Usher Syndrome: Likelihood Ratios

Introduction:

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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Prevalence

Population / Calculator	Prevalence	Comments / Study / Link
Overall U.S. population	0.004%	PMID:6885960
Congenitally deaf	3%	3-6% of congenitally deaf PMID:6885960
Deaf-blind individuals in U.S.	50%	50% of deaf-blind individuals in U.S. PMID:6885960
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The likelihood ratios of findings for Usher Syndrome are listed below. See the left navigation bar to change the display.

Finding	+LR	-LR	Comments, Study
Sensorineural hearing loss (SNHL)	No accuracy specified.		Study: no study specified.
retinitis pigmentosa	No accuracy specified.		Progressive Study: no study specified.
vestibular dysfunction	No accuracy specified.		Study: no study specified.