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Findings With Unspecified Accuracy

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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    Population / CalculatorPrevalence Comments / Study / Link
    Overall U.S. population 0.004% PMID:6885960
    Congenitally deaf 3% 3-6% of congenitally deaf


    Deaf-blind individuals in U.S. 50% 50% of deaf-blind individuals in U.S.


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    The sensitivity and specificity of findings for Usher Syndrome are listed below. See the left navigation bar to change the display.

    Findings With Unspecified Accuracy

    Finding SensitivitySpecificity Comments, Study
    Sensorineural hearing loss (SNHL) Edit No accuracy specified.

    Study: no study specified.

    retinitis pigmentosa Edit No accuracy specified.


    Study: no study specified.

    vestibular dysfunction Edit No accuracy specified.

    Study: no study specified.