Vision loss in an infantryman with complex hearing loss: Case report and review of Susac syndrome

Abstract: Susac syndrome can resemble various disorders resulting in a delayed or missed diagnosis and subsequent delays in treatment. Here, we present how successful consideration of patient history, symptoms, and ancillary testing led to prompt diagnosis and treatment of Susac syndrome by ophthalmologists. A 27-year-old active duty infantryman presented with sudden vision loss in the right eye during strenuous exercise after experiencing similar symptoms in the left eye 5 months earlier. The patient's medical history was notable for mixed conductive and sensorineural hearing loss, confounded by a history of concussions, syncope, frequent ear infections, tympanic membrane perforation requiring tympanoplasty, and loud noise exposures. Identification of branch retinal artery occlusions on fluorescein angiography and low-to-mid frequency hearing loss confirmed the diagnosis of Susac syndrome. The patient was immediately treated with high-dose oral prednisone and subsequently transitioned to rituximab infusions to preserve vision. Susac syndrome should be considered when a patient presents with vision loss, hearing deficits, and or cognitive changes, even if symptoms are separated by time and seemingly attributed to more common causes.

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