Otology & Neurotology 37:e187–e188 ß 2014, Otology & Neurotology, Inc.
Imaging Case of the Month
Inner Ear Inflammatory Pseudotumor With Middle Ear Cholesteatoma Hyo Vin Jung, yJin Woo Choi, Jung Eun Shin, and Chang-Hee Kim Departments of Otorhinolaryngology–Head and Neck Surgery; and yRadiology, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul, Republic of Korea
Inflammatory pseudotumor is a histologically benign and locally destructive mass lesion that is composed of fibrovascular tissue mixed with chronic inflammatory cell infiltration (1). A consensus has not been reached regarding etiology, but chronic and post–infectious inflammatory processes are generally considered important pathophysiologic correlates. Although inflammatory pseudotumor involving the temporal bone is extremely rare, this condition has an aggressive nature, such as rapid growth and bony destruction, in cases of temporal bone involvement (1–3). This imaging case study describes an inflammatory pseudotumor involving the otic capsule and internal auditory canal on the left side and bilateral middle ear cholesteatomas. A 59-year-old woman presented with progressive hearing loss and otorrhea on both sides with a duration of 8 years. The patient did not complain of vertigo or facial paralysis. Otoscopic examination showed attic destruction with bilateral automastoidectomy. Pure-tone audiometry revealed profound hearing loss in both ears. High-resolution computed tomography (CT) of the temporal bones revealed soft tissue density in the middle ear and mastoid cavity with severe bony destruction, creating a single large cavity on both sides (Fig. 1, A and B). The inner ear structures within the otic capsule showed an unusual centrifugally expansile appearance in the left ear. The cochlea was dilated and cystic, and the vestibular organs were enlarged and
dehiscent into the middle ear. Magnetic resonance imaging (MRI) showed contrast-enhancing soft tissue intensity in the internal auditory canal, cochlea, vestibular organs, and part of the middle ear cavity (Fig. 1, C and D). Nonenhancing soft tissue intensity suggestive of a cholesteatoma was observed in both the middle ear and mastoid cavity. The patient underwent tympanomastoidectomy on the right side and mass removal via a modified transotic approach on the left side. The histologic diagnosis of the left inner ear mass was consistent with an inflammatory pseudotumor. Patients with an inflammatory pseudotumor involving the inner ear structures may manifest all of the possible otologic symptoms, such as otalgia, hearing loss, vertigo, and facial palsy (1–3). Making a diagnosis of inner ear inflammatory pseudotumor may be challenging because of the rarity of this condition. The differential diagnoses include a cholesteatoma invading the inner ear structures; granulomatous diseases such as Wegener’s granulomatosis; neoplasms such as hemangioma, facial neuroma, meningioma, and paraganglioma; and tuberculosis (4). All of the patients with an inner ear inflammatory pseudotumor in previous reports (1,2) and the patient in this report had concomitant acute or chronic otitis media (with or without cholesteatoma), and temporal bone imaging showed characteristic findings. The typical high-resolution CT findings are dilatation of the inner ear structures and centrifugal expansion of the otic capsule, resulting in focal dehiscence into the middle ear cavity. On MRI, an inflammatory pseudotumor shows iso- or low-signal intensity on T2-weighted images and homogeneous contrast enhancement on T1-weighted images (5). An inner ear inflammatory pseudotumor is an extremely rare condition, and the typical changes of the inner ear structures found from CT and MRI, especially in patients with chronic middle ear infection, may be pathognomonic.
