From the Guidelines
Otosyphilis is characterized by sudden unilateral or bilateral sensorineural fluctuating hearing loss, tinnitus, and/or vertigo, with vertigo attacks not typically associated with aural symptoms immediately before or after attacks. The key features of otosyphilis include sudden or progressive sensorineural hearing loss, vestibular symptoms such as vertigo and disequilibrium, and tinnitus 1. Patients may experience fluctuating hearing loss that can progress to permanent deafness if left untreated. Otosyphilis typically occurs during the late stages of syphilis infection, often years after the initial infection.
Diagnosis and Treatment
Diagnosis requires both serologic evidence of syphilis (positive VDRL/RPR and treponemal-specific tests) and otologic symptoms that cannot be attributed to other causes. Audiometric testing typically reveals sensorineural hearing loss, and patients may have abnormal vestibular function tests. Treatment consists of intravenous penicillin G, and adjunctive corticosteroids are often added to prevent inflammatory response to dying spirochetes 1.
Clinical Presentation
The clinical presentation of otosyphilis can be variable, but it often includes a combination of hearing loss, tinnitus, and vertigo. The vertigo attacks in otosyphilis are not typically associated with aural symptoms immediately before or after attacks, which can help distinguish it from other causes of vertigo such as Ménière's disease 1.
Importance of Early Treatment
Early treatment is crucial as hearing loss may become permanent despite therapy. The pathophysiology involves direct invasion of the inner ear by Treponema pallidum spirochetes or immune-mediated damage to cochlear structures. All patients who have syphilis should be tested for HIV, and many specialists recommend treating patients who have evidence of auditory disease caused by syphilis in the same manner as patients who have neurosyphilis, regardless of CSF examination results 1.
From the Research
Features of Otosyphilis
- Otosyphilis is a less recognized complication of syphilis that can lead to irreversible sensorineural hearing loss 2
- It can cause sudden or fluctuating sensorineural hearing loss, tinnitus, and vertigo 2, 3, 4
- The condition can lead to asymmetric sensorineural hearing loss, as seen in some cases 5, 3
- Patients with otosyphilis may experience unilateral hearing loss, tinnitus, headache, and nausea 3
- Otosyphilis can be associated with neurosyphilis, as indicated by cerebrospinal fluid (CSF) analysis 3
Diagnosis and Treatment
- A high index of suspicion is needed for an accurate diagnosis of otosyphilis 5
- Patients with syphilis should be screened routinely for hearing loss, and all patients with new, sudden, or fluctuating sensorineural hearing loss should be evaluated for syphilis 2
- Treatment with intravenous penicillin can lead to complete recovery of hearing in some cases 5
- Oral doxycycline may be an effective alternate regimen for otosyphilis, with a response rate of 47.3% in one study 6
- Treatment with penicillin and corticosteroids has achieved improvement in hearing, tinnitus, and vertigo in some patients, but not in all 4