Progressive visual and hearing loss secondary to neurosyphilis

There is a rising incidence of syphilis in economically advanced countries. Early diagnosis can spare the patient devastating vision and hearing loss, as well as other significant morbidity. Ocular presentations of neurosyphilis are varied and numerous, requiring syphilis to be considered in many clinical situations, even if a negative medical history is reported. Features of Argyll Robertson pupils, however, are virtually pathognomonic of syphilis; therefore, careful pupil testing can be the key to making a correct and timely diagnosis.

A 51-year-old woman with a history of unexplained hearing loss presented for a third opinion for progressive vision loss OU, which was previously labeled as functional loss. A review of records indicated significant worsening of visual acuity and visual fields over 6 months, with minimal remaining visual function. She exhibited features of optic neuropathy as well as 2 mm miotic pupils, with evident light-near dissociation bilaterally. Evaluation showed a reactive fluorescent treponemal antibody absorption and rapid plasma reagin (1:8 titer) and positive cerebral spinal fluid venereal disease research laboratory test. A diagnosis of neurosyphilis was made, and a 2-week course of intravenous penicillin treatment was completed. Despite treatment, her vision decreased to no light perception OU owing to the advanced course of the disease at the time of treatment.

Neurosyphilis can occur at any stage of infection and needs to be considered in the differential diagnosis for many visual and ocular problems, especially in the setting of other systemic signs and symptoms. In this case, the combination of light-near dissociation and optic neuropathy with hearing loss was instrumental in dismissing the past diagnosis of functional vision loss and in pursuing the diagnosis of neurosyphilis. A timely diagnosis of neurosyphilis needs to be made to prevent devastating vision loss as seen in this case.