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JAMA Neurology Clinical Challenge
November 2018

Hearing and Vision Loss in an Older Man

Author Affiliations
  • 1Department of Neurology, University of California, Los Angeles
JAMA Neurol. 2018;75(11):1439-1440. doi:10.1001/jamaneurol.2018.1868

A 63-year-old man with a history of well-controlled hypertension, lumbar stenosis, and hip arthroplasty with revisions presented with bilateral loss of vision and hearing for several weeks. He also reported a history of painful paresthesias at his feet beginning about 1 year prior.

On examination, his visual acuity was initially 20/80 OD and 20/200 OS; it deteriorated over the next 2 months to finger counting bilaterally. The Weber test gave a nonlateralizing result, and bone conduction hearing was absent bilaterally. There was length-dependent loss of vibratory sensation and absent reflexes at both lower extremities. Magnetic resonance imaging demonstrated abnormal enhancement of bilateral optic nerves (Figure, A). Optical coherence tomography and fluorescein angiogram were normal. Nerve conduction studies showed mild sensory axonal polyneuropathy. Laboratory tests were notable for elevated thyrotropin (63 mlU/ml), low levels of free thyroxine (T4) (0.2 ng/dL), and elevated levels of cerebrospinal fluid protein (94 mg/dL). Cerebrospinal fluid IgG index, oligoclonal bands, aquaporin 4 antibody, paraneoplastic panel, and protein electrophoresis test results were all normal. He was treated empirically with high-dose steroids and plasmapheresis, with no improvement in symptoms.

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