From Sound to Stability: Lessons Learned From the CRUSH Study on Hearing Loss Progression and Vestibular Phenotype in Usher Syndrome Type 2A
This analysis provides robust evidence of measurable hearing loss progression in PTA 0.5-4kHz thresholds in USH2a patients beyond presbycusis. Speech understanding, as measured by SRT, remains relatively stable. Vestibular evaluation revealed no semicircular canal dysfunction, although VEMP results suggest a potential subclinical otolith organ impairment in USH2a patients.
Authors
- F. L. J. Cals (ORCID: https://orcid.org/0000-0003-2092-2793)
- Rolien H. Free (ORCID: https://orcid.org/0000-0002-8564-5734)
- Jeroen J. Smits (ORCID: https://orcid.org/0000-0002-4241-0900)
- Dirk H. Wijn
- Cris Lanting
- Sybren M.M. Robijn
- Andy J. Beynon (ORCID: https://orcid.org/0000-0002-3191-6113)
- S Yzer
- Hedwig M. Velde
- Mirthe L.A. Fehrmann
- Carel B. Hoyng
- Erwin van Wijk
Institutions
- Radboud University Nijmegen (NL)
- Radboud University Medical Center (NL)
Publication Details
- Journal
- Otology & Neurotology
- Published
- 2026-02-23
- DOI
- https://doi.org/10.1097/mao.0000000000004851
- Primary Topic
- Hearing, Cochlea, Tinnitus, Genetics
- Type
- article
- Field-Weighted Citation Impact
- 0.00