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Superficial hemosiderosis of the central nervous system (SH-CNS) is a relatively rare condition caused by chronic or repeated subarachnoid bleeding. The aim of this article was to present hearing disorders related to SH-CNS, and to describe results of magnetic resonance imaging (MRI) to confirm the diagnosis. We performed a retrospective case review at a tertiary referral center. Clinical presentation, auditory manifestations and MRI findings of two patients with SH-CNS are reported. The two patients experienced bilateral sensorineural hearing loss (SNHL) that progressively worsened. MRI revealed typical aspects of SH-CNS on T2-weighted images. Progressive bilateral SNHL is the most common manifestation of SH-CNS. The otolaryngologist should be aware of this disease that can be easily diagnosed with MRI.