A person with ONH has optic nerves which are small and poorly developed. Instead
of having over 1 million connections (nerve fiber) from each eye to the brain,
people with ONH have far fewer connections. The more connections between the
eye and brain the better vision. Some people with ONH have near normal
vision in one eye, others have decreased vision in both eyes, and others are severely
affected and nearly blind.

An eye doctor (ophthalmologist) can diagnose optic nerve hypoplasia by looking inside the eye with an ophthalmoscope. The front of the optic nerve (optic disc) appears smaller than normal.

Your child will undergo a number of eye examinations to determine his or her vision. The younger the child the more difficult it is to tell the amount of vision present. Depending on your child's ability to cooperate with the examination, an experienced ophthalmologist can usually tell your child's ability to see. However this usually can not be done with children under the age of 3-4 years. It takes a number of years to be able to tell what a child's vision will be like. The vision does not usually worsen over time from ONH. It may, however, improve over time.

Most children with ONH have unusual eye movements (nystagmus). They may have eyes that seem to move around with no real pattern or purpose. This occurs because the eyes are not able to focus well enough to hold still; this pattern is often seen with children who have poor vision.

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