S7 with bilateral EVA deteriorated into a bilateral SNHL at 8 months of age (MRI at 7 months of age [20]), and the subject now have bilateral cochlear implants at 2.5 years of age, and has been diagnosed with biallelic pathogenic variants of SLC26A4, and one found in each parent, indicating Pendred syndrome. The gene discussed is SLC26A4; the disease is sensorineural hearing loss disorder.