Prelingual non-syndromic genetic hearing loss
MONDO:0016297A rare, genetically highly heterogeneous otorhinolaryngologic disease, resulting from inner and/or middle ear or hearing nerve anomalies, typically characterized by bilateral, severe to profound hearing loss (mean sensorineural hearing impairment of 60 dB or more for 500-, 1,000-, and 2,000-Hz frequency tones in the better ear) which occurs before the onset of speech development and is not associated with visible external ear abnormalities or any other medical problems. It is usually nonprogressive and impedes oral language acquisition.
Also known as: prelingual non-syndromic genetic hearing loss, isolated prelingual genetic deafness, prelingual non-syndromic genetic deafness
34 clinical trials for this condition and its sub-types.
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Broader categories
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New cell therapy aims to boost hearing restoration in cochlear implant patients
Disease control Not yet recruitingThis early-stage trial tests a new cell therapy called Rincell-1 for adults with certain types of hearing loss who are getting a cochlear implant. The study will compare the safety and early effectiveness of receiving the cell therapy plus the implant versus the implant alone. Ab…
Phase: PHASE1, PHASE2 • Sponsor: Rinri Therapeutics • Aim: Disease control
Last updated Aug 13, 2026 00:00 UTC
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Could an app tune your hearing aids as well as an audiologist?
Disease control Not yet recruitingThis trial tests whether two self-assessment methods in a smartphone app can generate hearing aid settings that match those from a standard hearing test. Adults with perceived mild-to-moderate hearing loss will use the app to self-test their hearing and also undergo conventional …
Phase: NA • Sponsor: Rehear Audiology Company LTD • Aim: Disease control
Last updated Aug 05, 2026 00:00 UTC
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Seeing sound: new eye on ear bones could sharpen hearing loss diagnosis
Diagnosis Not yet recruitingThis pilot study tests a new imaging system that uses OCT and vibrometry to let doctors see the middle ear bones and measure how they move in response to sound. The study enrolls 500 adults with conductive or sensorineural hearing loss. The goal is to compare these detailed motio…
Sponsor: Audioptics Medical Incorporated • Aim: Diagnosis
Last updated Jul 31, 2026 00:00 UTC
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Robot vs. surgeon: which cochlear implant method gives better hearing?
Symptom relief Not yet recruitingThis study compares two ways to insert a cochlear implant: using a robotic device or the standard manual method. Researchers want to see which approach leads to better hearing one year after surgery. The study will enroll 32 adults with hearing loss, randomly assigning them to on…
Phase: NA • Sponsor: AdventHealth • Aim: Symptom relief
Last updated Jun 27, 2026 08:08 UTC
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Could remote Check-Ups replace frequent clinic visits for cochlear implant users?
Symptom relief Not yet recruitingThis study tests a new care pathway for adults getting a cochlear implant. Instead of the usual nine clinic visits in the first year, the new plan uses early activation of the implant and remote monitoring visits. The goal is to see if this approach is at least as good for hearin…
Phase: NA • Sponsor: Hospices Civils de Lyon • Aim: Symptom relief
Last updated Jun 26, 2026 15:51 UTC
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Real-World data sheds light on Long-Term cochlear implant performance
Knowledge-focused Not yet recruitingThis study looks back at the medical records of 200 people who received a SONATA 2 cochlear implant. The goal is to understand how well the implant works over time in real-world settings, including speech recognition in quiet and noisy environments. Researchers will also analyze …
Sponsor: MED-EL Elektromedizinische Geräte GesmbH • Aim: Knowledge-focused
Last updated Aug 12, 2026 00:00 UTC
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Which ear gets the implant? new study aims to reduce dizziness
Knowledge-focused Not yet recruitingThis study looks at 10 adults over 50 with hearing loss and one weak balance ear. It compares implanting the device in the weak-balance ear versus the good-balance ear. The goal is to see if choosing the weaker side helps reduce dizziness after surgery.
Phase: NA • Sponsor: University of Virginia • Aim: Knowledge-focused
Last updated Jun 27, 2026 11:00 UTC