Usher Syndrome: Sensitivity and Specificity
The Usher syndromes (US) are a genetically and clinically heterogeneous group of diseases characterized by SNHL, retinitis pigmentosa, and often vestibular dysfunction.
Type 1: severe to profound congenital hearing loss, vestibular dysfunction, and retinitis pigmentosa that develops in childhood
Type 2: moderate to severe congenital hearing loss, with uncertainty related to progression, no vestibular dysfunction, and retinal degeneration that begins in the third to fourth decade
Type 3: progressive hearing loss, variable vestibular dysfunction, and variable onset of retinitis pigmentosa
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The sensitivity and specificity
of findings for Usher Syndrome are listed below.
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