Timothy C. Hain, MD Return to Index. Page last modified: November 24, 2011
Syphilis is presently uncommon in the United States although it is making a comeback in some populations (persons with HIV infection). Syphilis is caused by infection with Treponema Pallidum, a spirochete. It usually is spread through contact with infectious lesions or body fluids, and generally through sexual contact.
About 21 days after exposure, patients develop a skin lesion called a chancre at the site of exposure. These are often on the sexual organs. Syphilitic chancres are typically nontender, hard, non-purulent ulcers. The chancres typically heal without therapy.
Secondary syphilis begins 4-10 weeks after the chancre. A rash is the main complaint. It often affects the hands and or soles. The rash is rarely vesicular (blistering). There are many less specific findings such as sore throat, headache and the like. Secondary syphilis can cause neurologic, renal, ophthalmologic, gastrointestinal and hepatic disease. Secondary syphilis again, typically resolves without treatment.
There may then be a latent period, with reappearance of symptoms late in the course. In Tertiary syphilis, patients may develop cardiovascular symptoms (such as aortitis), gumma's, and neurosyphilis. Neurosyphilis may include general paresis (a type of dementia) and tabes dorsalis (a spinal cord disease)
Otosyphilis can closely resemble Meniere's disease, perilymph fistula, sudden hearing loss, autoimmune inner ear disease, and bilateral vestibular loss. Because the inner ear communicates with spinal fluid via the cochlear aqueduct, otosyphilis is a variety of neurosyphilis and neurological symptoms are also possible.
Early neurosyphilis mainly presents as meningitis with or without cranial nerve involvement and meningovascular disease or stroke. Hearing symptoms of early neurosyphilis might be a sudden hearing loss.
Late neurosyphilis may affect the brain (general paresis), or spinal cord (tabes dorsalis). In the ear, late neurosyphilis may present as hearing loss, fluctuating hearing, or vestibular imbalance/weakness (vertigo).
There are several methods of diagnosing syphilis. The non-treponemal serologic tests are the RPR (rapid plasma reagin) and VDRL. Treponemal tests include FTA-abs (Fluorescent treponemal antibodies).
False positives occur in 1-2% of the US population, and are associated with autoimmune diseases, other infections, and IV drug use.
False negative tests can appear in persons with very high titer RPR or VDRL tests (the titer is too high to measure) as well in immunosuppressed people.
Although conventional teaching is that RPR/VDRL tests go negative after treatment, and FTA tests persist forever, there are numerous exceptions to this general rule.
Neurosyphilis and otosyphilis are diagnosed by CSF pleocytosis (increased cell count) or elevated spinal fluid protein, reactive CSF RPR, or identification of T-pallidum in CSF by PCR or by infectivity in rabbits.
Many patients with syphilis have HIV and testing for this should be considered in a person with syphilis. Testing for other STD's such as gonorrhea and chlamydia is also recommended.
The treatment of otosyphilis is the same as the treatment of neurosyphilis.
The CDC recommends aqueous penicillin G, 18-24 million units/day administered as 3-4 million units IV every 4 hours or continuously, for 10-14 days.
Alternatively, procaine penicillin 2.4 million units may be given IM daily plus probenicid 500 mg by mouth, 4 times/day, both for 10-14 days.
Persons with non-life threatening allergies to PCN should ideally be desensitized.
According to Goldman (2003), all patients require clinical and blood testing followup 6 and 12 months after treatment. Patients with HIV should also be evaluated at 3, 9 and 24 months after treatment. Treatment failure is defined as failure of RPR/VDRL to decline by at least 2 dilutions within 6 months of treatment, or a sustained 4-fold increase in RPR/VDRL at some point after treatment. If treatment failure is not noted, all patients with otosyphilis should undergo repeat CSF examinations at 3 and 6 months following therapy and then every 6 months until CSF is normal.
In the study of Gleich et al, hearing responded in only about 35%. (1992). Vertigo improved to a much greater extent. Hearing may respond initially but relapse (Dobbin and Perkins, 1983). In congenital syphilis, hearing may not show any lasting improvement.
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