Akouos IPO Presentation Deck

Dual Vector Delivery of AK-OTOF Led to Restoration of ABR Thresholds and Long-Term Hearing Recovery in Mice 29 Restoration of ABR Thresholds in Otof Knock-Out Mice Receiving a Dual AAV Vector Expressing OTOF Click ABR threshold (db SPL) 90 80 70 60 50 40 30 20 10 0 25 4 10 15 Weeks Post-Injection 30 Otof/-(n=6) Otof (5' alone; n=3) Otof (5'+3'; n=8) Wild-type (n=8) Click ABR threshold (db SPL) CONFIDENTIAL Intracochlear Delivery of AK-OTOF Resulted in Significant, Long-term Hearing Recovery in Otof Knock-Out Mice 100 80 60 40 20 0 1 mo AKOUOS Untreated Otof(n=5) Wild-Type (n=6 at 1 mo; n-3 at 10 mo) Otof/ + AAVAnc80-hOTOF (n=3 per group) 3 mo 7 mo 10 mo Approximate Age at ABR Test (Administration at 1 week old) AK-OTOF utilizes a dual vector approach to restore long-term hearing in knock-out mouse models - one AAVAnc80 vector carries the 5' fragment of OTOF gene and the other AAVAnc80 vector carries the 3' fragment of the OTOF gene

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