Late Syphilitic Hearing Loss: A Diagnostic and Therapeutic Dilemma

Abstract

Cochleovestibular dysfunction may occur as the only manifestation of late syphilis. It may occur singly, in combination, and be unilateral or bilateral. A presumptive diagnosis may be made on the basis of excluding other known etiologies of inner ear dysfunction and on a positive serological test for syphilis. The combined experience of the author and a literature review suggests that a combination of penicillin and prednisone is the treatment of choice. Since no controlled clinical trials have been reported, antibiotic and corticosteroid dosage, timing and route of administration are conjectural. All patients with a presumptive diagnosis of late otitic syphilis should be treated regardless of previous syphilitic treatment. Duration of symptoms and magnitude of functional loss are not contraindications. Penicillin is the antibiotic of choice and the minimal treatment should be the United States Public Health Service schedule for late syphilis, i.e., benzathine penicillin G, 2.4 million units by intramuscular injection weekly for three successive weeks. Since penicillin acts on dividing cells only and since treponemes probably divide very slowly in late syphilis, prolonged penicillinemia is desirable. This may be maintained with injectable depo‐penicillin (benzathine penicillin G) or penicillin by mouth, depending upon patient compliance. Duration of treatment of one year is suggested. Prednisone, 40 to 60 mgm/day by mouth is started and continued for at least two weeks. If symptomatic improvement occurs the dose is tapered to a level which will maintain this improvement.

Recent Trends in the Serologic Diagnosis of Syphilis

“…It should be noted, however, that the results of a CSF examination are frequently normal in patients with auditory involvement (78). Whenever the eye or ear or both are involved, the patient case is managed as a neurosyphilis infection regardless of CSF findings (76).…”

Neurosyphilis: A current review

“…The hearing loss is sensorineural and often unilateral. Sensorineural deafness has been attributed to syphilis infection, particularly when other causes have been excluded and there is positive serologic evidence of syphilis infection [17]. CSF examination is usually normal in these patients [17].…”

“…Syphilitic otitis is a more nebulous entity. It is thought to occur when the otic branch of cranial nerve VIII is involved in infection; sudden onset (over a period of weeks) of hearing loss is the usual presentation [14,17]. The hearing loss is sensorineural and often unilateral.…”

Otoneurosyphilis Masquerading as Neurofibromatosis Type II

“…This may be fluctuating but usually progressive over time. Conductive deafness may result from osteitic lesions of ossicles [11]. Vestibular involvement may present as acute labyrinthitis.…”