Microtia-atresia is a congenital malformation of the external ear, often affecting one side and being associated with severe-to-profound unilateral conductive hearing loss (UCHL). Although the impact of unilateral hearing loss (UHL) on speech recognition, sound localization and brain plasticity has been intensively investigated, less is known about the subjects with unilateral microtia-atresia (UMA). Considering these UMA subjects have hearing loss from birth, we hypothesize it has a great effect on brain organization. A questionnaire on speech recognition and spatial listening ability was administered to 40 subjects with UMA and 40 age- and sex-matched controls. UMA subjects showed poorer speech recognition in laboratory and poorer spatial listening ability. However, cognitive scores determined by the Montreal Cognitive Assessment (MoCA) and Wechsler Intelligence Scale for Children (WISC-IV) did not differ significantly in these two groups. The impact of hearing loss in UMA on brain functional organization was examined by comparing resting-state fMRIs (rs-fMRI) in 27 subjects with right-sided UMA and 27 matched controls. UMA subjects had increased nodal betweenness in visual networks and DMN but decreases in auditory and attention networks. These results indicate that UCHL in UMA causes significant abnormalities in brain organization. The impact of UCHL on cognition should be further examined with a battery of tests that are more challenging and better focused on the cognitive networks identified.

小耳症闭锁是一种先天性外耳畸形，常累及一侧并与重度至重度单侧传导性听力损失 (UCHL) 相关。虽然单侧听力损失 (UHL) 对语音识别、声音定位和大脑可塑性的影响已被深入研究，但对单侧小耳畸形闭锁 (UMA) 的受试者知之甚少。考虑到这些 UMA 受试者出生时就有听力损失，我们假设它对大脑组织有很大影响。对 40 名 UMA 受试者和 40 名年龄和性别匹配的对照组进行了一份关于语音识别和空间聆听能力的问卷调查。UMA 受试者在实验室中表现出较差的语音识别能力和较差的空间聆听能力。然而，由蒙特利尔认知评估 (MoCA) 和韦氏儿童智力量表 (WISC-IV) 确定的认知分数在这两组中没有显着差异。通过比较 27 名右侧 UMA 受试者和 27 名匹配对照的静息状态 fMRI (rs-fMRI)，检查了 UMA 中听力损失对大脑功能组织的影响。UMA 受试者在视觉网络和 DMN 中增加了节点介数，但在听觉和注意力网络中减少了。这些结果表明 UMA 中的 UCHL 会导致大脑组织出现显着异常。UCHL 对认知的影响应该通过一系列更具挑战性和更专注于识别的认知网络的测试来进一步检查。通过比较 27 名右侧 UMA 受试者和 27 名匹配对照的静息状态 fMRI (rs-fMRI)，检查了 UMA 中听力损失对大脑功能组织的影响。UMA 受试者在视觉网络和 DMN 中增加了节点介数，但在听觉和注意力网络中减少了。这些结果表明 UMA 中的 UCHL 会导致大脑组织出现显着异常。UCHL 对认知的影响应该通过一系列更具挑战性和更专注于识别的认知网络的测试来进一步检查。通过比较 27 名右侧 UMA 受试者和 27 名匹配对照的静息状态 fMRI (rs-fMRI)，检查了 UMA 中听力损失对大脑功能组织的影响。UMA 受试者在视觉网络和 DMN 中增加了节点介数，但在听觉和注意力网络中减少了。这些结果表明 UMA 中的 UCHL 会导致大脑组织出现显着异常。UCHL 对认知的影响应该通过一系列更具挑战性和更专注于识别的认知网络的测试来进一步检查。这些结果表明 UMA 中的 UCHL 会导致大脑组织出现显着异常。UCHL 对认知的影响应该通过一系列更具挑战性和更专注于识别的认知网络的测试来进一步检查。这些结果表明 UMA 中的 UCHL 会导致大脑组织出现显着异常。UCHL 对认知的影响应该通过一系列更具挑战性和更专注于识别的认知网络的测试来进一步检查。