The present disclosure relates to a method of treating sensorineural deafness. The disclosure provides a method of treating sensorineural deafness in a mammalian subject (e.g., human) in need thereof. The method comprises administering to a subject having a mutation in a CLDN9 gene a composition that comprises a polynucleotide that encodes a CLDN9 peptide, a CLDN9 peptide, an agent that blocks expression of a mutant CLDN9 gene, an agent that corrects the mutation in the CLDN9 gene.
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