Reporting and Description for Congenital Middle Ear Malformations to Facilitate Surgical Management

Abstract

Introduction: The aim of this work was to report and describe the different types of congenital middle ear malformations in order to guide surgical treatment approaches and improve outcomes for affected patients. Methods: The authors reviewed patients with congenital middle ear malformations who received surgical treatment between September 2010 and March 2017. Patient characteristics, middle ear deformities, and surgical procedures were documented. Results: In this retrospective study, 35 patients were reviewed. A description of middle ear malformation was proposed that considers ear embryogenesis and focuses on stapes deformity, with the main purpose of facilitating surgical approach selection to reconstruct the ossicular chain. Patients were classified into 3 categories: type I (19 cases), mobile stapes footplate, which included type Ia with normal stapes suprastructure and type Ib with abnormal stapes suprastructure; type II (4 cases), fixed stapes footplate, which included type IIa with normal ossicular chain and type IIb with abnormal ossicular chain; and type III (12 cases), oval window bony atresia or aplasia, with or without round window atresia. Types II and III could have concomitant aberrant facial nerve. Different surgical approaches are described. Conclusions: The authors describe the different types of congenital middle ear malformations. This category description considers ear embryogenesis and is focused on stapes deformity. It may provide better understanding of disease development and guide modern hearing reconstructive surgery.