"Petrified ears" with idiopathic adult-onset pituitary insufficiency.

"Petrified ears" or calcification of auricular cartilage is an uncommonly reported condition. The most common causes of this phenomenon are local trauma, frost bite, and inflammation. Adrenal insufficiency is the most frequent systemic disease associated with auricular calcification. We present a case of idiopathic adult-onset pituitary insufficiency with hypocortisolism and bilateral auricular calcification. Recognition of the association between auricular calcification and adrenal insufficiency can be an important step toward the identification of a life-threatening cortisol deficiency.

INTRODUCTION

“Petrified ears” or calcification of auricular cartilage is an uncommonly reported condition. It is characterized by the development of stony-hard auricular cartilage of one or both the ears without visible changes in the appearance of the ear. The most common causes of this phenomenon are local trauma, frost bite, and inflammation.[1] Adrenal insufficiency is the most frequent systemic disease leading to the development of stiffening of the auricular cartilage.[1] We report such a case.

CASE REPORT

A 53-year-old male presented with 6 months history of chronic diarrhea, general weakness, easy fatiguability, cold intolerance, and deterioration in mental function. He also noted that his ears were “turning to stone” in the past 1 year. He had consulted numerous physicians for this complaint but to no avail. He had no family history of neurological disorders or any other disease. Physical examination disclosed pale skin, periorbital puffiness, absent pubic hair with soft testes, and Chovstek's negative with no carpopedal spasm. His ears were generally stiff. Neurological examination revealed absent ankle jerk with extensor plantar and positive Rhomberg's sign. Biochemistry showed serum sodium 128 mEq/L, potassium 3.3 mEq/L, calcium (corrected) 7.2 mEq/L, phosphate 1.8 mEq/L, and magnesium 1.5 mEq/L with normal renal function and liver function. Endocrine work-up revealed cortisol 0.20 μg/dl (normal 5–23 μg/dl), free T3 1.67 pg/ml (normal 2.3–4.2 pg/ml), free T4 0.69 ng/dl (normal 0.7–1.51 ng/dl), TSH 5.33 (normal 0.5–5 μU/ml), positive TPO (106.3 IU/ ml), prolactin 47 ng/ml (normal 4–30 ng/ml), testosterone 76 ng/dl (normal 241–827 ng/dl) with LH 1.74 mIU/ml (normal 1.50–9.30 mIU/ml), and FSH 1.43 mIU/ml (normal 1.40–18.10 mIU/ml). There was no evidence of central diabetes insipidus (no polyuria, serum osmolality 285 mOsm/kg, urine osmolality 235 mOsm/kg). He was vitamin D insufficient with 25-hydroxy vitamin D level of 26 ng/ml (30–74 ng/ml). iPTH level was 20 pg/ml (10– pg/ml) Contrast-enhanced MRI sella revealed partially empty sella with normal stalk. Vitamin B12 levels were low–normal with a value of 311 pg/ml (normal 187–1059 pg/ml). Pernicious anemia screen was undertaken, his antiparietal cell antibody was positive, and biopsy revealed nonspecific gastritis. His serology for retroviral disease, syphilis, and rest of the autoimmune markers was negative. Non-contrast CT head revealed auricular calcification but no basal ganglia calcification. Patient also had dilated cardiomyopathy with global hypokinesia and ejection fraction of 35–40%. With this clinical and biochemical profile, a diagnosis of panhypopituitarism possibly due to adult-onset pituitary insufficiency, subacute combined degeneration, and dilated cardiomyopathy was considered. Patient was treated with IV hydrocortisone, l-thyoxine and testosterone replacement, vitamin B12 supplementation, ACE inhibitor, and beta-blocker. He experienced a rapid recovery. The patient remains well, but his ears remain unchanged [Figures 1–3].

