title: Auditory manifestations of superficial hemosiderosis of the central nervous system.
abstract: 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.
authors.affiliation: Otology-Neurotology Unit, Department of Otolaryngology Head and Neck Surgery, Fondation Adolphe de Rothschild, 25-29 rue Manin, 75019, Paris, France. dayache@fo-rothschild.fr
authors.forename: Denis
authors.initials: D
authors.lastname: Ayache
authors.forename: Catherine
authors.initials: C
authors.lastname: Blaivie
authors.forename: Asma
authors.initials: A
authors.lastname: El Kohen
authors.forename: Lucrecia
authors.initials: L
authors.lastname: Tosello
authors.forename: Marc T
authors.initials: MT
authors.lastname: Williams
mesh_terms: D000328:Adult; D002493:Central Nervous System Diseases / Q000150:complications* / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D006310:Hearing Aids; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology*; D006486:Hemosiderosis / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male