The mutation that we found in EYA4 may impair the formation of the complex that is regulated by EyaVR, which impedes complex transportation to the nucleus, where is acts as a transcription factor, and the haploinsufficiency of EyaVR may lead to inadequate cochlear transcriptional regulation and function maintenance and result in SNHL. The gene discussed is EYA4; the disease is sensorineural hearing loss disorder.