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  6. 2026 ICD-10-CM Code Q99.81

2026 ICD-10-CM Diagnosis Code Q99.81

Usher syndrome

ICD-10-CM Code:
Q99.81
ICD-10 Code for:
Usher syndrome
Is Billable?
Not Valid for Submission
Code Navigator:

Q99.81 is a non-specific and non-billable diagnosis code code, consider using a code with a higher level of specificity for a diagnosis of usher syndrome. The code is not specific and is NOT valid for the year 2026 for the submission of HIPAA-covered transactions. Category or Header define the heading of a category of codes that may be further subdivided by the use of 4th, 5th, 6th or 7th characters.

Specific Coding Applicable to Usher syndrome

Non-specific codes like Q99.81 require more digits to indicate the appropriate level of specificity. Consider using any of the following ICD-10-CM codes with a higher level of specificity when coding for usher syndrome:

  • Use Q99.811 for Usher syndrome, type 1 - BILLABLE CODE

  • Use Q99.812 for Usher syndrome, type 2 - BILLABLE CODE

  • Use Q99.813 for Usher syndrome, type 3 - BILLABLE CODE

  • Use Q99.818 for Other Usher syndrome - BILLABLE CODE

  • Use Q99.819 for Usher syndrome, unspecified - BILLABLE CODE

Code Classification

  • Congenital malformations, deformations and chromosomal abnormalities
    Q00-Q99
    • Chromosomal abnormalities, not elsewhere classified
      Q90-Q99
      • Other chromosome abnormalities, not elsewhere classified
        Q99

Clinical Information

  • Clarin-1|CLRN1|Clarin 1|Usher Syndrome Type-3 Protein

    clarin-1 (232 aa, ~26 kda) is encoded by the human clrn1 gene. this protein is involved in the organization of the cytoskeleton in photoreceptor and auditory receptor cells.

  • CLRN1 wt Allele|Clarin 1 wt Allele|RP61|USH3|USH3A|Usher Syndrome 3A Gene

    human clrn1 wild-type allele is located in the vicinity of 3q25.1 and is approximately 47 kb in length. this allele, which encodes clarin-1 protein, plays a role in sight and hearing. mutations in the gene are associated with usher syndrome 3a and retinitis pigmentosa 61.

  • HARS1 wt Allele|CMT2W|HARS|HRS|Histidine Translase Gene|Histidine tRNA Ligase 1, Cytoplasmic Gene|Histidyl-tRNA Synthetase 1 wt Allele|Histidyl-tRNA Synthetase Gene|Jo-1 Antigen Gene|USH3B|Usher Syndrome 3B Gene

    human hars1 wild-type allele is located in the vicinity of 5q31.3 and is approximately 19 kb in length. this allele, which encodes histidine-trna ligase, cytoplasmic protein, is involved in trna aminoacylation. mutation of the gene is associated with axonal charcot-marie-tooth disease type 2w and usher syndrome type 3b.

  • MYO7A wt Allele|DFNA11|DFNB2|MYOVIIA|MYU7A|Myosin VIIA (Usher Syndrome 1B (Autosomal Recessive, Severe)) Gene|Myosin VIIA wt Allele|Myosin, Unconventional Family VII, Member A Gene|NSRD2|USH1B

    human myo7a wild-type allele is located in the vicinity of 11q13.5 and is approximately 87 kb in length. this allele, which encodes unconventional myosin-viia protein, plays a role in intracellular transport affecting sight and hearing. mutations in the gene are associated with autosomal dominant deafness 11, autosomal recessive deafness 2 and usher syndrome type 1b.

  • USH1G Gene|USH1G|USH1G|Usher Syndrome 1G (Autosomal Recessive) Gene

    this gene plays a role in both sight and hearing.

  • USH1G wt Allele|ANKS4A|FLJ33924|SANS|Sans|Usher Syndrome 1G (Autosomal Recessive) wt Allele

    human ush1g wild-type allele is located in the vicinity of 17q25.1 and is approximately 7 kb in length. this allele, which encodes usher syndrome type-1g protein, is involved in the development of both the retina and cochlear hair cells. mutation of the gene is associated with usher syndrome 1g.

  • USH2A wt Allele|RP39|US2|USH2|Usher Syndrome 2A (Autosomal Recessive, Mild) Gene|Usherin wt Allele|dJ1111A8.1

    human ush2a wild-type allele is located in the vicinity of 1q41 and is approximately 801 kb in length. this allele, which encodes usherin protein, plays a role in hearing and sight. mutations in the gene are associated with retinitis pigmentosa 39 and usher syndrome type 2a.

  • Usher Syndrome

    a rare, autosomal recessive inherited syndrome caused by mutations in the cdh23, clrn1, gpr98, myo7a, pcdh15, ush1c, ush1g, and ush2a genes. it is characterized by hearing loss or deafness and progressive loss of vision. the loss of vision is the result of retinitis pigmentosa.

  • Usher Syndrome Type 1

    a syndrome characterized by congenital, bilateral, severe sensorineural hearing loss, abnormalities in the vestibular system, and adolescent-onset retinitis pigmentosa.

  • Usher Syndrome Type 2

    a syndrome characterized by congenital, bilateral sensorineural hearing loss that is mild to moderate in the low frequencies and severe to profound in the higher frequencies, no abnormalities in the vestibular system, and retinitis pigmentosa.

  • Usher Syndrome Type 2C|USH2C

    an autosomal recessive sub-type of usher syndrome caused by homozygous or compound heterozygous mutation(s) in the adgrv1 gene, encoding adhesion g protein-coupled receptor v1. it may also result from biallelic digenic mutation(s) in adgrv1 and pdzd7, which encodes pdz domain-containing protein 7.

  • Usher Syndrome Type 3

    a syndrome characterized by postlingual progressive hearing loss, abnormalities in the vestibular system, and onset of retinitis pigmentosa symptoms usually by the second decade of life.

  • Usher Syndrome Type-1G Protein|Scaffold Protein Containing Ankyrin Repeats and SAM Domain|USH1G

    usher syndrome type-1g protein (461 aa, ~51 kda) is encoded by the human ush1g gene. this protein plays a role in retinal and cochlear development.

  • Usherin|USH2A|Usher Syndrome Type IIa Protein|Usher Syndrome Type-2A Protein

    usherin (5202 aa, ~576 kda) is encoded by the human ush2a gene. this protein is involved in the functionality of cochlear hair cells and retinal photoreceptor cells.

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.


Use Additional Code

Use Additional Code
The “use additional code” indicates that a secondary code could be used to further specify the patient’s condition. This note is not mandatory and is only used if enough information is available to assign an additional code.
  • code to identify any auditory and visual manifestations

Code History

  • FY 2026 - No Change, effective from 10/1/2025 through 9/30/2026