Clinical Research Directory

Genetic and Epigenetic Background of Inner Ear Dysfunction in Turner Syndrome

The goal of this case-control study is to pave the way for new revolutionary treatment measures within hearing loss that could either replace or delay the need for hearing aids. The study focuses on people with Turner syndrome (TS). The aim is to find out if there are specific DNA methylation patterns and/or RNA expression profiles linked to sensorineural hearing loss (SNHL) in people with TS. Additionally, the structure and function of the inner ear in these individuals will be examined to see if there is a connection to their epigenetic profile. The main question it aims to answer is: Does epigenetics constitute a common denominator for some of the unexplained SNHL cases? Turner Syndrome (TS) represents an ideal model for studying epigenetics related to sensorineural hearing loss (SNHL). Participants will undergo the following tests: * Ear examinations * Hearing tests * Balance tests * Blood tests * MRI scans * CBCT (cone-beam computed tomography) scans

Vestibular and Postural Function in an Unselected Group of Children With Sensorineural Hearing Loss

The goal of this prospective cohort study is to investigate the vestibular function in children with unilateral or bilateral sensorineural hearing loss. The main hypothesis of the study is that abnormal vestibular test results will be found in 20-30 % of the children with sensorineural hearing loss. The participants will be children in the age of 3-10 years with sensorineural hearing loss. The test protocol consists of questionnaires and vestibular and postural assessments.

Genetic Analysis of Hereditary Disorders of Hearing and Balance

This study will try to identify the genetic causes of hereditary hearing loss or balance disorders. People with a hearing or balance disorder that affects more than one family member may be eligible for this study. They and their immediate family members may undergo some or all of the following procedures: * Medical and family history, including questions about hearing, balance and other ear-related issues, and review of medical records. * Routine physical examination. * Blood draw or buccal swab (brushing inside the cheek to collect cells) - Tissue is collected for DNA analysis to look for changes in genes that may be related to hearing loss. * Hearing tests - The subject listens for tones emitted through a small earphone. * Balance tests to see if balance functions of the inner ear are associated with the hearing loss In one test the subject wears goggles and watches moving lights while cold or warm air is blown into the ears. A second test involves sitting in a spinning chair in a quiet, dark room. * Photograph - A photograph may be taken as a record of eye shape and color, distance between the eyes, and hair color. * Computed tomography (CT) and magnetic resonance imaging (MRI) scans - These tests show the structure of the inner ear. For CT, the subject lies still for a short time while X-ray images are obtained. For MRI, the patient lies on a stretcher that is moved into a cylindrical machine with a strong magnetic field. The magnetic field and radio waves produce images of the inner ear. The radio waves cause loud thumping noises that can be muffled by the use of earplugs.

Randomized Controlled Trial of Valganciclovir for Cytomegalovirus Infected Hearing Impaired Infants

The overall goal of this study is to determine the clinical benefit and safety of antiviral therapy for asymptomatic congenital cytomegalovirus (cCMV) infected hearing-impaired infants. We will conduct a multi-center double-blind randomized placebo-controlled trial to determine whether hearing-impaired infants with asymptomatic cCMV have better hearing and language outcomes if they receive valganciclovir antiviral treatment. We will also determine the safety of antiviral valganciclovir therapy for asymptomatic cCMV-infected hearing impaired infants. This study will be unique in that the cohort enrolled will only include hearing-impaired infants with asymptomatic cCMV. Primary Objective: To determine if treatment of cCMV-infected hearing impaired infants with isolated hearing loss with the antiviral drug valganciclovir reduces the mean slope of total hearing thresholds over the 20 months after randomization compared to untreated cCMV-infected infants with isolated hearing loss. Main Secondary Objectives: 1. To determine if valganciclovir treatment improves the following outcomes when compared to the control group: 1. The slope of best ear hearing thresholds over the 20 months after randomization. 2. The MacArthur-Bates Communicative Development Inventory (CDI) percentile score for words produced at 20 months of age. 2. To evaluate safety measures based on all grade 3 or greater new adverse events designated by the NIAID Division of AIDS (DAIDS) toxicity tables.

