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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.

    Pre-Test Probability (Prevalence): %. Post-Test Probability (Predictive Value): %. Switch to display mode.
    **Note that calculating probabilities from more than one finding is inherently inaccurate because findings are not independent. (For example, using two positive findings that share a common pathogenesis is likely to overestimate the true probability.)
    Findings With Unspecified Accuracy
    FindingResult SensitivitySpecificity
    [ + ] Sensorineural hearing loss (SNHL) No accuracy specified.
    [ + ] retinitis pigmentosa No accuracy specified.
    [ + ] vestibular dysfunction No accuracy specified.