Prognosis of Vestibular Dysfunction in Patients With Idiopathic Sudden Sensorineural Hearing Loss...
Vestibular DisorderSudden Hearing LossIdiopathic sudden sensorineural hearing loss (ISSNHL) refers to idiopathic sensorineural hearing loss of at least 30 dB over at least three test frequencies occurring over a 72-hour period. Vertigo has been considered a risk factor of poor prognosis in patients with ISSNHL. However, the clinical outcome and development of vestibular function in these patients have not been reported yet. We'd like to conduct a study on the problem whether these patients resulted in a complete recovery of the peripheral vestibular functions or compensation of the central vestibular system. If the answer is the former one, these cases might be supportive evidence of regeneration of hair cells in vestibular disorders.
A Study of Web and Tablet-based Interactive Audiometry in Adults With CF
Hearing LossSensorineural1 moreAdults with CF regularly receive anti-infective therapy that can result in impaired hearing. Recent studies have identified that up to 50% of adults with CF have hearing loss with resulting significant impact on quality of life. At present, however, there is no national recommendation to screen for hearing loss within adult CF. Formal hearing tests are costly, require specialist staff and equipment and require further outpatient visits resulting in significant impact on health, social and financial resource. We propose to analyse over a 12 month period at two adult cystic fibrosis centres in London whether outpatient tablet based audiometry testing and web-based hearing apps are able to accurately screen for ototoxicity in adults with CF, and whether these methods of screening are acceptable. The results of this study will aim to streamline audiometry screening to improve detection rates, reduce cost of healthcare, enable equity in service delivery, and minimize disruption to the patient's social and work-life using smart technology.
Analysis of Risk Factors of Neurodevelopmental Disorder in Deaf Infants Under Ten Months of Age....
Congenital Sensorineural Hearing LossAmong risk factors of neurodevelopmental disorder is congenital hearing loss. However, congenital deafness is neither necessary nor sufficient for the occurrence of warning signs of neurodevelopmental disorder. The investigative team hypothesizes that the co-occurrence of these two clinical entities results from a common origin, within a syndromic diagnosis including other medical issues. These situations would therefore be very different from those of babies suffering from isolated sensorineural deafness. This study aims to identify which factors are statistically correlated with the association of congenital deafness and early symptoms of neurodevelopmental disorder, thanks to a partnership between a regional center for psychiatry of deaf children (Therapeutic Childhood and Deafness Unit of St Maurice Hospitals, UTES) and a pediatric audiophonology department (ENT department of the Necker-Enfants Malades hospital). This work is based on a cohort of 26 children under ten months of age diagnosed with severe to profound congenital sensorineural hearing loss. As part of their pre-implantation cochlear assessment, a consultation was filmed to precisely analyze the interactional and sensorimotor skills of these children (clinical observations, administration of a specific evaluation grid called Olliac Grid), after reviewing the films. The investigative team will then be able to grade the neurodevelopmental risk. In order to clarify what factors are linked with a neurodevelopmental risk, medical data concerning the child's family context, birth, and audiological profile will also be collected, and put in perspective with the assessed neurodevelopmental risk gradation.
Compassionate Use Arm - ABI541 ABI for 10 NF2 Patients
Neurofibromatosis Type 2Severe Profound Sensorineural Hearing LossThe Nucleus 24 Auditory Brainstem Implant (ABI) is the only FDA approved device for restoration of meaningful hearing in Neurofibromatosis Type 2 (NF2) patients. This device has been discontinued, meaning that there is no commercially approved device currently available. The replacement model, the ABI541 (an unapproved device), is being investigated in ongoing clinical trials. A compassionate use arm of a clinical trial allows patients with NF2 to be implanted with this new ABI.
Hyperbaric Oxygen Therapy Registry
Air or Gas EmbolismCarbon Monoxide Poisoning13 moreThe purpose of the Hyperbaric Oxygen Therapy Registry (HBOTR) is to provide real world patient outcome and side effect information from electronic health records submitted to a specialty specific hyperbaric registry as part of "Stage 2 of Meaningful Use," including data provided to meet PQRS requirements via the registry's QCDR mission. Goals include understanding the value of HBOT among patients treated for a variety of conditions in relation to the frequency and severity of HBOT side effects. While randomized, controlled trials can establish the efficacy of treatments like HBOT, because they routinely exclude patients with co-morbid conditions common to those patients seen in usual clinical practice, the results of RCTs are usually non-generalizable. Real world data can be used to better understand the effectiveness of HBOT among typical patients, as well as the risks associated with treatment.
