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Oval Window Atresia
H. Ric Harnsberger, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Oval window atresia (OWA)
            • Synonyms

              • Congenital absence of oval window (OW)
            • Definitions

              • Absent space between lateral semicircular canal above and cochlear promontory below associated with anomalous stapes and malpositioned CNVII
              • Normal oval window: Stapes footplate + annular ligament

            IMAGING

            • 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. 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
                                  2. 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
                                  3. 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
                                  4. 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
                                  5. de Alarcon A et al: Congenital absence of the oval window: diagnosis, surgery, and audiometric outcomes. Otol Neurotol. 29(1):23-8, 2008
                                  6. 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
                                  7. Booth TN et al: Imaging and clinical evaluation of isolated atresia of the oval window. AJNR Am J Neuroradiol. 21(1):171-4, 2000
                                  8. Zeifer B et al: Congenital absence of the oval window: radiologic diagnosis and associated anomalies. AJNR Am J Neuroradiol. 21(2):322-7, 2000
                                  9. 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

                                            TERMINOLOGY

                                            • Abbreviations

                                              • Oval window atresia (OWA)
                                            • Synonyms

                                              • Congenital absence of oval window (OW)
                                            • Definitions

                                              • Absent space between lateral semicircular canal above and cochlear promontory below associated with anomalous stapes and malpositioned CNVII
                                              • Normal oval window: Stapes footplate + annular ligament

                                            IMAGING

                                            • 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. 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
                                                                  2. 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
                                                                  3. 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
                                                                  4. 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
                                                                  5. de Alarcon A et al: Congenital absence of the oval window: diagnosis, surgery, and audiometric outcomes. Otol Neurotol. 29(1):23-8, 2008
                                                                  6. 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
                                                                  7. Booth TN et al: Imaging and clinical evaluation of isolated atresia of the oval window. AJNR Am J Neuroradiol. 21(1):171-4, 2000
                                                                  8. Zeifer B et al: Congenital absence of the oval window: radiologic diagnosis and associated anomalies. AJNR Am J Neuroradiol. 21(2):322-7, 2000
                                                                  9. Lambert PR: Congenital absence of the oval window. Laryngoscope. 100(1):37-40, 1990