Usher syndrome (US) phenotype
| US 1 | US 2 | US 3 | |
| Age at diagnosis of hearing loss | 0–2 years | 0–6 years | During first and up to fourth decade |
| Audiogram | Profound hearing loss/ non-recordable threshold | Moderate to profound high frequency hearing loss. Non-progressive | Progressive hearing loss |
| Cold caloric test | Absent | Nystagmus | Variable |
| Speech | Unintelligible | Intelligible | Intelligible |
| Pigmentary degeneration | Present | Present | Present |
| ERG | Reduced | Reduced | Reduced |