Perimodiolar Implant Performance in Adults With Low-Frequency Residual Hearing

The purpose of the feasibility study is to investigate hearing performance (audiometry and speech perception) using the CI632 in a group of adults (n=15) with low-frequency residual hearing who meet inclusion criteria.

First In Human Randomised Trial of Rincell-1 in Adults With a Cochlear Implant

Rinri Therapeutics is conducting a clinical trial of a new cell therapy called Rincell-1. Rincell-1 is being developed to treat adults with neural hearing loss, either age related hearing loss or auditory neuropathy, who also meet criteria for a cochlear implant. The goals of this study are: * To learn about the safety profile of Rincell-1, the procedure used to inject it and the medications given to promote the growth of the cells * To evaluate how well Rincell-1 works by measuring changes in the function of auditory neurons. * To understand if Rincell-1 can be easily and successfully given at the same time as cochlear implant surgery Participants will be randomly assigned to one of two groups: one group that receives a standard care cochlear implant, and the other that will receive an injection of Rincell-1 at the same time as their standard care cochlear implant. Researchers will compare the safety of Rincell-1 in combination with a cochlear implant to a cochlear implant on its own. Participants will take part in the trial for 52 weeks after CI surgery. During that time, they will have regular follow-ups and will take daily measurements at home of their hearing health.

Retrospectively Investigating Longitudinal Outcomes in MED-EL SONATA 2 Cochlear Implant Recipients.

MED-EL Cochlear Implants (CI) provide auditory sensations via electrical stimulation of the auditory pathways for severely to profoundly hearing-impaired individuals who obtain little or no benefit from acoustic amplification in the best aided condition. However, there is only few evidence from large long-term observational studies available to gain knowledge on clinical effectiveness of cochlear implants. With continued pressure on healthcare system budgets, there is an increasing demand for evidence-based research that demonstrates an intervention is effective in the real-world environment across large and diverse populations. Currently, there is a paucity of data concerning the long-term outcomes of subjects implanted with a SONATA 2 cochlear implant from representative samples of the population. This retrospective study aims at analyzing SONATA 2 cochlear implant data from clinical routine.

Clinical and Genetic Analysis of Enlarged Vestibular Aqueducts

This study will try to identify and understand the genetic factors that lead to an inner ear malformation called "enlarged vestibular aqueducts", that can be associated with hearing loss. Patients with sensorineural hearing loss with or without inner ear malformations and their parents and siblings may be eligible for this study. Participants and their immediate family members, may undergo some or all of the following tests and procedures: * Medical and family history, including questions about hearing, balance and other ear-related issues, and review of medical records. * Routine physical examination. * Blood draw or buccal swab (brushing inside the cheek to collect cells) - Tissue is collected for DNA analysis to look for changes in genes that may be related to hearing loss. * Hearing tests - The subject listens for tones emitted through a small earphone. * Balance test (VEMP) to see if balance functions of the inner ear are associated with the hearing loss Electrodes will be placed behind your ear and at the base of your neck. From a reclining position, you will be asked to raise your head while clicking sounds are played into your ears. - Ultrasound tests - An inner ear malformation called EVA (enlargement of the vestibular aqueduct) indicates that a genetic disorder called Pendred syndrome may be the cause. Because thyroid abnormalities are also associated with Pendred syndrome, an ultrasound examination of the thyroid gland may be done. * Computed tomography (CT) and magnetic resonance imaging (MRI) scans - These tests show the structure of the inner ear. For CT, the subject lies still for a short time while X-ray images are obtained. For MRI, the patient lies on a stretcher that is moved into a cylindrical machine with a strong magnetic field. The magnetic field and radio waves produce images of the inner ear. The radio waves cause loud thumping noises that can be muffled by the use of earplugs.

