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

Trial statusRecruiting
Trial phaseNot listed
Trial typeObservational
Biological sexAll
AgeUp to 16
SponsorSensorion

About this trial

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.

Eligibility criteria

Qualifiers

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.

Disqualifiers

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.

Trial design

Treatments tested in this trial

  • Pure Tone Audiometry Assessment
  • Quality of Life Questionnaires

Treatment groups

180 Participants
are divided into 3 treatment groups

Sponsors and collaborators