Usher syndrome causes sensori-neural or nerve deafness, which affects the transmission of sound to the brain. According to the type of Usher syndrome, the individual may experience moderate to profound deafness.

Routine auditory assessments are important to monitor any changes in hearing, the impact of further vision loss on hearing, and to assess the need for or modify existing hearing technologies used.

Some children with a profound hearing loss may benefit from a cochlear implant or implants, which successfully help many children to develop speech. Cochlear implants for both ears are becoming the norm, especially for visually-impaired children.

Sign language is a first choice of language for many people with Usher and is a useful additional skill for those who choose to use speech. 

Children who have partial hearing will benefit from the use of hearing aids. With early diagnosis and the introduction of hearing aids at an early age they will usually develop speech and be able to attend mainstream schools.

People with Usher type three who develop profound deafness over time, often benefit from cochlear implants. Since they also have a recognised visual impairment, they may be eligible for two cochlear implants, one in each ear, which should improve hearing to some degree.