Usher Syndromes

**Definition:** Autosomal recessive hereditary disorders characterized by congenital SENSORINEURAL HEARING LOSS and RETINITIS PIGMENTOSA. Genetically and symptomatically heterogeneous, clinical classes include type I, type II, and type III. Their severity, age of onset of retinitis pigmentosa and the degree of vestibular dysfunction are variable.

**Tree numbers:** - C09.218.458.341.186.500.500 - C09.218.458.341.887.886 - C10.597.751.418.341.186.500.500 - C10.597.751.418.341.887.886 - C10.597.751.941.162.625.500 - C11.768.585.658.500.813 - C11.966.075.375.500 - C16.131.077.299.500 - C16.320.290.684.500 - C23.888.592.763.393.341.887.886

**Synonyms:** - Usher Syndrome - Deafness-Retinitis Pigmentosa Syndrome - Dystrophia Retinae Pigmentosa-Dysostosis Syndrome - Retinitis Pigmentosa-Deafness Syndrome - Hallgren Syndrome - Usher's Syndrome - Graefe-Usher Syndrome