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Cochlear hearing loss pattern in Susac syndrome (audiogram)

Audiogram showing bilateral low-frequency sensorineural hearing loss, rising to normal at higher frequencies. This is the typical cochlear hearing loss pattern seen in Susac syndrome, likely reflecting microinfarction of the striae vascularis at the apex of the cochlea. Legend: X = left ear; O = right ear. (Source: Rennebohm RM, Lubow M, Rusin J, Martin L, Grzybowski DM, Susac JO. Aggressive immunosuppressive treatment of Susac's syndrome in an adolescent: using treatment of dermatomyositis as a model. Pediatr Rheumatol Online J 2008;6:3. Creative Commons Attribution [CC BY] license, creativecommons.org/licenses/by/2.0.)

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Associated Disorders

  • Cerebral microinfarcts
  • Deafness
  • Encephalopathy
  • Retinal arterial occlusion
  • Retinopathy