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

Compare with another code for Q99.81Check this code on a claim

Billing Status: NO. This is a non-billable ICD-10-CM code: report a more specific billable code beneath it.

Coding at a Glance

Official instructions
1 Excludes2 · 1 use-additional instruction

What these terms mean →

Not billable · FY2027A non-billable heading in the tabular list: report a more specific code beneath it.

What you need to know

Source: CMS/NCHS Official ICD-10-CM tabular notes, quoted. From the CMS/NCHS tabular list for the release in force. A note the category or block publishes applies to this code too, and is marked with where it comes from.

Use additional codeReport with this code when documented
  • Use additional code to identify any auditory and visual manifestations
Excludes2from Q90-Q99Not included here; may be reported together
  • mitochondrial metabolic disorders (E88.4-)

Where it sits Source: CMS/NCHS

Under:Chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities (Q00-QA1) (Q00-QA1)Q90-Q99 Chromosomal abnormalities, not elsewhere classifiedQ99 Other chromosome abnormalities, not elsewhere classifiedQ99.8 Other specified chromosome abnormalities

Codes under Q99.81 (5)

  • Q99.811Usher syndrome, type 1Billable
  • Q99.812Usher syndrome, type 2Billable
  • Q99.813Usher syndrome, type 3Billable
  • Q99.818Other Usher syndromeBillable
  • Q99.819Usher syndrome, unspecifiedBillable

Most relevant related codes MedCoder-derived

Read off the official notes above and this code’s own position in the tabular list. Which to report is a documentation question; Compare shows the two side by side.

CompareCheck ClaimView Related Codes

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: FY2027Effective: October 1, 2026

Trace:FY2027 changesChange historyRelease, file and checksum

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. Where the codes beneath it include an unspecified code, that code applies when the record goes no further; otherwise query for the missing detail.

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

Report the code beneath Q99.81 that the documentation supports, not Q99.81 itself. Then work the Use Additional Code note, and confirm that code is valid for the date of service on its own page.

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 the code beneath 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.

Don’t confuse Q99.81 with…

MedCoder analysis — pairs from this code’s official notes, distinctions written by MedCoder

The codes most likely to be picked instead of Q99.81 — named in its own official notes, or sharing its laterality or encounter family — with what separates them and which documentation moves the case. Each links to the full comparison.

  • E88.4— Excludes2 relationship (Informational)

    Distinct conditions — both may be reported.

    A distinct condition — both may be reported when both are documented.

    What moves you there: Documentation of mitochondrial metabolic disorders adds E88.4; it is not part of this code and both may be reported when both are documented.

    Full comparison: Q99.81 vs E88.4 →

Relationships are read from this code's own official notes, its laterality siblings and its encounter family; the wording of each distinction is MedCoder editorial, and the documentation named in each “what moves you there” line is the official note phrase or sibling title itself. Source: CMS ICD-10-CM tabular instructional notes; ICD-10-CM Official Guidelines, Section I.A.12.a.

Coding context

Guidelines, coding notes, decision aids, relationships (with MS-DRG and CCSR classification), hierarchy, HCC, coverage and the context map: what a coder reaches for after the core. Each section names whether it is official source data, a MedCoder-derived relationship or MedCoder editorial.

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-derived 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

Reference

Index terms and tables, published questions and FAQ, every source behind this page with its release and checksum, the date-of-service check and the complete change history.

Common coding questions

MedCoder editorial

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 FY2027 tabular list, index and tables, effective October 1, 2026 Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v44.0 (October 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-derived 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 editorial 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.

Current data releases:ICD-10-CM FY2027 · ICD-10-PCS FY2027 · HCPCS October 2026 · MS-DRG v44 · Medicare Code Editor v44.0 · NCCI PTP Q4 2026 · MUE Q4 2026 · NCD code lists 2026-01 · LCD export September 28, 2026 · All releases and sources

Labels on this page: Official source data · MedCoder-derived relationship · MedCoder editorial explanation. How to read the labels · All data sources and release dates · CMS coding rules

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 FY2027. 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