34. Marfan's Syndrome Arachnodactyly, ectopia lentis, and an appearance similar to that of the homocystinuria

Hunter's syndrome has a similar habitus but does not show corneal clouding. It is X-linked, so the disease is expressed only in males. The onset of signs and symptoms usually occurs around age 2, and most patients die by the age of 20. However, some have lived into their sixties. About 50% of these cases are accompanied by progressive hearing loss. The loss usually is not severe (159) and is most commonly mixed or sensorineural.