Cochlear Implantation and Electric Acoustic Stimulation in Children With TMPRSS3 Genetic Mutation.

Mutations in the TMPRSS3 gene, although rare, can cause high frequency hearing loss with residual hearing at low frequencies. Several previous studies have reported cochlear implant (CI) outcomes for adults with TMPRSS3 mutation with mixed results. Although some studies have suggested that TMPRSS3 is expressed in spiral ganglion cells, it remains unclear if previously reported poor CI outcomes in this population were secondary to long durations of deafness or to the effects of the TMPRSS3 mutation. To date, no studies in the literature have reported CI outcomes for children with TMPRSS3 mutation treated with CI.