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Clinical Pathologic Conference |

A Young Man With Progressive Vision and Hearing Loss

Nathan H. Kung, MD1; Robert C. Bucelli, MD, PhD1; Renee B. Van Stavern, MD1; Joel A. Goebel, MD2; Gregory P. Van Stavern, MD3
[+] Author Affiliations
1Department of Neurology, Washington University in St Louis, St Louis, Missouri
2Department of Otolaryngology–Head and Neck Surgery, Washington University in St Louis, St Louis, Missouri
3Department of Ophthalmology, Washington University in St Louis, St Louis, Missouri
JAMA Neurol. 2016;73(7):880-883. doi:10.1001/jamaneurol.2016.1336.
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A 37-year-old man with a history of progressive bilateral sensorineural hearing loss presented to a neuro-ophthalmology clinic with an acute left homonymous hemianopsia. In this article, we discuss the clinical approach and differential diagnosis of progressive combined vision and hearing loss and guide the reader to discover the patient’s ultimate diagnosis.

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Figure.
Fundus Photographs, Humphrey Visual Fields, and Computed Tomography

Fundus photographs of the right eye (A) and left eye (B), showing normal optic discs without evidence of occult optic atrophy, pigmentary retinopathy, or maculopathy. Humphrey 30-2 Swedish Interactive Threshold Algorithm–fast visual fields of the left eye (C) and right eye (D), showing a homonymous visual field defect consistent with a left homonymous hemianopsia. E, Noncontrast head computed tomography showing the symptomatic right medial occipital hypodensity responsible for the patient’s left homonymous hemianopsia. F, Noncontrast head computed tomography showing bilateral basal ganglia calcifications, which can be seen in a number of inherited, infectious, toxic, or metabolic conditions.

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