Background: There are two types of central auditory disorders due to pathology of the bilateral auditory cortices in adult patients: auditory agnosia with residual hearing; cortical deafness with total hearing loss. However, long-term changes of hearing acuity over physical development time are unknown.

Objective: The aim of this case report was to illustrate the pathophysiology of auditory changes in a 1 year-3 months old child who was diagnosed with auditory agnosia as a sequel of herpes encephalitis and later developed cortical deafness during a 36-year follow-up.

Materials and methods: Brain MRI, hearing tests, ABR, DPOAE and developmental tests of speech/language were periodically performed.

Results: Brain imaging revealed extensive damage in the bilateral auditory cortices. His auditory speech perception was lost and his language development was seriously delayed. His hearing threshold progressively decreased over time from normal at first to profound hearing loss as a teenager. However, at 29 years of age his ABR was normal. He was able to communicate and converse by sign language or writing.

Conclusions and significance: Later developing cortical deafness with profound hearing loss could be caused by retrograde degeneration of the auditory radiation overtime to the bilateral medial geniculate bodies.

Keywords: ABR; Auditory agnosia; auditory cortex; cortical deafness; herpes encephalitis; retrograde degeneration.

背景：

成年患者的双侧听皮层病理导致两种类型的中枢性听力障碍：残余听力的听觉失认症；全聋的皮层耳聋。然而，随着生理发育时间的变化，听力敏锐度的长期变化尚不清楚。

目的：

本案例报告旨在阐述一名1岁3个月大的儿童因疱疹性脑炎后遗症被诊断为听觉失认症，并在随后长达36年的随访中发展出皮层耳聋的听力病理生理变化情况。

材料和方法：

定期进行大脑MRI、听力测试、ABR（听觉脑干反应）、DPOAE（畸变产物耳声发射）以及言语/语言发育评估。

结果：

大脑成像显示双侧听皮层有广泛的损伤。他的言语听力感知能力丧失，语言发展严重延迟。随着时间的推移，他的听力阈值从最初的正常逐渐下降至青少年时期的重度听力损失。然而，在29岁时，他的ABR测试结果是正常的。他能够通过手语或书写进行交流。

结论与意义：

迟发性的皮层耳聋和严重的听力丧失可能是由于随着时间推移听辐射向双侧内侧膝状体的逆行性退行导致的。

关键词：

ABR；听觉失认症；听觉皮层；皮层耳聋；疱疹性脑炎；逆行性退化。