eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > External Ear Diseases

External Ear, Benign Tumors

Author: Ravi N Samy, MD, FACS, Assistant Professor, Pediatric and Adult Otology, Neurotology, and Skull Base Surgery, Department of Otolaryngology, University of Cincinnati Medical Center, Cincinnati Children's Hospital
Coauthor(s): Bradley Marple, MD, Vice Chairman, Department of Otolaryngology, University of Texas Southwestern Medical Center
Contributor Information and Disclosures

Updated: Mar 12, 2009

Introduction

Medical practitioners often encounter benign lesions of the external ear. Primary care physicians and specialists must be able to distinguish these disease processes and institute proper therapy or referral, if indicated. This article outlines several benign lesions and tumors of the external ear and gives the reader a basic understanding of their clinical features and treatment options.

Anatomy of the External Ear

The external ear consists of the auricle and the external auditory canal (EAC).

Auricle

• Components
  • Cartilage
  • Elastic tissue
  • Skin
• Topography
  • Helix
  • Antihelix
  • Tragus
  • Antitragus
  • Concha
  • Triangular fossa
  • Scaphoid fossa
  • Lobule
• Musculature
  • Superior, anterior, and posterior auricular muscles
  • Innervated by seventh cranial nerve (facial nerve)
  • Serves to attach and support auricle
• Blood supply
  • Derived from external carotid artery
  • Supply from postauricular and superficial temporal branches off external carotid
• Sensory innervation
  • Anterior - Auriculotemporal nerve
  • Posterior - Greater auricular nerve and lesser occipital nerve
  • Concha - Branches from vagus and facial nerves
• Lymphatics
  • Anteriorly into parotid region
  • Inferiorly into superficial and deep cervical chains
  • Posteriorly into postauricular and occipital regions
  • Of utmost importance in the treatment of malignant lesions, as parotidectomy and neck dissection may be necessary

External auditory canal

• General anatomy
  • S-shaped and approximately 4 cm in length from tragus to tympanic membrane
  • Cartilaginous outer third and osseus inner two thirds
• Vasculature and lymphatics
  • Vasculature - Supplied by postauricular, superficial temporal, and deep auricular arteries
  • Lymphatics - Drain anterior (parotid gland lymph nodes), posterior, and inferior to the auricle
• Innervation
  • Vagus - Inferior and posterior regions of the canal
  • Auriculotemporal - Anterior and superior
  • Facial nerve - Movement of auricula by the extrinsic muscles, the sensory innervation of conchal, and portions of the posterior EAC
• Glandular structures
  • Sebaceous - Numerous and open into the follicles of fine vellus hairs, primarily in outer third
  • Ceruminous - Variable in number, secrete cerumen into outer third of EAC

Benign Lesions of the External Ear

Chondrodermatitis nodularis chronica helicis

Chondrodermatitis nodularis chronica helicis (CNCH) is uncommon and has a male predominance. Lesions typically present as a solitary tender erythematous nodule with central crusting or ulceration. They most commonly are located on the helical rim in men and on the antihelix and antitragus in women. Nodules are dome shaped, firm, reddish gray, and measure 3-18 mm. They appear spontaneously and reach maximum size in a short time. The lesions can last for several years and rarely regress spontaneously.

The etiology of CNCH remains unknown. Some authors think that it begins with some minor trauma to the ear that then incites chronic inflammation of the perichondrium, resulting in local vascular compromise. This vascular disruption then creates the observed clinical sequelae of painful nodules.

The pathophysiology of CNCH is thought to be related to the anatomy of the ear. The skin over the lateral and anterior aspect of the auricle is thin and tightly stretched over the underlying cartilage. Subcutaneous tissue is lacking in this region, which creates an area of limited vascularity. Various insults, such as trauma, frostbite, and tight-fitting headgear, can result in ischemia. This ischemia is thought to create a chronic perichondritis, which in turn leads to the clinical symptoms of pain and a nodule.

