Phase
Condition
Hearing Impairment
Hearing Loss
Deafness
Treatment
N/AClinical Study ID
Ages < 7 All Genders
Study Summary
Eligibility Criteria
Inclusion
Inclusion Criteria:
Participant is no older than 7 years (inclusive) at the time of the parent/legalguardian signing the informed consent form (ICF)
Participant has 1 of the following genotypes and meets the associated audiologiccriteria based on physiologic and/or behavioral measures of inner ear function:
Biallelic pathogenic or likely pathogenic OTOF mutations with severe to profoundsensorineural hearing loss (SNHL; ≥ 85 dB HL) and confirmed presence of OAEs
Biallelic pathogenic and truncating GJB2 mutation(s) with moderate ormoderately-severe SNHL (≤41 to <71 dB HL)
Digenic pathogenic or likely pathogenic mutations in GJB2 and GJB6 genes with mildor worse SNHL (>26 dB HL)
Participant has at least 1 ear that has not received a cochlear implant (CI)
Exclusion
Exclusion Criteria:
History of or active participation in an interventional trial related to hearingloss
History or presence of any other permanent/untreatable hearing loss conditions,including genetic conditions other than those involving biallelic OTOF or GJB2mutations, or digenic GJB2/GJB6 mutations
History of treatment with ototoxic drugs
Study Design
Study Description
Connect with a study center
Phoenix Children's Hospital
Phoenix, Arizona 85016
United StatesSite Not Available
Phoenix Childrens Hospital
Phoenix, Arizona 85016
United StatesSite Not Available
Nationwide Children's Hospital
Columbus, Ohio 43205
United StatesSite Not Available
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