Congenital malformations of the inner ear: a classification based on embryogenesis

Laryngoscope. 1987 Mar;97(3 Pt 2 Suppl 40):2-14. doi: 10.1002/lary.5540971301.


Approximately 20% of patients with congenital sensorineural hearing loss have radiographic abnormalities of the inner ear. A broad spectrum of anomalous patterns have been described, most of which have been lumped together under the term "Mondini's dysplasia." We feel that this grouping of many dissimilar entities under a single umbrella term is unwarranted. Based on a review of 63 patients with 98 congenitally malformed ears, we have been able to recognize a number of distinct anatomic patterns from their radiographic appearance. A remarkable similarity between these morphologies and the appearance of the inner ear at various stages of embryogenesis was found. This led us to propose a classification system based upon the theory that these deformities result from an arrest of development during varying stages of inner ear organogenesis.

MeSH terms

  • Congenital Abnormalities / classification*
  • Congenital Abnormalities / diagnostic imaging
  • Congenital Abnormalities / embryology
  • Ear, Inner / abnormalities*
  • Ear, Inner / diagnostic imaging
  • Humans
  • Radiography