ICD-10-CM Code H55.01

Congenital nystagmus

Version 2021 Billable Code

Valid for Submission

H55.01 is a billable code used to specify a medical diagnosis of congenital nystagmus. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code H55.01 might also be used to specify conditions or terms like 2q partial trisomy syndrome, autosomal recessive cerebellar ataxia, pyramidal signs, nystagmus, oculomotor apraxia syndrome, congenital esotropia, congenital esotropia, congenital nystagmus, congenital nystagmus, etc

Short Description:Congenital nystagmus
Long Description:Congenital nystagmus

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code H55.01 are found in the index:


The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • 2q partial trisomy syndrome
  • Autosomal recessive cerebellar ataxia, pyramidal signs, nystagmus, oculomotor apraxia syndrome
  • Congenital esotropia
  • Congenital esotropia
  • Congenital nystagmus
  • Congenital nystagmus
  • Congenital nystagmus with sensory abnormality
  • Congenital nystagmus without sensory abnormality
  • Epiphyseal dysplasia, microcephalus, nystagmus syndrome
  • Esotropia with nystagmus
  • Esotropia with nystagmus block
  • Partial trisomy of chromosome 2
  • Pendular nystagmus
  • Syndactyly, nystagmus syndrome due to 2q31.1 microduplication

Clinical Information

  • NYSTAGMUS CONGENITAL-. nystagmus present at birth or caused by lesions sustained in utero or at the time of birth. it is usually pendular and is associated with albinism and conditions characterized by early loss of central vision. inheritance patterns may be x linked autosomal dominant or recessive. adams et al. principles of neurology 6th ed p275

Diagnostic Related Groups

The ICD-10 code H55.01 is grouped in the following groups for version MS-DRG V38.0 What are Diagnostic Related Groups?
The Diagnostic Related Groups (DRGs) are a patient classification scheme which provides a means of relating the type of patients a hospital treats. The DRGs divides all possible principal diagnoses into mutually exclusive principal diagnosis areas referred to as Major Diagnostic Categories (MDC).
applicable from 10/01/2020 through 09/30/2021.


Convert H55.01 to ICD-9

Code Classification

  • Diseases of the eye and adnexa (H00–H59)
    • Other disorders of eye and adnexa (H55-H57)
      • Nystagmus and other irregular eye movements (H55)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021

Information for Patients

Eye Movement Disorders

When you look at an object, you're using several muscles to move both eyes to focus on it. If you have a problem with the muscles, the eyes don't work properly.

There are many kinds of eye movement disorders. Two common ones are

  • Strabismus - a disorder in which the two eyes don't line up in the same direction. This results in "crossed eyes" or "walleye."
  • Nystagmus - fast, uncontrollable movements of the eyes, sometimes called "dancing eyes"

Some eye movement disorders are present at birth. Others develop over time and may be associated with other problems, such as injuries. Treatments include glasses, patches, eye muscle exercises, and surgery. There is no cure for some kinds of eye movement disorders, such as most kinds of nystagmus.

  • Cranial mononeuropathy III (Medical Encyclopedia)
  • Cranial mononeuropathy VI (Medical Encyclopedia)
  • Eye muscle repair (Medical Encyclopedia)
  • Nystagmus (Medical Encyclopedia)
  • Strabismus (Medical Encyclopedia)
  • Supranuclear ophthalmoplegia (Medical Encyclopedia)

[Learn More]

X-linked infantile nystagmus X-linked infantile nystagmus is a condition characterized by abnormal eye movements. Nystagmus is a term that refers to involuntary side-to-side movements of the eyes. In people with this condition, nystagmus is present at birth or develops within the first six months of life. The abnormal eye movements may worsen when an affected person is feeling anxious or tries to stare directly at an object. The severity of nystagmus varies, even among affected individuals within the same family. Sometimes, affected individuals will turn or tilt their head to compensate for the irregular eye movements.
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