Long-Term Outcomes of Cochlear Implantation in Usher Syndrome.
Mirthe L A Fehrmann, Cris P Lanting, Lonneke Haer-Wigman and 4 others
PMID 38987893WHAT IT FOUND
Early cochlear implantation in Usher syndrome yields excellent long-term speech recognition, especially in children implanted before age 8.
Adults implanted later gain sound awareness but not oral communication skills. Outcomes are stable for USH2 and USH3, though variable in USH3.
Key findings
01Early implanted children with USH1 achieved median phoneme scores of 100% at long-term follow-up, significantly better than late-implanted adults.
02Simultaneous bilateral implantation in USH1 children resulted in significantly higher binaural phoneme scores compared to sequential implantation.
03Late-implanted USH1 adults reported satisfaction with the implant for sound detection and safety, despite poor speech recognition.
STILL TO COME
How it was doneWhat they foundWhat it means for SLPs
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What it does not show
Small sample sizes in specific subgroups, particularly late-implanted USH1 and USH3, limit statistical power. Retrospective design resulted in missing data for some participants. The comparison between simultaneous and sequential implantation in USH1 relied on very few subjects in the sequential group.
Declared interests
The authors declared no conflicts of interest. The study was funded by Cochlear.
The easy way to misread this
Do not assume that cochlear implantation will restore speech recognition in adults with Usher syndrome who have had profound hearing loss since childhood. In late-implanted USH1 subjects, speech scores remained low, and the primary benefit reported was sound awareness and safety, not oral communication.