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Early Childhood Intervention
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Optic Nerve Hypoplasia
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The optic nerve serves as a connector, carrying visual
information from the eye to the brain. Those that have optic nerve
hypoplasia (ONH) have an underdeveloped optic nerve in one or both
eyes. Typically each optic nerve has about 1 million connections.
However, those with optic nerve hypoplasia have far fewer
connections. Optic nerve hypoplasia is one of the three most
frequently diagnosed visual impairments in children in the United
States.
Often associated with optic nerve hypoplasia are hormonal deficiencies.
Therefore, it is critical for a child to be seen by an endocrinologist at
the time of diagnosis and then followed up regularly as hormonal
deficiencies can develop at any time. The ophthalmologist may also recommend
an MRI and/or CT to check for any brain abnormalities, which are also
associated with ONH. In some cases children have a poorly formed or missing
septum pellucidum (area in the brain that divides the ventricles). This is
referred to as septo-optic dysplasia (SOD) or DeMorsier’s syndrome. Other
areas of the brain may also be affected.
Optic nerve hypoplasia is a congenital defect. It is
thought that there may be an exaggeration of the natural dying of nerve
fibers as the child develops in utero.
For the majority of cases there is no known cause for why this
condition occurs. A few cases
have been associated with young maternal age (under 20 years of age),
maternal diabetes, maternal alcohol use, and maternal anti-epileptic drug
use. ONH affects all races and socio-economic groups.
Learn more about
Optic Nerve
Hypoplasia under Visual
Development
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Diagnosis
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