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Congenital Ossicular Anomalies and Fixation
Caroline D. Robson, MBChB
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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

                                      1. 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
                                      2. Kuhn JJ et al: Congenital incudostapedial anomalies in adult stapes surgery: a case-series review. Am J Otolaryngol. 32(6):477-84, 2011
                                      3. 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
                                      4. 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
                                      5. Park K et al: Isolated congenital ossicular anomalies. Acta Otolaryngol. 129(4):419-22, 2009
                                      6. Magliulo G et al: Osteopathia striata-cranial sclerosis: otorhinolaryngologic clinical presentation and radiologic findings. Am J Otolaryngol. 28(1):59-63, 2007
                                      7. Koike T et al: An apparatus for diagnosis of ossicular chain mobility in humans. Int J Audiol. 45(2):121-8, 2006
                                      8. Martin C et al: Malleus head fixation: histopathology revisited. Acta Otolaryngol. 126(4):353-7, 2006
                                      9. 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
                                      10. Nakajima HH et al: Experimental and clinical studies of malleus fixation. Laryngoscope. 115(1):147-54, 2005
                                      11. Seidman MD et al: A new approach for malleus/incus fixation: no prosthesis necessary. Otol Neurotol. 2004
                                      12. Watanabe A et al: CT findings of bilateral congenital absence of the long process of the incus. Neuroradiology. 46(10):859-61, 2004
                                      13. Huber A et al: Fixation of the anterior mallear ligament: diagnosis and consequences for hearing results in stapes surgery Ann Otol Rhinol Laryngol. 2003
                                      14. Harris JP et al: Malleus fixation: clinical and histopathologic findings. Ann Otol Rhinol Laryngol. 111(3 Pt 1):246-54, 2002
                                      15. Vincent R et al: Malleus ankylosis: a clinical, audiometric, histologic, and surgical study of 123 cases. Am J Otol. 20(6):717-25, 1999
                                      16. 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
                                      17. Meiteles LZ et al: Columella stapes. Otolaryngol Head Neck Surg. 114(6):833-4, 1996
                                      18. 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

                                                                          1. 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
                                                                          2. Kuhn JJ et al: Congenital incudostapedial anomalies in adult stapes surgery: a case-series review. Am J Otolaryngol. 32(6):477-84, 2011
                                                                          3. 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
                                                                          4. 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
                                                                          5. Park K et al: Isolated congenital ossicular anomalies. Acta Otolaryngol. 129(4):419-22, 2009
                                                                          6. Magliulo G et al: Osteopathia striata-cranial sclerosis: otorhinolaryngologic clinical presentation and radiologic findings. Am J Otolaryngol. 28(1):59-63, 2007
                                                                          7. Koike T et al: An apparatus for diagnosis of ossicular chain mobility in humans. Int J Audiol. 45(2):121-8, 2006
                                                                          8. Martin C et al: Malleus head fixation: histopathology revisited. Acta Otolaryngol. 126(4):353-7, 2006
                                                                          9. 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
                                                                          10. Nakajima HH et al: Experimental and clinical studies of malleus fixation. Laryngoscope. 115(1):147-54, 2005
                                                                          11. Seidman MD et al: A new approach for malleus/incus fixation: no prosthesis necessary. Otol Neurotol. 2004
                                                                          12. Watanabe A et al: CT findings of bilateral congenital absence of the long process of the incus. Neuroradiology. 46(10):859-61, 2004
                                                                          13. Huber A et al: Fixation of the anterior mallear ligament: diagnosis and consequences for hearing results in stapes surgery Ann Otol Rhinol Laryngol. 2003
                                                                          14. Harris JP et al: Malleus fixation: clinical and histopathologic findings. Ann Otol Rhinol Laryngol. 111(3 Pt 1):246-54, 2002
                                                                          15. Vincent R et al: Malleus ankylosis: a clinical, audiometric, histologic, and surgical study of 123 cases. Am J Otol. 20(6):717-25, 1999
                                                                          16. 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
                                                                          17. Meiteles LZ et al: Columella stapes. Otolaryngol Head Neck Surg. 114(6):833-4, 1996
                                                                          18. Moon CN Jr et al: Primary malleus fixation: diagnosis and treatment. Laryngoscope. 91(8):1298-307, 1981