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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Congenital middle ear cholesteatoma (CMEC)
Synonyms
Primary cholesteatoma, epidermoid
Definitions
Cholesteatoma in middle ear (ME) behind intact TM in patient with no history of surgery, otitis media, or otorrhea
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Bacciu A et al: Open vs closed type congenital cholesteatoma of the middle ear: two distinct entities or two aspects of the same phenomenon? Int J Pediatr Otorhinolaryngol. 78(12):2205-9, 2014
Más-Estellés F et al: Contemporary non-echo-planar diffusion-weighted imaging of middle ear cholesteatomas. Radiographics. 32(4):1197-213, 2012
Ganaha A et al: Efficacy of diffusion-weighted magnetic resonance imaging in the diagnosis of middle ear cholesteatoma. Auris Nasus Larynx. 38(3):329-34, 2011
Park KH et al: Congenital middle ear cholesteatoma in children; retrospective review of 35 cases. J Korean Med Sci. 24(1):126-31, 2009
De Foer B et al: The value of single-shot turbo spin-echo diffusion-weighted MR imaging in the detection of middle ear cholesteatoma. Neuroradiology. 49(10):841-8, 2007
Isaacson G: Diagnosis of pediatric cholesteatoma. Pediatrics. 120(3):603-8, 2007
Kutz JW Jr et al: Congenital middle ear cholesteatoma. Ear Nose Throat J. 86(11):654, 2007
Lazard DS et al: Congenital cholesteatoma: risk factors for residual disease and retraction pockets--a report on 117 cases. Laryngoscope. 117(4):634-7, 2007
Kojima H et al: Congenital cholesteatoma clinical features and surgical results. Am J Otolaryngol. 27(5):299-305, 2006
Shirazi MA et al: Surgical treatment of pediatric cholesteatomas. Laryngoscope. 116(9):1603-7, 2006
El-Bitar MA et al: Congenital middle ear cholesteatoma: need for early recognition--role of computed tomography scan. Int J Pediatr Otorhinolaryngol. 67(3):231-5, 2003
Darrouzet V et al: Congenital middle ear cholesteatomas in children: our experience in 34 cases. Otolaryngol Head Neck Surg. 126(1):34-40, 2002
Koltai PJ et al: The natural history of congenital cholesteatoma. Arch Otolaryngol Head Neck Surg. 128(7):804-9, 2002
Nelson M et al: Congenital cholesteatoma: classification, management, and outcome. Arch Otolaryngol Head Neck Surg. 128(7):810-4, 2002
Potsic WP et al: A staging system for congenital cholesteatoma. Arch Otolaryngol Head Neck Surg. 128(9):1009-12, 2002
Potsic WP et al: Congenital cholesteatoma: 20 years' experience at The Children's Hospital of Philadelphia. Otolaryngol Head Neck Surg. 126(4):409-14, 2002
Yammine FG et al: Anterior and posterior middle ear congenital cholesteatomas in children. J Otolaryngol. 30(1):29-33, 2001
Yeo SW et al: The clinical evaluations of pathophysiology for congenital middle ear cholesteatoma. Am J Otolaryngol. 22(3):184-9, 2001
Tos M: A new pathogenesis of mesotympanic (congenital) cholesteatoma. Laryngoscope. 110(11):1890-7, 2000
De la Cruz A et al: Detection and management of childhood cholesteatoma. Pediatr Ann. 28(6):370-3, 1999
Cholesteatoma in middle ear (ME) behind intact TM in patient with no history of surgery, otitis media, or otorrhea
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Bacciu A et al: Open vs closed type congenital cholesteatoma of the middle ear: two distinct entities or two aspects of the same phenomenon? Int J Pediatr Otorhinolaryngol. 78(12):2205-9, 2014
Más-Estellés F et al: Contemporary non-echo-planar diffusion-weighted imaging of middle ear cholesteatomas. Radiographics. 32(4):1197-213, 2012
Ganaha A et al: Efficacy of diffusion-weighted magnetic resonance imaging in the diagnosis of middle ear cholesteatoma. Auris Nasus Larynx. 38(3):329-34, 2011
Park KH et al: Congenital middle ear cholesteatoma in children; retrospective review of 35 cases. J Korean Med Sci. 24(1):126-31, 2009
De Foer B et al: The value of single-shot turbo spin-echo diffusion-weighted MR imaging in the detection of middle ear cholesteatoma. Neuroradiology. 49(10):841-8, 2007
Isaacson G: Diagnosis of pediatric cholesteatoma. Pediatrics. 120(3):603-8, 2007
Kutz JW Jr et al: Congenital middle ear cholesteatoma. Ear Nose Throat J. 86(11):654, 2007
Lazard DS et al: Congenital cholesteatoma: risk factors for residual disease and retraction pockets--a report on 117 cases. Laryngoscope. 117(4):634-7, 2007
Kojima H et al: Congenital cholesteatoma clinical features and surgical results. Am J Otolaryngol. 27(5):299-305, 2006
Shirazi MA et al: Surgical treatment of pediatric cholesteatomas. Laryngoscope. 116(9):1603-7, 2006
El-Bitar MA et al: Congenital middle ear cholesteatoma: need for early recognition--role of computed tomography scan. Int J Pediatr Otorhinolaryngol. 67(3):231-5, 2003
Darrouzet V et al: Congenital middle ear cholesteatomas in children: our experience in 34 cases. Otolaryngol Head Neck Surg. 126(1):34-40, 2002
Koltai PJ et al: The natural history of congenital cholesteatoma. Arch Otolaryngol Head Neck Surg. 128(7):804-9, 2002
Nelson M et al: Congenital cholesteatoma: classification, management, and outcome. Arch Otolaryngol Head Neck Surg. 128(7):810-4, 2002
Potsic WP et al: A staging system for congenital cholesteatoma. Arch Otolaryngol Head Neck Surg. 128(9):1009-12, 2002
Potsic WP et al: Congenital cholesteatoma: 20 years' experience at The Children's Hospital of Philadelphia. Otolaryngol Head Neck Surg. 126(4):409-14, 2002
Yammine FG et al: Anterior and posterior middle ear congenital cholesteatomas in children. J Otolaryngol. 30(1):29-33, 2001
Yeo SW et al: The clinical evaluations of pathophysiology for congenital middle ear cholesteatoma. Am J Otolaryngol. 22(3):184-9, 2001
Tos M: A new pathogenesis of mesotympanic (congenital) cholesteatoma. Laryngoscope. 110(11):1890-7, 2000
De la Cruz A et al: Detection and management of childhood cholesteatoma. Pediatr Ann. 28(6):370-3, 1999