|Year : 2020 | Volume
| Issue : 4 | Page : 248-249
Bilateral auricular ossificans with stenosis of external ear canals and hearing loss caused by primary hyperparathyroidism: A case report
Tzu-Kun Lo1, Chao-Kai Hsu1, Su-Ting Hsin2, Jiunn-Liang Wu3, Julia Yu-Yun Lee1
1 Department of Dermatology, National Cheng Kung University Hospital, Tainan, Taiwan
2 College of Medicine, National Cheng Kung University, Tainan, Taiwan
3 Department of Otolaryngology, National Cheng Kung University Hospital, Tainan, Taiwan
|Date of Submission||11-Feb-2020|
|Date of Decision||14-Apr-2020|
|Date of Acceptance||04-May-2020|
|Date of Web Publication||16-Dec-2020|
Dr. Julia Yu-Yun Lee
Department of Dermatology, National Cheng Kung University Hospital, Tainan
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Lo TK, Hsu CK, Hsin ST, Wu JL, Lee JY. Bilateral auricular ossificans with stenosis of external ear canals and hearing loss caused by primary hyperparathyroidism: A case report. Dermatol Sin 2020;38:248-9
|How to cite this URL:|
Lo TK, Hsu CK, Hsin ST, Wu JL, Lee JY. Bilateral auricular ossificans with stenosis of external ear canals and hearing loss caused by primary hyperparathyroidism: A case report. Dermatol Sin [serial online] 2020 [cited 2021 Jan 21];38:248-9. Available from: https://www.dermsinica.org/text.asp?2020/38/4/248/303694
A petrified ears (PE) is a rare condition in which one or both auricles become stony hard and cannot be bent. Histologically, it usually shows calcification of the auricular cartilage; rarely is ossification responsible. PE may be secondary to local tissue injury, underlying endocrine diseases, and congenital disorders or of unknown etiology.
| Report of a Case|| |
A 59-year-old Taiwanese male was referred by an otolaryngologist for the evaluation of a 5-year history of progressively hardening of both auricles with progressive hearing loss. He denied any history of local trauma, frostbite, or inflammation.
Physical examination revealed that, in addition to the widespread lesions of psoriasis and both auricles were stony hard and thickened with stenosis of the external ear canals [Figure 1]a. Pure-tone audiometry showed conductive hearing loss of the left ear and bilateral high tone hearing loss. A biopsy specimen from a hard area of the left auricle [Figure 1]b showed normal-appearing skin with a piece of trabecular bone present at the level where the auricular cartilage would normally be seen. Laboratory evaluation revealed anemia (11.0 g/dL), elevated parathyroid hormone (PTHi) 132.6 pg/mL and serum calcium 10.7 mg/dL, low serum phosphorus 1.2 mg/dL, Vitamin D (25-OH) 16.2 ng/mL, and low urinary calcium (2.7 mg/24 h).
|Figure 1: The patient presenting with (a) thickened and hardened auricles. Note the severe narrowing of the external ear canals. Histological section of an incisional biopsy specimen from a hard area of the left auricle showing no visible cartilage beneath the skin (b, ×40); instead there is a piece of bone consisting of mature bony trabeculae with intervening fatty tissue|
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|Figure 2: Axial (a) computer tomography showing the minute radiolucent air spaces within the bony opacity of bilateral auricles, indicating partial ossification of the external auricular cartilage. Coronal (b) image revealing narrowed external ear canals|
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Computer tomography (CT) of the head [Figure 2]a showed ossification of bilateral auricular cartilage with normal soft-tissue density of the overlying skin, and stenosis of the external ear canals with narrowing of the joint space of the bilateral tympanic membrane joints [Figure 2]b. A 1.0 cm enlarged right parathyroid gland was suspected by a thyroid ultrasound. Based on the diagnosis of parathyroid adenoma, the endocrinologist prescribed Vitamin D supplementation with calcitriol 0.25 mcg daily for 2 months. Canalplasty was suggested by the otolaryngologist to treat the stenosis of the bilateral external ear canal.
| Discussion|| |
In this report, we describe a case of petrified ears with hearing loss associated with primary hyperparathyroidism (PHPT) caused by the parathyroid adenoma. The skin biopsy revealed ossification beneath the skin, leading to the diagnosis of ossification of the auricle, which was further confirmed by imaging studies.
Petrified ears (PE) is characterized by the development of partial or total rigidity either due to calcification or, rarely, ossification of the auricle. Calcification can either be dystrophic or metastatic in origin. Dystrophic calcification describes calcium deposition onto altered or necrotic tissue despite normal serum calcium levels and is thought to be secondary to tissue injury, leading to increased tissue alkalinity and subsequent abnormal calcium deposition into damaged tissue. In contrast, metastatic calcium deposition stems from alterations in calcium and phosphorus metabolism, with subsequent deposition of calcium into tissues.
The auricular cartilages are often affected bilaterally with sparing of the earlobes. The shape of the ear does not change. Some may experience the discomfort of the auricle when compressed, and ulceration can rarely occur.
Approximately 140 cases of PE have been reported with evidence of either calcification or ossification of the elastic auricular cartilage by radiography and/or histopathologic examination. Auricular ossification occurs much less frequently than calcification, with <20 pathologically confirmed cases documented. PE occurs more commonly in men than in women. The predisposing factors or etiologies include local trauma, frostbite, addison's disease, hypo or hyperthyroidism, hypopituitarism, hyperparathyroidism, Vitamin D intoxication, sarcoidosis, scleroderma, diabetes mellitus, and alkaptonuria. The most common cause of auricular calcification and ossification is frostbite. In PHPT, there is an over secretion of PTHi, which acts on the bone and kidney to produce a hypercalcemic state in the absence of a secondary cause for PTHi elevation.
PE is a rare cause of conductive hearing impairment. In the present case, the ear canal and tympanic membrane joints were involved, resulting in hearing loss. Rarely, patients have subjective and/or objective hearing loss. Only 3 cases had conductive hearing impairment;, two of them with canal stenosis.
With the diagnosis of PE, it is important to search for an underlying systemic disorder. Laboratory evaluation includes a complete blood count, serum calcium, phosphate, Vitamin D, and parathyroid hormone levels, liver, renal, and thyroid function tests, and fasting plasma glucose levels. Radiography is usually diagnostic. Skull X-ray will show hyperdense areas where calcification or new bone formation has occurred. To differentiate calcification versus ossification, a temporal CT scan may be performed. The former shows uniform hyperdensity while the latter shows radiolucent spaces within hyperdense areas represent the trabecular bone formation seen in ossification. The histopathological examination differentiates between calcification and ossification but is not necessary for the diagnosis., The present case is the 13th case, in which the ossification was confirmed histopathologically.
Clinicians should think of PE whenever the patient is presented with stiffness of ears with or without pain. PE can be a sign of the underlying systemic disorder. Identifying and treating the underlying metabolic or endocrine disease are important to prevent disease progression and potential complication of hearing loss.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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