Histologic findings include dermal inflammation and fibrosis associated with either a central hyperkeratotic plug or ulceration. The cartilage is disrupted, hemorrhagic, and even necrotic.

Surgical excision generally is considered the treatment of choice for CNCH. Surgical options include curettage, electrocautery, carbon dioxide laser ablation, and excision of skin and cartilage with closure. Munnoch et al recommend a minimal skin excision combined with a more extensive cartilage resection to avoid recurrence. Healing can be hampered and infection can result if the remaining cartilage is not meticulously trimmed and smoothed in a patient with CNCH.

Medical options used to treat CNCH have included radiation therapy, cryotherapy, topical antibiotics, and injection of procaine hydrochloride; however, these have been abandoned because of high recurrence rates. Standard medical therapy consists of a trial of intralesional injections of 0.1-0.2 mL triamcinolone acetate (10-40 mg/mL). Surgical excision is used when other treatment fails.

Patients with CNCH can expect a good prognosis, but lesions recur at the edges of the resected cartilage in as many as 10% of patients, even with wide removal of diseased cartilage.

Idiopathic cystic chondromalacia

Idiopathic cystic chondromalacia (ICC) is uncommon and generally affects men of all ages, but it most commonly is observed in the third decade of life. As its name suggests, ICC is of unknown etiology. Theories involving embryologic malformation of the auricle have been offered to explain its occurrence. During development of the external ear, the 6 hillocks of His are thought to fuse and fold around the first branchial groove. ICC is believed to originate during this fusion and folding process when potential planes and spaces are created in areas of decreased structural resistance. Cysts can occur in these potential planes and spaces of the cartilage. The other main theory to explain ICC is related to abnormal lysozymal enzyme release that causes cartilage degradation and cyst formation.

Lesions of ICC present as unilateral asymptomatic swelling on the auricle and appear in the scaphoid fossa in 80% of patients. The lesions contain viscous yellow fluid that causes slight fluctuance. Usually, no ulceration is present. Histology reveals an intracartilaginous cavity filled with granulation tissue. No epithelial lining of the cavity is observed, and the surrounding cartilage is benign.

ICC can be treated with intralesional steroids; however, the high incidence of a resulting cosmetic deformity limits use of this treatment. Simple aspiration of the cyst fluid has been tried, but reaccumulation is universal. Complete excision of the cyst can lead to significant cosmetic deformities. The most common surgical technique involves excising the anterior cyst wall followed by sclerosing the posterior cyst wall with 1% iodine solution and applying a bolster dressing postoperatively. The sclerosing creates inflammation and scarring that prevents the cyst from reforming, and the preserved posterior cartilage gives cosmetic support to the auricle. The surgeon should be careful to avoid extensive posterior cyst wall resection because floppy ear deformities can result. No significant recurrence is associated with complete excision of ICC.

Kimura disease

Kimura disease is rare but most commonly observed in individuals of Asian descent. According to the Armed Forces Institute of Pathology (AFIP) series, the average age of presentation is 36 years. Kimura disease has a male predominance; the etiology is unknown. Immune dysregulation and atopic reaction to a continuous antigenic stimulus are being investigated as causes. One prominent theory claims that Candida albicans acts as a source of persistent antigen stimulus. This results in production of interleukin 5 (IL-5), which serves as an inflammatory mediator and chemotactic agent for eosinophils. A parasite has been suggested as a potential cause, but none has been demonstrated.

Kimura disease usually occurs in the head and neck but can involve other parts of the body. Lesions present as nontender subcutaneous swellings with a smooth or nodular surface. Color varies from deep red to skin colored. Ear involvement includes the auricle, postauricular sulcus, EAC, and meatus; the average lesion is 1.2 cm. Lesions can be associated with firm and palpable posterior cervical or supraclavicular lymph nodes. Kimura disease can be associated with nephrotic syndrome, and renal failure has been reported in patients with aggressive cases. Referral to a nephrologist may be indicated.

