Slide 1: Corporate Presentation: Unlocking The Potential Of Advanced Therapies For Hearing Loss
Corporate Presentation Unlocking The Potential Of Advanced Therapies For Hearing Loss May 2025
Slide 2: Disclaimer
DISCLAIMER • This document has been prepared by Sensorion (the “Company”) and is provided for information • This document contains certain forward-looking statements. These statements are not guarantees purposes only. This document does not purport to contain comprehensive or complete information of
Slide 3: Sensorion
1 SENSORION Our vision is to help people with inner ear hearing disorders to live life with unlimited connections 3
Slide 4: Establishing Global Leadership In Hearing Loss With Strong And Diversified Pipeline
Sensorion Establishing Global Leadership In Hearing Loss With Strong And Diversified Pipeline • Unmet clinical need: 1.5bn people affected by hearing loss (HL) • Multiple causes: genetic, environmental, idiopathic • Modality agnostic approach to hearing loss disorders • World-leading and exclusive p
Slide 5: Experienced Leadership Team, Board of Directors and SAB
Sensorion Experienced Leadership Team, Board of Directors and SAB NAWAL OUZREN GERALDINE HONNET LAURENE DANON BERND SCHMIDT STEPHANIE FILIPE LAURENT DESIRE Chief Executive Officer Chief Medical Officer Chief Financial Officer Chief Technical Officer Head of Business Ops & Head of Preclinical Portfol
Slide 6: Best-In-Class Partners And Internal Capabilities To Transform Standard Of Care
Sensorion Best-In-Class Partners And Internal Capabilities To Transform Standard Of Care TRANSLATIONAL CLINICAL DIAGNOSIS AND RESEARCH RESEARCH PATIENT JOURNEY PARTNERS GLOBAL CLINICAL CENTERS OF EXCELLENCE IN-HOUSE PRECLINICAL - CLINICAL EXPERIENCE CMC GENE THERAPY REGULATORY EXPERTISE PATIENT ACCE
Slide 7: Portfolio Of Advanced Hearing Loss Therapies
Sensorion Portfolio Of Advanced Hearing Loss Therapies Product Indication Discovery In-vivo POC Preclinical Phase 1 Phase 2 Phase 3 Milestones (estimated) Phase 1/2 2nd Cohort Completed SENS-501* Otoferlin Deficiency in H1 2025 GENE THERAPIES RESTORE Adult Onset CTA/IND Enabling GJB2-GT* (presbycusi
Slide 8: Gene Therapy Programs
2 GENE THERAPY PROGRAMS 8
Slide 9: Gene Therapy Programs Target Rare Auditory Diseases
Sensorion Gene Therapy Programs Target Rare Auditory Diseases FIRST PROGRAMS RESULTING FROM THE INSTITUT PASTEUR COLLABORATION OTOFERLIN DEFICIENCY GJB2-RELATED HEARING LOSS • Pediatric patients with mutations in OTOF gene suffer from severe We have identified three forms of hearing loss associated
Slide 10: Otoferlin Deficiency
2.1 OTOFERLIN DEFICIENCY 2.1 10
Slide 11: SENS-501 (OTOF-GT) Is The Perfect Pilot Program
SENS-501 SENS-501 (OTOF-GT) Is The Perfect Pilot Program • SENS-501 is the pilot program that has the potential to demonstrate that GT is a relevant medical approach for • Well understood biology and pathology of the the inner ear otoferlin deficiency • SENS-501 will establish understanding of GT in
Slide 12: OTOF Gene Encodes Otoferlin, A Key Ca2+ Sensor Protein
SENS-501 OTOF Gene Encodes Otoferlin, A Key Ca2+ Sensor Protein Increase in Ca2+ SNARE Proteins VAMP Ca2+ Ca2+ Ca2+ Ca2+ Vesicle Ca2+-Channel Otoferlin Pre-synaptic membrane neurotransmitters release Restricted SNARE interaction Otoferlin SNARE interaction SNARE-complex binding Primed vesicle contai
Slide 13: Long-Term Hearing Recovery In A Standardized Translational Model Of Otoferlin Deficiency
SENS-501 Long-Term Hearing Recovery In A Standardized Translational Model Of Otoferlin Deficiency Long-Term Hearing Restoration Hearing Restoration Correlates With De Novo OTOF Expression AAV-mOTOF injected in mice before hearing onset SENS-501 injected in mice after hearing onset * 10 months after
Slide 14: Restoration Of Efficient Sound Processing In Behavioural Test
SENS-501 Restoration Of Efficient Sound Processing In Behavioural Test Behavior Test Based On Hearing Recovery Implemented In Mouse White noise pulse (2-20 kHz, 115 dB) Wild type Non-injected Otof ko/ko Restored Otof ko/ko Olivier et al. ASGCT 2023 Copyright by Sensorion - 2025 - All Rights Reserved
