SNOMED CT, US Edition

Last uploaded: March 22, 2026
Id http://purl.bioontology.org/ontology/SNOMEDCT/720749004
http://purl.bioontology.org/ontology/SNOMEDCT/720749004
Preferred Name

Congenital hereditary endothelial dystrophy and perceptive deafness syndrome

Definitions
Corneal dystrophy-perceptive deafness (CDPD) or Harboyan syndrome is a degenerative corneal disorder characterized by the association of congenital hereditary endothelial dystrophy with progressive, postlingual sensorineural hearing loss. Corneal dystrophy-perceptive deafness (CDPD) or Harboyan syndrome is a degenerative corneal disorder characterised by the association of congenital hereditary endothelial dystrophy with progressive, postlingual sensorineural hearing loss. A degenerative corneal disorder characterized by the association of congenital hereditary endothelial dystrophy with progressive postlingual sensorineural hearing loss. The ocular manifestations include diffuse bilateral corneal edema occurring with severe corneal clouding, blurred vision, visual loss and nystagmus. Caused by mutations in the SLC4A11 gene located at the CHED2 locus on chromosome 20p13p12. A degenerative corneal disorder characterised by the association of congenital hereditary endothelial dystrophy with progressive postlingual sensorineural hearing loss. The ocular manifestations include diffuse bilateral corneal oedema occurring with severe corneal clouding, blurred vision, visual loss and nystagmus. Caused by mutations in the SLC4A11 gene located at the CHED2 locus on chromosome 20p13p12.
Synonyms
Harboyan syndrome
Corneal dystrophy with progressive deafness
Corneal dystrophy and perceptive deafness syndrome
Congenital hereditary endothelial dystrophy and perceptive deafness syndrome (disorder)
Type http://www.w3.org/2002/07/owl#Class
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