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

Retrospective record review with no control group. Patients were not assigned to hearing aids or implants; they got whatever their clinical team chose, so the comparison between treatments is a description of what happened, not a test of which is better. Some subtypes rest on one or two people. There were 3 patients with USH type 3, 1 with USH2C and 1 with USH1G, and no patients at all with USH1C, USH1D or USH2D, so the type-by-type results are thin. Many patients with a clinical diagnosis of Usher syndrome had to be left out because no genetic testing records existed or because records held elsewhere could not be accessed, which may have skewed who is in the cohort. Follow-up was very uneven: the shortest observation was a single audiometry on one day and the longest was 546 months (45.5 years), and the number of hearing tests per patient varied widely. Speech testing was only done at some centres, often sporadically, and at many different loudness levels, which is why only tests at 65 and 80 dB HL were analysed. There are no pre-implantation speech scores, so speech perception could not be tracked from before to after surgery. The paper has no information on which hearing aids were used, when they were first fitted, or how long patients wore them, all of which affect how well they work. Implant-aided hearing thresholds were measured from the implanted ear alone with no hearing aid in the other ear, which does not reflect how a patient hears in everyday life. Every implant in the study was made by one manufacturer, so nothing here compares devices.

The authors declare no conflicts of interest. Funding is named from the Christian Doppler Research Society and the Austrian National Foundation for Research, Technology and Development, and the paper says nothing further about what role the funders had. Every cochlear implant used in the study was made by MED-EL.

Do not read the finding that age at implantation did not affect outcome as evidence that implanting children early is unnecessary. Only 12 patients were implanted, each was operated on as part of routine care with no control group, and the paper itself still describes early bilateral implantation in congenitally deaf children as the standard of care.