Prevention and/or treatment of hearing loss or impairment

Abstract

The present invention relates to the use of gasdermin, in particular of gasdermin A, gasdermin B, gasdermin C, gas-dermin D, DFNA5 or DFNB59 (or pejvakin), and more particularly pejvakin for modulating cellular redox homeostasis. A particularly preferred use of gasdermin, in particular of gasdermin A, gasdermin B, gasdermin C, gasdennin D, DFNA5 or DFNB59 (or pejvakin), and more particularly pejvakin in the context of the present invention is as an antioxidant. The present invention also concerns a virally-mediated gene therapy for restoring genetically-impaired auditory and vestibular functions in subjects suffering from an Usher syndrome. More precisely, this gene therapy takes advantage of an AA V2/8 vector expressing at least one USH1 gene product, preferably SANS.

1 - 31 . (canceled) 
     
     
         32 . A viral vector for transduction of inner hair cells comprising a nucleic acid sequence encoding an gasdermin. 
     
     
         33 . The viral vector of  claim 32 , wherein the gasdermin is pejvakin. 
     
     
         34 . The viral vector of  claim 33 , wherein the pejvakin has the amino acid sequence of SEQ ID NO:1 or an amino acid sequence having at least 80% identity with SEQ ID NO:1. 
     
     
         35 . The viral vector of  claim 33 , wherein the pejvakin has the amino acid sequence of SEQ ID NO:1 or an amino acid sequence having at least 90% identity with SEQ ID NO:1. 
     
     
         36 . The viral vector of  claim 33 , wherein the pejvakin has the amino acid sequence of SEQ ID NO:1. 
     
     
         37 . The viral vector of  claim 32 , wherein the vector is selected from the group consisting of lentivirus vectors, adenovirus vectors, and adeno-associated virus (AAV) vectors. 
     
     
         38 . The viral vector of  claim 33 , wherein the vector is selected from the group consisting of lentivirus vectors, adenovirus vectors, and adeno-associated virus (AAV) vectors. 
     
     
         39 . The viral vector of  claim 34 , wherein the vector is selected from the group consisting of lentivirus vectors, adenovirus vectors, and adeno-associated virus (AAV) vectors. 
     
     
         40 . The viral vector of  claim 35 , wherein the vector is selected from the group consisting of lentivirus vectors, adenovirus vectors, and adeno-associated virus (AAV) vectors. 
     
     
         41 . The viral vector of  claim 36 , wherein the vector is selected from the group consisting of lentivirus vectors, adenovirus vectors, and adeno-associated virus (AAV) vectors. 
     
     
         42 . The viral vector of  claim 37 , wherein the vector is an AAV vector. 
     
     
         43 . The viral vector of  claim 38 , wherein the vector is an AAV vector 
     
     
         44 . The viral vector of  claim 39 , wherein the vector is an AAV vector. 
     
     
         45 . The viral vector of  claim 40 , wherein the vector is an AAV vector. 
     
     
         46 . The viral vector of  claim 41 , wherein the vector is an AAV vector. 
     
     
         47 . The viral vector of  claim 42 , wherein the vector is an AAV2/8 vector. 
     
     
         48 . The viral vector of  claim 43 , wherein the vector is an AAV2/8 vector. 
     
     
         49 . The viral vector of  claim 44 , wherein the vector is an AAV2/8 vector. 
     
     
         50 . The viral vector of  claim 45 , wherein the vector is an AAV2/8 vector. 
     
     
         51 . The viral vector of  claim 46 , wherein the vector is an AAV2/8 vector.