A Phase I/II clinical trial with SENS-501 in children suffering hearing loss due to OTOF mutations

  • Research type

    Research Study

  • Full title

    A Phase I/II, adaptative, open label dose escalation and expansion clinical trial to evaluate the efficacy and safety of unilateral intracochlear injection of SENS-501 using an injection system in children with severe to profound hearing loss due to Otoferlin gene mutations

  • IRAS ID

    1008280

  • Contact name

    Lionel Hovsepian

  • Contact email

    lionel.hovsepian@sensorion-pharma.com

  • Sponsor organisation

    Sensorion

  • ISRCTN Number

    N/A

  • Research summary

    The purpose of this research study is to test the safety and effectiveness of a new investigational drug. This drug is called SENS-501.
    SENS-501 is a new gene therapy, which intends to treat a rare form of hearing loss, which was caused by the
    deficiency of the protein otoferlin (OTOF). This deficiency is due to a malfunction of the gene that produces otoferlin,
    which enables the transmission of speech and sounds, from the inner ear to the brain.
    SENS-501 may be able to repair the gene defect causing the otoferlin deficiency, and recover physiological hearing. It includes a piece of DNA, which has the correct information to produce the otoferlin protein.
    SENS-501 will be administered unilateraly into the cochlea of children between 6 and 31 months of age with a pre-lingual hearing loss due to a muation in the otoferlin gene. The administration will be done using a high precision investigational pump and an investigational catheter, developed specially for this procedure.
    Participation in this study will last approximately five years. At least 12 children will participate in this study in several countries.
    The Study is comprised of 2 Parts:
    - Part 1 (dose escalation) during which 2 doses of SENS-501 will be tested to select the most adequate one.
    - Part 2 (dose expansion) during which patients will received the dose deemed the most adequate after Part 1.

  • REC name

    South Central - Oxford A Research Ethics Committee

  • REC reference

    23/SC/0262

  • Date of REC Opinion

    21 Dec 2023

  • REC opinion

    Further Information Unfavourable Opinion