WHO-ICD-10 version:2010
Diseases of the nervous system
Systemic atrophies primarily affecting the central nervous system
Mode of Inheritance
Autosomal recessive, X-linked recessive?
Gene Map Locus
Not Mapped
Ataxia-Deafness-Retardation Syndrome is a rare genetic syndrome characterized by intellectual disability, deafness, ataxia, developmental delay and progressive sensorineural hearing loss. It is different from the Richards-Rundle syndrome, although both share the ataxia, deafness, and intellectual disability. The condition shows an autosomal recessive pattern of inheritance. However, one of the studies has also suggested X-linked inheritance as a possible inheritance pattern.