Deafness, aminoglycoside-induced

This study tests a new type of stem cell transplant for children with primary immune deficiencies or inherited metabolic disorders. The transplant uses stem cells from a half-matched family donor, which are specially processed to remove certain immune cells. The goal is to see if…

This study tests a bone marrow transplant from a family donor for children under 21 with severe non-cancer blood disorders like sickle cell disease, bone marrow failure, or immune problems. The goal is to see if the transplant can replace the diseased cells with healthy donor cel…

This study tests whether placing a cochlear implant's ground electrode near the vagus nerve behind the ear works as well as the standard placement under a muscle. Twelve adults getting a cochlear implant will participate. The main goal is to see if speech understanding in noise i…

This study follows 15 adults (ages 22–90) with severe, long-term balance disorders caused by inner ear damage. Participants have already received a vestibular implant, a device that electrically stimulates the balance nerve to help restore steadiness and clear vision during movem…

This study tests a vestibular implant in 15 older adults (ages 65-90) with chronic balance issues due to bilateral vestibular hypofunction. The implant electrically stimulates the inner ear to improve balance and vision. Researchers will measure changes in gait and eye reflexes o…

This study tests a vestibular implant, a device surgically placed in the inner ear to electrically stimulate balance nerves. It aims to improve balance, posture, and vision in up to 8 adults with bilateral vestibular hypofunction, a condition causing chronic dizziness and instabi…

This program offers extra stem cells from a half-matched family donor to patients who are getting a cord blood transplant for serious blood cancers or immune disorders. The goal is to help the bone marrow recover more quickly while the cord blood cells take over permanently. It i…

This study tests an auditory brainstem implant in 20 children who are deaf because they are missing the inner ear or the nerve that carries sound to the brain. The device is surgically placed to stimulate the brainstem directly, bypassing the missing parts. Researchers will monit…

This study compares two common hearing screening methods—pure-tone audiometry and otoacoustic emissions—in over 13,000 preschool children. The goal is to determine which method is better at identifying hearing loss early, so children can get the help they need to avoid speech and…

This study is testing whether changing the frequency settings on a cochlear implant can improve sound quality and speech understanding for people who use both a cochlear implant and a hearing aid. Thirteen experienced bimodal users will try an experimental frequency setting for o…

This Phase 2 trial tests whether KL1333 can reduce fatigue and improve leg strength in adults with primary mitochondrial disease, a genetic condition that affects energy production. About 180 participants will receive either KL1333 or a placebo twice daily for 48 weeks. The study…

This study tests a new way for hearing aids to process soft sounds, aiming to make quiet speech clearer. Sixteen adults with hearing loss will try two different hearing aid settings in a lab and rate which one sounds more natural and understandable. The goal is to improve hearing…

This study tests whether automatic sound processing in cochlear implants helps children with hearing loss understand speech in noisy environments. Twenty children aged 6 to 16 who use cochlear implants will compare three different sound settings. The goal is to see which setting …

This study tests whether a new AI-based noise reduction feature in Phonak hearing aids helps people with hearing loss or cochlear implants hear speech more clearly in noisy settings. Researchers will measure how well 500 participants repeat words and sentences in quiet and noisy …

This study is testing a new software algorithm called SEDA that reduces background noise for cochlear implant users. Researchers will measure how well 60 participants (ages 12 and older) understand speech in quiet and noisy settings with and without the algorithm. The goal is to …

This study tests a new hearing aid, Audiocap, in 100 adults with mild-to-moderate hearing loss who have never used a hearing aid before. The goal is to see if it improves hearing better than a placebo device after one month of use. Participants will undergo standard hearing tests…

This study compares using virtual reality glasses versus the standard 'show-do' technique to reduce dental anxiety in 54 children with moderate to severe hearing impairment. The children will have their pulse and anxiety scores measured during a dental cleaning. The goal is to se…

This study tests whether community healthcare workers can effectively fit hearing aids using a smartphone app in low- and middle-income communities. 90 adults with mild to severe hearing loss will be randomly assigned to receive either a smartphone-based fitting, a pre-set fittin…

This study aims to better understand and treat people with certain inherited metabolic or genetic disorders. Researchers will use standard medical tests like blood work and imaging to diagnose and care for participants, who may also join other related studies. The goal is to expa…

This study at the National Institutes of Health looks at how infections can worsen symptoms in people with mitochondrial disease, a group of disorders that affect energy production in cells. Researchers will evaluate participants' immune systems through blood tests, physical exam…

This study looks at children with suspected or confirmed genetic and metabolic disorders to find new disease-causing gene mutations. Researchers will analyze blood samples for DNA and metabolites, and in some cases take a small skin sample. The goal is to better understand these …