ABR Characteristics and Salivary Cortisone Concentration in Noise-exposed Workers
Sensorineural Hearing LossNoise Induced Hearing Loss1 moreThe aim of the research: Investigate the association of auditory evoked brainstem response characteristics with salivary cortisone concentrations in noise-exposed workers. Purpose of the research: Investigate the magnitude of the amplitudes and latencies of auditory brainstem responses. Investigate the concentration of cortisone in saliva. To investigate the correlation of the characteristics of auditory evoked brainstem potentials with the age of the subjects or the length of work in noise. Investigate the association of salivary cortisone concentration with subjects' age or length of work in noise. Investigate the prevalence of mild, moderate and severe hearing impairment in the 19-35 age group. Investigate the relationship of hearing threshold shift with the age of the subjects or the length of work in noise. To investigate the difference in the characteristics of auditory evoked brainstem potentials and the difference in salivary cortisone concentration between subjects with a bilaterally normal hearing threshold (≤25 dB) at high frequencies and subjects with unilaterally or bilaterally increased hearing threshold (> 25 dB) at high frequencies . Investigate the perceived stress and psychosocial risk factors in the workplace and their relationship to salivary cortisone concentrations. Research Hypothesis: There is an association between the characteristics of auditory evoked brainstem response and salivary cortisone concentrations in noise-exposed workers. Research participants Inclusion criteria: male and female ages 19-35 exposure to noise level ≥ 85 dB (A) per week at the workplace work in noise from 1 to 16 years workplace not involving exposure to carbon disulfide or a mixture of organic solvents that have toxic effects on the ear (toluene, xylene and styrene) unilaterally or bilaterally normal otoscopic findings unilaterally or bilaterally tympanogram: peak pressure value ± 50 daPa at 226 Hz with eardrum mobility of 0.3 to 1.3 mL Exclusion criteria: information about an earlier sudden hearing loss information on chronic middle ear inflammation information on ear surgery (except placement of ventilation tubes in the eardrum) information on dizziness associated with hearing loss and noise information on insomnia current use of oral corticosteroids bilaterally found air-bone gap greater than 15 dB
Electrocochleography Function for Monitoring Residual Hearing
Hearing LossCochlearThis trial is a pilot study to assess the feasibility of using Electrocochleography recorded from a cochlear implant intra-operatively in real time to monitor the progress of its insertion. Correlation between: Observed changes in this signal during surgery and residual hearing loss post-operatively and Recordings of this signal post-operatively and actual pure tone audiometry results will also be assessed.
Investigation of the Automatic Technology on the Naída M Hearing Devices
Cochlear Hearing LossIn this clinical trial, an un-controlled, repeated measures open design with within-subject comparison will be used to evaluate the effect of the device under investigation on sound perception. This design was shown to be successful in previous studies for the evaluation of sound coding strategies. Furthermore, a within-subject comparison decreases the variance in the results allowing for fewer subjects when the population using the investigational device is not large in general.
The SeaSHeL National Prospective Cohort Study
Sensory Hearing LossSensorineural Hearing Loss3 moreEach year, approximately 15,000 people in the United Kingdom experience sudden loss of hearing that is sensorineural in nature. In the majority of cases, the cause is unknown despite investigation, and these cases are termed idiopathic 'sudden onset sensorineural hearing loss' (SSNHL). Treatment options for idiopathic SSNHL mainly include steroid treatments, with considerable limitations in their effectiveness and evidence base. There are a number of new treatments being developed for idiopathic SSNHL based upon recent discoveries in underlying molecular mechanisms. These treatments require rigorous testing in clinical trials before they can become available for clinical use. To allow for such trials to be run effectively, there is an urgent need for information on patient numbers, geographical distribution, demographics, patient and treatment pathways, as well as outcomes. This study proposes to collect these data through an ENT trainee and Audiologist led nationwide prospective cohort study of adult patients presenting with SSNHL within the National Health Services (NHS). The study will take place at 97 NHS sites across England with Ear, Nose, and Throat (ENT) and Audiology services. Data once collected will be analysed to: Establish the patient pathway for patients presenting with SSNHL in the NHS Develop a tool that will help predict recovery for patients with idiopathic SSNHL. Establish the impact of idiopathic SSNHL on people's quality of life.
Enlarged Vestibular Aqueduct Registry
Enlarged Vestibular AqueductSensorineural Hearing LossPeople with the congenital anatomical malformation known as "Enlarged Vestibular Aqueduct" (EVA) may experience decline in hearing ability. This is an online registry to gather patient experiences with EVA, with the goal of providing future researchers with the information necessary to better evaluate and make recommendations for patients with EVA. Data will be obtained from volunteers who enroll and submit their information on a website, https://eva.uhhospitals.org, and a limited data set may be provided to researchers interested in analyzing repository data. The limited data set would not contain any identifiable information except for ages, dates such as date of diagnosis, or city/state of residence. EVA Research Project Website: http://rainbow.org/EVAResearch EVA Patient Registry Website: https://eva.uhhospitals.org