Stable long-term outcomes after cochlear implantation in subjects with TMPRSS3 associated hearing loss: a retrospective multicentre study.
M L A FehrmannW J HuinckM E G ThijssenL Haer-WigmanH G YntemaL J C RotteveelJ C C WiddershovenT GoderieM F van DoorenE H HoefslootM P van der SchroeffE A M Mylanusnull nullC P LantingR J E PenningsPublished in: Journal of otolaryngology - head & neck surgery = Le Journal d'oto-rhino-laryngologie et de chirurgie cervico-faciale (2023)
Subjects with TMPRSS3-associated SNHL have adequate and stable long-term outcomes after cochlear implantation, equal to the performance of genotyped patient with affected genes not expressed in the SGN. These findings are not in line with the spiral ganglion hypothesis. However, more recent studies showed that TMPRSS3 is mainly expressed in the hair cells with only limited SGN expression. Therefore, we cannot confirm nor refute the spiral ganglion hypothesis.