Petrified Ear Auricles with Isolated Adrenocorticotropic Hormone Deficiency

A 22-year-old man was hospitalized with nausea. His serum sodium, adrenocorticotropic hormone (ACTH), and cortisol levels were 108 mmol/L (range, 138-145), 2.2 pg/mL (range, 7.2-63.3), and 0.08 μg/dL (range, 4.64-24.56), respectively. The levels of other anterior pituitary hormones were as follows: growth hormone 2.46 ng/mL (range, 0.663.68), thyroid-stimulating hormone 0.625 μIU/mL (range, 0.50-5.00), prolactin 8.26 ng/mL (range, 3.1-20.5), luteinizing hormone 8.12 mIU/mL (range, 1.71-8.59), and folliclestimulating hormone 5.88 mIU/mL (range, 1.5-12.4). An anterior pituitary stimulation test revealed that ACTH reactivity to corticotropin-releasing hormone was selectively impaired (plasma hormone levels at 60 minutes after intravenous injection of 100 μg corticotropin-releasing hormone: ACTH 2.7 pg/mL and cortisol 0.03 μg/dL). Adequate responses were observed for all other pituitary hormones. The patient was diagnosed with an isolated ACTH deficiency. Further physical examinations revealed inflexible stiffness in both ears. X-ray of the lateral skull view showed double auricular edges (Picture 1). Three-dimensional computed tomography revealed petrified auricles of both ears (Picture 2). After hydrocortisone supplementation for six years, all symptoms improved, except for ear stiffness. Adrenal insufficiency is a frequent systemic disorder associated with calcification of the auricular cartilage, which results from a chronic cortisol deficiency (1). Auricular petrifaction has also been reported in patients with primary and secondary adrenal insufficiency, suggesting that excessive ACTH is not a causative factor in auricular petrifaction (2). Although the pathogenesis remains unclear, adrenal insufficiency should be considered when patients present with petrified ear auricles. The presence of double auricular edges on X-ray in the lateral skull view is a major sign of petrified ear auricles.