Otology & Neurotology 35:e90Ye91 Ó 2014, Otology & Neurotology, Inc.
Imaging Case of the Month
Bilateral Temporal Bone Otosyphilis *†‡Ilkka Kiveka¨s, †‡Juha-Pekka Vasama, and §Jari Hakoma¨ki *Boston Children’s Hospital, Department of Otolaryngology, Harvard Medical School, Boston, Massachusetts, U.S.A.; ÞDepartments of Otorhinolaryngology, Tampere University Hospital, and þDepartment of Otorhinolaryngology, University of Tampere; and §Department of Radiology, Tampere University Hospital, Tampere, Finland
The incidence of syphilis has increased during the past decade in the United States and many European countries
(1,2). Congenital syphilis may be associated with a latent infection without specific signs or symptoms. Typical
FIG. 1. Axial HRCT of temporal bones (A), where symmetric chronic syphilitic osteitis is visible in both otic capsules (arrows). Axial (B, C) and coronal (D) HRCT scans of the left temporal bone showing chronic osteitis of otic capsule. There is ‘‘moth-eaten’’ permeative demineralization (arrows) typical for otosyphilis.
findings in late-onset syphilis may include rapidly progressive or even sudden bilateral hearing loss, ocular changes, dental deformities, and pathologic bone alterations (3). Hearing loss is seen in 3% to 38% of cases with congenital syphilis (4) and may be due to endolymphatic hydrops (5). Typical otopathologic findings of otosyphilis are inflammatory resorptive osteitis of temporal bone and
Address correspondence and reprint requests to Ilkka Kiveka¨s, M.D., Ph.D., Department of Otolaryngology, Boston Children’s Hospital, Harvard Medical School, Boston, Massachusetts; E-mail: ilkka.kivekas@ childrens.harvard.edu Conflicts of Interest and Source of Funding: The authors have no conflicts of interest to disclose. I. K. has received grants for postdoctoral Research Fellow year from the Jenny and Antti Wihuri Foundation and the Finnish Otolaryngology Foundation.
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Copyright © 2014 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
BILATERAL TEMPORAL BONE OTOSYPHILIS atrophy of organ of Corti, spiral ganglion, and stria vascularis (5). Skull, sternal, clavicular, tibial, fibular, ulnar, and radial bones are most commonly affected, and typical pathologic findings are gummosa tumors, osteomyelitis, and periosteitis. This imaging case describes bilateral temporal bone changes because of congenital syphilis. Our patient is a 75-year-old woman, whose hearing started to deteriorate at the age of 10 years, and her hearing aid rehabilitation began at the age of 26 years. Chronic interstitial keratitis in the 1950s led to severe visual impairment and syphilis diagnosis. She eventually developed profound bilateral sensorineural hearing loss. High-resolution computed tomography of the temporal bones revealed demineralization of the otic capsules bilaterally and no ossicular involvement (Fig. 1). She underwent uneventful right ear cochlear implant surgery in 2007 with full electrode insertion via cochleostomy approach. Her open set bisyllabic word scores in Finnish are 76% on her most recent testing with the implanted ear only. Typical high-resolution computed tomographic findings in otosyphilis are osteomyelitis of the otic capsule and ‘‘moth-eaten’’ permeative demineralization of the temporal bone or rarely bony erosion in ossicles (6). Obliterative endarteritis and mononuclear infiltration are both
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seen in temporal bone osteitis, compromising the vascular supply and leading to varying degrees of bony necrosis. The differential diagnosis includes otosclerosis, Paget‘s disease, and osteogenesis imperfecta (6). Labyrinthitis and internal auditory canal meningoneuritis can be present with otosyphilis. Congenital syphilis is a rare condition that should be included in the differential diagnosis of the demineralized or ossified inner ear. Diagnosis should be confirmed by either serology or study of the cerebrospinal fluid. REFERENCES 1. Centers for Disease Control and Prevention (CDC). Congenital syphilisVUnited States, 2003Y2008. MMWR Morb Mortal Wkly Rep 2010;59:413Y7. 2. Simms I, Broutet N. Congenital syphilis re-emerging. J Dtsch Dermatol Ges 2008;6:269Y72. 3. Mayer O, Fraser J. Pathological changes in the ear in late congenital syphilis. J Laryngol Otol 1936;51:683Y714. 4. Darmstadt GL, Harris JP. Luetic hearing loss: clinical presentation, diagnosis, and treatment. Am J Otolaryngol 1989;10:410Y21. 5. Miller ME, Makary C, Lopez IA, Ishiyama A. Endolymphatic hydrops in otologic syphilis: a temporal bone study. Otol Neurotol 2010; 31:681Y6. 6. Sonne JE, Zeifer B, Linstrom C. Manifestations of otosyphilis as visualized with computed tomography. Otol Neurotol 2002;23:806Y7.
