AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2025 Issue 4; New/Revised ICD-10-CM Codes
Usher Syndrome
Subcategory Q99.81, Usher syndrome, was created to identify specific types of Usher syndrome. The new codes are as follows:Q99.811 Usher syndrome, type 1 Q99.812 Usher syndrome, type 2 Q99.813 Usher syndrome, type 3 Q99.818 Other Usher syndrome Q99.819 Usher syndrome, unspecified Usher syndrome (USH) is an inherited disorder that causes hearing and vision loss (i.e., retinitis pigmentosa, night blindness, tunnel vision or blindness), and problems with balance in some individuals. USH symptoms are usually present at birth or may develop during childhood. Symptoms vary and develop over time depending on the type...
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Article Overview
This article provides a coding-focused overview of Usher syndrome, including the creation of a dedicated ICD-10-CM subcategory and the broad clinical distinctions among the condition’s main types. It is relevant to coders, clinical documentation specialists, and providers who document hereditary hearing and vision disorders and need to recognize how the diagnosis is organized in the classification system.
Why This Topic Matters
Usher syndrome can present with different patterns of hearing loss, vision loss, and balance involvement over time, so documentation must be specific enough to support the correct diagnosis category. The article helps readers understand the general structure of the code family and the clinical context behind it without relying on detailed source material.
What You Will Learn
- How Usher syndrome is categorized in ICD-10-CM
- The broad clinical features associated with the major Usher syndrome types
- Why subtype documentation matters for this diagnosis
- The general role of supportive management for the condition
Who Should Read This
- Medical coders
- Clinical documentation specialists
- Healthcare providers
- Coding educators
Codes Discussed
Code Ranges Discussed
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