Usher syndrome, type 2

This code represents Usher syndrome type 2, a genetic disorder characterized by moderate to severe congenital sensorineural hearing loss and progressive retinitis pigmentosa, typically manifesting as night blindness in adolescence. Vision loss progresses over time, often leading to legal blindness by mid-adulthood.

Use this code when documentation specifies a diagnosis of Usher syndrome and explicitly identifies it as type 2. This diagnosis is typically made based on clinical findings, audiometry, electroretinography (ERG), and often confirmed by genetic testing.

• Differentiate from other Usher syndrome types (e.g., type 1, type 3) which have distinct clinical presentations and separate codes.
• Ensure documentation clearly states "type 2" to support this specific code.
• Code any associated manifestations, such as specific types of hearing loss (e.g., H90.3 for sensorineural hearing loss, bilateral) or visual impairments (e.g., H53.6 for night blindness), as secondary diagnoses.