Usher syndrome, type 3

This code identifies Usher syndrome type 3, a rare, progressive genetic disorder characterized by postlingual sensorineural hearing loss that typically begins in adolescence or early adulthood, followed by progressive vision loss due to retinitis pigmentosa. Individuals with Usher type 3 may also experience vestibular dysfunction leading to balance issues.

Assign this code for patients diagnosed with Usher syndrome type 3 based on clinical findings, genetic testing, or a family history consistent with this specific subtype. Documentation should clearly specify the type of Usher syndrome to differentiate it from other types.

• Ensure documentation specifies "type 3" to distinguish from other Usher syndrome types (e.g., type 1, type 2).
• Code any associated hearing loss (e.g., H90.3, H90.5) and retinitis pigmentosa (e.g., H35.52) separately as secondary diagnoses.
• Consider genetic counseling (Z71.83) if genetic testing confirms the diagnosis or for family planning.