Slide 15: Dedicated Surgical Approach For Gene Therapy
SENS-501 Dedicated Surgical Approach For Gene Therapy Non-Human Primates injected through the round window membrane with or without stapedotomy 1 Fenestration Used for cochlear implant • Overpressure Surgical Approach • Limited volume • Backflow • Surgical procedure is similar to cochlear • Irregula
Slide 16: Raising The Bar With The SENS-501 Audiogene Study
SENS-501 Raising The Bar With The SENS-501 Audiogene Study Generating a compelling value story showing that SENS-501 treatment is able to: • Demonstrate by itself hearing restoration in toddlers • Enable infants to have normal language acquisition and development • Improve Patient Reported Outcomes
Slide 17: Audiogene The First Phase 1/2 Study With A Homogeneous Population
SENS-501 Audiogene The First Phase 1/2 Study With A Homogeneous Population Audiogene, a Phase 1/2 clinical trial in homogenous population of infants and toddlers, aged 6 to 31 months, naive of cochlear implants, to assess safety, tolerability, and efficacy of SENS-501 following unilateral injection
Slide 18: Audiogene Study Status
SENS-501 Audiogene Study Status • Patient recruitment going as planned with first cohort of infants and toddlers (three patients) injected in H2 2024 • In the first three patients so far, good initial safety: FIRST • No dose-limiting toxicities, no Serious Adverse Events COHORT • Vestibular function
Slide 19: SENS-501 Program Status
SENS-501 Program Status Pediatric CTA Approval 1st Cohort 2nd Cohort Investigational IND in Various Completed Completed Plan agreed process Countries H2 2024 H1 2025 in EU Copyright by Sensorion - 2025 - All Rights Reserved 19
Slide 20: GJB2-Related Hearing Loss
2.2 GJB2-RELATED HEARING LOSS 20
Slide 21: Leveraging SENS-501 Program For GJB2-GT Program Success
GJB2-GT Leveraging SENS-501 Program For GJB2-GT Program Success SENS-501 Is Paving The Way For GJB2-GT SENS-501 GJB2-GT Aiming To Develop Best-In Class And First-In Class Gene Therapy • AAV capsid selected for high-level of target cells specificity • GT product showing high level of target cells tra
Slide 22: Connexin Is Encoded By GJB2 Gene And Is Responsible For Tissue Homeostasis
GJB2-GT Connexin 26 Is Encoded By GJB2 Gene And Is Responsible For Tissue Homeostasis Mutations In The GJB2 gene Lead To Deafness GJB2 Expression In The Cochlea • Connexin 26 and Connexin 30 proteins are the dominating connexins in the cochlea; heteromeric or heterotypic hexamers forming Gap Junctio
Slide 23: Lead Candidate Was Selected To Answer Specific Development Criteria
GJB2-GT Lead Candidate Was Selected To Answer Specific Development Criteria CRITERIA LEAD CANDIDATE Natural and synthetic AAV capsid libraries screening for broad coverage of target cells Expression cassette design for high-level of target cells transduction, correct cellular localization, active ga
Slide 24: Lead Candidate Can Deliver Connexin 26 In The Appropriate Target Cells
GJB2-GT Lead Candidate Can Deliver Connexin 26 In The Appropriate Target Cells Correct Delivery Of Connexin 26 Using Lead Candidate Flag In Non-Human Primate Cochlea Cell Types Claudius Cells Deiters Cells Great Epithelial Ridge Cells Hensen Cells Inner Border Cells Inner Hair Cells Inner Phalangeal
Slide 25: Lead Candidate Prevents Hearing Loss In Relevant Mouse Model
T+7w - 1X GJB2-GT 100 ABR Threshold (dB SPL) 80 Lead Candidate Prevents Hearing Loss In Relevant Mouse Model 60 Proof Of Concept In Mice With Progressive Hearing Loss Proof Of Concept In Mice With 40 Congenital Hearing Loss Mouse model provides congenital and Progressive Model Congenital model 20 pr
Slide 26: GJB2-GT Program Status
GJB2-GT Program Status Update on Ongoing Ongoing Additional European Natural History IND/CTA Clinical Trial PoC Efficacy Natural History Study Enabling Applications and Safety Data Study with Sonova Studies Q1 2026 Oct 2024 OTOCONEX SONG (ESGCT) Copyright by Sensorion - 2025 - All Rights Reserved 26
Slide 27: SENS-401 Programs
3 SENS-401 PROGRAMS Multiple Indications To Treat And Prevent Hearing Loss 27
