DIHYDROTESTOSTERONE REGULATION OF SEMEN IN MALE PSEUDOHERMAPHRODITES WITH 5-ALPHA-REDUCTASE-2 DEFICIENCY

Semen analyses were performed in nine male pseudohermaphrodites with i nherited 5 alpha-reductase-2 deficiency and decreased dihydrotestoster one (DHT) production. The semen samples were characterized by extremel y low volume (range, <0.05 to 1.0 mL), increased viscosity, and poor l iquefaction. Surgical correction of pseudovaginal perineoscrotal hypos padias in four subjects did not result in an increase in semen volume or a change in viscosity. Inexplicably, semen liquefaction reverted to normal. Affected males have rudimentary prostates and small seminal v esicles. Six subjects had bilaterally descended testes, one subject ha d bilaterally retractile testes, and two subjects had unilaterally und escended testes. Semen from one subject with bilaterally descended tes tes had a normal sperm concentration, normal total sperm count, and no rmal motility and morphology. Semen from another subject who was oligo spermic at baseline demonstrated a normal sperm concentration after hy pospadias repair, with a low total sperm count. The other subjects stu died were oligospermic or azospermic. In summary, DHT appears to regul ate semen volume and viscosity through its action on the development a nd function of the prostate and seminal vesicles. The finding of norma l sperm concentrations in two subjects with 5 alpha-reductase-2 defici ency suggests that DHT does not play a major role in spermatogenesis. However, the possibility that low levels of DHT might be sufficient fo r normal spermatogenesis must also be considered.