Active clinical trials for "Hearing Loss"

In the UK, cochlear implantation is the standard treatment for adults with 'severe-to-profound' deafness. This level of deafness means that without a cochlear implant they have difficulty understanding what people are saying even when they wear hearing aids. The NHS provides a cochlear implant for one ear even though these adults are deaf in both ears. Two cochlear implants could help them understand speech in noisy environments, know where to look to see who is talking, and avoid hazards outdoors. The decision about who can receive cochlear implants and how many they can receive is based on guidance from the National Institute for Health and Care Excellence (NICE). NICE agreed that providing two cochlear implants can provide additional benefits over providing just one implant. However, they decided that there is not enough evidence to show that these additional benefits are worth the additional cost of providing the second implant. NICE recommended that a clinical trial should be conducted to gather this evidence. This study will consult with patients to explore whether the trial would be acceptable to future implant recipients. The study will also work with clinicians to design a trial comparing bilateral and unilateral implantation in adults. These clinicians will also be surveyed to assess the acceptability of the trial. A group of clinicians that span the range of professions involved in delivering the care pathway will be invited to attend a trial design workshop to establish a consensus on acceptable trial design elements from a clinical perspective. Representatives from cochlear implant manufacturers will be consulted to determine possible strategies for securing industry support for a future trial. Those who run cochlear implantation services will be engaged in a working group to gather information on the current care pathway to inform future assessments of whether bilateral implantation could be cost-effective.

One of the main factors affecting the ability to maximize the full potential of a cochlear implant is an accurate map.The goal of mapping is to enable cochlear implant recipients to perceive a desired range of acoustic signals. The process includes programming of the minimum and the maximum stimulation levels that are based on subjective measurements of thresholds and the most comfortable level . The use of objective measures in the CI process has greatly contributed to the definition of the dynamic field, as they provide specific values that serve as the basis for the start of the mapping process, especially in cases of infants and young children. Some examples of these measures are electrically evoked stapedius reflex threshold , neural response telemetry , brainstem auditory evoked potential , and P300, among others.

The study is part of the Post Market Clinical Follow (PMCF) activity. The investigational device is the CE-marked Ponto 3 SuperPower (available on the market since December 2016) already fitted to the subjects, and data is collected retrospectively from clinical routine visits. The study is a retrospective study. Therefore there will be no study visits for treatment or follow-up in the study. Subject inclusion for this clinical investigation will be performed among subjects who has been fitted with the Ponto 3 SuperPower. To have a wide representative selection of subjects, enrollment will be spread over the 4 years since the device was available on the market since December 2016. The endpoints in the study will investigate the improvement of hearing with the Ponto 3 SuperPower. The data will be collected from the subjects in the time period from the clinical routine visit where the Ponto 3 SuperPower where fitted or just prior, and from the clinical routine visit following the fitting, where audiologic fitting follow-up procedures are performed (approximately 3 months).

Introduction: Bilateral-bimodal users may benefit from bilateral-binaural and a bimodal effect . The two modes of stimulation complement each other and may overcome the limitation of electric hearing alone. However, alongside the expected benefits of having two hearing systems in bilateral bimodal hearing, there are several inherent mismatches in combing two different stimulation methods, which may negatively impact the processing of the binaural cues. Aim: To determine whether bimodal listening with a hearing aid programmed using a dedicated bimodal fitting formula, which aligns the frequency response, loudness growth functions, and automatic gain control (AGC) characteristics between the cochlear implant speech processor and the hearing aid brings additional benefit over bimodal listening with the subject's clinical hearing instrument via the use of using a comprehensive battery of speech perception tests aimed at different aspects of binaural hearing as well as the contribution of the unique bimodal complementary effect. A secondary goal is to identify the tests that are best suited to predicting and evaluating bimodal benefit. Method: The study will include 20 Hebrew and 20 Arabic speakers' adult bilateral-bimodal users that their audiometric thresholds in the non implanted ear will be no worse than 75 dB HL at 250 and 85 dB HL at 500 Hz, who use their hearing aids for at least 75% of their waking hours. The added benefit of the hearing aid programmed using a dedicated bimodal fitting formula will be evaluated using six task-specific tests designed to sensitively assess the bilateral-binaural and bimodal complementary effects using roving speech and noise and different types of background noises (maskers), pitch-related task and subjective questionnaire. Participants will be tested twice in quick succession, at two-weekly intervals, in establishing a baseline score with their clinical HAs. Then participants will be tested again twice in quick succession, at two-weekly intervals following three month experience with the HA fitted with the bimodal fitting formula. All tests will be administered the CI/HA listening condition.

