What’s not working is the optic nerve which functions similarly to a wire that transmits information from the eye to the brain and back. With LHON the cells in the optic nerve are overdoing it on apoptosis—preprogrammed cell death—too many cells are committing suicide, causing the optic nerve to atrophy. It’s like a TV cord that’s been chewed by a mouse. Since optic nerve cells can’t regenerate, the ‘cord’ can’t be fixed (though some with LHON are fortunate in that their optic nerve cells don’t die, they’re just sick, hence there can be spontaneous recovery in some cases).
Someone affected by LHON may not “look blind,” and if they don’t use a cane or a guide dog, people may not understand their problems. When people walk by and the person with LHON vision “ignores them,” or doesn’t say hi back because they can’t see the person who said “hello”, it’s awkward for everyone. Teaching friends to say “hi, this is so-and-so” upon approaching someone with LHON vision, and encouraging other friends ‘in the know’ to tell the person with LHON vision someone they know is approaching, can be a big help in overcoming this awkwardness.
Since those with LHON vision usually have some peripheral vision, they tend to use that peripheral vision to see. They may look straight ahead so that others don’t find their gaze disconcerting, yet will look upward or to the side to really see.