Histologically, reactive prominent germinal centers are observed along with a cellular infiltrate of eosinophils, plasma cells, and histiocytes. Microabscesses can be present, and vascular proliferation with plump endothelium also is noted. Heavy immunoglobulin G (IgG) and immunoglobulin E (IgE) deposits can be seen on immunohistochemistry. Laboratory tests can be important for the evaluation of patients with Kimura disease. Renal function is assessed with creatinine and urinary protein levels. In addition, peripheral eosinophil counts and serum IgG and IgE levels can be elevated. Imaging may be helpful in Kimura disease. Contrasted CT scans can highlight the lesion and any associated lymphadenopathy. MRI shows increased signal in T1 and T2 sequences.

Treatment of Kimura disease includes medical and surgical options. Complete surgical excision is generally recommended for patients with Kimura disease. The prognosis is generally good, but recurrence after surgery can be a problem. Renal involvement indicates a worse prognosis. Several medical regimens have been tried, including steroids and radiation therapy. Intralesional steroids have not proven very successful. Systemic steroids are reserved for patients in whom surgery fails or for those with renal involvement. Radiation therapy is used as a last resort.^1,2

Exostoses

Exostoses are the most common bony abnormality of the EAC; they are present in approximately 1 out of every 150 patients examined for otolaryngologic problems. Bony exostoses manifest as a gradual narrowing of the bony canal by broad-based mounds of bone that arise from the anterior and posterior canal walls. They are most often observed in individuals with a history of cold-water exposure (such as swimmers or surfers). The bone deposition responsible for exostosis is thought to be secondary to a chronic periostitis due to exposure to cold temperatures. The bone mounds usually occur bilaterally and are generally asymptomatic. Symptoms such as conductive hearing loss and otitis externa can arise if the canal becomes occluded.

Histologic findings include a dense stratified arrangement of new bone that is remodeled over time into normal lamellar bone. Exostoses can be managed surgically if canal obstruction and symptoms arise. A canalplasty can be performed to remove the offending lesions. Split-thickness skin grafts may be needed for denuded canal skin. Tympanic membrane perforation can be avoided by raising medially based canal skin flaps and reflecting them onto the tympanic membrane. Bone curettes and diamond burrs are used. Patients undergoing surgical removal can expect a good prognosis unless cold-water exposure recurs.

Premalignant Lesions of the External Ear

Actinic keratosis

Actinic keratosis (AK) is one of the more common premalignant lesions encountered on the ear. It most often is observed in fair-complexioned white individuals; the average age of onset is 62 years. Both sexes are affected equally. Sun exposure is thought to be the cause of AK.

AK presents as round or irregularly shaped lesions that are scaly, keratotic, and flat topped. The color varies from gray to deep brown. Basophilic degeneration in the dermis with a chronic nonspecific inflammatory infiltrate and hyperkeratosis of the epidermis are seen on histology. Dysplastic cells can be present. Complete surgical excision is recommended, and a good prognosis is expected with complete removal.

Benign Tumors of the External Ear

Osteoma

An osteoma manifests as a discrete, pedunculated bony mass that arises from the tympanosquamous suture line adjacent to the bony-cartilaginous junction. The masses are solitary, unilateral, and slow growing. These are the most common bony neoplasms of the temporal bone. The incidence of osteoma formation or presentation peaks in the fourth decade of life. Osteomas are usually asymptomatic; however, symptoms can arise if canal obstruction occurs. Osteomas are more common in men than in women. No known definite link to cold-water exposure or radiation has been demonstrated.

Histology reveals well-formed lamellae with multiple fibrovascular channels throughout the interlamellar spaces. Surgical removal of osteomas is similar to that of exostoses. Canal skin flaps are raised, and the lesion is drilled away, preferably with a diamond burr. Recurrence is unusual but necessitates more aggressive removal of bone.