This study at Necker Hospital in Paris is checking how well patients with inherited metabolic diseases follow their daily oral medication routines. About 200 patients (children aged 7 and up, teens, and adults) will fill out a questionnaire during a regular visit. The goal is to …

This study enrolls up to 15,000 children with known or suspected endocrine or metabolic disorders, along with their family members. Researchers will collect medical records, blood, saliva, and other samples to identify genetic changes linked to these conditions. The goal is to be…

This study is creating a large collection of blood, urine, stool, and heart tissue samples from 10,000 people with and without heart or metabolic conditions. The goal is to store these samples along with medical information to speed up future research into what causes these disea…

This study will follow 5,000 children and teens with hearing loss and normal hearing over time. Researchers will measure changes in hearing, speech, language, and brain scans to see how different treatments—like hearing aids, cochlear implants, or gene therapy—affect development.…

This study tests a new communication tool called 'Let's Get REAL' that helps children and teens (ages 8-17) and their families talk together about stem cell transplant or cellular therapy decisions. The goal is to see if the tool is easy to use and helpful for families. About 60 …

This study looks at how people with normal hearing, hearing loss, or cochlear implants use nearby sounds to recognize speech. Researchers will test participants' ability to identify words and sentences in quiet and noisy settings. The goal is to better understand how hearing aids…

This study tests a patient navigation program to help rural adults in Kentucky get hearing tests. 500 participants from primary care clinics will receive guidance every two weeks to overcome barriers like travel and cost. The goal is to see if more people complete diagnostic hear…

This study looks at how people with hearing aids perceive the timbre (quality) of sounds and understand complex sound scenes, like music or crowded rooms. Researchers will compare 75 adults aged 60-75 with and without hearing loss. Participants will listen to sounds and rate how …

This study looks at DNA methylation patterns (chemical tags on DNA) in fetuses with rare genetic diseases. Researchers will analyze DNA from amniotic fluid and tissue samples to create reference signatures. The goal is to improve diagnosis of these conditions before birth. The st…

This study aims to improve how children with cochlear implants perceive sound by testing new sound coding strategies. Researchers will compare hearing performance between children and adults, and between those with implants and those with normal hearing. The goal is to understand…

This nationwide study aims to understand how hearing loss affects thinking, language, reading, and emotional wellbeing in 256 deaf or hard-of-hearing adolescents. Researchers will use tests and questionnaires to measure speech perception, cognitive skills, and school burnout. No …

This study is gathering information from 100 children and adults who use MED-EL hearing devices, including implants and non-implantable devices. Researchers will collect data on hearing ability, speech understanding, and quality of life through tests and questionnaires. The goal …

This study uses MRI scans to look for brain differences in people with metabolic diseases compared to healthy volunteers. Researchers will track changes over time and link them to body fat and other health measures. About 126 adults will take part at Ulm University Hospital. No d…

This study is creating a large registry and tissue bank for people with mitochondrial disorders. Researchers will collect medical information and samples from up to 1,000 participants, including those diagnosed with or suspected to have a mitochondrial disease. The goal is to gat…

This research study looks at how children and adults who use cochlear implants or auditory brainstem implants hear and understand sounds. Participants will listen to words, sentences, and tones to see how well they identify speech and tell differences in loudness or pitch. The go…

This study looks at whether inner-ear balance (vestibular) problems make reading harder for children with hearing loss. Researchers will test 90 children, some with normal hearing and some with severe hearing loss, measuring their ability to see clearly during head movements and …

This study looks at how people with cochlear implants understand speech in everyday situations, like conversations with background noise. Researchers will test 460 adults (both with and without hearing loss) using listening tasks that measure effort and comprehension. The goal is…

This study looks at how often young children (ages 1 to 6) with inherited metabolic diseases develop eating disorders when they are on special diets. Researchers will use a feeding scale to measure eating problems in 200 children. The goal is to understand the link between these …

The SMILE study is tracking 10,000 adults aged 18 and older to see how sarcopenia (age-related muscle loss) and metabolic diseases like diabetes influence overall health and lifespan. Researchers will collect data from medical records, tests, and surveys over time. This is an obs…

This study collects blood and skin samples from 100 people with primary mitochondrial diseases and healthy volunteers aged 3 to 85. Researchers will study how different mitochondrial mutations affect cell function and look for biomarkers. The samples will also help test a new the…

This study is testing whether using rapid genetic sequencing can help doctors diagnose and treat birth defects in newborns in intensive care. Researchers will enroll 2,000 babies and compare death rates, disability rates, and genetic findings. The goal is to see if personalized t…

This study follows 100 children under 18 who are already on a ketogenic diet for conditions like drug-resistant epilepsy, autism, chronic migraine, or brain tumors. Researchers want to see if the diet supports healthy growth, improves symptoms, and boosts quality of life. The die…