Loading clinical trials...

Natural History in Children Up to 16 Years with Mild to Profound Hearing Loss Due to Mutations in GJB2 / OTOF Genes | Clinical Trials | Clareo Health

RECRUITING

Natural History in Children Up to 16 Years with Mild to Profound Hearing Loss Due to Mutations in GJB2 / OTOF Genes

Longitudinal Study of the Natural History of Two Autosomal Recessive Non Syndromic Deafness (DFNB1A and DFNB9) in Children Up to 16 Years of Age

NCT05402813•Sensorion

View on ClinicalTrials.gov

Summary

The purpose of this study is to follow the natural history of non-syndromic hearing loss caused by mutations in two genes (GJB2 or OTOF) in children up to 16 years of age.

Detailed Description

The study aims to: * better describe the prevalence of cases of DFNB1A and DFNB9, including the type of mutations, and to assess the clinical course of the disease in children up to 16 years of age who have a mild to profound deafness. * better understand the audiological and genetic characteristics of the participants with congenital versus evolutive DFNB1A and DFNB9 deafness.

Eligibility

Age

0 - 16 years

Sex

ALL

Healthy Volunteers

Inclusion Criteria

•Aged ≤ 16 years on the date of signed informed consent for cohort 1 and ≤ 10 years for cohort 2;
•With a diagnosis of non-syndromic, bilateral, mild to profound, sensorineural hearing loss (according to the American Speech Language-Hearing Association);
•With documented genotyping results showing mutation(s) in GJB2 or OTOF genes;
•Written informed consent as required by local regulations.
•Either without Cochlear Implant, or with unilateral or bilateral Cochlear Implant(s)

Exclusion Criteria

•Other type of deafness, such as unilateral deafness, persistent conductive deafness, malformation syndrome, syndromic deafness, known familial deafness linked to mutations in other genes than OTOF or GJB2;
•Documented genotyping results showing pathogenic mutation(s) in other gene(s) than GJB2 or OTOF genes in the tested panel;
•Unable and/or unwilling to comply with all the protocol requirements and/or study procedures.

Locations (1)

Necker Hospital

Paris, France

Key Dates

Start Date

November 18, 2022

Primary Completion Date

November 18, 2028

Completion Date

November 18, 2028

Last Updated

February 14, 2025

Enrollment

180

ESTIMATED participants

Conditions

Sensorineural Hearing Loss, BilateralAUNB1DFNB1ACongenital DeafnessDFNB9OTOF Gene MutationGJB2 Gene Mutation

Interventions

Pure Tone Audiometry Assessment

OTHER

Quality of Life Questionnaires

OTHER

Contacts

Lionel HOVSEPIAN, MD

CONTACT

+33 (0)7 86 31 13 76 lionel.hovsepian@sensorion-pharma.com

Géraldine HONNET, MD

CONTACT

geraldine.honnet@sensorion-pharma.com

Auditory Performances With Different Stimulation Depths in Cochlear Implanted Subjects Using a Fine Structure Strategy

NCT04591093

Sensorineural Hearing Loss, Bilateral

View study

COMPLETEDNA

Study of Sound and Speech Perception in New Cochlear Implanted Subjects Using or Not an Anatomy-based Fitting

NCT05230498

Sensorineural Hearing Loss, Bilateral

View study

COMPLETEDNA

Clinical Investigation of New CI Delivery Models in an Adult Nucleus CI Population

Data Source & Attribution

This clinical trial information is sourced from ClinicalTrials.gov, a service of the U.S. National Institutes of Health.

ClinicalTrials.gov last update: February 14, 2025Data synced to Clareo: March 29, 2026

Modifications: This data has been reformatted for display purposes. Eligibility criteria have been parsed into inclusion/exclusion sections. Location data has been geocoded to enable distance-based search. For the authoritative and most current information, please visit ClinicalTrials.gov.

Neither the United States Government nor Clareo Health make any warranties regarding the data. Check ClinicalTrials.gov frequently for updates.