Figure 1

Non Contrast CT scan of the head showing auricular calcification

Figure 3

MRI sella showing the partially empty sella

Petrified ears-stiff ears but with no visible changes

DISCUSSION

We describe a patient of idiopathic adult-onset pituitary insufficiency with associated auricular calcification. Idiopathic adult-onset pituitary insufficiency usually follows as a sequel to head injury, subarachnoid hemorrhage, cerebrovascular accident, postradiation, and lymphocytic hypophysitis. These other causes of nontumoral panhypopituitarism in adults like head injury, SAH, and CVA were carefully excluded in the index patient. The presumptive diagnosis in this patient leading to idiopathic adult-onset pituitary insufficiency seems to be lymphocytic hypophysitis. The patient had panhypopituitarism. He presented with ACTH deficiency, which is a universal feature of lymphocytic hypophysitis and had hyperprolactinemia suggestive of stalk involvement. The presence of normal sized empty sella virtually excludes the presence of pituitary adenoma; also a background history of autoimmunity (positive TPO antibodies and antiparietal cell antibody) suggests the diagnosis of lymphocytic hypophysitis even in the absence of tissue diagnosis. These other causes of nontumoral panhypopituitarism in adults like head injury, SAH, and CVA were carefully excluded in the index patient. Lymphocytic hypophysitis is usually a disease of women, particularly in peripartum period. The usual presentation is with ACTH deficiency, diabetes insipidus, and hyperprolactinemia. Diagnosis is usually based on a combination of clinical constellation of symptoms and signs with MRI findings and underlying features suggestive of autoimmunity, as was seen in our patient.

Tissue diagnosis is not a prerequisite for lymphocytic hypophysitis as “No—Autoimmune Hypophysitis cannot be diagnosed with certainty without pituitary biopsy” but also “No—Pituitary biopsy is not always clinically necessary for effective clinical management of the patient.”[2] Panhypopituitarism with hyperprolactinemia and concurrent autoimmunity were strong pointers to the diagnosis of lymphocytic hypophysitis even in the absence of tissue diagnosis. With other diagnoses effectively excluded, especially when patient was clinically well on simple replacement therapy, biopsy was hard to justify, hence biopsy was not undertaken.

Calcification of the auricular cartilage was first recognized as early as 1866 by Bochdalek.[3] Since then, for more than a century this cutaneous condition has been reported under a variety of terms. Although it seems that the disease is infrequently seen in clinical practice, the true incidence of the condition is a matter of debate. In 1932, Scherrer[4] examined 800 apparently healthy patients and no evidence of calcification was documented. In 1963, in a randomized study of a series of 300 patients, Gordon[1] examined for inflexibility of the ears. He found radiological evidence of calcification of the ear cartilage in 11 patients (3%). More recently, in 1998, Bowers and Gould[5] reported that auricular calcification is found more frequently in older people, especially those who have worked outdoors.

Calcification of soft tissues may be the result of dystrophic calcification or metastatic calcification. Dystrophic calcification is due to deposition of the mineral in damaged tissue. The auricle of the ears is vulnerable to local trauma and frost bite, which are the common etiological factors in the hardening of the auricular cartilage,[1] but such history of previous trauma or frost bite was not evident in our case.

Metastatic calcification is deposition of mineral in normal soft tissue due to presence of high calcium–phosphorous product in the serum. Metastatic calcification may be a causative factor, and hypercalcemia may be a feature of adrenal insufficiency.[6] The uniqueness in presentation of our patient was that both his serum calcium and phosphorous levels were below normal which has not been reported so far. The level of calcium was either high[6] or normal[7] in the cases reported so far. Neither did the index patient have acute signs of hypocalcemia nor did he have features suggestive of chronic hypocalcemia in the form of cataract or basal ganglia calcification. He was vitamin D insufficient with normal level of the parathyroid hormone.

Adrenal insufficiency is the most frequent systemic disease leading to the development of stiffening of the auricular cartilage.[1] Jarvis et al.[8] reported a high incidence of this phenomenon in patients with Addison's disease, with varying severity from moderate stiffness to stony hard ears. The etiology of this phenomenon in adrenal insufficiency is not clear. Long-term 11-desoxycorticosterone acetate (DOCA) therapy was implicated,[9] but this is unlikely, as stiffening of the auricular cartilage has been found in patients who like our patient had never received DOCA.[78] As calcification of auricular cartilage has been described in patients with both primary adrenal insufficiency as well as in secondary adrenal insufficieny and adrenogenital syndrome,[78] its etiology is believed to be cortisol deficiency.[7] This was indeed evident in our patient.

The exact pathophysiologic mechanism of hypocortisolism-related calcification of the elastic cartilage remains uncertain. Of note, calcification of external ear has not been reported in patients receiving adequate replacement of glucocorticoids.[7] As auricular calcification has only been described in males, gonadal steroids may play a role.[7]

Other endocrinopathies reported with auricular calcification are diabetes mellitus, hypothyroidism, and acromegaly.[10] Systemic diseases associated with the condition are familiar cold hypersensitivity, hypertension, alkaptonuria, scleroderma, and systemic chondromalacia.[11112]

Most cases are asymptomatic hence there are no guidelines for treatment of this condition. An improvement has been reported after conchal reduction by surgery.[13]