Visible features of the condition include:[1] Iris atrophy and glaucoma are more common than average.

[1] The size of the eyes often interferes with learning to read; special eyeglasses may be required.

The neurologic changes may appear earlier in each subsequent generation[3] and can include abnormal white matter, conductive deafness, and various kinds of paresis,[4] including ataxia, spastic paraplegia, difficulty controlling the eyes, and bladder and bowel disturbances.

[5][6][7] ODD is typically an autosomal dominant condition, but can be inherited as a recessive trait.

[8] It is generally believed to be caused by a mutation in the gene GJA1, which codes for the gap junction protein connexin 43.