Squamous papilloma

This is not an uncommon lesion of the external ear. An AFIP series from 1976-1985 found 66 patients with squamous papilloma. Both sexes and all ages are equally affected. Squamous papilloma may be a result of exposure to the human papillomavirus, with resulting virally induced changes of the genome that create cell proliferation.

The lesion presents as a warty, verrucous, and elevated lesion that may be pigmented. It may have an overlying thickened keratin layer. Histology indicates benign proliferation of uniform squamous cells with a central core of vascular fibrous tissue. Surgical excision is recommended. No recurrence or malignant degeneration was observed in the AFIP series of 66 patients.

Seborrheic keratosis

Seborrheic keratosis (SK) is common in the head and neck region, but ear involvement is rare. SK results from intracellular changes that create intraepidermal basal cell proliferation. It presents as an oval verrucous plaque. Early, the lesion appears light yellow, and it becomes more brown as its size increases. Histologically, thickened epidermis secondary to accumulation of immature keratinocytes between the basal layer and the surface as well as invaginations of the thickened epithelium that create keratin tunnels are seen. The tumor protrudes above the horizontal plane of the skin. Complete surgical excision is recommended for SK, and a good prognosis is expected. Malignant transformation has been reported.

Sebaceous adenoma

Sebaceous adenoma (SA) is rare in any part of the body and even rarer in the ear. Rulon and Helwig reported a series of 95 patients with SA, but only 3 of the lesions were located on the ear. SA has a male-to-female ratio of 2.5:1.

These tumors arise from sebaceous glands located in the skin of the ear and EAC; they present as a smooth, elevated, pedunculated, firm-to-soft lesion. The lesions usually are solitary and smaller than 0.5 cm. Color varies; tan, skin-colored, pink, and red lesions have been observed. SA lesions can bleed from trauma, but they rarely ulcerate.

On histology, the cells replace surface epithelium and represent a duplicate progression of sebaceous cell maturation. Pleomorphism of cells and nuclei can be seen with occasional mitotic figures. Complete surgical excision is recommended for SA, and a good prognosis is noted. Recurrence is rare.

Pilomatrixoma

Pilomatrixoma is relatively rare and has a higher incidence in females than in males. It most commonly presents in the first 2 decades of life and is believed to arise from the hair follicles. Characteristics include necrosis, keratinization, and proliferation of small epithelial cells. Calcification or ossification is sometimes observed. The lesion presents as a slow-growing, bluish, solitary, and well-circumscribed nodule within the dermis of the auricle or preauricular region. Pilomatrixoma can have a history of several years and can appear in other parts of the body. Growth can arrest spontaneously.

Plain film or CT radiography can be useful in pilomatrixoma because it can reveal areas of prominent stippled calcification. Histology reveals irregular islands of progressively degenerating epithelial cells separated by fibrous stroma. The examination reveals 2 main cell types: basaloid cells at the margin of epithelial islands and anucleated shadow cells in the inner core. Calcium deposits are seen as fine basophilic granules. Complete surgical excision is recommended for pilomatrixoma. A good prognosis is noted, but malignant transformation rarely occurs and is associated with multiple local recurrences.

Keratoacanthoma

Keratoacanthomas are relatively common and occur most often in white adults; the male-to-female ratio is 2:1. Keratoacanthoma has been linked to sun exposure, chemical carcinogens, trauma, and possibly a viral etiology. Keratoacanthoma arises from the hair follicles and involves 3 phases: proliferation, the developed tumor, and involution.

Neoplastic epithelium accumulates in the proliferative phase and results in keratin-filled invaginations or in a crater on the epidermal surface. The lesion presents as an elevated mass that appears flesh colored or slightly reddish; tightly stretched skin gives it a slightly translucent appearance. Over time, a central, umbilicated keratinous plug develops; this plug can ulcerate. A puckered or pitted scar can be left after involution. Histology shows a discrete, highly keratinized crater with epidermis extending across borders. Characteristic marginal cupping is present.

