Table 6.

Recommended Surveillance for Individuals with Usher Syndrome Type I

System/ConcernEvaluationFrequency
Hearing loss Audiometry & tympanometry w/cochlear implant or hearing aids to assure adequate auditory stimulation
  • For persons w/o profound loss, annual testing allows appropriate hearing aid adjustment.
  • For cochlear implant recipients, annual follow up is necessary to assure appropriate implant function & programming.
  • Children w/profound loss & cochlear implants can still develop fluid & chronic ear infection issues, which are less evident to the child because of hearing loss. Annual otoscopic exam w/tympanometry avoids potential serious complications of chronic otitis media.
Cataracts Ophthalmologic evalAnnually from age 20 yrs or from age at diagnosis
Cystoid
macular edema
Retinitis
pigmentosa 		Fundus photography, VA, VF (Goldmann perimetry, Humphrey perimetry, dark-adapted rod perimetry), ERG, OCT, & FAF

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

From: Usher Syndrome Type I

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