Usher syndrome is a rare inherited disorder primarily characterized by deafness due to an impaired ability of the auditory nerves to transmit sensory input to the brain (sensorineual hearing loss) accompanied by retinitis pigmentosa, a disorder that causes progressive loss of vision.
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- A-Z Deafblindness An in-depth article by Mary Guest, Head of Usher Services at Sense. Includes a detailed description of Usher syndrome, the symptoms, genetics, transmission, associated problems and what can be done.
- National Institutes of Health: Usher Syndrome Provides information including a description of the condition, resources, how to join a NIH clinical study, and funding opportunities for researchers studying the condition.
- NIDCD: Usher Syndrome Factsheet on this condition that affects both hearing and vision, its causes, characteristics and treatment.
- Boys Town National Research Hospital: Usher Syndrome Provides information on types 1, 2 and 3 of this condition, retinitis pigmentosa, hearing loss, balance, gene studies, and other conditions which may have similar symptoms.
- Deaf woman’s shock as she hears for first time The BBC reports on a video of the moment a 39-year-old woman heard for the first time, thanks to cochlear implants.
- Losing your sight as a deaf person The BBC reports on deaf people who then become blind.
- Patient: Usher Syndrome Provides information on this genetic defect causing retinitis pigmentosa and congenital deafness and often vestibular dysfunction. Includes classification, presentation, differential diagnosis and management.