ICD-10-CM Diagnosis Code Q99.818 - Other Usher syndrome (original) (raw)

ICD List Logo

ICD List 2025-2026 Edition

  1. Home
  2. ICD-10-CM Codes
  3. Q00-Q99
  4. Q90-Q99
  5. Q99
  6. 2026 ICD-10-CM Code Q99.818

Other Usher syndrome

ICD-10-CM Code:

Q99.818

ICD-10 Code for:

Other Usher syndrome

Is Billable?

Yes - Valid for Submission

Code Navigator:

Q99.818 is a billable diagnosis code used to specify a medical diagnosis of other usher syndrome. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2025 through September 30, 2026. The code is exempt from present on admission (POA) reporting for inpatient admissions to general acute care hospitals.

  1. Code Information
  2. Clinical Information
  3. 2026 ICD-10 Code
  4. Tabular List of Diseases and Injuries
  5. Diagnostic Related Groups Mapping
  6. Present on Admission (POA)
  7. Replacement Code
  8. Patient Education
  9. Other Codes Used Similar Conditions
  10. Code History

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.

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.

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.

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.

this gene plays a role in both sight and hearing.

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.

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.

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.

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

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.

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.

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

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.

Q99.818 is new to ICD-10-CM code set for the FY 2026, effective October 1, 2025. The National Center for Health Statistics (NCHS) has published an update to the ICD-10-CM diagnosis codes which became effective October 1, 2025. This is a new and revised code for the FY 2026 (October 1, 2025 - September 30, 2026).

Q99.818 is exempt from POA reporting - The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement. Review other POA exempt codes here.

CMS POA Indicator Options and Definitions

POA Indicator: Y

Reason: Diagnosis was present at time of inpatient admission.

CMS Pays CC/MCC DRG? YES

POA Indicator: N

Reason: Diagnosis was not present at time of inpatient admission.

CMS Pays CC/MCC DRG? NO

POA Indicator: U

Reason: Documentation insufficient to determine if the condition was present at the time of inpatient admission.

CMS Pays CC/MCC DRG? NO

POA Indicator: W

Reason: Clinically undetermined - unable to clinically determine whether the condition was present at the time of inpatient admission.

CMS Pays CC/MCC DRG? YES

POA Indicator: 1

Reason: Unreported/Not used - Exempt from POA reporting.

CMS Pays CC/MCC DRG? NO

Q99818 replaces the following previously assigned ICD-10-CM code(s):