Address correspondence and reprint requests to Chang-Hee Kim, M.D., Ph.D., Department of Otorhinolaryngology–Head and Neck Surgery, Konkuk University Medical Center, Konkuk University School of Medicine 120-1 Neungdong-ro (Hwayang-dong), Gwangjin-gu, Seoul, Republic of Korea 143-729; E-mail: [email protected] This research was supported by the Basic Science Research Program through the National Research Foundation of Korea funded by the Ministry of Education, Science and Technology (2012R1A1A2044883). The authors disclose no conflicts of interest.
e187
Copyright © 2016 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
e188
H. V. JUNG ET AL.
FIG. 1. The axial images of high-resolution CTof the temporal bone (A, B) demonstrate a large soft tissue density filling the middle ear and mastoid cavity, with visible severe bony destruction in both ears (arrowheads). The left internal auditory canal is enlarged (thin arrow), and the vestibules and horizontal semicircular canal have an expansile appearance, with focal dehiscence into the middle ear cavity (dotted arrow). The cochlea is widened on the left side (thick arrow). The axial image of T2-weighted MRI shows that the pathologic soft tissue, which is isointense with the brain, is filling the internal auditory canal, cochlea, vestibule, and horizontal and posterior semicircular canals (C, split arrows). The contrast-enhanced T1-weighted axial image shows a moderately enhancing mass lesion filling the abovementioned inner ear structures (D, dotted split arrows).
REFERENCES 1. Curry JM, King N, O’Reilly RC, et al. Inflammatory pseudotumor of the inner ear: are computed tomography changes pathognomonic? Laryngoscope 2010;120:1252–5. 2. Mulder JJ, Cremers WR, Joosten F, et al. Fibroinflammatory pseudotumor of the ear. A locally destructive benign lesion. Arch Otolaryngol Head Neck Surg 1995;121:930–3.
3. Williamson RA, Paueksakon P, Coker NJ. Inflammatory pseudotumor of the temporal bone. Otol Neurotol 2003;24:818–22. 4. Schonermark MP, Issing P, Stover T, et al. Fibroinflammatory pseudotumor of the temporal bone. Skull Base Surg 1998;8: 45–50. 5. Park SB, Lee JH, Weon YC. Imaging findings of head and neck inflammatory pseudotumor. AJR Am J Roentgenol 2009;193: 1180–6.
Otology & Neurotology, Vol. 37, No. 6, 2016
Copyright © 2016 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
Imaging Case of the Month
Inner Ear Inflammatory Pseudotumor With Middle Ear Cholesteatoma Hyo Vin Jung, yJin Woo Choi, Jung Eun Shin, and Chang-Hee Kim Departments of Otorhinolaryngology–Head and Neck Surgery; and yRadiology, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul, Republic of Korea
Inflammatory pseudotumor is a histologically benign and locally destructive mass lesion that is composed of fibrovascular tissue mixed with chronic inflammatory cell infiltration (1). A consensus has not been reached regarding etiology, but chronic and post–infectious inflammatory processes are generally considered important pathophysiologic correlates. Although inflammatory pseudotumor involving the temporal bone is extremely rare, this condition has an aggressive nature, such as rapid growth and bony destruction, in cases of temporal bone involvement (1–3). This imaging case study describes an inflammatory pseudotumor involving the otic capsule and internal auditory canal on the left side and bilateral middle ear cholesteatomas. A 59-year-old woman presented with progressive hearing loss and otorrhea on both sides with a duration of 8 years. The patient did not complain of vertigo or facial paralysis. Otoscopic examination showed attic destruction with bilateral automastoidectomy. Pure-tone audiometry revealed profound hearing loss in both ears. High-resolution computed tomography (CT) of the temporal bones revealed soft tissue density in the middle ear and mastoid cavity with severe bony destruction, creating a single large cavity on both sides (Fig. 1, A and B). The inner ear structures within the otic capsule showed an unusual centrifugally expansile appearance in the left ear. The cochlea was dilated and cystic, and the vestibular organs were enlarged and
