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Q99.81 ICD-10-CM Code: Usher syndrome

Billing Status: NO. This is a clinician non-billable / parent hierarchy grouping in the ICD-10-CM system.

Coding at a Glance

Tabular directives
1 Excludes2 · 1 use-additional code

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for Q99.81 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2026Effective: October 1, 2025

Notes without a marker are published on Q99.81 itself; “inherited from” names the category or block whose note applies here.

Excludes2 — Not Included Here

Conditions not covered by this code, but which may be reported alongside it when both are present.

Source: inherited from Q90-Q99

Use Additional Code

Supplementary codes the tabular list directs you to add.

  • Use additional code to identify any auditory and visual manifestations

Coder workflow for Q99.81

MedCoder structured workflow — derived from this code’s own official record

Before you code Q99.81

  1. Q99.81 is not reportable as written. Select the more specific code beneath it that the documentation supports. Codes are reported to the highest level of specificity the classification provides (Guidelines I.B.2).

    ReviewQ99.811, Q99.812, Q99.813, Q99.818, Q99.819

    See the relationships section · Guide: How to choose an ICD-10-CM code →

Choose the right path

  1. Does the documentation support one of the more specific codes beneath Q99.81?
    Yes → Select that code and continue the checks below on its own page.
    No → Q99.81 cannot be reported as written; query for the specificity its subcategory needs.

    ReviewQ99.811, Q99.812, Q99.813, Q99.818, Q99.819

Consider Q99.81. Then work the Use Additional Code note, and confirm the code is valid for the date of service in the Verify section.

Documentation check

The provider’s diagnostic statement
Codes are assigned from the provider’s documented diagnosis, not from clinical criteria, test values or a medication list (Guidelines I.A.19).
The conditions the Use Additional Code note names
Reported with this code when documented; a conditional instruction (“if applicable”, “if known”) applies only when the record supports it.

Official instructions as workflow

  • Excludes2 — not part of Q99.81(1 note)

    Coding workflow: The conditions named in this note are not included in Q99.81. When the record documents both, both may be reported; the note is a boundary, not a prohibition.

    CompareE88.4

    See the official tabular notes · Guidelines I.A.12.b

  • Use Additional Code — after identifying Q99.81(1 note)

    Coding workflow: Check whether the documentation supports the additional code(s) the note names, and report them with Q99.81 when it does. Where the instruction is conditional (“if applicable”, “if known”), it applies only when the record documents the condition.

    See the official tabular notes · Guidelines I.A.13

Coding decision scenarios

Pattern scenarios for this code’s structure — decision rules, not clinical cases

Documentation: A condition the Use Additional Code note names is documented.

Coding question: Is a second code reported with Q99.81?

Path: Review the Use Additional Code note and the code it names.

Reason: The additional code is reported when the record documents the condition; a conditional instruction applies only when its condition is met (Guidelines I.A.13).

Every row is derived from this code’s own record — its title, tabular notes, 7th-character family and same-category siblings — with fixed MedCoder wording; nothing is inferred about a patient. The official notes and guideline text are in the sections each row links to, and they control.

Code Overview

Usher syndrome is a non-billable ICD-10-CM category code (Q99.81). A more specific billable subcode must be selected for claims submission.

MedCoder summary Summary composed by MedCoder from this code's official ICD-10-CM record. The tabular instructional notes themselves appear verbatim below.

Verify Before Coding

From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim in Claim Check.

Relationships & Classification

MedCoder structured relationships — computed from published CMS and AHRQ datasets

Other codes that name Q99.81 or its code family, from the CMS ICD-10-CM tabular instructional notes. Tabular-note edges are stored at the code family level that carries each note.

Referenced by 10 Excludes1 notes across 3 chapters: E25 — Adrenogenital disorders (via Q99.-), Q50 — Congenital malformations of ovaries, fallopian tubes and broad ligaments (via Q99.-), Q50-Q56 — Congenital malformations of genital organs (Q50-Q56) (via Q99.-), Q51 — Congenital malformations of uterus and cervix (via Q99.-), Q52 — Other congenital malformations of female genitalia (via Q99.-), Q53 — Undescended and ectopic testicle (via Q99.-), Q54 — Hypospadias (via Q99.-), Q55 — Other congenital malformations of male genital organs (via Q99.-), Q56 — Indeterminate sex and pseudohermaphroditism (via Q99.-), Z15 — Genetic susceptibility to disease (via Q99.-).

These codes’ tabular lists mark this diagnosis as mutually exclusive — not reported together unless the conditions are documented as unrelated.

Referenced by 1 Code First instruction: D84.81 — Immunodeficiency due to conditions classified elsewhere (via Q99.-).

Each of these codes carries a Code First note naming this condition — when that code is reported, THIS code is sequenced first, ahead of it.

Contextual Map

Every relationship of Q99.81 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.

Run Q99.81 with these 9 related codes in Claim Check

Hierarchy

Referenced by Excludes1 notes (10)

Referenced by Code First instructions

Nearest codes (12)

Change history

Common coding questions

Can Q99.81 be billed directly?

No. Q99.81 (Usher syndrome) is a non-billable ICD-10-CM category code. A more specific billable subcode must be selected based on clinical documentation.

Sources for this page

Codes, titles, notes, index terms and mappings on this page are transcribed from the datasets below. Relationships MedCoder computed and text MedCoder wrote are labelled where they appear.

Code, title, tabular notes and index terms Official source data
CMS/CDC ICD-10-CM FY2026 tabular list, index and tables, effective October 1, 2025 Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v43.1 (April 2026) Release, file and checksum · Publisher’s page
Change history and date-of-service validity Official source data
CMS ICD-10-CM release addenda, ingested release by release into the change ledger Release, file and checksum · Publisher’s page
Comparisons, relationships and the contextual map MedCoder structured relationship
Computed by MedCoder from the tabular notes and tables above; every derived relationship is marked as derived where it appears
Summary and FAQ answers MedCoder explanation
Written by MedCoder to explain the sources above: drafted with AI assistance, checked by a person against the release files, and labelled as MedCoder text where it appears. Not official text.

Labels on this page: Official source data · MedCoder structured relationship · MedCoder explanation. How to read the labels · All data sources and release dates

Cite this page

Reference this page in a research guide, syllabus or article. The release is included because code content changes each year.

MedCoder.ai. "Q99.81 — Usher syndrome." ICD-10-CM FY2026. https://medcoder.ai/icd10/code/q99.81-usher-syndrome

Change history

  • FY2026 — October 1, 2025
    Added to the code set
    Usher syndrome
    FY2026 changes

Nearest Codes in This Family

Official ICD-10-CM classifications closest to Q99.81 in its code family, with their registry titles.

View all codes in the Q99 family