![]() However, this study included both clinical/demographics and genetic information of the patients in the manuscript thus, it is believed that information for future replication will be sufficient. We provide contact information for the IRB (Contact information: +82-3, for future readers with data access requests. B-2007-622-114) because the data include patient information that is potentially identified or sensitive (e.g., genetic information). ![]() This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.ĭata Availability: There are ethical or legal restrictions on disclosing anonymized data sets (e.g., website) under IRB regulations (IRB No. ![]() Received: NovemAccepted: Published: June 7, 2021Ĭopyright: © 2021 Lee et al. PLoS ONE 16(6):Įditor: Qian-Jie Fu, University of California, Los Angeles, UNITED STATES (2021) Central auditory maturation and behavioral outcomes after cochlear implantation in prelingual auditory neuropathy spectrum disorder related to OTOF variants (DFNB9): Lessons from pilot study. We believe that the current study opens a new path toward genome-based neuroimaging in the field of hearing research.Ĭitation: Lee S-Y, Han JH, Song H-K, Kim NJ, Yi N, Kyong J-S, et al. Collectively, our results suggest that central auditory maturation and successful outcome of CI in DFNB9 may have more demanding requirements, that is, earlier implantation and more sustained rehabilitation. Indeed, an additional follow-up study showed that a reduction in P1 latency was linked to an improvement in auditory performance. This suggests the importance of sustained rehabilitation in DFNB9 than in other etiologies. However, timely implantation, as early as 12 months of age per se, might be insufficient to achieve age-appropriate cortical maturation of DFNB9 in cases with six to seven months of device use. We observed that DFNB9 subjects who received CI after 2 years of age exhibited “absent” or “anomalous” P1 components that correspond to delayed language development. We conducted a preliminary study comprising 10 subjects diagnosed with OTOF-related ANSD who underwent CI by a single surgeon and subsequently underwent measurements of the P1 component. Here, we sought to provide a much better understanding of the brain perspectives (i.e., P1 maturation) in OTOF-associated ANSD subjects and set the stage for an optimal strategy to enhance language development. ![]() Thus, a focused evaluation of those carrying OTOF variants, which may be the salient molecular etiology of prelingual ANSD, would circumvent the issue of heterogeneity. However, varying degrees of neural dyssynchrony, resulting from the etiological heterogeneity of ANSD, may preclude uniform application of this hypothesis to ensure auditory cortical maturation. To date, early intervention primarily before the age of two years and six months of CI usage is necessary and sufficient to achieve age-appropriate cortical maturation and good prognosis. The cortical auditory evoked potential (CAEP)-based P1 component acts as a biomarker for cochlear implantation (CI) outcomes in children with auditory neuropathy spectrum disorder (ANSD). ![]()
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