Novel digenic inheritance of PCDH15 and USH1G underlies profound non-syndromic hearing impairment.

BMC Med Genet · 2018 · PMC6053831 · PMID 30029624

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+/ Pcdh15 av-3J +/ Ush1g js double heterozygous mice display hearing loss, with highly significant elevated auditory brainstem response (ABR) thresholds at 3–4 months [ 29 ], suggesting Pcdh15-Ush1g epistasis [ 29 ].

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