Slide 28: SENS-401 Mechanism Of Action
SENS-401 Mechanism Of Action Disrupted Ca2+ Calcineurin Neurodegenerative cascades INSULT homeostasis Activation Excitotoxicity • NFAT translocation: oxidative stress, survival, inflammation pathways Neuro Inflammation • Cell death pathways: BAD, mPTP, AIF, caspases activation • Structural degenerat
Slide 29: SENS-401 CI
3.1 SENS-401 CI Preservation Of Residual Hearing Following Cochlear Implantation 29 29
Slide 30: SENS-401 To Preserve Residual Hearing After Cochlear Implantation
CI SENS-401 To Preserve Residual Hearing After Cochlear Implantation COMBINATION OF COCHLEAR IMPLANT WITH SENS-401 TO PREVENT CELL-DEATH POST COCHLEAR IMPLANT PROCEDURE BURDEN OF DISEASE KEY FIGURES Growing understanding of the link between healthy hearing and healthy ageing 80, 000 Implants sold gl
Slide 31: Residual Low Frequency Hearing Benefits For Cochlear Implant Users
CI Residual Low Frequency Hearing Benefits For Cochlear Implant Users Initial Initial shift* shift* Second shift* (2-4 weeks (2-4 weekspostoperative) postoperative) (3-6 months postoperative) between surgery between andand surgery initial activation initial activationofofthe thedevice device Attribu
Slide 32: Primary Endpoint of The Phase 2a Study For Residual Hearing Preservation Has Been Met
CI Primary Endpoint of The Phase 2a Clinical Study For Residual Hearing Preservation Has Been Met Perilymph Concentrations Data Treated with SENS-401 (n=16) n (%) SENS-401 levels ≤ LLOQ 0 SENS-401 levels > LLOQ 14*(100) *Among the 16 participants who underwent surgery, 15 have a perilymph samples an
Slide 33: SENS-401 CI Study Design - Study Completed
CI SENS-401 CI Study Design - Study Completed A Phase 2a, Multicenter, Randomized, Controlled, Open-label Study to Evaluate the Presence of SENS-401 in the Perilymph and to Assess Its Efficacy to Prevent Residual Hearing Loss After Cochlear Implantation SCREENING PERIOD EVALUATION PERIOD W-6 W-1 W0
Slide 34: SENS-401 Provides Hearing Protection 6 & 14 Weeks Post-Cochlear Implantation
CI SENS-401 Provides Hearing Protection 6 & 14 Weeks Post-Cochlear Implantation LS Mean change from baseline of hearing threshold Mean of the 3-frequency average (250-750 Hz) hearing values at 6 and 14 weeks post CI threshold values at baseline, 6 weeks and 14 weeks post CI 0 40 10 Treated group (n=
Slide 35: SENS-401 Provides Residual Hearing Preservation 6 & 14 Weeks Post-Cochlear Implantation
CI SENS-401 Provides Residual Hearing Preservation* 6 & 14 Weeks Post-Cochlear Implantation W6 Post-CI W14 Post-CI Complete: HP>75% Partial: HP of 25 to 75% 3-frequency average (250-750 Hz) 27% 40% 14% 53% 40% 43% 29% • Patients in the SENS-401 treated group are twice as likely to show complete or p
Slide 36: SENS-401 CI Final Results - Conclusion
CI SENS-401 CI Final Results - Conclusion SENS-401 can cross the labyrinthine barrier to target cochlear hair cells in all patients sampled, confirming primary endpoint is met. SENS-401, present in the perilymph fluid, reaches concentrations that are pharmacologically active. A complete hearing pres
Slide 37: SENS-401 CIO
3.2 SENS-401 CIO Prevention Of Cisplatin-Induced Ototoxicity 37
Slide 38: Cisplatin Administration Damages The Inner Ear And Leads To Hearing Loss
CIO Cisplatin Administration For Chemotherapies Damages The Inner Ear And Leads To Hearing Loss, Tinnitus And Dizziness WHAT IS CIO? Hearing loss caused by cisplatin administration as chemotherapeutic treatment. Risk factors include young age as well as individual and cumulative cisplatin doses. CIO
Slide 39: SENS-401 Phase 2a Proof-Of-Concept Study NOTOXIS
CIO SENS-401 Phase 2a Proof-Of-Concept Study NOTOXIS Recruitment Completed – Positive Preliminary Safety Data A Phase 2a, Multicenter, Randomized, Controlled, Open-label Study to Evaluate the Efficacy of SENS-401 to Prevent the Ototoxicity Induced by Cisplatin in Adult Subjects with a Neoplastic Dis
Slide 40: Preliminary Results Show Patients With High Exposure To Cisplatin May Benefit Most
