Study Tracking Hearing Loss Caused by Otoferlin Gene Changes Observing Individuals Up to 44 Years Old

What this Trial Is About

Researchers are studying individuals with Otoferlin Gene-Mediated Hearing Loss, specifically those with bilateral sensorineural hearing loss SNHL, including auditory neuropathy or auditory neuropathy spectrum disorder. This observational study combines both retrospective and prospective approaches to better understand the natural history of this condition. The study is sponsored by Akouos, Inc., and aims to gather detailed audiologic data over several years. Participants are observed through a natural history study involving no active treatment but careful monitoring of hearing function. The study includes retrospective data collection from medical records and a prospective phase lasting up to five years. During the prospective phase, participants must have certain hearing test results such as Otoacoustic Emissions OAE or cochlear microphonics CM present and abnormal auditory brainstem responses ABRs in at least one ear that does not have a cochlear implant. Participants will undergo audiologic assessments including ABR and OAE recordings from their first audiologic data available in medical records through the five-year follow-up. Researchers track hearing changes and other relevant clinical features over time. The study involves regular evaluations and follow-up visits to collect and monitor hearing data, with the total participation duration extending up to five years.

Otoferlin Gene-mediated Hearing Loss Natural History Study