Major anomaly: Congenital external and middle ear malformation (CEMEM)
Minor anomaly: Isolated middle ear (ME) anomaly
Ossicular fixation: Congenital ankylosis; rigid bar or fibrous band connects ossicle to wall of ME
Ossicular malformation: Anomaly in shape, size, &/or orientation of ossicle
Columellar stapes: 1 central broad stapedial crus
IMAGING
General Features
Imaging Recommendations
CT Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Reporting Tips
Selected References
Henkemans SE et al: Congenital anomalies of the ossicular chain: surgical and audiological outcomes. Ann Otol Rhinol Laryngol. 131(4):388-96, 2022
Kang BC et al: Hearing outcomes of stapes surgery in children with stapes fixation and ossicular anomalies. Otol Neurotol. 42(7):1039-43, 2021
Zhang N et al: Isolated congenital middle ear malformations: comparison of preoperative high-resolution ct and surgical findings. Ann Otol Rhinol Laryngol. 129(3):216-23, 2020
Gentric JC et al: High-resolution computed tomography of isolated congenital anomalies of the stapes: a pictural review using oblique multiplanar reformation in the "axial stapes" plane. J Neuroradiol. 39(1):58-64, 2012
Kuhn JJ et al: Congenital incudostapedial anomalies in adult stapes surgery: a case-series review. Am J Otolaryngol. 32(6):477-84, 2011
Potti TA et al: A comprehensive review of reported heritable noggin-associated syndromes and proposed clinical utility of one broadly inclusive diagnostic term: NOG-related-symphalangism spectrum disorder (NOG-SSD). Hum Mutat. 32(8):877-86, 2011
Thomeer HG et al: Congenital stapes ankylosis associated with another ossicular chain anomaly: surgical results in 30 ears. Arch Otolaryngol Head Neck Surg. 137(9):935-41, 2011
Park K et al: Isolated congenital ossicular anomalies. Acta Otolaryngol. 129(4):419-22, 2009
Koike T et al: An apparatus for diagnosis of ossicular chain mobility in humans. Int J Audiol. 45(2):121-8, 2006
Martin C et al: Malleus head fixation: histopathology revisited. Acta Otolaryngol. 126(4):353-7, 2006
Oktay MF et al: Histologic changes in the anterior mallear ligament and the head of the malleus in otosclerosis. Otolaryngol Head Neck Surg. 134(2):232-5, 2006
Nakajima HH et al: Experimental and clinical studies of malleus fixation. Laryngoscope. 115(1):147-54, 2005
Seidman MD et al: A new approach for malleus/incus fixation: no prosthesis necessary. Otol Neurotol. 2004
Watanabe A et al: CT findings of bilateral congenital absence of the long process of the incus. Neuroradiology. 46(10):859-61, 2004
Huber A et al: Fixation of the anterior mallear ligament: diagnosis and consequences for hearing results in stapes surgery Ann Otol Rhinol Laryngol. 2003
Harris JP et al: Malleus fixation: clinical and histopathologic findings. Ann Otol Rhinol Laryngol. 111(3 Pt 1):246-54, 2002
Vincent R et al: Malleus ankylosis: a clinical, audiometric, histologic, and surgical study of 123 cases. Am J Otol. 20(6):717-25, 1999
Kurosaki Y et al: Malleus bar as a rare cause of congenital malleus fixation: CT demonstration. AJNR Am J Neuroradiol. 19(7):1229-30, 1998
Meiteles LZ et al: Columella stapes. Otolaryngol Head Neck Surg. 114(6):833-4, 1996
Moon CN Jr et al: Primary malleus fixation: diagnosis and treatment. Laryngoscope. 91(8):1298-307, 1981
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Synonyms
Lateral fixation
Type II columellar stapes = monopod stapes
Definitions
Major anomaly: Congenital external and middle ear malformation (CEMEM)
Minor anomaly: Isolated middle ear (ME) anomaly
Ossicular fixation: Congenital ankylosis; rigid bar or fibrous band connects ossicle to wall of ME
Ossicular malformation: Anomaly in shape, size, &/or orientation of ossicle
Columellar stapes: 1 central broad stapedial crus
IMAGING
General Features
Imaging Recommendations
CT Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Reporting Tips
Selected References
Henkemans SE et al: Congenital anomalies of the ossicular chain: surgical and audiological outcomes. Ann Otol Rhinol Laryngol. 131(4):388-96, 2022
Kang BC et al: Hearing outcomes of stapes surgery in children with stapes fixation and ossicular anomalies. Otol Neurotol. 42(7):1039-43, 2021
Zhang N et al: Isolated congenital middle ear malformations: comparison of preoperative high-resolution ct and surgical findings. Ann Otol Rhinol Laryngol. 129(3):216-23, 2020
Gentric JC et al: High-resolution computed tomography of isolated congenital anomalies of the stapes: a pictural review using oblique multiplanar reformation in the "axial stapes" plane. J Neuroradiol. 39(1):58-64, 2012
Kuhn JJ et al: Congenital incudostapedial anomalies in adult stapes surgery: a case-series review. Am J Otolaryngol. 32(6):477-84, 2011
Potti TA et al: A comprehensive review of reported heritable noggin-associated syndromes and proposed clinical utility of one broadly inclusive diagnostic term: NOG-related-symphalangism spectrum disorder (NOG-SSD). Hum Mutat. 32(8):877-86, 2011
Thomeer HG et al: Congenital stapes ankylosis associated with another ossicular chain anomaly: surgical results in 30 ears. Arch Otolaryngol Head Neck Surg. 137(9):935-41, 2011
Park K et al: Isolated congenital ossicular anomalies. Acta Otolaryngol. 129(4):419-22, 2009
Koike T et al: An apparatus for diagnosis of ossicular chain mobility in humans. Int J Audiol. 45(2):121-8, 2006
Martin C et al: Malleus head fixation: histopathology revisited. Acta Otolaryngol. 126(4):353-7, 2006
Oktay MF et al: Histologic changes in the anterior mallear ligament and the head of the malleus in otosclerosis. Otolaryngol Head Neck Surg. 134(2):232-5, 2006
Nakajima HH et al: Experimental and clinical studies of malleus fixation. Laryngoscope. 115(1):147-54, 2005
Seidman MD et al: A new approach for malleus/incus fixation: no prosthesis necessary. Otol Neurotol. 2004
Watanabe A et al: CT findings of bilateral congenital absence of the long process of the incus. Neuroradiology. 46(10):859-61, 2004
Huber A et al: Fixation of the anterior mallear ligament: diagnosis and consequences for hearing results in stapes surgery Ann Otol Rhinol Laryngol. 2003
Harris JP et al: Malleus fixation: clinical and histopathologic findings. Ann Otol Rhinol Laryngol. 111(3 Pt 1):246-54, 2002
Vincent R et al: Malleus ankylosis: a clinical, audiometric, histologic, and surgical study of 123 cases. Am J Otol. 20(6):717-25, 1999
Kurosaki Y et al: Malleus bar as a rare cause of congenital malleus fixation: CT demonstration. AJNR Am J Neuroradiol. 19(7):1229-30, 1998
Meiteles LZ et al: Columella stapes. Otolaryngol Head Neck Surg. 114(6):833-4, 1996
Moon CN Jr et al: Primary malleus fixation: diagnosis and treatment. Laryngoscope. 91(8):1298-307, 1981
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