Clinical Evaluation of Hearing Aid OTC Self-Assessment Methods for Adults With Perceived Mild-to-Moderate Hearing Loss

The goal of this clinical trial is to compare two methods of self assessment of hearing with conventional audiometry in an OTC Hearing Aid for adults with perceived mild to moderate hearing loss. The main questions it aims to answer are: 1. To evaluate agreement between prescribed gain outputs generated from conventional audiometry and those generated from the self-assessment response method. 2. Determine whether either self-assessment response method provides acceptable estimated audibility. 3. Evaluate participant preference between the detection response method and the audibility-release response method. Researchers will compare the two novel methods with conventional audiometry to determine levels of agreement. Participants will self assess their hearing methods using two novel methods of hearing assessment as well as receive a conventional audiometric evaluation of hearing levels.

Middle Ear Optical Coherence Tomography Imaging and Vibrometry in Patients With Hearing Loss

This study involves imaging ears with hearing loss with a new system that allows the user to see the middle ear bones and measure their movement in response to sound.

Anatomy-Based Fitting in Bimodal Cochlear Implant Users

The purpose of the study is to investigate the effect of an anatomy-based frequency distribution in comparison to a default frequency distribution in bimodal CI patients, i.e. wearing a cochlear implant and a contralateral hearing aid. Speech perception in quiet, speech perception in noise, consonant/vowel discrimination and pitch/timbre perception will be assessed in an acute set-up with both the default fitting map and the anatomy-based fitting map.

Evaluation of Electrode-modiolus Distance and Cochlear Fibrosis Using Depth Sounding and Spectroscopy Tools

Cochlear fibrosis development can compromise the success and the outcomes of the cochlear implantation (CI) thus affecting the quality of life of the implanted patient. Correlating the results of the Transimpedance Matrix (TIM) measurements to the implant electrode location determined by the Cone Beam Computer Tomography (CBCT), this study aims to identify a range of TIM profiles within the implanted population, certain profiles suggesting the growth of the fibrosis tissue in cochlea

Long-term Follow-up of a Cochlear Implant With Dexamethasone Eluting Electrode Array

A long-term follow-up of Cochlear's cochlear implant electrode array which passively elutes dexamethasone for a defined period of time to help reduce inflammatory responses.

Perceptual Adaptation Following Cochlear Implantation (Aim 3a)

The goal of this study is to study the adaptation process in newly implanted Single-Sided Deaf Cochlear Implant (SSD-CI) users. Subjects will use four frequency maps for the first month after CI activation. One of these maps will be standard of care and the other three will have higher low frequency edges than the standard of care default maps. These maps will alternate daily during the first month (with exceptions made for situations when the subject is allowed to use a preferred map rather than the map predetermined for use that day), and after that point each subject will make a selection at the beginning of the week to compare all four maps and decide which one to use at the beginning of the following week. The goal of this study is to investigate how reducing place-pitch mismatch in SSD CI users affects 1) sound quality, 2) device use, and 3) speech perception.

Anatomy-Based Fitting in Unexperienced Cochlear Implant Users

Severe to profound hearing loss affects 0,8% of the global population. For these people, a conventional hearing aid often does not provide sufficient benefit. However, these people can benefit from a cochlear implant (CI). A CI needs to be individually programmed (fitted) for each recipient. A fitting "map" is defined as a set of electrical parameters that are individually adapted to a recipient's needs to achieve optimal sound perception. At present, most CI recipients are fitted with a default frequency allocation map that doesn't take individual variability in size and shape of the cochlea into account. In this study, a fitting strategy based on the post-operative CT scan, that will allow the audiologist to set a frequency-band distribution for CI fitting that may be more closely aligned to the natural tonotopic frequency distribution of a normal hearing cochlea, will be evaluated.

