognition, and the secondary endpoints are the steepness of the detection threshold versus phase duration functions, and the width of psychophysically estimated neural excitation.
European Alpha-Mannosidosis Participant
ImmunodeficiencySkeletal Abnormalities4 moreInternational, multicenter, observational, longitudinal monitoring study to investigate the prevalence of Alpha-Mannosidosis in participants at risk for Alpha-Mannosidosis.
Cochlear Implants for Adults With Single-sided Deafness
Single-sided DeafnessThe purpose of this investigation is to determine the safety and preliminary efficacy of implanting a cochlear implant (CI) in the profoundly deaf ear of an adult with one normal hearing (NH) ear (termed "single-sided deaf" person, or SSD). The potential subjects will have been deafened post-lingually, thus, at one point the now deafened ear did conduct sound from the periphery. The MED-EL CI system will be implanted in ten (10) SSD patients.The long-term goal of this research program is to determine whether the CI, in combination with the NH ear, may provide improved localization ability and better speech understanding in noise, relative to performance before cochlear implantation (i.e., with the NH ear alone). A secondary long-term goal is to determine whether CI stimulation may reduce tinnitus severity, compared to tinnitus experienced prior to cochlear implantation or when the CI is turned off, after implantation.