Orphanet: Erfelijke sensorische neuropathie doofheid dementie syndroom

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Hereditary sensory neuropathy-deafness-dementia syndrome

Disease definition

A rare genetic neurological disorder characterized by sensorineural hearing loss, sensory neuropathy, behavioral abnormalities, and dementia. Occurrence of seizures has also been reported. Age of onset is between adolescence and adulthood. The disease is progressive, with fatal outcome typically in the fifth to sixth decade.


Classification level: Disorder
  • Synonym(s):
    • HSAN1E
    • HSN1E
    • Hereditary sensory neuropathy-sensorineural hearing loss-dementia syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Adolescent, Adult
  • ICD-10: G60.8
  • OMIM: 614116
  • UMLS: -
  • MeSH: -
  • GARD: 11927
  • MedDRA: -

Detailed information


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