Early hearing loss is sensorineural, mild and asymmetric.[1] By adolescence, high-frequency hearing loss begins to appear. Hearing loss is severe, symmetric, and broad-spectrum by the age of 35. However, studies show that while the hearing loss is deteriorating, the ability to speak well is highly preserved.[2] The slowly progressing hearing loss is more problematic to adjust to than the congenital blindness for most people with Norrie disease.[1]