2026 ICD-10-CM Diagnosis Code H47.22Hereditary optic atrophy

ICD-10-CM Codes›H00–H59›H46-H47›H47

ICD-10-CM H47.22
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

H47.22 is a billable ICD-10-CM diagnosis code for hereditary optic atrophy. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Neuro-ophthalmology.

Code Identity

ICD-10-CM Code
H47.22
Billable Status
Yes — Valid for Submission
Code Describes
Hereditary optic atrophy
Short Description
Hereditary optic atrophy
Same as the full description in the CMS dataset.
Parent Code
H47.2
Optic atrophy

Code Classification

ChapterH00–H59Diseases of the eye and adnexa
SectionH46-H47Disorders of optic nerve and visual pathways
CategoryH47Other disorders of optic [2nd] nerve and visual pathways
This CodeH47.22Hereditary optic atrophy

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Absence of teeth
  • Auditory neuropathy, optic atrophy syndrome
  • Autosomal dominant optic atrophy and cataract
  • Autosomal dominant optic atrophy and peripheral neuropathy syndrome
  • Autosomal dominant optic atrophy classic form
  • Autosomal dominant optic atrophy plus syndrome
  • Autosomal recessive bilateral optic atrophy
  • Autosomal recessive isolated optic atrophy
  • Autosomal recessive optic atrophy type 6
  • Autosomal recessive optic atrophy type 7
  • Autosomal recessive spastic ataxia, optic atrophy, dysarthria syndrome
  • Axonal neuropathy
  • Bilateral optic atrophy of eyes
  • Bilateral primary optic atrophy
  • CAMOS syndrome
  • Cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss syndrome
  • Childhood-onset autosomal dominant optic atrophy
  • Congenital atrophy of optic nerve
  • Diffuse atrophy of cerebrum
  • Dominant hereditary optic atrophy
  • Early-onset progressive diffuse brain atrophy, microcephaly, muscle weakness, optic atrophy syndrome
  • Early-onset X-linked optic atrophy
  • False anodontia
  • GAPO syndrome
  • Generalized dystonia
  • Genetic anomaly of leukocyte
  • Global brain atrophy
  • Hereditary bilateral optic atrophy
  • Hereditary left optic atrophy
  • Hereditary motor and sensory neuropathy with optic atrophy
  • Hereditary optic atrophy
  • Hereditary right optic atrophy
  • Infantile-onset axonal motor and sensory neuropathy, optic atrophy, neurodegenerative syndrome
  • Leber plus disease
  • Leber's optic atrophy
  • Left primary optic atrophy
  • MEPAN syndrome
  • Optic atrophy, ataxia, peripheral neuropathy, global developmental delay syndrome
  • Optic atrophy, intellectual disability syndrome
  • Pelger-Huët anomaly
  • Pelger-Huët cell
  • Right primary optic atrophy
  • Second cranial nerve finding
  • Severe X-linked intellectual disability Gustavson type
  • Short stature, optic nerve atrophy, Pelger-Huët anomaly syndrome
  • Spastic paraplegia, optic atrophy, neuropathy syndrome
  • SPOAN and SPOAN-related disorder
  • Tooth absent
  • Wolfram syndrome
  • Wolfram syndrome type 1
  • Wolfram syndrome type 2
  • X-linked optic atrophy

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Leber's optic atrophy

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Atrophy, atrophic(of)
      • Leber's optic (hereditary)
    • Atrophy, atrophic(of)
      • optic (nerve)
        • hereditary
    • Leber's
      • optic atrophy (hereditary)
    • Neuroretinopathy, hereditary optic

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR EYE006
Neuro-ophthalmology
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Wolfram Syndrome

    a hereditary condition characterized by multiple symptoms including those of diabetes insipidus; diabetes mellitus; optic atrophy; and deafness. this syndrome is also known as didmoad (first letter of each word) and is usually associated with vasopressin deficiency. it is caused by mutations in gene wfs1 encoding wolframin, a 100-kda transmembrane protein.

  • Hereditary Optic Atrophy

    a family of inherited disorders characterized by progressive loss of vision secondary to death of the retinal ganglion cell axons that comprise the optic nerve.

  • Leber Hereditary Optic Atrophy

    a hereditary disorder caused by mitochondrial mutations, resulting in the degeneration of the retinal ganglion cells and optic atrophy. it is characterized by an acute or subacute loss of central vision. it may initially affect one eye only, but eventually the central loss of vision becomes bilateral.

  • Acute Motor and Sensory Axonal Neuropathy|Acute Motor And Sensory Axonal Neuropathy|Acute Motor-Sensory Axonal Neuropathy|Acute Motor-Sensory Axonal Neuropathy

    a subtype of guillain-barre syndrome that targets sensory motor axons, and is characterized by acute onset of quadriparesis, distal sensory loss, areflexia, and respiratory insufficiency.

  • Acute Motor Axonal Neuropathy|AMAN

    a subtype of guillain-barre syndrome that targets motor axons, and is characterized by symmetric limb weakness, diffuse areflexia, facial and oropharyngeal muscle weakness, and respiratory insufficiency.

  • Axonal Neuropathy

    any nerve disorder affecting the axon of a nerve.

  • GAN wt Allele|GAN1|Giant Axonal Neuropathy (Gigaxonin) Gene|Gigaxonin wt Allele|KLHL16

    human gan wild-type allele is located in the vicinity of 16q24.1 and is approximately 65 kb in length. this allele, which encodes gigaxonin protein, is involved in both ubiquitination and neurofilament structure. mutation of the gene is associated with giant axonal neuropathy.

  • Giant Axonal Neuropathy

    a rare inherited disorder affecting the neurofilaments. it is caused by mutations in the gan gene. it is characterized by the presence of abnormally large nerve cell axons. signs and symptoms include difficulty walking, sensory disturbances, lack of motor coordination and abnormal reflexes in the limbs.

  • Spinocerebellar Ataxia, Autosomal Recessive, with Axonal Neuropathy 2|AOA2|Ataxia with Oculomotor Apraxia Type 2|SCAN2

    an autosomal recessive condition caused by mutation(s) in the setx gene, encoding probable helicase senataxin. it is characterized by juvenile onset progressive cerebellar ataxia, axonal sensorimotor peripheral neuropathy, and increased concentrations of serum alpha-fetoprotein. oculomotor apraxia is common, but is not always present.

Patient EducationClinical

Optic Nerve Disorders

The optic nerve is a bundle of more than 1 million nerve fibers that carry visual messages. You have one connecting the back of each eye (your retina) to your brain. Damage to an optic nerve can cause vision loss. The type of vision loss and how severe it is depends on where the damage occurs. It may affect one or both eyes.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert H47.22 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
377.16 Hereditary optic atrophy
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About H47.22Overview

Is H47.22 (Optic atrophy) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report hereditary optic atrophy on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of H47.22?

Under the General Equivalence Mappings, hereditary optic atrophy converts to ICD-9-CM 377.16 (hereditary optic atrophy). The mapping is a direct match.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.