Actively Recruiting

Phase 1
Phase 2
Age: 6Months - 31Months
All Genders
ID06370351

A Phase I/II Clinical Trial to Evaluate Safety and Efficacy of Unilateral Intracochlear Injection of SENS-501 in Children With Severe to Profound Hearing Loss Due to Otoferlin Gene Mutations

Led by Sensorion · Updated on 2024-09-26

12

Participants Needed

2

Research Sites

208 weeks

Total Duration

On this page

AI-Summary

What this Trial Is About

Researchers are evaluating the safety, tolerability, and effectiveness of the gene therapy SENS-501 in children aged 6 to 31 months who have severe to profound hearing loss caused by mutations in the Otoferlin gene. This open-label Phase I/II study focuses on children with pre-lingual hearing loss due to these genetic mutations, aiming to better understand how this treatment works in this young population. The study involves administering SENS-501 directly into one ear using a special injection system. It includes a dose-escalation phase where children receive either a low or high dose, followed by a dose expansion phase where the dose recommended from earlier phases is given. The treatment is given as a single unilateral intracochlear injection, and the administration system's safety, performance, and usability are also evaluated. Participants will be followed for up to 5 years to monitor safety and tolerability and to assess hearing improvement using auditory brainstem response (ABR) and pure tone audiometry (PTA). Additional evaluations include clinical checks of the administration system shortly after treatment. Throughout the study, children will undergo hearing tests and medical assessments to measure outcomes and track any side effects or complications from the treatment and procedure.

CONDITIONS

Brief Title

A Phase I/II Clinical Trial with SENS-501 in Children Suffering from Severe to Profound Hearing Loss Due to Otoferlin (OTOF) Mutations

Who Can Participate

Age: 6Months - 31Months
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Children (male or female) aged 6 to 31 months at the time of inclusion
  • Severe to profound hearing loss confirmed by auditory brainstem response (ABR)
  • Biallelic mutation in the Otoferlin gene
  • Presence of Otoacoustic emissions (OAEs)
  • Documented normal cochlea and internal auditory canals
  • Intact vestibular function
Not Eligible

You will not qualify if you...

  • History of chronic, acute, or major disease or other reasons making participation unsafe or unsuitable
  • Previous dosing in a gene therapy clinical trial
  • Prior or current cochlear implant
  • Any contraindication to surgery or anesthesia or history of prolonged ototoxic therapy (e.g., cisplatin, aminoglycosides over 2 weeks)
  • Participation in another interventional clinical trial
  • Any condition that may compromise safety, compliance, or interfere with treatment evaluation
  • Anticipated noncompliance with study requirements

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Your Study Journey

Screening

Duration - 2 to 4 weeks

Participants are screened for eligibility to participate in the trial.

Treatment

Duration - Up to 5 years

Participants receive a single unilateral intracochlear injection of SENS-501 with a dedicated administration system to assess safety, tolerability, and efficacy.

1 administration visit and multiple follow-up visits over 5 years

Trial Site Locations

Total: 2 locations

1

Childrens Hospital Westmead

Westmead, Australia, NSW 2145

Actively Recruiting

2

Hopital Necker Enfants Malades

Paris, France, 75015

Actively Recruiting

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Research Team

L

Lionel HOVSEPIAN, MD

G

Géraldine HONNET, MD

How is the study designed?

Study Type

INTERVENTIONAL

Masking

NONE

Allocation

NON_RANDOMIZED

Model

SEQUENTIAL

Primary Purpose

TREATMENT

Number of Arms

3

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Frequently Asked Questions

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