Androglottia in a young female adolescent with congenital adrenal hyperplasia and 21-hydroxylase deficiency

J Pediatr Endocrinol Metab. 2000 Jul-Aug;13(7):959-62. doi: 10.1515/jpem.2000.13.7.959.

Abstract

Vocal disturbances in women with congenital adrenal hyperplasia and androgen excess should be extremely rare today since effective substitution with glucocorticoids is available. We present a 17 year-old female with congenital adrenal hyperplasia due to 21-hydroxylase deficiency and severe virilization because of long-term insufficient therapy. Laboratory data showed elevated serum levels of testosterone, 17-hydroxyprogesterone, plasma ACTH and a high excretion of urinary pregnanetriol. The phoniatric aspect showed a masculine voice. We discuss the different effects of androgens on the pubertal larynx and various hormonal disturbances that may cause voice changes as well as therapeutic options of voice therapy. From the pediatric point of view it might be important to perform a phoniatric examination in girls with congenital adrenal hyperplasia during puberty in order to monitor androgen effects.

MeSH terms

  • 17-alpha-Hydroxyprogesterone / blood
  • Adolescent
  • Adrenal Hyperplasia, Congenital / blood
  • Adrenal Hyperplasia, Congenital / complications*
  • Adrenal Hyperplasia, Congenital / enzymology
  • Adrenocorticotropic Hormone / blood
  • Female
  • Humans
  • Pregnanetriol / urine
  • Steroid 21-Hydroxylase / genetics
  • Steroid 21-Hydroxylase / metabolism*
  • Testosterone / blood
  • Voice Disorders / complications*
  • Voice Disorders / physiopathology

Substances

  • Pregnanetriol
  • Testosterone
  • 17-alpha-Hydroxyprogesterone
  • Adrenocorticotropic Hormone
  • Steroid 21-Hydroxylase