Hearing Loss and Bilateral Recurrent Peripheral Facial Nerve Palsy in Superficial Siderosis

Abstract

Superficial siderosis of the central nervous system (SSCN) is a very rare disorder. The clinical syndrome of SSNC consists of sensorineural hearing loss, cerebellar ataxia and myelopathy. A 58 year-old man who has a bilateral sensorineural hearing loss, recurrent peripheral facial nerve palsy and gait instability for 10 years with a history of recurrent head trauma is presented. The neurological examination revealed bilateral sensorineural hearing loss, cerebellar ataxia, neurogenic bladder, anosmia and mild spasticity of the lower extremities. In patients with progressive bilateral cochleo-vestibular deficit and recurrent peripheral facial nerve palsy of unknown etiology, MRI is the examination of choice to confirm SS.