Otoferlin Gene-mediated Hearing Loss Natural History Study

Inclusion Criteria:

1. Clinical presentation of bilateral sensorineural hearing loss (SNHL), includingauditory neuropathy (AN) / auditory neuropathy spectrum disorder (ANSD) phenotype ormedical history of AN / ANSD phenotype earlier in life

2. Mutation(s) in the otoferlin gene

3. Able and willing to comply with all study requirements, as evidenced by successfulcompletion of the informed consent (and assent, if applicable) process Additional Criteria for Inclusion in the Prospective Phase:

4. Presence of OAE / CM and absent / abnormal ABRs in at least one ear (that does nothave a cochlear implant) within 12 months prior to or at the Month 0 visit

Exclusion Criteria:

1. Unwillingness or inability of the potential participant and/or legally authorizedrepresentative to comply with all protocol requirements

2. Presence of cochlear nerve deficiency and/or cochlear nerve dysplasia Additional Criteria for Exclusion from the Prospective Phase:

3. Presence of bilateral cochlear implants at the time of record review or plannedwithin the next 6 months

4. Presence of middle ear or auditory brainstem implant(s) at the time of record reviewor planned within the next 6 months

5. Any condition that would not allow the potential participant to complete follow-upassessments during the course of the study and/or, in the opinion of theInvestigator, makes the potential participant unsuitable for the study

Note: Potential participants will not be excluded based on their sex, gender, race, or ethnicity