Intracranial vascular malformations can be classified as telangiectasis, varices, cavernous malformations, venous malformations (VMs), or arteriovenous malformations. VMs are congenital vascular malformations of the brain thought to be anomalies of the normal venous drainage. VMs are the most common intracranial vascular malformations documented by brain imaging and by autopsy series. However, vascular lesions of the internal auditory canal (IAC) are extremely rare. We report here a case of a VM arising within the IAC and expanding to the brainstem, causing a unilateral profound sensorineural hearing loss (SNHL). To the best of our knowledge, this is the first documented case of a VM as a rare cause of a unilateral SNHL in a child.
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