Otocephaly, also called agnathia-otocephaly complex, is a very rare and lethal disorder of head development. It is characterized by absence of the lower jaw, known as agnathia, together with ears that are fused just below the chin, known as synotia. It is caused by a disruption to the development of the first branchial arch, the embryonic structure from which the jaw arises, and it is reported to occur in about one in every 70,000 embryos. This entry records what is held about the condition as a congenital malformation and does not describe management.
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