Conditions / Syndrome

Usher syndrome type 1

info · Syndrome · ICD-10: Q99.811

An Usher syndrome characterized by profound congenital deafness, vestibular dysfunction and early development of retinitis pigmentosa.

Signs and symptoms

  • Visual loss
  • Nystagmus
  • Sensorineural hearing impairment
  • Rod-cone dystrophy
  • Abnormal electroretinogram
  • Undetectable electroretinogram
  • Motor delay
  • Absent vestibular function

Also known as: US1; USH1