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April 23, 2026Ear and HearingOpen Access

Multicenter Natural History Study and Long-Term Cochlear Implant Outcomes in Usher Syndrome Subtypes

Paul Emmerich Krumpoeck Anselm Joseph Gadenstaetter Natsumi Uehara Takeshi Fujita Nadia Giarbini Maximilian Pawloff Markus Ritter Anna Landegger Thomas Parzefall Dominik Riss Wolf-Dieter Baumgartner Wolfgang Gstoettner Christoph Arnoldner Lukas D. Landegger

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Key Points

The aim is to provide a benchmark for comparing new gene therapies to existing treatments for Usher syndrome.
Conducted a multicenter natural history study
Monitored hearing and speech perception abilities
Involved extensive genetic testing across Usher syndrome subtypes.
Current cochlear implant outcomes serve as benchmarks for future gene therapies
Increased genetic testing may clarify treatment benefits for specific subtypes of Usher syndrome.

Implication

Multicenter natural history study assesses cochlear implant outcomes in Usher syndrome subtypes, suggesting benchmarks for gene therapy comparisons.

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Cite This Study

Krumpoeck et al. (2026) studied this question.

synapsesocial.com/papers/69ec59fc88ba6daa22dab9bf https://doi.org/10.1097/aud.0000000000001822

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Keep reading

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

1THE BENEFITS OF EARLY COCHLEAR IMPLANTATION FOR SPEECH DEVELOPMENT IN CHILDREN WITH USHER SYNDROME: LITERATURE REVIEW2024
2Usher Syndrome and Cochlear ImplantationCohort Study · 2003 · 92 citations
3Navigating the Usher Syndrome Genetic Landscape: An Evaluation of the Associations between Specific Genes and Quality Categories of Cochlear Implant Outcomes2024 · 4 citations
4From Sound to Stability: Lessons Learned From the CRUSH Study on Hearing Loss Progression and Vestibular Phenotype in Usher Syndrome Type 2AOriginal Research · 2026
5[Genetic and clinical phenotypic analysis of Usher syndrome-associated gene variants].2025