Other Usher syndrome

This code represents a rare, inherited disorder characterized by combined hearing and vision loss that does not fit the specific types of Usher syndrome (Type 1, 2, or 3). Patients typically present with sensorineural hearing impairment and progressive retinitis pigmentosa, leading to tunnel vision and eventual blindness. The specific genetic mutation or clinical presentation deviates from the well-defined categories.

Use this code when documentation clearly indicates a diagnosis of Usher syndrome, but the specific type (e.g., Type 1, 2, or 3) is not identified, or the clinical presentation does not align with the established criteria for those types. This code is appropriate when the physician explicitly diagnoses "other Usher syndrome" or "atypical Usher syndrome" after comprehensive audiological and ophthalmological evaluations.

• Ensure the medical record specifies "Usher syndrome" and clarifies that it is not Type 1, 2, or 3.
• Avoid using this code if a more specific Usher syndrome type (Q87.01-Q87.03) can be identified from the documentation.
• Always sequence this code as a primary diagnosis when it is the reason for the encounter, or as a secondary diagnosis when it is a co-morbidity.