- Males: 1 in 7500
- Females: 1 in 50,000
- Secondary to LHRH deficiency (GnRH deficiency)
- Results in isolated Hypogonadotropic Hypogonadism
- Kallmann's Syndrome is most common cause
- Partial LHRH deficiency results in fertile eunuch
- Anosmia or Hyposmia
- Delayed Puberty
- Small Testes (size correlates with LHRH deficiency)
- Micropenis (50% of cases)
- Other findings seen in Hypogonadotropic Hypogonadism
- Cleft Lip and Palate
- Seizure Disorder
- Short Metacarpals
- Pes cavus
- Sensorineural Hearing Loss
- Cerebellar Ataxia
- Wilson (1998) Williams Endocrinology, p. 1555-6