Decibel Therapeutics, a clinical-stage biotechnology company dedicated to discovering and developing transformative treatments to restore and improve hearing and balance, announced a partnership with Invitae, a medical genetics company, to launch Amplify, a no-charge genetic testing program to screen for the genetic cause of congenital hearing loss in children diagnosed with auditory neuropathy.

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“We are pleased to collaborate with Invitae to introduce Amplify, which is designed to bring patients one step closer to molecular diagnosis and clinical management of auditory neuropathy, a disorder that affects approximately 10% of children who are born with hearing loss,” said Jonathon Whitton, AuD, PhD, Vice President of Clinical Research at Decibel. “This program seeks to provide much-needed answers to patients and families of patients who experience congenital, monogenic hearing loss. We hope that Amplify will provide those patients with a better understanding of their diagnosis and their treatment options.”

Auditory neuropathy is a hearing disorder in which the cochlea, the hearing organ located in the inner ear, receives sound normally, yet the transmission of sound to the brain is interrupted. The most common genetic cause of auditory neuropathy is insufficient production of a protein called otoferlin, which facilitates communication between the inner ear sensory cells and the auditory nerve. When this protein is lacking, the ear cannot communicate with the auditory nerve and the brain, resulting in profound hearing loss. Decibel’s lead investigational gene therapy program, DB-OTO, is designed to treat congenital, monogenic hearing loss caused by a deficiency in the otoferlin gene.

Amplify Program Eligibility
Amplify is available to individuals who meet the following criteria:

  • Absent or highly abnormal Auditory Brainstem Response (ABR) in both ears;
  • Presence of Distortion Product Otoacoustic Emissions (DPOAE) in at least three frequencies in at least one ear;
  • Less than five years of age.

Amplify is a no-charge program that offers genetic testing for those who qualify. Although genetic testing can confirm a potential diagnosis, the absence of a genetic alteration does not preclude a diagnosis of genetic hearing loss. For more information about the program, please visit the Amplify program page.

Source: Decibel Therapeutics, Invitae