CIO Preliminary Results Show Patients With High Exposure To Cisplatin May Benefit The Most From SENS-401’s Otoprotective Effects : SENS-401 (n=7) Groups n Variables Mean SD Maximum mean PTA change for 3 most : CONTROL (n=9) severe consecutives frequencies* (dB) Cisplatin dose 305 110.0 Contro
Slide 41: Key Takeaways From Preliminary Study Data
CIO Key Takeaways From Preliminary Study Data Cumulative dose of cisplatin is a key factor of ototoxicity severity. SENS-401 has a favorable safety profile when administered continuously for up to 23 weeks in adult patients undergoing cisplatin-based chemotherapy. Based on preliminary data, no signi
Slide 42: SENS-401 Programs Status
CI & CIO SENS-401 Programs Status SENS-401 with SENS-401 CIO SENS-401 SENS-401 SENS-401 with cochlear implants NOTOXIS NOTOXIS NOTOXIS cochlear implants - - - - - Full Data Readout Preliminary Safety End of Enrollment Topline Data Final Results Sept 20, 2024 and Efficacy Data H1 2025 End of H2 2025
Slide 43: Sensorion Newsflow [Estimated Timelines]
Sensorion Newsflow [Estimated Timelines] H1 2025 SENS-501 Gene Therapy Program Audiogene 2nd cohort enrollment completion Q1 2026 GJB2-GT Program Clinical Trial Applications H2 2025 SENS-401 Program CIO NOTOXIS: Topline Data Copyright by Sensorion - 2025 - All Rights Reserved 143
Slide 44: Conclusion
Sensorion Conclusion • Developing hearing loss therapeutics to treat, prevent and restore hearing – an area of high unmet clinical need • Combining extensive internal capabilities with world-leading exclusive partnerships • Advancing a robust and diversified pipeline with multiple upcoming milestone
Slide 45: Thank You
THANK YOU E:contact@sensorion-pharma.com
Slide 46: Hearing Loss
HEARING LOSS 46
Slide 47: Access And Clarity Are Mandatory For Optimal Outcomes
Access And Clarity Are Mandatory For Optimal Outcomes (Hz) (dB HL) O = Right ear threshold Normal X = Left ear threshold Slight th f p s Mild t Hearing aid fitting range* z v h g k m d b ch j n o sh Moderate i a ng r Adults* e u Moderately-severe Children O Severe > 2 years* X O X O O O X O X X X Co
Slide 48: Internal Capabilities
Internal Capabilities 48
Slide 49: We Have Established Internal Capabilities To Ensure Successful Execution
We Have Established Internal Capabilities To Ensure Successful Execution PRECLINICAL - CMC GENE THERAPY PATIENT ACCESS SMALL MOLECULES & CLINICAL EXPERIENCE REGULATORY EXPERTISE FACILITIES GT PROGRAMS • Audiology, inner ear surgery and • 600 subjects enrolled in • Process development: non- • Develop
Slide 50: SENS-401 Programs Back-Up
SENS-401 PROGRAMS Back-Up Multiple Indications To Treat And Prevent Hearing Loss 50
Slide 51: SSNHL: Sudden Sensorineural Hearing Loss
SSNHL Sudden Sensorineural Hearing Loss 51
Slide 52: SSNHL is a Severe Disease Affecting more than 200,000 Patients Per Year
Sudden Sensorineural Hearing Loss (SSNHL) is a Severe Disease Affecting more than 200,000 Patients Per Year WHAT IS SSNHL? The sudden onset of a significant hearing loss due to dysfunction of the cells of the cochlea and central auditory structures. Hearing loss develops over less than 72 hrs, heari
Slide 53: SSNHL AUDIBLE-S Phase 2 Design
SSNHL AUDIBLE-S Phase 2 Design A Phase 2b, Multicenter, Randomized, Controlled, Double-blind Study to Evaluate the Efficacy of SENS-401 to Treat Patients with Severe to Profound Sudden Sensorineural Hearing Loss 50 clinical sites globally 1 Primary endpoint +/- oral corticosteroids Audiometry 15 dB
Slide 54: SENS-401 Induces Complete PTA Recovery In 50% Of Patients
SSNHL SENS-401 Induces Complete PTA Recovery In 50% Of Patients COMPLETE PTA RECOVERY 60,0% * p=0.046 50,0% 40,0% Complete PTA SENS-401 30,0% Placebo recovery (n/n total) (2 doses pooled*) 20,0% Day 28 9/28 25/49 10,0% Day 84 8/26 22/40 0,0% DAY 28 DAY 84 *As SENS-401 plasmatic concentration is simi
Slide 55: SSNHL Phase 2 Results Summary
SSNHL Phase 2 Results Summary Complete PTA recovery is achieved in 50% of the SENS-401 treated patients. SENS-401 shows a clinically meaningful and statistically significant effect on PTA change (at least 10 dB) over time in a large homogeneous idiopathic population of patients treated with corticos