RecruitingNCT05572073

Otoferlin Gene-mediated Hearing Loss Natural History Study

A Natural History Study in Individuals With Otoferlin Gene-mediated Hearing Loss

Sponsor

Akouos, Inc.

Enrollment

150 participants

Start Date

Jul 14, 2022

Study Type

OBSERVATIONAL

Conditions

Sensorineural Hearing Loss, Bilateral

Summary

This is a retrospective and prospective longitudinal study in participants with Otoferlin Gene-Mediated Hearing Loss.

Eligibility

Max Age: 44 Years

Inclusion Criteria5

Clinical presentation of bilateral sensorineural hearing loss (SNHL), including auditory neuropathy (AN) / auditory neuropathy spectrum disorder (ANSD) phenotype or medical history of AN / ANSD phenotype earlier in life
Mutation(s) in the otoferlin gene
Able and willing to comply with all study requirements, as evidenced by successful completion of the informed consent (and assent, if applicable) process
Additional Criteria for Inclusion in the Prospective Phase:
Presence of OAE / CM and absent / abnormal ABRs in at least one ear (that does not have a cochlear implant) within 12 months prior to or at the Month 0 visit

Exclusion Criteria7

Unwillingness or inability of the potential participant and/or legally authorized representative to comply with all protocol requirements
Presence of cochlear nerve deficiency and/or cochlear nerve dysplasia
Additional Criteria for Exclusion from the Prospective Phase:
Presence of bilateral cochlear implants at the time of record review or planned within the next 6 months
Presence of middle ear or auditory brainstem implant(s) at the time of record review or planned within the next 6 months
Any condition that would not allow the potential participant to complete follow-up assessments during the course of the study and/or, in the opinion of the Investigator, makes the potential participant unsuitable for the study
Note: Potential participants will not be excluded based on their sex, gender, race, or ethnicity

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Interventions

OTHERNatural History Study

Natural History Study

Locations(10)

University of Iowa

Iowa City, Iowa, United States

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Vanderbilt University Medical Center

Nashville, Tennessee, United States

Murdoch Children's Research Institute

Parkville, Victoria, Australia

University Hospital in Tübingen

Tübingen, Germany

Sant Joan de Déu Barcelona Hospital

Esplugues de Llobregat, Barcelona, Spain

National Taiwan University Hospital

Taipei, Taiwan

Ankara University

Ankara, Turkey, Turkey (Türkiye)

University College London

London, United Kingdom, United Kingdom

View Full Details on ClinicalTrials.gov

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NCT05572073

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