usher syndromes/hearing loss

A cochlear implant (CI) is a small, complex electronic device used by the profoundly deaf or severely hard-of-hearing. The CI has two main components: 1. The externally worn microphone, sound processor and transmitter system; 2. The implanted receiver and electrode system sending electrical currents

This natural history study of patients with USH2A mutations will accelerate the development of outcome measures for clinical trials. Sensitive, objective outcome measures of retinal degeneration will greatly facilitate development of treatments for Usher syndrome patients. Together these approaches

Background and objectives: The Usher syndromes are a group of clinically variable and genetically heterogeneous autosomal recessive syndromes. On the basis of clinical findings, at least three types exist. Patients with Usher type I are born deaf, have vestibular problems, and are thought to

The Usher Syndromes (USH), characterized by autosomal recessive inheritance, are genotypically distinct diseases which share specific phenotypic characteristics. Affected individuals have congenital neurosensory hearing impairment of variable severity and a progressive pigmentary retinal