To explore how auditory and speech function developed post-cochlear implantation (pCI) in prelingually deaf children with white matter lesions (WML). Patients (41 males, 29 females; mean age at implantation 2y 11mo [SD 7.5mo], range 1y 8mo-5y) were divided into the following groups according to preoperative brain magnetic resonance imaging evaluation: mild WML, moderate WML, severe WML, and control. We assessed auditory and speech performance at baseline, 6months, 12months, and 24months pCI. As well as auditory event-related potentials, topographic maps and electroencephalography source imaging were recorded and analysed at 24months pCI. For children with WML (any level), postoperative auditory or speech performance at 6months was significantly below that of control participants. After stratification, auditory and speech performance was highly related to WML grading. Auditory or speech performance in mild WML or control groups was comparatively better than the moderate WML and severe WML groups. The recovery rate of speech performance fell behind that of the auditory perception. With the increasing severity of WML, N1 amplitude was significantly smaller with a consistent presentation in the topographic map, which was similar in the mild WML and control group. The dominant auditory centre was activated in the control or mild WML groups, but not in the moderate WML and severe WML groups. WML gradually affect auditory and speech development, and electrophysiological performance pCI in prelingually deaf children. Auditory and speech performance in prelingually deaf children with white matter lesions (WML) was significantly worse than those without WML. Postoperative auditory and speech performance in children with WML was highly related to WML grading. Grand N1 amplitude in auditory event-related potentials was negatively related to the severity of WML. Non-dominant areas close to the auditory cortex were potentially activated in severe WML.
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