Congenital defects of the middle ear--uncommon cause of pediatric hearing loss.

INTRODUCTION In children, hypoacusis, or conductive hearing loss, is usually acquired; otitis media with effusion is the most common etiology. However, in some cases this condition is congenital, ranging from deformities of the external and middle ear to isolated ossicular chain malformations. The non-ossicular anomalies of the middle ear, for instance, persistent stapedial artery and anomaly of the facial nerve, are uncommon but may accompany the ossicular defects. OBJECTIVE This study aimed to describe the clinical presentation, diagnostic tests, and therapeutic options of congenital malformations of the middle ear. METHODS This was a retrospective study of cases followed in otolaryngologic consultations since 2007 with the diagnosis of congenital malformation of the middle ear according to the Teunissen and Cremers classification. A review of the literature regarding the congenital malformation of the middle ear and its treatment is presented. CONCLUSION Middle ear malformations are rarely responsible for conductive hearing loss in children. As a result, there is often a late diagnosis and treatment of these anomalies, which can lead to delays in the development of language and learning.