Progressive Bilateral Visual and Hearing Loss in an Elderly Woman

Abstract

Dr. Vaphiades:A 63-year-old white woman with a history of previously treated tuberculosis and a 10-year history of bilateral hearing loss presented with a complaint of progressive visual loss in both eyes over the last 2 years. She denied tobacco, alcohol, or drug abuse. At the time of initial vision loss, she had been found to have a swollen right optic disc; however, 2 weeks later, she was noted to have bilateral optic disc swelling. Initial brain and orbital MRI was interpreted as showing no abnormalities. Repeat MRI 9 months later showed marked bilateral optic nerve enhancement. The patient was treated with high-dose corticosteroids without improvement. In the meantime, a variety of serologic and blood studies were performed, including complete blood count, erythrocyte sedimentation rate, C-reactive protein, Lyme, Bartonella, Brucella, Toxoplasma, rickettsial, and antinuclear antibody titers, serology for syphilis, and testing for human immunodeficiency virus. All gave negative or normal results. A lumbar puncture revealed no abnormalities other than 10 white blood cells, all monocytes. The cerebrospinal fluid (CSF) glucose was 73 mg/dL (normal: 40-80 mg/dL), and the CSF protein was 34 mg/dL (normal: 15-45 mg/dL). The fluid was negative for cryptococcal antibody, there were no oligoclonal bands, and cultures for bacteria, including acid-fast bacteria, showed no growth. Cytology was negative for malignancy but showed small mature lymphocytes. CT of the chest and a gallium scan showed no lesions. Bilateral temporal artery biopsies showed no evidence of vasculitis. The patient was referred to our institute for an assessment.On examination, the patient's vital signs were normal. Her visual acuity was light perception, right eye, and no light perception, left eye. Color vision was absent on the right. The pupils measured 7 mm with the right pupil minimally reactive to direct light stimulation, and the left was nonreactive. There was a left relative afferent pupillary defect. Extraocular movements were full, but there was a 40-prism diopter comitant right exotropia by Krimsky testing. There was no proptosis. Slit-lamp examination revealed normal corneas, anterior segments, and intraocular pressures. The ocular fundi showed bilateral optic disc pallor. There was no vascular sheathing, and there were no retinal hemorrhages or exudates. Trigeminal and facial nerve function were normal bilaterally.The patient was treated with another course of systemic steroids without improvement in vision. In the meantime, further blood tests were ordered, including tests for the common Leber hereditary optic neuropathy mitochondrial mutations and assays for collapsin response mediator protein-5, angiotensin-converting enzyme (ACE), and aquaporin-4 antibody. All gave negative or normal results. A third brain and orbital MRI was performed (Figs. 1-3).Section Editor: Neil R. Miller, MD FIG. 1. Fat-suppressed gadolinium-enhanced T1 axial (A) and coronal (B) MRI of the orbits demonstrate uniform thickening and enhancement of both optic n...