![]() Progressive loss of peripheral vision impairs the ability to safely drive a car.Įvaluation of relatives at risk: The hearing of at-risk sibs should be assessed as soon after birth as possible to allow early diagnosis and treatment of hearing loss. Because of the high risk for disorientation when submerged in water, swimming needs to be undertaken with caution. Annual ophthalmologic evaluation, fundus photography, visual acuity, visual field testing, electroretinography, optical coherence tomography, and fundus autofluorescence from age 20 years.Īgents/circumstances to avoid: Competition in sports requiring acute vision and/or good balance may be difficult and possibly dangerous. Annual otoscopic exam with tympanometry in children with profound loss to evaluate for chronic otitis media. Surveillance: Annual audiometry and tympanometry in those with cochlear implant or hearing aids to assure adequate auditory stimulation. Standard treatments for retinitis pigmentosa. Vestibular compensation therapy for children with residual balance function and sensory substitution therapy for individuals with complete absence of vestibular function. Specialized training from educators of the hearing impaired. Sign language and tactile signs (once visual loss occurs) for families who choose non-auditory communication. ![]() Cochlear implantation should be considered as young as medically feasible. ![]() Treatment of manifestations: In infants: an initial trial of hearing aids to stimulate residual hearing and accustom the infant to auditory stimulation. ![]()
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