Otology & Neurotology, Vol. 35, No. 2, 2014
Copyright © 2014 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
Imaging Case of the Month
Bilateral Temporal Bone Otosyphilis *†‡Ilkka Kiveka¨s, †‡Juha-Pekka Vasama, and §Jari Hakoma¨ki *Boston Children’s Hospital, Department of Otolaryngology, Harvard Medical School, Boston, Massachusetts, U.S.A.; ÞDepartments of Otorhinolaryngology, Tampere University Hospital, and þDepartment of Otorhinolaryngology, University of Tampere; and §Department of Radiology, Tampere University Hospital, Tampere, Finland
The incidence of syphilis has increased during the past decade in the United States and many European countries
(1,2). Congenital syphilis may be associated with a latent infection without specific signs or symptoms. Typical
FIG. 1. Axial HRCT of temporal bones (A), where symmetric chronic syphilitic osteitis is visible in both otic capsules (arrows). Axial (B, C) and coronal (D) HRCT scans of the left temporal bone showing chronic osteitis of otic capsule. There is ‘‘moth-eaten’’ permeative demineralization (arrows) typical for otosyphilis.
findings in late-onset syphilis may include rapidly progressive or even sudden bilateral hearing loss, ocular changes, dental deformities, and pathologic bone alterations (3). Hearing loss is seen in 3% to 38% of cases with congenital syphilis (4) and may be due to endolymphatic hydrops (5). Typical otopathologic findings of otosyphilis are inflammatory resorptive osteitis of temporal bone and
Address correspondence and reprint requests to Ilkka Kiveka¨s, M.D., Ph.D., Department of Otolaryngology, Boston Children’s Hospital, Harvard Medical School, Boston, Massachusetts; E-mail: ilkka.kivekas@ childrens.harvard.edu Conflicts of Interest and Source of Funding: The authors have no conflicts of interest to disclose. I. K. has received grants for postdoctoral Research Fellow year from the Jenny and Antti Wihuri Foundation and the Finnish Otolaryngology Foundation.
e90
Copyright © 2014 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
BILATERAL TEMPORAL BONE OTOSYPHILIS atrophy of organ of Corti, spiral ganglion, and stria vascularis (5). Skull, sternal, clavicular, tibial, fibular, ulnar, and radial bones are most commonly affected, and typical pathologic findings are gummosa tumors, osteomyelitis, and periosteitis. This imaging case describes bilateral temporal bone changes because of congenital syphilis. Our patient is a 75-year-old woman, whose hearing started to deteriorate at the age of 10 years, and her hearing aid rehabilitation began at the age of 26 years. Chronic interstitial keratitis in the 1950s led to severe visual impairment and syphilis diagnosis. She eventually developed profound bilateral sensorineural hearing loss. High-resolution computed tomography of the temporal bones revealed demineralization of the otic capsules bilaterally and no ossicular involvement (Fig. 1). She underwent uneventful right ear cochlear implant surgery in 2007 with full electrode insertion via cochleostomy approach. Her open set bisyllabic word scores in Finnish are 76% on her most recent testing with the implanted ear only. Typical high-resolution computed tomographic findings in otosyphilis are osteomyelitis of the otic capsule and ‘‘moth-eaten’’ permeative demineralization of the temporal bone or rarely bony erosion in ossicles (6). Obliterative endarteritis and mononuclear infiltration are both
e91
seen in temporal bone osteitis, compromising the vascular supply and leading to varying degrees of bony necrosis. The differential diagnosis includes otosclerosis, Paget‘s disease, and osteogenesis imperfecta (6). Labyrinthitis and internal auditory canal meningoneuritis can be present with otosyphilis. Congenital syphilis is a rare condition that should be included in the differential diagnosis of the demineralized or ossified inner ear. Diagnosis should be confirmed by either serology or study of the cerebrospinal fluid. REFERENCES 1. Centers for Disease Control and Prevention (CDC). Congenital syphilisVUnited States, 2003Y2008. MMWR Morb Mortal Wkly Rep 2010;59:413Y7. 2. Simms I, Broutet N. Congenital syphilis re-emerging. J Dtsch Dermatol Ges 2008;6:269Y72. 3. Mayer O, Fraser J. Pathological changes in the ear in late congenital syphilis. J Laryngol Otol 1936;51:683Y714. 4. Darmstadt GL, Harris JP. Luetic hearing loss: clinical presentation, diagnosis, and treatment. Am J Otolaryngol 1989;10:410Y21. 5. Miller ME, Makary C, Lopez IA, Ishiyama A. Endolymphatic hydrops in otologic syphilis: a temporal bone study. Otol Neurotol 2010; 31:681Y6. 6. Sonne JE, Zeifer B, Linstrom C. Manifestations of otosyphilis as visualized with computed tomography. Otol Neurotol 2002;23:806Y7.
Otology & Neurotology, Vol. 35, No. 2, 2014
Copyright © 2014 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
