Usher syndrome

Overview

Usher syndrome is an inherited condition that causes 1) a serious hearing loss that is usually present at birth or shortly thereafter

Symptoms

Hearing impairment Retinitis pigmentosa - this leads to symptoms such as: o Night-blindness o Peripheral vision loss o Progressive retinal degeneration

Causes

Retinitis pigmentosa can be classified according to its inheritance pattern: autosomal dominant, autosomal recessive, and X-linked. Typically, in all forms of retinitis pigmentosa, the retinal rods slowly deteriorate. Clumps of pigment resembling bone corpuscles aggregate in the peripheral region of the retina and later involve the macular and peripheral areas. Visual symptoms usually appear between ages 10 and 30, though some children may become blind within the first year of life. Retinitis pigmentosa affects 1 of every 4,000 people in the United States.

Diagnosis

The signs and symptom information on this page attempts to provide a list of some possible signs and symptoms of Usher Syndrome. This medical information about signs and symptoms for Usher Syndrome has been gathered from various sources, may not be fully accurate, and may not be the full list of Usher Syndrome signs or Usher Syndrome symptoms. Furthermore, signs and symptoms of Usher Syndrome may vary on an individual basis for each patient. Only your doctor can provide adequate diagnosis of any signs or symptoms and whether they are indeed Usher Syndrome symptoms.