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Congenital malformations of the inner ear: A classification based on embryogenesis
Author(s) -
Jackler Robert K,
Luxfor William M.,
House William F.
Publication year - 1987
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1002/lary.5540971301
Subject(s) - inner ear , anatomy , dysplasia , embryogenesis , congenital malformations , medicine , biology , embryo , pathology , pregnancy , genetics
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.