Understanding Optic Nerve Hypoplasia (ONH)

Read this overview of Optic Nerve Hypoplasia (ONH) to learn about signs, symptoms and causes.  Also learn how you can take advantage of various assistance options, from support groups to the use of adaptive technology for the visually impaired.


What is Optic Nerve Hypoplasia?


Optic nerve hypoplasia is a congenital condition in which the optic nerve is underdeveloped in one eye or both. ONH accounts for approximately 25% of vision loss in infants, according to a report from the Oman Journal of Ophthalmology

You see things as your eyes receive impulses from the optic nerves. Your vision function is diminished when optic nerves are underdeveloped, as in the case of ONH. ONH patients may also have nystagmus, which is characterized by involuntary, abnormal eye movements. When you have ONH, you may barely receive light in one or both of your eyes and the amount of disability can range from minor to profound.

ONH was first described in medical literature in 1884. ONH is not inherited. 

Shape of a women in a dark room.
Those living with ONH may barely receive light in one or both eyes


Prevalence of Optic Nerve Hypoplasia


Researchers believe that optic nerve hypoplasia affects females and males at the same rate. The prevalence of this disease is approximately 1 in 10,000 children, according to the National Organization of Rare Diseases


Common Signs and Symptoms


While optic nerve hypoplasia is manifested at the time of birth, symptoms might not be evident until the infant reaches adolescence. Vision disturbance in one or both eyes is common. 

One sign of having ONH is an optic disk that is smaller than average. The optic disk is the part of the eye responsible for the “blind spot,” where there are no light-detecting cells because it is where retina nerve fibers meet to create the optic nerve, connecting from the back of the eye to the brain. An eye doctor can determine the size of a patient’s optic disc by investigating with an ophthalmoscope.

Read about this Florida hockey fan living with ONH who uses eSight to watch the games.


A report from the Children’s Hospital of Los Angeles indicates that patients with ONH sometimes lack a corpus callosum (or have an abnormal one), which connects the left and right sides of the brain. Or, patients may have a malformed septum pellucidum, which is a membrane that connects lateral ventricles in the brain. Neither of these defects will affect the function of the brain, but they do sometimes occur with ONH.

Children may experience delays in acquiring skills to coordinate their muscular and mental activity when they have ONH. In many cases, children with ONH are also diagnosed with autism.


Common Causes


The fundamental cause of optic nerve hypoplasia is not yet understood by science yet, according to the National Organization of Rare Diseases, which noted, “in most cases, the disorder appears to occur randomly for unknown reasons.” However, researchers do know that ONH is a congenital condition.


Options and Support for Optic Nerve Hypoplasia


Having a better understanding of optic nerve hypoplasia will naturally motivate you to find out about what options may be available. You can explore using software and low vision aids as well as locate a support group.

* Low Vision Apps

The smartphone that you carry with you everywhere can be a handy resource by installing low vision apps and the operating system may already come with options for disabled individuals. The iPhone, for example, includes VoiceOver technology that helps visually impaired users navigate more easily. BrightFocus Foundation offers an overview of apps for people with low vision to get started.

* Low Vision Aids

In addition to software, you may find benefit in using devices for the blind. For example, recent innovations in medical technology have led to the team at eSight developing the eSight class 1 medical device for enhancing vision.

It’s non-surgical solution that users wear to bring more visual information into the brain to compensate for visual gaps. It works by stimulating synaptic activity (the brain’s internal connections) from the patient’s existing photoreceptors (that receive visual information from the world). Of course, you will need to consult with experts to determine if this type of device is the right solution for you or your loved one. Read reviews and testimonials from others who have already tried the technology as you evaluate whether it is a good fit for you.

* Support Groups

Feeling isolated is common when dealing with a medical condition such as ONH. You can improve the situation by finding and joining a local support group. You can also find a support group by visiting the American Association for Pediatric Ophthalmology & Strabismus patient resources page. Sharing stories with others who are going through similar difficulties with optic nerve hypoplasia can inspire you as you weigh the options for potentially enhancing your vision with low vision glasses.


Advice from Professionals About Optic Nerve Hypoplasia


It’s understandable that you might be concerned about whether there could ever be a solution to a visual disorder like ONH. Doctors that you visit will say that there is no surgical or medical option to address their loss in vision. But with new low vision products on the market, there may be an answer to your particular situation.

When it comes to optic nerve hypoplasia, it’s a good idea to learn as much as you can about available options to enhance existing eyesight capability, ranging from low vision apps and low vision aids to joining a local support group. You should know that you’re not alone. If you have questions about low vision aids for optic nerve hypoplasia, help is a quick phone call away. Our professionals will be happy to go over the details of eSight technology. For more information, click here to connect with the team at eSight today.


Did you find this post helpful? Continue reading about ocular albinism here.

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