X-linked syndromic intellectual disability hereditary optic neuropathy Genetic neurodegenerative disease Rare genetic developmental defect during embryogenesis Mohr-Tranebjaerg syndrome DDON syndrome orphanet Deafness - dystonia - optic neuronopathy syndrome OMIM:311150 Mohr-Tranebjaerg syndrome (MTS) is an X-linked recessive neurodegenerative syndrome characterized by clinical manifestations commencing with early childhood onset hearing loss, followed by adolescent onset progressive dystonia or ataxia, visual impairment from early adulthood onwards and dementia from the 4th decade onwards. UMLS:C0796074 ICD10:G31.8 OMIM:304700 MESH:C535808 Disorder of energy metabolism Syndromic genetic deafness Mitochondrial disease with eye involvement