Yiyuancong for the Treatment of Sudden Sensorineural Hearing Loss

This project aims to evaluate the clinical efficacy of Yiyuancong, an independently developed traditional Chinese medicine (TCM) formula based on the homology of medicine and food, in participants with sudden sensorineural hearing loss (SSNHL) and to elucidate its potential underlying mechanisms. This study is designed as a multicenter, randomized controlled trial (RCT), utilizing dynamic randomization for participant allocation. Due to the requirement for the experimental group to continuously administer the TCM preparation for 1-2 months, this study is conducted as an open-label (non-blinded) trial. All participants will undergo audiological evaluations at baseline, day 7 of treatment, and upon completion of the first and second treatment courses. Based on previous research regarding integrated traditional Chinese and Western medicine for SSNHL, the estimated effect size (ES) for the improvement of pure-tone thresholds compared with Western medicine alone ranges from 0.5 to 0.8. Assuming an effect size of 0.5 for the difference in pure-tone audiometry (PTA) improvement between the experimental and control groups, with a significance level (α) of 0.05 (one-tailed) and a statistical power (1-β) of 0.8, the required sample size calculated via G\*Power software is 51 per group. Accounting for a 20% dropout rate, the investigators plan to enroll 64 participants per group (total N = 128).

Regenerative Medicine and Stem Cell-Based Interventions for Inner Ear Trauma, Tinnitus, and Sensorineural Hearing Loss

The HEAR-RESTORE study is an exploratory clinical research program designed to evaluate new diagnostic and regenerative approaches for individuals experiencing inner ear injury, tinnitus, and temporary or permanent hearing loss. Inner ear disorders can result from acoustic trauma, infections, inflammation, or other conditions that affect the cochlea, auditory nerve, or vestibular system. These conditions may lead to muffled hearing, ringing in the ears (tinnitus), balance disturbances, or reduced hearing sensitivity. The purpose of this study is to better understand how inner ear injuries develop and to evaluate potential treatments that may support hearing recovery or protect auditory function. Participants will undergo comprehensive ear and hearing evaluations including audiometry, speech recognition testing, tympanometry, and other diagnostic assessments used in audiology and otolaryngology. These tests help measure hearing thresholds, ear pressure, and auditory nerve responses. The study may also explore emerging regenerative medicine strategies aimed at restoring inner ear function. These approaches may include investigational therapies designed to promote repair of cochlear hair cells, reduce inflammation in the inner ear, or support neural recovery in the auditory pathway. Research may include evaluation of regenerative biological therapies, stem cell-based approaches, and other experimental technologies intended to support recovery of hearing or reduce symptoms such as tinnitus. Participants will be monitored over time to assess hearing outcomes, safety, and changes in auditory function. Study assessments may include repeat audiology tests, imaging studies, symptom questionnaires, and other clinical measurements used to track hearing health. The results of this research may contribute to improved diagnostic methods and potential future treatments for individuals with hearing loss or inner ear disorders.

Pivotal Clinical Trial Evaluating the Safety and Efficacy of the Fully Implanted Acclaim Cochlear Implant

The goal of this clinical trial is to learn if the Acclaim CI works to treat severe to profound sensorineural hearing loss in adults. It was also learn about the safety of the Acclaim CI implant. The main questions it aims to answer are: * Does the Acclaim CI device help participants hear words better compared to before the implants? * What medical problems do participants have after the Acclaim CI device was implanted? Participants will: * Have the Acclaim CI implanted; and * Visit the clinical site for checkups and tests at 1-Month, 3-Months, 6-Months, and at 1-year and 2-years after the device has been turned on.