dehiscent into the middle ear. Magnetic resonance imaging (MRI) showed contrast-enhancing soft tissue intensity in the internal auditory canal, cochlea, vestibular organs, and part of the middle ear cavity (Fig. 1, C and D). Nonenhancing soft tissue intensity suggestive of a cholesteatoma was observed in both the middle ear and mastoid cavity. The patient underwent tympanomastoidectomy on the right side and mass removal via a modified transotic approach on the left side. The histologic diagnosis of the left inner ear mass was consistent with an inflammatory pseudotumor. Patients with an inflammatory pseudotumor involving the inner ear structures may manifest all of the possible otologic symptoms, such as otalgia, hearing loss, vertigo, and facial palsy (1–3). Making a diagnosis of inner ear inflammatory pseudotumor may be challenging because of the rarity of this condition. The differential diagnoses include a cholesteatoma invading the inner ear structures; granulomatous diseases such as Wegener’s granulomatosis; neoplasms such as hemangioma, facial neuroma, meningioma, and paraganglioma; and tuberculosis (4). All of the patients with an inner ear inflammatory pseudotumor in previous reports (1,2) and the patient in this report had concomitant acute or chronic otitis media (with or without cholesteatoma), and temporal bone imaging showed characteristic findings. The typical high-resolution CT findings are dilatation of the inner ear structures and centrifugal expansion of the otic capsule, resulting in focal dehiscence into the middle ear cavity. On MRI, an inflammatory pseudotumor shows iso- or low-signal intensity on T2-weighted images and homogeneous contrast enhancement on T1-weighted images (5). An inner ear inflammatory pseudotumor is an extremely rare condition, and the typical changes of the inner ear structures found from CT and MRI, especially in patients with chronic middle ear infection, may be pathognomonic.
Address correspondence and reprint requests to Chang-Hee Kim, M.D., Ph.D., Department of Otorhinolaryngology–Head and Neck Surgery, Konkuk University Medical Center, Konkuk University School of Medicine 120-1 Neungdong-ro (Hwayang-dong), Gwangjin-gu, Seoul, Republic of Korea 143-729; E-mail: [email protected] This research was supported by the Basic Science Research Program through the National Research Foundation of Korea funded by the Ministry of Education, Science and Technology (2012R1A1A2044883). The authors disclose no conflicts of interest.
e187
Copyright © 2016 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
e188
H. V. JUNG ET AL.
FIG. 1. The axial images of high-resolution CTof the temporal bone (A, B) demonstrate a large soft tissue density filling the middle ear and mastoid cavity, with visible severe bony destruction in both ears (arrowheads). The left internal auditory canal is enlarged (thin arrow), and the vestibules and horizontal semicircular canal have an expansile appearance, with focal dehiscence into the middle ear cavity (dotted arrow). The cochlea is widened on the left side (thick arrow). The axial image of T2-weighted MRI shows that the pathologic soft tissue, which is isointense with the brain, is filling the internal auditory canal, cochlea, vestibule, and horizontal and posterior semicircular canals (C, split arrows). The contrast-enhanced T1-weighted axial image shows a moderately enhancing mass lesion filling the abovementioned inner ear structures (D, dotted split arrows).
REFERENCES 1. Curry JM, King N, O’Reilly RC, et al. Inflammatory pseudotumor of the inner ear: are computed tomography changes pathognomonic? Laryngoscope 2010;120:1252–5. 2. Mulder JJ, Cremers WR, Joosten F, et al. Fibroinflammatory pseudotumor of the ear. A locally destructive benign lesion. Arch Otolaryngol Head Neck Surg 1995;121:930–3.
3. Williamson RA, Paueksakon P, Coker NJ. Inflammatory pseudotumor of the temporal bone. Otol Neurotol 2003;24:818–22. 4. Schonermark MP, Issing P, Stover T, et al. Fibroinflammatory pseudotumor of the temporal bone. Skull Base Surg 1998;8: 45–50. 5. Park SB, Lee JH, Weon YC. Imaging findings of head and neck inflammatory pseudotumor. AJR Am J Roentgenol 2009;193: 1180–6.
Otology & Neurotology, Vol. 37, No. 6, 2016
Copyright © 2016 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
