Usher Syndromes

Autosomal recessive hereditary disorders characterized by congenital SENSORINEURAL HEARING LOSS and RETINITIS PIGMENTOSA. Genetically and symptomatically heterogeneous, clinical classes include type I, type II, and type III. Their severity, age of onset of retinitis pigmentosa and the degree of vestibular dysfunction are variable.

Also Known As:

Usher Syndrome; Usher Syndrome, Type I; Usher Syndrome, Type III; Deafness-Retinitis Pigmentosa Syndrome; Dystrophia Retinae Pigmentosa-Dysostosis Syndrome; Graefe-Usher Syndrome; Hallgren Syndrome; Retinitis Pigmentosa And Congenital Deafness; Retinitis Pigmentosa-Deafness Syndrome; Usher Syndrome, Type 1; Usher Syndrome, Type 1A; Usher Syndrome, Type 3; Usher Syndrome, Type I, French Variety; Usher Syndrome, Type II; Usher Syndrome, Type IId; Usher's Syndrome; Deafness Retinitis Pigmentosa Syndrome; Deafness-Retinitis Pigmentosa Syndromes; Dystrophia Retinae Pigmentosa Dysostosis Syndrome; Graefe Usher Syndrome; Pigmentosa Syndromes, Deafness-Retinitis; Retinitis Pigmentosa Deafness Syndrome; Retinitis Pigmentosa-Deafness Syndromes; Syndrome, Deafness-Retinitis Pigmentosa; Syndrome, Graefe-Usher; Syndrome, Hallgren; Syndrome, Retinitis Pigmentosa-Deafness; Syndrome, Usher; Syndrome, Usher's; Syndromes, Deafness-Retinitis Pigmentosa; Syndromes, Retinitis Pigmentosa-Deafness; Syndromes, Usher; Ushers Syndrome

Otorhinolaryngologic Diseases: 203
- Ear Diseases: 261
  - Hearing Disorders: 325
    - Hearing Loss: 12265
      - Deafness: 5416
        Deaf-Blind Disorders: 69
        Usher Syndromes: 357
      - Sensorineural Hearing Loss: 3974
        Usher Syndromes: 357
Nervous System Diseases: 14178
- Neurologic Manifestations: 7102
  - Sensation Disorders: 117
    - Vision Disorders: 2977
      - Blindness: 5548
        Deaf-Blind Disorders: 69
        Usher Syndromes: 357
    - Hearing Disorders: 325
      - Hearing Loss: 12265
        Deafness: 5416
        Deaf-Blind Disorders: 69
        Usher Syndromes: 357
        Sensorineural Hearing Loss: 3974
        Usher Syndromes: 357
Eye Diseases: 3522
- Retinal Diseases: 3014
  - Retinal Degeneration: 4078
    - Retinal Dystrophies: 743
      - Retinitis Pigmentosa: 3975
        Usher Syndromes: 357
- Vision Disorders: 2977
  - Blindness: 5548
    - Deaf-Blind Disorders: 69
      - Usher Syndromes: 357
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Congenital Abnormalities: 25723
  - Multiple Abnormalities: 211
    - Deaf-Blind Disorders: 69
      - Usher Syndromes: 357
- Inborn Genetic Diseases: 11939
  - Hereditary Eye Diseases: 18
    - Retinitis Pigmentosa: 3975
      - Usher Syndromes: 357
Signs and Symptoms Pathological Conditions
- Signs and Symptoms
  - Neurologic Manifestations: 7102
    - Sensation Disorders: 117
      - Hearing Disorders: 325
        Hearing Loss: 12265
        Sensorineural Hearing Loss: 3974
        Usher Syndromes: 357

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