Otocephaly is a type of cephalic disorder

Cephalic disorder

Cephalic disorders are congenital conditions that stem from damage to, or abnormal development of, the budding nervous system...

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This is a lethal condition in which the primary feature is agnathia

Micrognathism

Micrognathism is a condition where the jaw is undersized. It is also sometimes called "Mandibular hypoplasia". It is common in infants, but is usually self-corrected during growth, due to the jaws increasing in size. It may be a cause of abnormal tooth alignment and in severe cases can hamper...

 — a developmental anomaly characterized by total or virtual absence of the lower jaw

Jaw

The jaw is any opposable articulated structure at the entrance of the mouth, typically used for grasping and manipulating food. The term jaws is also broadly applied to the whole of the structures constituting the vault of the mouth and serving to open and close it and is part of the body plan of...

. The "oto" in the name refers to the relationship of the ears to the face in this disorder.

The condition is considered lethal because of a poorly functioning airway

Airway

The pulmonary airway comprises those parts of the respiratory system through which air flows, conceptually beginning at the nose and mouth, and terminating in the alveoli...

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In otocephaly, agnathia may occur alone or together with holoprosencephaly

Holoprosencephaly

Holoprosencephaly is a cephalic disorder in which the prosencephalon fails to develop into two hemispheres. Normally, the forebrain is formed and the face begins to develop in the fifth and sixth weeks of human pregnancy...

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External links

• NINDS Overview