Orphanet: X linked hereditary sensory and autonomic neuropathy with deafness

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X-linked hereditary sensory and autonomic neuropathy with deafness

Disease definition

This syndrome is characterized by the association of an axonal sensory and autonomic neuropathy with hearing loss.


Classification level: Disorder
  • Synonym(s):
    • X-linked HSAN with deafness
    • X-linked auditory neuropathy with peripheral sensory neuropathy type 1
  • Prevalence: <1 / 1 000 000
  • Inheritance: X-linked recessive 
  • Age of onset: Childhood
  • ICD-10: G60.8
  • OMIM: 300614
  • UMLS: -
  • MeSH: -
  • GARD: 12731
  • MedDRA: -
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