Petrified Ear in Adrenal Insufficiency: Systematic Literature Review

Background: Adrenal insufficiency has been recognized as a condition linked to petrified ear. To further explore this issue, we conducted a review of the literature. Methods: The study was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines after pre-registration. Excerpta Medica, the National Library of Medicine, and Web of Science databases without language restrictions were used. Inclusion criteria comprised adrenal insufficiency and the presence of petrified ear. Data extraction included demographics, clinical and laboratory features, and outcome. Results: Thirty-six reports were identified, encompassing 40 cases: 38 males and 2 females, aged between 22 and 79 years. All cases exhibited bilateral petrified ears, with 18 cases of primary adrenal insufficiency and 20 cases of central insufficiency. The type of adrenal insufficiency was not specified in two cases. Sixteen patients had concurrent endocrine disorders. In primary adrenal insufficiency, petrified ear was typically (N = 13; 72%) detected two or more years after the endocrine diagnosis. In central adrenal insufficiency, auricular calcification was identified either prior to or at the time (N = 17; 85%) of the endocrine diagnosis. Petrified ear was never associated with hearing impairment and never improved with therapy. Conclusions: In adrenal insufficiency, petrified ear is always bilateral, affects adult males, occurs in both primary and central forms of the disease and does not improve on therapy. In primary insufficiency, this feature is mostly observed two years after the endocrine diagnosis, whereas in central cases, it is identified before or concurrently with the endocrine diagnosis.