Table 5. [Treatment of Manifestations in Individuals with Usher Syndrome Type I]. - GeneReviews®

Manifestation/ Concern	Treatment	Considerations/Other
Hearing loss	An initial trial of hearing aids even w/profound loss stimulates any residual hearing & accustoms the infant to auditory stimulation for auditory/oral language development. Cochlear implantation should be considered as early as is medically feasible. ¹	Hearing aids are usually inadequate in severe-to-profound hearing loss. Sign language such as American Sign Language and tactile signs (once visual loss occurs) are communication options for families who choose nonauditory communication.
Specialized training from educators of the hearing impaired	Recommended for affected children & all family members to improve communication skills
Balance difficulties & ↑ risk of accidental injury	Vestibular compensation therapy for children w/residual balance function & sensory substitution therapy for individuals w/complete absence of vestibular function	Well-supervised sports activities may help develop somatosensory component of the balance system.
Vision loss	See Nonsyndromic Retinitis Pigmentosa Overview, Management.	Adults w/USH1 age >20 yrs use sign language & lip reading for communication. As vision loss progresses, these methods become increasingly difficult & tactile signing may ultimately be required.

Manifestation/
Concern

Treatment

Considerations/Other

Hearing loss

An initial trial of hearing aids even w/profound loss stimulates any residual hearing & accustoms the infant to auditory stimulation for auditory/oral language development.
Cochlear implantation should be considered as early as is medically feasible. ¹

Hearing aids are usually inadequate in severe-to-profound hearing loss. Sign language such as American Sign Language and tactile signs (once visual loss occurs) are communication options for families who choose nonauditory communication.

Specialized training from educators of the hearing impaired

Recommended for affected children & all family members to improve communication skills

Balance
difficulties
& ↑ risk of
accidental
injury

Vestibular compensation therapy for children w/residual balance function & sensory substitution therapy for individuals w/complete absence of vestibular function

Well-supervised sports activities may help develop somatosensory component of the balance system.

Vision loss

See Nonsyndromic Retinitis Pigmentosa Overview, Management.

Adults w/USH1 age >20 yrs use sign language & lip reading for communication. As vision loss progresses, these methods become increasingly difficult & tactile signing may ultimately be required.

From: Usher Syndrome Type I

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Adam MP, Feldman J, Mirzaa GM, et al., editors.

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