The purpose of this evaluation is to receive the greatest benefit of new custom made hearing product technology and shell modification for the end customer and to continual improve the custom made hearing products.

The purpose of this study is to identify factors that may contribute to conductive hearing loss. Conductive hearing loss is a type that is due to a problem with the outer or middle ear. Because of this, sound does not travel through the ear normally. Perforated tympanic membranes of study patients will be photographed during the patient's routine visit using a digital otoscope. These pictures will be used to analyze perforation size as an absolute value as well as a percentage of the tympanic membrane. Audiometric results and CT scans of temporal bone (reports and images) collected as standard of care will be evaluated. Images of CT scans will be imported into a medical imaging software for creation of anatomically realistic 3D models of the middle ear and mastoid air space. Structural analysis on each 3D model will be conducted and analyzed, the volume of middle ear and mastoid air spaces will be recorded, and data points will be correlated with perforation size and location to audiogram results.

Development of a new mass spectrography-based biomarker for the early and sensitive diagnosis of Fabry disease from the blood

The aim of this study is to investigate short and long term consequences from early postnatal HCMV infection transmitted via human milk in very preterm infants (birth weight < 1500 g or gestational age < 32 weeks). These infants are at high risk of early death or survival with chronic disease and neurodevelopmental impairment if infected with HCMV. Infection is a common complication in this group of patients and reported to be the most frequent cause of death after the second week of life. Systemic infection in the newborn period is reported as representing an independent risk factor for survival with neurodevelopmental impairment among very preterm infants.

Congenital hypothyroidism (CH) occurs in 1:4000 live births. Neurological disturbances like speech delay, motor delay and lower IQ were reported in children with CH. Evidence from animal experiments indicate that CH is associated with high frequency of deafness. Morphological changes of the external and internal cholera hairs were reported in mutagenic mice with CH. Anatomical changes of the internal ear and low hearing threshold were reported in mice bearing Dual oxidase 2 (Duox2) gene mutations, which is responsible of oxidation of iodide. Knockout of Pax8 gene in mice resulted in both agenesis of thyroid gland and deafness. Early L-thyroxin therapy prevented the development of deafness in those mice. The expression of thyroid receptor α (TRα) in the external and internal cholera hairs in mice indicates that the thyroid hormones have an important role in the development of the internal ear. The etiology of deafness in human is both genetic and environmental. The prevalence of deafness in live births is 1:1000 and among them 60% is genetic. Connexin 26 gene mutations are the most common cause of inherited deafness and account for about 40% of the genetic cases. In two conditions in human the coexistence of hypothyroidism and deafness were reported; Pendred syndrome and thyroid hormone resistance syndrome. The prevalence of hearing loss in human with CH was explored in only few studies and the results are contrary. The aim of the current study is to study the prevalence of hearing loss among children with CH and to compare the clinical, biochemical and genetic characteristics between subjects with hearing loss to those without hearing loss.

Next Generation Sequencing (NGS) strategy is a powerful tool to identify genes implicated in very rare diseases for which the previous genetic explorations remain negative to date. The aim of this project is based on groups of patients with original clinical phenotypes including neurosensory impairment without genetic cause identified to date. The investigators will study these families using whole exome sequencing to potentially identify new genes and new underlying biological pathways involved in neurosensory diseases.