Article
Multicenter Natural History Study and Long-Term Cochlear Implant Outcomes in Usher Syndrome Subtypes.
Ear and hearing - 1 Jan 2026
Krumpoeck Paul Emmerich, Gadenstaetter Anselm Joseph, Uehara Natsumi, Fujita Takeshi, Giarbini Nadia, Pawloff Maximilian, Ritter Markus, Landegger Anna, Parzefall Thomas, Riss Dominik, Baumgartner Wolf-Dieter, Gstoettner Wolfgang, Arnoldner Christoph, Landegger Lukas David
Abstract excerpt
OBJECTIVES: Usher syndrome (USH) is an inherited disorder that causes bilateral sensorineural hearing loss (SNHL), retinitis pigmentosa, and vestibular defects. It represents the most common cause of combined deaf-blindness worldwide. Mutations in the nine known underlying genes are categorized into three types: USH type 1 has the earliest, most severe onset, while the much more common USH type 2 is comparatively...
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