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**OTOV101** is an investigational dual-adeno-associated virus gene-replacement therapy developed for autosomal recessive deafness 9 caused by pathogenic **OTOF** variants. The product comprises the paired AAV vectors OTOV101N and OTOV101C, which deliver complementary portions of the human *OTOF* coding sequence to cochlear inner hair cells. Intracellular reconstruction and expression of full-length otoferlin is intended to restore synaptic vesicle exocytosis at inner-hair-cell auditory synapses and thereby improve hearing. OTOV101 has been evaluated in an investigator-initiated clinical study using intracochlear administration for DFNB9 deafness.
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