Usher Syndrome: An Inherited Condition With Severe Complications in Hearing and Eyesight

Usher syndrome is a hereditary condition with its primary characteristic being partial or total hearing loss (neurosensory hearing loss).

The condition does not only cause hearing problems to the individual, but it also creates another serious complication which is the loss of eyesight. The loss of vision is gradual (pigmentary retinopathy).

What are the causes of Usher syndrome?

Hearing and vision loss are caused by gene mutations (autosomal recessive pattern) that affect the retina of the eye as well as the cochlea inside the ear. Mutations affect a total of 6 genes encoding proteins that are associated with normal healing, vision and balance.

Based on the symptoms and the age of onset of the disease distinguish three types for this syndrome:

  • USHER I: Where people are deaf by birth and they often have severe balance problems. Eyesight is usually affected after the age of 8 years.
  • USHER II: Patients with partial or complete hearing loss but less problematic balance. During adolescence, the onset of problems with vision develops more slowly compared to type I.
  • USHER III: In this type, patient’s symptoms are less severe than in the other two types and the age of onset is between the second and fourth decade of the individual’s life.

Diagnosis of Usher Syndrome

Τhe development of this disease can lead to blindness, an early a diagnosis is fundamental as it will greatly help the patient's mental state. The main symptoms that can lead to a valid diagnosis are: severe balance problems, increased sensitivity to changes in lightingnarrow field of vision and nocturnal blindness. Specialists who examine a child with hearing loss (the teacher, the otolaryngologist, the family doctor) should be able to realize based on a child’s deafness symptoms, this child is likely to have Usher syndrome.


Τhe cure for Usher syndrome has not been found yet. Science is able to offer a number of aids, which can help the person in discomfort up to a certain point.

  • Ear implants
  • Orientation and mobility training.
  • Training in communication skills.
  • Access to services for low vision.
  • Counseling assistance.


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