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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Congenital cholesteatoma (CCh)
Synonyms
- Primary cholesteatoma, epidermoid
Definitions
- Benign mass secondary to epithelial rests of embryonal origin
- Most commonly occurs in middle ear (ME), behind intact tympanic membrane (TM) in patient without history of surgery, chronic 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
- Misale P et al: Congenital cholesteatoma in adults-interesting presentations and management. Indian J Otolaryngol Head Neck Surg. 70(4):578-82, 2018
- Morita Y et al: Risk factors of recurrence in pediatric congenital cholesteatoma. Otol Neurotol. 38(10):1463-9, 2017
- Mierzwinski J et al: Cochlear implant and congenital cholesteatoma. J Otolaryngol Head Neck Surg. 45:8, 2016
- van Egmond SL et al: A systematic review of non-echo planar diffusion-weighted magnetic resonance imaging for detection of primary and postoperative cholesteatoma. Otolaryngol Head Neck Surg. 154(2):233-40, 2015
- Chung J et al: Congenital cholesteatoma and cochlear implantation: implications for management. Cochlear Implants Int. 14(1):32-5, 2013
- Más-Estellés F et al: Contemporary non-echo-planar diffusion-weighted imaging of middle ear cholesteatomas. Radiographics. 32(4):1197-213, 2012
- Choi HG et al: Clinical experience of 71 cases of congenital middle ear cholesteatoma. Acta Otolaryngol. 130(1):62-7, 2010
- De Foer B et al: Diffusion-weighted magnetic resonance imaging of the temporal bone. Neuroradiology. 52(9):785-807, 2010
- De Foer B et al: Middle ear cholesteatoma: non-echo-planar diffusion-weighted MR imaging versus delayed gadolinium-enhanced T1-weighted MR imaging--value in detection. Radiology. 255(3):866-72, 2010
- 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
- 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
- El-Bitar MA et al: Bilateral occurrence of congenital middle ear cholesteatoma. Otolaryngol Head Neck Surg. 127(5):480-2, 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
- 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
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Congenital cholesteatoma (CCh)
Synonyms
- Primary cholesteatoma, epidermoid
Definitions
- Benign mass secondary to epithelial rests of embryonal origin
- Most commonly occurs in middle ear (ME), behind intact tympanic membrane (TM) in patient without history of surgery, chronic 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
- Misale P et al: Congenital cholesteatoma in adults-interesting presentations and management. Indian J Otolaryngol Head Neck Surg. 70(4):578-82, 2018
- Morita Y et al: Risk factors of recurrence in pediatric congenital cholesteatoma. Otol Neurotol. 38(10):1463-9, 2017
- Mierzwinski J et al: Cochlear implant and congenital cholesteatoma. J Otolaryngol Head Neck Surg. 45:8, 2016
- van Egmond SL et al: A systematic review of non-echo planar diffusion-weighted magnetic resonance imaging for detection of primary and postoperative cholesteatoma. Otolaryngol Head Neck Surg. 154(2):233-40, 2015
- Chung J et al: Congenital cholesteatoma and cochlear implantation: implications for management. Cochlear Implants Int. 14(1):32-5, 2013
- Más-Estellés F et al: Contemporary non-echo-planar diffusion-weighted imaging of middle ear cholesteatomas. Radiographics. 32(4):1197-213, 2012
- Choi HG et al: Clinical experience of 71 cases of congenital middle ear cholesteatoma. Acta Otolaryngol. 130(1):62-7, 2010
- De Foer B et al: Diffusion-weighted magnetic resonance imaging of the temporal bone. Neuroradiology. 52(9):785-807, 2010
- De Foer B et al: Middle ear cholesteatoma: non-echo-planar diffusion-weighted MR imaging versus delayed gadolinium-enhanced T1-weighted MR imaging--value in detection. Radiology. 255(3):866-72, 2010
- 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
- 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
- El-Bitar MA et al: Bilateral occurrence of congenital middle ear cholesteatoma. Otolaryngol Head Neck Surg. 127(5):480-2, 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
- 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
