COCHLEAR IMPLANTSHearing Preservation and Spatial Hearing Outcomes After Cochlear Implantation in Children With TMPRSS3 MutationsPeng, Z. Ellen∗; Garcia, Alejandro†; Godar, Shelly P.∗; Holt, Jeffrey R.‡; Lee, Daniel J.†; Litovsky, Ruth Y.∗,§ Author Information ∗Waisman Center, University of Wisconsin-Madison, Madison, Wisconsin †Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts ‡Boston Children’s Hospital & Harvard Medical School, Boston, Massachusetts §Department of Communication Sciences & Disorders, University of Wisconsin-Madison, Madison, Wisconsin Address Correspondence and reprint requests to Z. Ellen Peng, Ph.D. Boys Town National Research Hospital, 555 North 30th Street, Omaha, NE 68131; E-mail: [email protected] J.R.H. is a consultant for Rescue Hearing and several other biotech companies developing inner ear therapeutics. The remaining authors disclose no conflicts of interest. Sources of support and disclosure of funding: This work was supported by the National Institute of Health (NIH) National Institute on Deafness and Other Communication Disorders grants R01DC003083, R01DC008365 (RYL), R01DC013521 (JRH) and in part by a core grant to the Waisman Center at University of Wisconsin-Madison from the NIH Eunice Kennedy Shriver National Institute of Child Health and Human Development grant P50HD105353. Otology & Neurotology 44(1):p 21-25, January 2023. | DOI: 10.1097/MAO.0000000000003747 Buy Metrics Abstract Objective Investigate hearing preservation and spatial hearing outcomes in children with TMPRSS3 mutations who received bilateral cochlear implantation. Study Design and Methods Longitudinal case series report. Two siblings (ages, 7 and 4 yr) with TMPRSS3 mutations with down-sloping audiograms received sequential bilateral cochlear implantation with hearing preservation with low-frequency acoustic amplification and high-frequency electrical stimulation. Spatial hearing, including speech perception and localization, was assessed at three time points: preoperative, postoperative of first and second cochlear implant (CI). Results Both children showed low-frequency hearing preservation in unaided, acoustic-only audiograms. Both children demonstrated improvements in speech perception in both quiet and noise after CI activations. The emergence of spatial hearing was observed. Each child's overall speech perception and spatial hearing when listening with bilateral CIs were within the range or better than published group data from children with bilateral CIs of other etiology. Conclusion Bilateral cochlear implantation with hearing preservation is a viable option for managing hearing loss for pediatric patients with TMPRSS3 mutations. © 2022, Otology & Neurotology, Inc.