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RECRUITINGNCT05402813

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

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.

Lead sponsor: Sensorion•1 locations•View source on ClinicalTrials.gov

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Contacts

Lionel HOVSEPIAN, MD

CONTACT

+33 (0)7 86 31 13 76 [email protected]

Géraldine HONNET, MD

CONTACT

[email protected]

Sponsors

Lead Sponsor

Sensorion

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.

Age

0 - 16 years

Sex

ALL

Healthy Volunteers

No

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.

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Data from ClinicalTrials.govLast updated on ClinicalTrials.gov: February 14, 2025Terms

Trial snapshot

StatusRECRUITING

Phase—

Enrollment180

Start dateNov 2022

Last updatedFeb 14, 2025

Condition

Sensorineural Hearing Loss, Bilateral AUNB1 DFNB1A Congenital Deafness DFNB9+2 more

Locations shown

Necker Hospital

Paris

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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: May 25, 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.

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