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Round window atresia – a case report


Authors: K Vitoul ;  Richard Salzman
Authors‘ workplace: Klinika otorinolaryngologie a chirurgie hlavy a krku, LF UP a FN Olomouc
Published in: Otorinolaryngol Foniatr, 71, 2022, No. 3, pp. 162-168.
Category: Case Reports
doi: https://doi.org/10.48095/ccorl2022162

Overview

Only a few cases of a round windows atresia have been reported in the literature so far. The dia gnosis is based on the preoperative CT scan and local findings during a middle ear exploration. The complete round window closure leads to a significant conductive hearing loss. The hearing test result might be similar to the one found in otosclerosis. We report a case of 30-year-old woman, who previously underwent three surgeries for suspected otosclerosis without any hearing improvement. A round window atresia was only identified during following revision surgery, as well as a missing stapedial muscle, an abnormal shape and movement of the stapes footplate. In order to achieve a functional inner ear hydrodynamics, a cochlear fenestration was performed. Although there was a significant improvement in the cochlear reserve at speech frequencies after the operation, the patient didn’t report any hearing improvement. Although otosclerosis may be considered as a cause of the conductive hearing loss preoperatively, rare diagnoses should not be neglected. The aim of this case report is to share the experience and highlight mistakes made. After each unsuccessful stapedoplasty with unsatisfying hearing gain, a high resolution CT should be performed to exclude third window syndrome or another rare abnormality.

Keywords:

atresia – cochlear fenestration – round window – stapes surgery


Sources

1. Borrmann A, Arnold W. Non-syndromal round window atresia: an autosomal dominant genetic disorder with variable penetrance? Eur Arch Otorhinolaryngol 2007; 264(9): 1103–1108. Doi: 10.1007/ s00405-007-0305-1.

2. Harrison WH, Shambaugh GE Jr, Derlacki EL. Congenital Absence of the Round Window: Case Report with Surgical Reconstruction by Cochlear Fenestration. Laryngoscope 1964; 74: 967–978. Doi: 10.1288/ 00005537-196407000-00 005.

3. Linder TE, Ma F, Huber A. Round window atresia and its effect on sound transmission. Otol Neurotol 2003; 24(2): 259–263. Doi: 10.1097/ 00 129492-200303000-00021.

4. Martin C, Tringali S, Bertholon P et al. Isolated congenital round window absence. Ann Otol Rhinol Laryngol 2002; 111(9): 799–801. Doi: 10.1177/ 000348940211100907.

5. Pappas DG, Jr., Pappas DG, Sr., Hedlin G. Round window atresia in association with congenital stapes fixation. Laryngoscope 1998; 108(8 Pt 1): 1115–1118. Doi: 10.1097/ 00005537- 199808000-00002.

6. Richards SH. Congenital absence of the round window treated by cochlear fenestration. Clin Otolaryngol Allied Sci 1981; 6(4): 265–269. Doi: 10.1111/ j.1365-2273.1981.tb01546.x.

7. Wong WK, Salkeld L, Flint D. Isolated Congenital Round Window Atresia: Report of 2 Cases. Ear Nose Throat J 2020; 99(7): 433–436. Doi: 10.1177/ 0145561319840541.

8. Clifford AR, Fagan PA, Doust BD. Isolated congenital round window absence. J Laryngol Otol 1990; 104(12): 980–981. Doi: 10.1017/ s00222 15100114549.

9. Ombredanne M. [Congenital absence of the round window in certain minor aplasias. Further cases]. Ann Otolaryngol Chir Cervicofac 1968; 85(5): 369–378.

10. Stewart TJ, Belal A. Surgical anatomy and pathology of the round window. Clin Otolaryngol Allied Sci 1981; 6(1): 45–62. Doi: 10.1111/ j.1365- 2273.1981.tb01785.x.

11. Tonndorf J. Bone conduction. Studies in experimental animals. Acta Otolaryngol 1966: Suppl 213: 211+.

12. Scarpa A. Anotomicae Observationes de Structura Fenestrae Rotundae et de Qmpano Secundario. Modena: Typographicam Societatum; 1772.

13. Heiland KE, Goode RL, Asai M et al. A human temporal bone study of stapes footplate movement. Am J Otol 1999; 20(1): 81–86.

14. Smyth GD, Patterson CC. Results of middle ear reconstruction: do patients and surgeons agree? Am J Otol 1985; 6(3): 276–279.

15. Vercruysse JP, Casselman J, De Foer B et al. Congenital bilateral oval and round window aplasia with a hypoplastic stapes. Otol Neurotol 2006; 27(3): 441–442. Doi: 10.1097/ 00129492- 200604000-00024.

Labels
Audiology Paediatric ENT ENT (Otorhinolaryngology)
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