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  1. 30 de mar. de 2022 · Usher syndrome (USH) is the most common hereditary form of deaf–blindness, with a global prevalence of 4 to 17 cases per 100,000 individuals; it accounts for more than half of all hereditary cases of deaf–blindness and 3–6% of all cases of childhood hearing loss (Hope et al. 1997; Kimberling et al. 2010 ).

  2. 17 de sept. de 2020 · Usher syndrome is a disorder with vast clinical and genetic heterogeneity, typically resulting in significant dual sensory loss causing great impact on patient quality of life. More than ever, the prospect of an available treatment for at least some Usher subtypes looks promising.

  3. Usher syndrome (USH) is a genetic disorder that is characterized by sensorineural hearing loss (HL) and visual abnormality, i.e., loss of night vision and side (peripheral) vision. Usher syndrome is categorized into four subtypes (USH1, USH2, USH3, USH4) on the basis of phenotypic spectrum.

  4. 11 de ene. de 2022 · Usher syndrome (USH) is the most common deafblind syndrome, with 50% of deaf-blindness in persons younger than 65 years of age. It is a genetic condition that includes hearing loss, retinopathy (retinitis pigmentosa), and vestibular areflexia with different entities and onset [ 2, 3, 4 ].

  5. 10 de dic. de 1999 · Usher syndrome type I (USH1) is characterized by congenital, bilateral, profound sensorineural hearing loss, vestibular areflexia, and adolescent-onset retinitis pigmentosa (RP). Unless fitted with a cochlear implant, individuals do not typically develop speech.

  6. Usher syndrome (USH) is a rare, autosomal recessively inherited disorder resulting in a combination of sensorineural hearing loss and a progressive loss of vision resulting from retinitis pigmentosa (RP), occasionally accompanied by an altered vestibular function.

  7. Usher syndrome (USH) is the most common cause of deaf-blindness in humans, with a prevalence of about 1/10,000 (~ 400,000 people worldwide). Cochlear implants are currently used to reduce the burden of hearing loss in severe-to-profoundly deaf patients, but many promising treatments including gene, ….