Optimizing Cochlear Implant Laterality in Patients With Unilateral Vestibular Weakness

In patients with symmetric hearing loss who meet traditional cochlear implant candidacy criteria yet demonstrate pre-operative unilateral vestibular weakness, the choice of which ear to implant may impact their post-operative vestibular course. The investigators aim to implement both objective videonystagmography metrics as well as subjective patient-reported outcome measures to assess whether selecting laterality for cochlear implantation as it relates to unilateral vestibular loss has a measurable impact on patients' post-operative vestibular function. In pursuing cochlear implantation on the ear that demonstrates worse vestibular function, the investigators hypothesize improved vestibular function in comparison to operating on the unaffected side. The randomized control pilot study plans to allocate 10 subjects to a treatment group that involves cochlear implantation on the ipsilateral side of vestibular hypofunction and a control group that will undergo implantation on the contralateral side. The investigators plan to compare scores derived from the Dizziness Handicap Inventory completed at pre-operative evaluation and at two subsequent post-operative visits between control and treatment groups as primary outcome. The investigators also plan to determine if there is a significant difference in measures derived from caloric vestibular testing pre- and post-operatively as well as between control and treatment groups.

Healthy heaAring for Healthy Ageing: Data-driven Hearing Rehabilitation Intervention to Promote Healthy Hearing

The goal of the HAHA trial is to prove that the treatment of HI and prevention of HI-related cognitive decline are most likely to be effective if HI is approached as a broader neurodegenerative entity with multifaceted manifestations currently unaddressed in clinical practice and managed using a novel individualised data-driven protocol for early hearing rehabilitation. The main hypothesis is that, compared with standard care, the data-driven rehabilitation will likely have broader benefits manifested in three key areas: hearing, cognition, and quality of life and psychosocial outcomes. Participants will be randomized 1:1 to either intervention (individualised data-driven hearing rehabilitation) or control group (standard care hearing rehabilitation). The primary objective of this study is to investigate the effect of an optimized data-driven hearing rehabilitation protocol versus standard care protocol on change in speech perception in noise (SPIN) in older adults with mild to moderately severe sensorineural HI and without dementia.

Protective Effect of Acetylcysteine Against Cisplatinum-Induced Ototoxicity: A Randomized Controlled Trial

The purpose of this study is to evaluate the efficacy of N-acetylcysteine (NAC) in preventing cisplatin-induced ototoxicity in patients receiving cisplatin-based chemotherapy. Cisplatin is a highly effective chemotherapeutic agent but often leads to permanent hearing loss (ototoxicity) as a significant side effect. This study investigates whether the administration of NAC as an otoprotective agent can reduce or prevent the decline in hearing sensitivity, particularly at extended high frequencies, as measured by audiometry and distortion product otoacoustic emissions (DPOAE).

Comparative Analysis of Hearing Outcomes: Robotic vs. Manual Insertion of Cochlear Implants

This research study is evaluating two different methods of cochlear implant (CI) insertion - robotic-assisted insertion and manual insertion - to better understand how they affect hearing outcomes. Participants in this study will be randomly assigned to receive their cochlear implant using one of these two techniques. Both methods are performed in a standard operating room by qualified surgeons, and both are considered safe and approved for use. The main goal is to compare how well participants hear one year after surgery based on the insertion method used. The study will also look at things like surgical time, inner ear health, and how the hearing nerve responds. All participants will receive the same type of cochlear implant device and follow-up care. This study may help guide future surgical techniques and improve outcomes for individuals receiving cochlear implants.

Quantification of Visually Evoked Cortical Potentials in Individuals With Hearing Loss

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.

Hearing Preservation in Cochlear Implantation Surgery

In order to preserve the residual hearing in patients with sensorineural hearing loss (SNHL) receiving a cochlear implant (CI), the insertion trauma to the delicate and microscopic structures of the cochlea needs to be minimized. The surgical procedure starts with the conventional mastoidectomy-posterior tympanotomy (MPT) approach to the middle ear, and is followed by accessing the cochlea, with either a cochleostomy (CO) or via the round window (RW). Both techniques have their benefits and disadvantages. Another aspect is the design of the electrode array. There are fundamentally two different designs: a \*straight\* lateral wall lying electrode array (LW), or a \*pre-curved\* perimodiolar cochlear lying electrode array (PM). Interestingly, until now, the best surgical approach and type of implant is unknown. Our hypothesis is that the combination of a RW approach and a LW lying electrode array minimizes insertion trauma, leading to better hearing outcome for SNHL patients.