Congenital Oval Window Aplasia: An Unusual Cause of Conductive Hearing Loss in an Adult

Authors

  • Charlotte M. Chiong Philippine National Ear Institute National Institutes of Health University of the Philippines Manila 2Department of Otorhinolaryngology College of Medicine - Philippine General Hospital University of the Philippines Manila; Department of Otolaryngology Manila Doctors Hospital
  • Rachel T. Mercado-Evasco Department of Otolaryngology Manila Doctors Hospital
  • Alessandra E. Chiong Department of Otorhinolaryngology College of Medicine - Philippine General Hospital University of the Philippines Manila
  • Mary Ellen C. Perez Department of Anesthesiology College of Medicine- Philippine General Hospital University of the Philippines Manila
  • Franco Louie L. Abes Philippine National Ear Institute National Institutes of Health University of the Philippines Manila; Department of Otolaryngology Manila Doctors Hospital
  • Abner L. Chan Philippine National Ear Institute National Institutes of Health University of the Philippines Manila; Department of Otorhinolaryngology College of Medicine - Philippine General Hospital University of the Philippines Manila; Department of Otolaryngology Manila Doctors Hospital

DOI:

https://doi.org/10.32412/pjohns.v31i1.315

Keywords:

: oval window absence, Cremers classification, congenital middle ear

Abstract

Objective: To report a case of congenital oval window aplasia (COWA) in a Filipino adult presenting with  unilateral maximal conductive hearing loss and discuss the diagnostic considerations, pathophysiology and management.

Methods:

            Study Design:  Case report

            Subjects: One (1)

            Setting:  Tertiary Public Referral Center

Results: Audiometric evaluation showed a maximal unilateral left conductive hearing loss. High resolution temporal bone CT showed absence of the oval window on the left along with facial and stapes abnormalities.  Exploratory tympanotomy showed an aberrant facial nerve, monopodal and abnormally located stapes and absent oval window.  Postoperative hearing gain achieved after a neo-oval window and Schuknecht piston wire prosthesis remained stable over two years.

Conclusion: A congenital minor ear anomaly classified as Cremers Class 4a in which a congenital oval window aplasia was associated with an aberrant facial nerve anomaly and a monopodal stapes is reported. Recent literature supported the view that congenital oval window aplasia can in selected cases be amenable to various surgical approaches and a stable postoperative hearing gain is achievable in the long term.

Keywords: oval window absence,  Cremers classification, congenital middle ear

Downloads

Download data is not yet available.

Downloads

Published

2016-06-24

How to Cite

1.
Chiong CM, Mercado-Evasco RT, Chiong AE, Perez MEC, Abes FLL, Chan AL. Congenital Oval Window Aplasia: An Unusual Cause of Conductive Hearing Loss in an Adult. Philipp J Otolaryngol Head Neck Surg [Internet]. 2016 Jun. 24 [cited 2024 Jun. 23];31(1):39-44. Available from: https://pjohns.pso-hns.org/index.php/pjohns/article/view/315

Similar Articles

1 2 3 > >> 

You may also start an advanced similarity search for this article.

Most read articles by the same author(s)

1 2 > >>