Hypogonadotropic eunuchoidism (H.E.) is a familial disorder characterized by selective gonadotropin deficiency and sexual infantilism. Cryptorchidism and olfactory dysfunction often accompany the hormonal abnormalities. An additional defect in testis, resulting in a lack of responsiveness to exogenous gonadotropin administration, has been suggested by the observations of some investigators, but not by those of others. The present study was designed to reassess whether men and women with H.E. do respond appropriately to exogenous gonadotropin stimulation. During acute studies, testosterone increments during a standard 4-day hCG stimulation test were compared in a group of 6 hypogonadotropic patients who had received exogenous gonadotropins until one month prior to study and in 5 normal men. Serum testosterone levels increased at least two-fold in each of 5 patients with scrotal testes as in normal men. However, no response was demonstrated in the sixth patient with H.E., a subject with bilateral cryptorchidism. During prolonged hCG administration (3 weeks-8 months) serum testosterone increased to normal adult male levels in each of 9 hypogonadotropic men with scrotal testes. In contrast, serum testosterone levels increased only minimally in four additional patients with bilateral cryptorchidism who received hCG for 3–8 weeks. Finally, three women with this disorder responded to gonadotropin therapy by increased estrogen production and by ovulation. It is concluded that both men and women with H.E. can respond appropriately to gonadal stimulation unless bilateral cryptorchidism is present.
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Santen et al. (1973) studied this question.