69743A>G which is predicted to result in a p.I663V amino acid substitution was observed in this study in a patient with bilateral, profound sensorineural hearing loss who is also homozygous for the pathogenic c.35delG mutation in GJB2. The detection of a homozygous c.35delG mutation in GJB2 in this patient provides a clear etiology for this patient's hearing loss without the need to invoke additional contributing factors. Here, GJB2 is linked to sensorineural hearing loss disorder.