Table 7.

Recommended Surveillance for Individuals with Usher Syndrome Type II

System/ConcernEvaluationFrequency
Hearing loss Audiometry & tympanometry w/hearing aids or cochlear implant to assure adequate auditory stimulationAnnually, incl testing w/hearing aids in place
Cataracts Ophthalmologic evalAnnually from age 20 yrs or age of diagnosis
Cystoid macular
edema
Ophthalmologic eval
Retinitis
pigmentosa
Fundus photography, VA, VF (Goldmann perimetry, Humphrey perimetry, dark adapted rod perimetry), ERG, OCT, FAFAnnually from age 10 yrs or age of diagnosis

ERG = electroretinography; FAF = fundus autofluorescence; OCT = optical coherence tomography; VA = visual acuity; VF = visual field

From: Usher Syndrome Type II

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