Optic Nerve Hypoplasia and Blindness

by Mar 27, 2023

Optic nerve hypoplasia is an inherited eye condition that severely impacts vision due to abnormalities in the retina and optic nerve. Typically optic nerve hypoplasia affects both eyes, although it may be slightly more severe in one eye than the other. Symptoms of optic nerve hypoplasia include extremely poor vision, nystagmus, and sensitivity to light. There is no treatment for optic nerve hypoplasia but low-vision services and devices can assist with improving daily activities.


What is the Optic Nerve?

The optic nerve is the main nerve that connects the eye to the brain and is responsible for collecting information and creating vision.

The optic nerve is located at the back of the eye in the retina, which is the back layer of the eye that is filled with photoreceptors.

In a healthy eye, the optic nerve has a large ring of visible nerve tissue that forms a circle around the core of the optic nerve that travels from the eye to the brain.

The optic nerve is the location of many eye conditions that can lead to blindness or otherwise very poor vision because if the optic nerve is damaged, vision in that eye will be severely reduced.

Conditions such as glaucoma, optic neuritis, and optic nerve hypoplasia can cause this reduced vision by damaging the optic nerve.


How Optic Nerve Hypoplasia Occurs

Optic nerve hypoplasia is a congenital malformation of the optic nerve and the nerve fibers around the optic nerve.

As a fetus, the optic nerve develops from fetal nervous tissue and forms what will eventually be the visible head of the optic nerve in the retina.

However, if this fetal nervous tissue does not form and grow properly, the optic nerve can be underdeveloped and cause optic nerve hypoplasia.

The nerve tissue which would typically carry visual information from the eyes to the brain is absent and the eye is left with diminished visual abilities and a poor prognosis.


Symptoms of Optic Nerve Hypoplasia

Optic nerve hypoplasia is often diagnosed very early on in childhood due to the lack of visual development and early onset of nystagmus.

In infants, vision develops quickly and begins to guide the child’s other behavioral development.

However, if optic nerve hypoplasia is present, vision is so poor that behavioral development is impacted.

Additionally, the eyes may develop a rhythmic shaking back and forth called nystagmus. The nystagmus is usually an adaptation to try to seek out any possible visual input in a very poor-seeing eye.

Early nystagmus is often enough to warrant an eye examination and may lead to the diagnosis of optic nerve hypoplasia.


Low Vision Services for Optic Nerve Hypoplasia

Low vision is usually the best treatment option for an individual with optic nerve hypoplasia. These providers can help identify areas of living that can be improved with adaptive equipment or devices.

Low-vision services may be available from the government or other organizations that support those with severely reduced vision.


Our eye doctors at Neal Eye Group in Conshohocken, PA excel in the prescription of contact lenses, glasses and various eye diseases.  Call our optometrists at (610) 828-9701 or schedule an appointment online if you would like to learn more about Optic Nerve Hypoplasia. Our optometrists provide the highest quality optometry services and eye exams in Conshohocken, Norristown, Plymouth Meeting, Lafayette Hill, and Philadelphia.

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