Optic Nerve Disorders

In Optic Atrophy, the optic nerve appears very pale.

In Optic Atrophy, the optic nerve appears very pale.

The Optic Nerve, which contains 1.2 million fibers each, carries the neural impulses created by the retina to the brain to enable us to see. Disorders of the optic nerve are caused either by developmental (genetic or abnormal development) or acquired factors (trauma or disease). Optic nerve disorders will always impact vision in some way and can affect one or both eyes. The optic nerve fibers from each eye combine at the optic chiasm located above the pituitary gland, located in the skull about 1 inch behind the bridge of the nose. Defects to the optic nerve in front of the chiasm will affect vision in only that eye, while defects at the chiasm and beyond (toward the back of the head) will affect vision in both eyes. Optic nerve atrophy can be diagnosed during an examination of the eyes. In optic atrophy patients will show a:

  • Reduction in visual acuity
  • Loss or reduction in color vision (especially to red)
  • Pupil response abnormality (the pupil of the affected eye will not perform normally- called an afferent pupillary defect)
  • The Optic Nerve will appear pale in color

Low vision care can be very effective to help individuals with Optic nerve Disease to lead very normal lives. Vision loss from Optic Nerve Disease responds very well to magnification which can make reading, seeing signs, faces, the computer, TV and even driving a possibility. There is a wide range of optical, electronic and software magnification options to address virtually every need.

Every individual with Optic Nerve Disease should have a low vision examination by a doctor skilled in low vision rehabilitation to help identify the most appropriate options to enhance their visual functioning, academic and vocational potential and their personal quality of life.

Types of Optic Nerve Disorders

There are a number of optic nerve disorders that can be inherited or can occur due to abnormal development.

Congenital Optic Atrophy is usually hereditary. The milder form is autosomal dominant and has a gradual onset of deterioration in childhood but little progression thereafter. The more severe form is autosomal recessive and is present at birth or within 2 years; this form is accompanied by nystagmus.

Leber’s Hereditary Optic Neuropathy has an unclear mode of inheritance but is suspected of being X-linked, since it rarely occurs in women; optic neuropathy occurs more commonly in 20-30 year old males; usually some level of vision is retained however there are varying degrees of impairment, sometimes with marked vision loss.

Image of Optic Nerve

Optic Nerve Hypoplasia has a white rim of tissue around the smaller than usual optic nerve head. The circle shows the actual nerve size.

Optic Nerve Hypoplasia (ONH) is an undeveloped optic nerve due to a neurological insult early in the prenatal developmental period; the optic nerve has started to develop, but regresses. Visual acuity ranges from normal to severely impaired. 

ONH is most often (approximately 80%) present in both eyes (bilateral) but can also be in one eye (unilateral). Because the unilateral cases tend to have better vision, they are typically diagnosed at a later age than those with bilateral ONH.

Children diagnosed with ONH generally present with vision problems which include nystagmus (involuntary pendular movement of the eyes), which tends to develop at 1 to 3 months and/or strabismus (inability to align the eyes), that becomes noticeable during the first year of life.

Acquired Optic Atrophy may be due to vascular disturbances (occlusions of the central retinal vein or artery or arteriosclerotic changes within the optic nerve itself), may be secondary to degenerative retinal disease (e.g., papilledema or optic neuritis), may be a result of pressure against the optic nerve, or may be related to metabolic diseases (e.g., diabetes), trauma, glaucoma, or toxicity (to alcohol, tobacco, or other poisons). Loss of vision is the only symptom.

Optic Neuritis most commonly occurs in individuals with multiple sclerosis (MS). Up to 50% of patients with MS will develop an episode of optic neuritis, and vision loss is the presenting symptom of the disease in 20-30% of cases.

Optic neuritis is an inflammation of the optic nerve. It may affect the part of the nerve and disc within the eyeball (papillitis) or the portion behind the eyeball (retrobulbar optic neuritis) in which case the optic nerve may appear normal.

The onset of optic neuritis is usually in one eye (unilateral) rather than both eyes (bilateral). There usually is a marked but temporary decrease in vision for several days or weeks, and there is pain in the eye when it is moved. Single episodes generally do not result in permanent vision loss; however, multiple episodes may.

Optic neuritis typically affects young adults, especially females, ranging from 18–45 years of age. In the U.S. the prevalence of ON is estimated to be 115/100,000 population.

Visual symptoms include a sudden loss of vision (partial or complete), or sudden blurred or “foggy” vision, and pain on movement of the affected eye. The vision might also look blacked-out rather than blurry. Color vision can also be reduced (especially to red), with colors appearing washed-out when compared to the other eye.