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Multicenter Natural History Study and Long-Term Cochlear Implant Outcomes in Usher Syndrome Subtypes.
Paul Emmerich Krumpoeck1,2, Anselm Joseph Gadenstaetter1,2, Natsumi Uehara3
1Christian Doppler Laboratory for Inner Ear Research, Department of Otolaryngology - Head and Neck Surgery, Vienna General Hospital, Medical University of Vienna, Vienna, Austria.
Ear and Hearing
|April 23, 2026
Summary
Cochlear implants significantly benefit Usher syndrome (USH) patients with severe hearing loss, offering long-term hearing improvement. This study provides a benchmark for evaluating new USH gene therapies.
Area of Science:
- Ophthalmology
- Genetics
- Audiology
Background:
- Usher syndrome (USH) causes inherited bilateral sensorineural hearing loss (SNHL), retinitis pigmentosa, and vestibular dysfunction, leading to combined deaf-blindness.
- Mutations in nine genes cause USH, classified into types 1 (severe, early onset) and 2 (milder, later onset), with type 3 being rare and variable.
- Current SNHL management for USH involves hearing aids or cochlear implants, but studies often lack long-term data and subtype differentiation.
Purpose of the Study:
- To evaluate the long-term effectiveness of conventional hearing aids and cochlear implantation in patients with genetically confirmed Usher syndrome.
- To establish a benchmark for comparing future gene therapies against current standard-of-care treatments for USH.
Main Methods:
- A retrospective multicenter cohort study analyzed 655 audiograms from 33 patients with genetically confirmed USH.
- Correlated patient characteristics and treatments with pure-tone average thresholds and speech perception over a mean follow-up of 7 years and 10 months.
- Specifically analyzed outcomes 8 years and 2 months post-cochlear implantation.
Main Results:
- Conventional hearing aids provided a modest average improvement of 20.1 dB HL but did not halt progressive SNHL.
- Cochlear implantation in patients with severe-to-profound SNHL or deafness restored hearing to average thresholds of 37.9 dB HL at 1 year, 37.3 dB HL at 2 years, and 27.5 dB HL at 15-20 years.
- All patients receiving cochlear implants showed benefit, regardless of age at implantation or USH mutation type.
Conclusions:
- Cochlear implantation is a highly effective treatment for severe SNHL in Usher syndrome, offering sustained benefits.
- These findings serve as a crucial benchmark for assessing emerging gene therapies for USH.
- Further large-scale studies with detailed monitoring are needed to refine understanding of treatment benefits across USH subtypes.
