Dysplasia Neuroectodermal Congenital

**Dysplasia of neuroepithelial origin, Beckley syndrome** (Beckley-Schaumann syndrome) is a hereditary neuropathy characterized by sensorineural deafness, loss of muscle tone and seizures.

The syndrome is characterized by a characteristic face - convex orbits, micrognathia, cleft lip, epicanthus, depressed nose, flat bridge of the nose; short neck; well-developed subcutaneous fat layer; scanty frontal and occipital tubercles of the skull, wider sagittal diameter; multiple dental changes due to enamel hypoplasia, delayed teething, myxodysplasia; overweight,

**Congenital neurosensory dysplasia**

*Dysplasia* is a disease in which the coordination of the processes of formation and development of tissues in the body is impaired. *Disa-* from Greek. dys – prefix meaning “disturbance” + plasis – formation + kinesis – movement. *Plasia* means “perversion” - it is a plastic deformation in which the proportions of organs or their parts are distorted and some loss or change in tissue structure. In this case, we mean a disturbance in human embryogenesis with a subsequent change in the differentiation of nerve cells of the brain stem and a violation of the formation of the skin. The term "neuroectodermal" is used to refer to the nervous