Global Aphasia and Auditory Agnosia Following Left Hemispheric Intracerebral Hemorrhage in Moyamoya Disease: A Case Report

Objectives: This study aimed to report a rare case of a patient with Moyamoya disease who developed left hemispheric intracerebral hemorrhage presenting with global aphasia accompanied by auditory agnosia. We also aimed to explore the clinical manifestations and diagnostic evaluation of combined severe language and auditory processing deficits caused by chronic hemorrhagic brain injury in the left middle cerebral artery territory in this context.Methods: Comprehensive clinical, neuropsychological, and audiological assessments were conducted, including language function tests (K-WAB), speech audiometry, brain imaging (MRI and MRA), and electrophysiological studies (BAEPs). The patient received herbal medicines, acupuncture, and rehabilitative therapy during the chronic phase after intracerebral hemorrhage.Results: The patient exhibited profound global aphasia with extremely low language comprehension and expression scores, along with auditory agnosia characterized by an inability to recognize sounds despite preserved peripheral hearing. Neuroimaging revealed encephalomalacia and a porencephalic cyst in the left parietotemporal lobe with an old hemorrhagic lesion in the left thalamus, corresponding to the language and auditory processing networks in the left middle cerebral artery territory. Audiological tests showed only mild high-frequency sensorineural hearing loss with normal brainstem auditory evoked potentials, supporting a central rather than peripheral origin of the auditory deficit.Conclusions: This case highlights the complex neurological impairment associated with Moyamoya disease involving both language and auditory processing centers. In particular, it illustrates that left hemispheric intracerebral hemorrhage in a patient with Moyamoya disease can lead to persistent global aphasia accompanied by auditory agnosia despite relatively preserved peripheral hearing. This underscores the necessity of multidisciplinary evaluation and integrated treatment approaches to manage such multifaceted cerebrovascular conditions effectively. The case also suggests that combining conventional rehabilitation with Korean medicine interventions may help optimize the recovery environment during the chronic post-hemorrhagic phase.