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This study aims to evaluate the effectiveness of ABI in pediatric patients with congenital non\u2010tumor hearing loss and elucidate the role of cortical plasticity in hearing and speech development. A prospective cohort study is conducted involving 112 consecutive pediatric patients who received ABI at a tertiary university\u2010based referral center from January 2019 to October 2023. The mean age at the time of surgery and postoperative follow\u2010up are 3.4\u00b11.9 years and 25.7\u00b111.7 months (3\u201348 months), respectively. The average percentage of elicited electrodes (eABR%) during the operation is 72.73\u00b117.99%. Post\u2010activation, hearing and speech outcomes improved steadily. Younger age at implantation (&lt;3 years), less severe inner\u2010ear malformation, and higher intraoperative eABR% (\u226560%) are significantly associated with better hearing and speech outcomes. Mismatch negativity (MMN) responses are evoked in all children aged 12\u201318 months. Cortical functional connectivity developed after activation, particularly within the bilateral temporal\/frontal lobes. Several factors contribute to hearing and speech development in children with ABI, and cortical plasticity plays a pivotal role. MMN amplitude and brain functional connectivity may serve as cortical indices for predicting long\u2010term outcomes.<\/jats:p>","DOI":"10.1002\/advs.202406092","type":"journal-article","created":{"date-parts":[[2025,3,30]],"date-time":"2025-03-30T15:21:46Z","timestamp":1743348106000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Neural Plasticity and Hearing\u2010Speech Development in Children with Auditory Brainstem Implants for Congenital Hearing Loss Due to Severe Inner Ear Malformation"],"prefix":"10.1002","volume":"12","author":[{"given":"Yu","family":"Zhang","sequence":"first","affiliation":[{"name":"Department of Otolaryngology\u2010Head and Neck Surgery Shanghai Ninth People's Hospital Shanghai Jiaotong University School of Medicine  No. 639, Zhizaoju Road Shanghai 200011 China"},{"name":"Ear Institute Shanghai Jiaotong University School of Medicine  No. 115, Jinzun Road Shanghai 200125 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