Usher syndrome, type 1

This code represents Usher syndrome type 1, a rare genetic disorder characterized by profound sensorineural hearing loss present from birth or early childhood, and progressive vision loss due to retinitis pigmentosa, typically manifesting in adolescence. Individuals with Usher syndrome type 1 also experience severe balance problems due to vestibular dysfunction.

Use this code for patients definitively diagnosed with Usher syndrome type 1, based on clinical findings of congenital profound deafness, progressive vision loss, and significant vestibular dysfunction. Documentation should clearly specify "type 1" to differentiate from other Usher syndrome types.

• Ensure documentation specifies "type 1" to avoid confusion with other Usher syndrome types (e.g., type 2 or 3).
• Code also for associated manifestations such as profound sensorineural hearing loss (H90.41 or H90.42) and retinitis pigmentosa (H35.52).
• Do not use this code for unspecified Usher syndrome; seek clarification from the provider if the type is not documented.