Eur Arch Otorhinolaryngol (2005) 262: 972–974 DOI 10.1007/s00405-005-0934-1
O TO L OG Y
Jae Jun Song Æ Heung-Man Lee Æ Sung Won Chae Soon Jae Hwang
Bilateral otosyphilis in a patient with HIV infection
Received: 26 August 2004 / Accepted: 10 January 2005 / Published online: 21 June 2005 Ó Springer-Verlag 2005
Abstract Otosyphilis is a rare but important cause of sensorineural hearing loss and dizziness, because this hearing loss can be reversed by early diagnosis and aggressive treatment. Moreover, HIV may alter the course of otosyphilis and hasten the development of otosyphilis by reducing host cellular immunity. We report the case of a 35-year-old HIV-infected patient with bilateral fluctuating sensorineural hearing loss and bilateral total vestibular loss caused by otosyphilis. We include a discussion of the relationship between otosyphilis and HIV infection. Keywords HIV Æ AIDS Æ Otosyphilis Æ Bilateral vestibulopathy
Introduction HIV infection is closely related with head and neck manifestations [10], and otosyphilis is a rare but important otologic comorbidity in HIV infection. Syphilis used to be a common cause of progressive or sudden sensorineural hearing loss, but its incidence in the general population decreased markedly after the introduction of penicillin. However, its incidence is now increasing in parallel with HIV infection. Many studies have substantiated the aggressive and rapidly progressive behavior of neurosyphilis, including otosyphilis, in the presence of HIV infection [5, 14]. We report the case of 35-year-old HIV-infected patient with bilateral fluctuating sensorineural hearing loss and bilateral total vestibular loss caused by otosyphilis, which was resistant to treatment. J. J. Song Æ H.-M. Lee Æ S. W. Chae Æ S. J. Hwang (&) Department of Otorhinolaryngology and Head and Neck Surgery, Korea University College of Medicine Guro Hospital, 80 Guro-dong Guro-gu, 152-703 Seoul, Korea E-mail:
[email protected] Tel.: +82-2-8186750 Fax: +82-2-8680475
Case report A 35-year-old homosexual man was referred for bilateral progressive hearing loss and dizziness. Six months previously, he had been diagnosed as a HIV carrier, and 4 months previously, he had been treated for secondary syphilis with a 3-week course of benzathine penicillin G of 2.4 million units per week by intramuscular injection. A neurotologic examination revealed moderately severe sensorineural hearing loss of the right ear and moderate sensorineural hearing loss of the left ear (Fig. 1). A caloric test revealed right side canal paresis. Other neurologic tests and imaging studies were normal. After consultation with internal medicine, he was treated with half-dose oral steroid for 2 weeks. His decreased hearing acuity was unchanged, but his dizziness improved. At a visit 2 months later, he complained of worsened imbalance, difficulty in recognizing peoples faces during head movement and an inability to read road signs while driving, which was improved by slowing down. A neurotologic examination revealed bilateral sensorineural hearing loss and bilateral total vestibular function loss (Fig. 2). The serum venereal disease research laboratory (VDRL) titer showed elevation versus the previous test. However, other viral marker tests were negative. Spinal tapping revealed increased protein and reactive fluorescent treponemal antibody absorption (FTA-ABS test). He was given a 10-day course of penicillin G of 24 million units by intravenous injection daily and three injections of intramuscular beszathine penicillin of 2.4 million units once a week for 3 weeks. After 6 months, his hearing was partially improved (Fig. 3), but mild imbalance persisted despite vestibular rehabilitation.
Discussion Patients with HIV infection or AIDS frequently have manifestations of the head and neck. Marcusen and Sooy showed a 41% incidence of head and neck
973 Fig. 1 Initial audiogram presenting right moderately severe and left moderate sensorineural hearing loss
manifestations, and a near 100% incidence of head and neck medical problems in patients with AIDS [10]. Syphilis was once a common disease before the development of penicillin, and otosyphilis was a common syphilitic manifestation. Following the development of penicillin, otosyphilis became rare. However, the incidence of syphilis is increasing among HIV patients. Through a literature review, Morris and Prasad listed otosyphilis among other causes of otologic disease in AIDS patients [12]. Another study found that 1.5% of hospitalized AIDS patients had neurosyphilis [6]. Smith and Canalis proposed that, after infection, HIV may alter the course of latent syphilis and hasten the development of otosyphilis [14]. Otosyphilis is usually diagnosed by various cochleovestibular symptoms, a positive serological test and the exclusion of other causes. Symptoms in the otosyphilis range from acute or fluctuating hearing loss, tinnitus, vertigo, dizziness and/or unsteadiness [4, 7, 13]. Hearing loss is unilateral or bilateral and sensorineural in type; it usually progresses rapidly and may occasionally have a sudden onset. The audiometric curve often demonstrates low frequency involvement suggestive of endolymphatic hydrops [14]. Basic screening includes venereal disease research laboratory (VDRL), Treponema pallidum microhemagFig. 2 Slow harmonic acceleration test showing a phase lead and a subsequent decrease in gain
glutinin assays (TPHA) and fluorescent treponemal antibody absorption (FTA-ABS) [16]. VDRL is a nontreponemal test that relies on the presence of antibodies that cross-react with antigens expressed on bovine cardiolipin, and has a high false positive rate among patients with collagen vascular disease. Serum fluorescent treponemal antibody absorption (FTA-ABS) or Treponema pallidum microhemagglutinin assays (TPHA) are more specific, but cannot discriminate between active and treated disease and may remain positive for life despite adequate treatment [1, 2]. Although the FTA-ABS test is often considered the ‘‘gold standard’’ confirmatory test, its sensitivity is slightly lower than that of certain other treponemal antigen tests, and serial quantitative cardiolipin antigen tests remain the method of choice for monitoring the efficacy of treatment [16]. In the current patient, disease activity and response to treatment were evaluated by serial VDRL titer tests. Early treatment is mandatory for an excellent outcome. The treatment of choice is penicillin. Benzathine penicillin G of 2.4 million units intramuscularly weekly for 3 weeks was considered curative. However, studies have shown that treatment with intramuscular penicillin fails to obtain treponemicidal levels in cerebrospinal fluid [11, 15]. In the current patient, the otologic symptom
974 Fig. 3 Posttreatment audiogram showing improved right sensorineural hearing loss with persistent high tone loss
aggravated rapidly over 3 months in spite of intramuscular penicillin treatment. Intravenous penicillin was used since it has been shown to achieve treponemicidal levels in the cerebrospinal fluid in patients with neurosyphilis of otosyphilis [3]. Benzathine penicillin G 24 million units per day by intravenous injection for 2 weeks followed by benzathine penicillin G 2.4 million units intramuscularly per week for 3 weeks was considered curative [2]. Moreover, the combination of penicillin with steroids is widely used [8]. However, their use in HIV-infected patients is generally contraindicated because further immunosuppression carries a risk of lethal opportunistic infections and neoplasia [9]. At the recommendation of virologists, the dose and duration of steroid treatment were reduced in this patient. The result of treatment is variable, and complete remission is not possible in all patients. In a study by Linstrom involving 18 patients with otosyphilis, tinnitus decreased in 71% and disequilibrium improved in 66%, but hearing improved in only 25% of those with hearing loss [8]. Otosyphilis may cause severe otologic sequelae, especially in HIV infected patients. Because of decreased cellular immunity, the clinical course of syphilis with HIV infection is more aggressive and resistant to treatment. Otosyphilis should always be considered in HIV seropositive patients presenting with otologic complaints, and optimal treatment should be investigated through additional clinical and temporal bone studies.
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