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FDA Approves First Gene Therapy for Inherited Deafness

Regeneron will supply the one-time treatment free in the U.S., easing a key barrier to access.

Overview

  • The FDA granted accelerated approval Thursday to Otarmeni (lunsotogene parvec) for OTOF-related severe-to-profound hearing loss in patients with preserved outer hair cells and no cochlear implant in the treated ear.
  • Regeneron said it will provide the drug at no cost to eligible U.S. patients, though families may still face charges for the surgical procedure used to deliver it.
  • The one-time dose uses an AAV vector to deliver a working OTOF gene directly into the cochlea, restoring otoferlin in inner-ear hair cells so sound signals can reach the brain.
  • A multicenter Nature study of 42 patients reported about 90% regained hearing within weeks, with gains building by 26 weeks and holding for as long as 2.5 years.
  • Young children saw the largest gains, adults improved less, about 10% did not respond, and regulators will track durability and speech outcomes in long-term follow-up.