Improving the Performance of Cochlear Implants for DFNB8/10 by accompanying Gene Therapy

• Applicant: Professorin Dr. Ellen Reisinger
• Subject Area: Otolaryngology, Phoniatrics and Audiology
• Term: since 2023
• Project identifier: Deutsche Forschungsgemeinschaft (DFG) - Project number 518273664

In cases of profound hearing loss, cochlear implantation (CI) can restore hearing to a level where most people can understand speech in everyday life. Electrodes on the CI electrically stimulate and thus activate spiral ganglion neurons (SGN), the first neurons of the auditory pathway. Therefore, intact SGN and a functioning auditory pathway are absolutely necessary for good CI performance. In the case of mutations in the gene TMPRSS3, which cause the recessively inherited hearing loss DFNB8/10, electrical stimulation evokes lower SGN responses. Cochlear implantation in childhood resulted in good hearing with CI, but the outcome in adults was highly variable. This is in line with the observation that a drastic reduction in SGN cell bodies was observed in the mouse model. My research project aims at deciphering the reason why CIs performance varies and declines in patients with mutations in the TMPRSS3 gene causing DFNB8/10. Subsequently, I aim to develop a gene therapy to improve CI performance in animal models for this form of hearing loss.

• DFG Programme: Research Grants