Usher Syndrome: Who is at Risk?

The first noticeable symptom of Usher syndrome is a sensorineural hearing loss. If the hearing loss is a result of any of the conditions listed below, the person is at low risk for Usher syndrome. There is always a small chance that Usher syndrome is a secondary cause of the hearing loss, but this is extremely rare.

While the individuals who have a hearing loss due to any of the conditions listed below are at low risk for Usher syndrome, they may suffer a vision loss for other reasons. This makes it especially important to have regular, comprehensive eye exams.

Low Risk Causes: 

  • Conductie loss Rubella (German Measles)
  • Kidney disease
  • Otitis Media
  • Trauma/accident
  • Scarring the the eardrum
  • Genetic deafness*
  • Noise-induced loss
  • Auditory neuritis
  • Oxotoxic drug therapy
  • Diabetes
  • Viral Infection
  • Disease (i.e., Meningitis Mumps, Scarlet Fever, Measles, Chicken Pox, Encephalitis)

    *The genes for deafness and for Usher syndrome are different. An individual at risk for Usher syndrome will probably not have another family member who is deaf, unless they too have Usher syndrome.