Complete surgical excision is recommended. Keratoacanthoma can behave like squamous cell carcinoma and can progress to carcinoma; however, spontaneous involution can also occur. A good prognosis with low recurrence is noted with complete excision. Topical and intralesional cytotoxic drug therapy, cryotherapy, and radiation have also been used to treat keratoacanthomas

Ceruminoma

Ceruminomas present in a few patients. Friedman reported on 13 patients (6 men and 7 women) aged 28-74 years. The lesion is a result of neoplasia of the ceruminous glands of the EAC, which are located in the lower two thirds of the dermis in the cartilaginous canal. Ceruminomas may be present for years and manifest as skin-covered, nonulcerated masses located in the outer half of the EAC. Ceruminomas can extend medially to involve the bony EAC or the middle ear. Symptoms result from EAC obstruction.

Histology reveals good demarcation from surrounding tissue but without a true capsule. A varied glandular pattern is observed, with an inner cuboidal eosinophilic epithelial component. Mitosis and pleomorphism are not characteristic. Complete surgical excision is recommended. Good prognosis with complete removal is noted; however, clear margins are imperative.

The term ceruminoma is no longer used by the World Health Organization (WHO), since it does not describe the benign or malignant nature of the lesion. Instead, the following classification is used by the WHO:

• Benign: ceruminous adenoma and pleomorphic adenoma
• Malignant: adenoid cystic carcinoma and adenocarcinoma

Mixed tumor

Ceruminous mixed tumors are rare. They present at an average age of 59 years. A 2:1 male-to-female ratio has been reported. Mixed tumors are believed to arise from the ceruminous glands of the EAC. An epithelial element is mixed with a mucoid-, myxoid-, or chondroid-appearing element. Clinical presentation is similar to that of ceruminoma. A slow-growing mass occurs, and EAC obstruction can result. Otitis externa can be present with canal obstruction. Histology reveals a lack of a capsule and biphasic histology similar to that of salivary gland mixed tumors. The epithelial cells create a tubular formation with a stroma that is fibrocellular with occasional myxoid or chondroid elements. Complete surgical excision is recommended for mixed ceruminous tumor. A good prognosis is obtained with clear margins.

Multimedia

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Media file 1: Classic chondrodermatitis nodularis chronica helicis on the superior helix.

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Media file 2: Close-up view of same patient as in Media File 1.

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Media file 3: Chondrodermatitis nodularis chronica helicis on the antihelix.

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Media file 4: Sharply circumscribed elevated seborrheic keratoses.

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Media file 5: Closer view of multiple seborrheic keratoses in an autosomally dominant mode of inheritance.

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Media file 6: Seborrheic keratoses projecting above the level of the epidermis. Cysts represent sections of hyperkeratotic follicles.

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Media file 7: Seborrheic keratosis showing lackluster surface and appearance of being stuck on the skin surface.

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Media file 8: Keratoacanthoma of the left forehead.

Keywords

external ear, benign tumors, ear, benign tumors of the external ear, ear tumors, external ear tumors, chondrodermatitis nodularis chronica helicis, CNCH, Winkler disease, Winkler's disease, chondrodermatitis helicis, idiopathic cystic chondromalacia, ICC, pseudocyst of the ear, enchondral pseudocyst, seroma of the ear, Kimura disease, Kimura's disease, angiolymphoid hyperplasia with eosinophilia, atypical pyogenic granuloma, papular angioplasia, exostosis, actinic keratosis, AK, solar keratosis, senile keratosis, osteoma, squamous papilloma, seborrheic keratosis, SK, seborrheic verrucous benign epithelioma, basal cell papilloma, sebaceous adenoma, SA, pilomatrixoma, calcifying epithelioma, Malherbe calcifying epithelioma, hair matrixoma, keratoacanthoma, molluscum sebaceum, molluscum verrucosum, vegetating sebaceous cyst, ceruminous gland adenoma, ceruminoma, hidradenoma, ceruminous gland mixed tumor, pleomorphic adenoma, chondroid syringoma

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Mark Newcomer, MD, to the development and writing of this article.

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