Akouos IPO Presentation Deck

AK-OTOF Overview 27 AK-OTOF Prevalence Indication Delivery Method Mechanism of Action Progress and Status AK-OTOF encodes otoferlin, a protein that enables the sensory cells of the ear to release. neurotransmitter vesicles, activating auditory neurons Estimated 20,000 cases of OTOF-mediated hearing loss in the US and EU • Treatment of sensorineural hearing loss due to mutations in the otoferlin, or OTOF, gene • Mutations in the OTOF gene are a major cause of genetic nonsyndromic hearing loss Use AAVAnc80 as a delivery vehicle for the OTOF gene Administer product candidate directly into the inner ear AKOUOS Encodes otoferlin to enable release of neurotransmitter vesicles to activate auditory neurons in response to sound • Auditory neurons then carry electronically encoded acoustic information to the brain allowing: us to hear Promising preclinical data in representative mouse models Pre-IND meeting with FDA. • Executing on agreed IND-enabling preclinical studies • Designing a Phase 1/2 trial for AK-OTOF and plan to file an IND in 2021 CONFIDENTIAL

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