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Oval Window Atresia
William T. O'Brien, Sr., DO, FAOCRSurjith Vattoth, MD, FRCRH. Ric Harnsberger, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Oval window atresia (OWA)
              • Synonyms

                • Congenital absence of oval window (OW)
              • Definitions

                • Absent space between lateral semicircular canal above & cochlear promontory below
                • Majority also have anomalous stapes & malpositioned CNVII
                • Normal OW: Stapes footplate + annular ligament

              IMAGING

              • General Features

                • CT Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    • Image Interpretation Pearls

                                      Selected References

                                      1. D'Arco F et al: Temporal bone and intracranial abnormalities in syndromic causes of hearing loss: an updated guide. Eur J Radiol. 123:108803, 2020
                                      2. Nguyen T et al: Conductive hearing loss with a "dry middle ear cleft"-a comprehensive pictorial review with CT. Eur J Radiol. 110:74-80, 2019
                                      3. Vincent R et al: Congenital oval or round window malformations in children: surgical findings and results in 17 cases. Laryngoscope. 126(11):2552-58, 2016
                                      4. Zhao S et al: Round window application of an active middle ear implant (AMEI) system in congenital oval window atresia. Acta Otolaryngol. 136(1):23-33, 2016
                                      5. Colletti L et al: Vestibulotomy with ossiculoplasty versus round window vibroplasty procedure in children with oval window aplasia. Otol Neurotol. 35(5):831-7, 2014
                                      6. Sennaro─člu L et al: Oval window atresia: a novel surgical approach and pathognomonic radiological finding. Int J Pediatr Otorhinolaryngol. 78(5):769-76, 2014
                                      7. Su Y et al: Congenital middle ear abnormalities with absence of the oval window: diagnosis, surgery, and audiometric outcomes. Otol Neurotol. 35(7):1191-5, 2014
                                      8. Ukkola-Pons E et al: Oval window niche height: quantitative evaluation with CT before stapes surgery for otosclerosis. AJNR Am J Neuroradiol. 34(5):1082-5, 2013
                                      9. de Alarcon A et al: Congenital absence of the oval window: diagnosis, surgery, and audiometric outcomes. Otol Neurotol. 29(1):23-8, 2008
                                      10. Martin C et al: Abnormal facial nerve course associated with stapes fixation or oval window absence: report of two cases. Eur Arch Otorhinolaryngol. 263(1):79-85, 2006
                                      11. Booth TN et al: Imaging and clinical evaluation of isolated atresia of the oval window. AJNR Am J Neuroradiol. 21(1):171-4, 2000
                                      12. Zeifer B et al: Congenital absence of the oval window: radiologic diagnosis and associated anomalies. AJNR Am J Neuroradiol. 21(2):322-7, 2000
                                      13. Lambert PR: Congenital absence of the oval window. Laryngoscope. 100(1):37-40, 1990
                                      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

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Oval window atresia (OWA)
                                                  • Synonyms

                                                    • Congenital absence of oval window (OW)
                                                  • Definitions

                                                    • Absent space between lateral semicircular canal above & cochlear promontory below
                                                    • Majority also have anomalous stapes & malpositioned CNVII
                                                    • Normal OW: Stapes footplate + annular ligament

                                                  IMAGING

                                                  • General Features

                                                    • CT Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Gross Pathologic & Surgical Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      DIAGNOSTIC CHECKLIST

                                                                      • Consider

                                                                        • Image Interpretation Pearls

                                                                          Selected References

                                                                          1. D'Arco F et al: Temporal bone and intracranial abnormalities in syndromic causes of hearing loss: an updated guide. Eur J Radiol. 123:108803, 2020
                                                                          2. Nguyen T et al: Conductive hearing loss with a "dry middle ear cleft"-a comprehensive pictorial review with CT. Eur J Radiol. 110:74-80, 2019
                                                                          3. Vincent R et al: Congenital oval or round window malformations in children: surgical findings and results in 17 cases. Laryngoscope. 126(11):2552-58, 2016
                                                                          4. Zhao S et al: Round window application of an active middle ear implant (AMEI) system in congenital oval window atresia. Acta Otolaryngol. 136(1):23-33, 2016
                                                                          5. Colletti L et al: Vestibulotomy with ossiculoplasty versus round window vibroplasty procedure in children with oval window aplasia. Otol Neurotol. 35(5):831-7, 2014
                                                                          6. Sennaro─člu L et al: Oval window atresia: a novel surgical approach and pathognomonic radiological finding. Int J Pediatr Otorhinolaryngol. 78(5):769-76, 2014
                                                                          7. Su Y et al: Congenital middle ear abnormalities with absence of the oval window: diagnosis, surgery, and audiometric outcomes. Otol Neurotol. 35(7):1191-5, 2014
                                                                          8. Ukkola-Pons E et al: Oval window niche height: quantitative evaluation with CT before stapes surgery for otosclerosis. AJNR Am J Neuroradiol. 34(5):1082-5, 2013
                                                                          9. de Alarcon A et al: Congenital absence of the oval window: diagnosis, surgery, and audiometric outcomes. Otol Neurotol. 29(1):23-8, 2008
                                                                          10. Martin C et al: Abnormal facial nerve course associated with stapes fixation or oval window absence: report of two cases. Eur Arch Otorhinolaryngol. 263(1):79-85, 2006
                                                                          11. Booth TN et al: Imaging and clinical evaluation of isolated atresia of the oval window. AJNR Am J Neuroradiol. 21(1):171-4, 2000
                                                                          12. Zeifer B et al: Congenital absence of the oval window: radiologic diagnosis and associated anomalies. AJNR Am J Neuroradiol. 21(2):322-7, 2000
                                                                          13. Lambert PR: Congenital absence of the oval window. Laryngoscope. 100(1):37-40, 1990