Active clinical trials for "Deafness"

This is a retrospective and prospective longitudinal study in participants with Otoferlin Gene-Mediated Hearing Loss.

The aim of this study is to display the predictive factors of hearing rehabilitation after cochlear implant surgery in severely to profoundly deaf adults.

The aim of this study is to establish the connection between the cochlear implant programming parameters with the auditory effort associated with the speech perception evaluated by pupillometry techniques in different audiometric conditions.

CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access. Visit sanfordresearch.org/CoRDS to enroll.

The cognitive profile of older adults with a severe to profound hearing impairment is determined by means of the Repeatable Battery for the Assessment of Neuropsychological Status, adjusted for Hearing impaired subjects (RBANS-H) before and after cochlear implantation. In this prospective, longitudinal study the participants are tested preoperatively, at six months and twelve months postoperatively and from then on yearly up to 10 years after implantation. In addition to the RBANS-H an audiological examination and an semistructured interview is conducted concerning the cochlear implant use and the self-reliance of the patient and subjective questionnaires are filled out by the subjects to assess quality of life and hearing benefit.

The study will follow a cohort of CI-candidates becoming CI-user, till two years postoperative. A through-out assessment of both their audiological and vestibular status will be carried out at multiple fixed timepoints over 2 years, evaluating both subjective (patients reported) and objective outcomes over time. For the audiological part of the study both pure-tone and speech audiometry results will be held against the patient perceived benefit of the treatment as assessed by the questionnaires NCIQ and SSQ-12. The vestibular part of the study will evaluate the function of the SCCs and the sacculus. This is will be put in concert with functional tests of the vestibular system, and the patient perceived outcome measure DHI. Correlations between vestibular and audiological performances will be examined. A collection of specific hypotheses will be tested by predefined statistical methods.

Evaluation of a cohort of deaf children looking for autosomal recessive deafness-9 (DFNB9). Clinical and audiologic evaluation of patients with known auditive neuropathy / auditory dys-synchrony (ANAD) or recently diagnosed congenital severe to profound hearing loss (HL), and assessing genetic analysis looking for DFNB9. The investigators expect to compile genotypic and phenotypic characterization of 25 children with DFNB9 within 4 years.

This research is being done to determine whether a test that measures a "Visual Evoked Potential" can be used in a new way for individuals that have hearing loss. This test measures the participant's brain's response (so called "brain waves") to specific visual images. This study will help the investigators determine whether this test could be used to improve treatments for patients with hearing loss. The "Visual Evoked Potential" measurement test is already used in the investigator's Neurology clinic at Dartmouth Hitchcock Medical Center for various conditions to measure "early" brain responses that occur in the first 1-2 seconds after a new cue. Our research aims to explore your brain's response just after that early 1-2 second period by looking at a specific response called the "P300". The P300 wave is a brain response to new or different images or sounds. A visual evoked P300 has not been studied in individuals with hearing loss. The investigators will compare the results of this test to standard auditory tests, tests of cognitive function, and cochlear implant patient outcomes to explore how these factors can predict successful use of a hearing aid or cochlear implant.

Age-related hearing loss, presbycusis, affects up to 50% of American adults. There are two main causes for presbycusis: 1) Progressive death of hair cells in the inner ear, and 2) Central hearing loss, or the reduced ability to decipher the sound source of interest from other competing sounds in a multi-source complex environment. The first cause is better understood and treatment options, such as hearing aids and cochlear implants, are available. However, central hearing loss is not as well understood and there is not a treatment available at this time. This study aims to advance our understanding of central hearing loss by evaluating the abilities of younger and older listeners in two primary outcome measures: to 1) neurologically process sound stimuli and 2) focus on conversational speech in the presence of spatially-separated competing background noise. A test using Auditory Brainstem Responses (ABR) will be used to evaluate the brain's response to clicking sounds. This study will assess all waveform data, but will focus particularly on wave III. Sentence in noise tests will be used to assess each subject's ability to process speech in noisy situations. Both the ABR and sentence in noise tests are non-invasive and are commonly used in audiology practices to diagnosis and treat a variety of audiological pathologies.

Acute cochleo-vestibular syndrome or labyrinthitis is characterized clinically by the sudden appearance of a great rotatory vertigo and a unilateral sensorineural hearing loss. In this clinical context, MRI is the examination to eliminate differential diagnoses and to make a positive diagnosis of labyrinthitis (supposedly infectious, immunologic or ischemic). The etiologies described are ischemic, infectious or autoimmune, so the risk factors are very variable (cardiovascular, autoimmune or infectious). Labyrinthitis has been little studied as a clinical entity in its own right. Indeed, studies mainly focus on sudden deafness with subgroups of patients with vertigo. The incidence of sudden deafness is of the order of 5 to 20 per 100,000 people per year but is probably under-diagnosed. The individual and medico-economic consequences are similar to those of hearing loss, with an increased risk of dementia, depression, premature death and an increase in health care consumption.