Background

The ear is affected by manY different pathologic processes which overlap with many different organ systems. It is divided into the external, middle, and inner ear. The external ear contains the auricle and external auditory meatus leading to the tympanic membrane (ear drum). The middle ear includes the tympanic membrane, the ossicles (malleus, incus, and stapes), eustachian tube, epitympanic recess, and the mastoid cavity. The inner ear leads to the brain with the auditory nerve (cranial nerve VIII).

• Cholesteatoma
• Low-grade adenocarcinoma of probable endolymphatic sac origin
  (Aggressive Papillary Tumor of Temporal Bone and Endolymphatic Sac)
• Middle ear adenoma
• Relapsing Polychondritis

OUTLINE

Gross Appearance and Clinical Variants
Histopathological Features and Variants
Prognosis
Treatment
Commonly Used Terms
Internet Links

CLINICAL VARIANTS	CHARACTERIZATION
HEARING LOSS
Screening and management of adult hearing loss in primary care: clinical applications. Bogardus ST Jr, Yueh B, Shekelle PG. Department of Medicine, Yale University School of Medicine, New Haven, Conn.	JAMA 2003 Apr 16;289(15):1986-90 Abstract quote Hearing loss is one of the most common chronic health conditions and has important implications for patient quality of life. However, hearing loss is substantially underdetected and undertreated. We present clinical cases to illustrate common situations in which primary care physicians may be called on to identify or to manage hearing loss. With the data reported in the companion scientific review as a guide, we present potential answers to important questions pertaining to hearing loss and suggest ways in which primary care physicians can improve the detection, evaluation, and treatment of hearing loss. The cases focus on screening for chronic hearing loss, evaluation of hearing loss, and treatment of patients with presbycusis.
Screening and management of adult hearing loss in primary care: scientific review. Yueh B, Shapiro N, MacLean CH, Shekelle PG. Veterans Affairs Puget Sound Health Care System, Departments of Otolaryngology-Head and Neck Surgery and Health Services, University of Washington, Seattle.	JAMA 2003 Apr 16;289(15):1976-85 Abstract quote CONTEXT: Hearing loss is the third most prevalent chronic condition in older adults and has important effects on their physical and mental health. Despite these effects, most older patients are not assessed or treated for hearing loss. OBJECTIVE: To review the evidence on screening and management of hearing loss of older adults in the primary care setting. Data Sources and STUDY SELECTION: We performed a search from 1985 to 2001 using MEDLINE, HealthSTAR, EMBASE, Ageline, and the National Guideline Clearinghouse for articles and practice guidelines about screening and management of hearing loss in older adults, as well as reviewed references in these articles and those suggested by experts in hearing impairment. DATA EXTRACTION: We reviewed articles for the most clinically important information, emphasizing randomized clinical trials, where available, and identified 1595 articles. DATA SYNTHESIS: Screening tests that reliably detect hearing loss are use of an audioscope, a hand-held combination otoscope and audiometer, and a self-administered questionnaire, the Hearing Handicap Inventory for the Elderly-Screening version. The value of routine screening for improving patient outcomes has not been evaluated in a randomized clinical trial. Screening is endorsed by most professional organizations, including the US Preventive Services Task Force. While most hearing loss in older adults is sensorineural and due to presbycusis, cerumen impaction and chronic otitis media may be present in up to 30% of elderly patients with hearing loss and can be treated by the primary care clinician. In randomized trials, hearing aids have been demonstrated to improve outcomes for patients with sensorineural hearing loss. Nonadherence to use of hearing aids is high. Prompt recognition of potentially reversible causes of hearing loss, such as sudden sensorineural hearing loss, is important to maximize the possibility of functional recovery. CONCLUSION: While untested in a clinical trial, older adults can be screened for hearing loss using simple methods, and effective treatments exist and are available for many forms of hearing loss.

 

HISTOPATHOLOGY	CHARACTERIZATION
CERUMINOUS ADENOMA
Ceruminous Adenomas: A Clinicopathologic Study of 41 Cases With a Review of the Literature Thompson, Lester D. R MD*; Nelson, Brenda L DDS, LCDR, DC, USNR*; Barnes, E Leon MD† From the *Departments of Endocrine-Otorhinolaryngic-Head & Neck Pathology, Armed Forces Institute of Pathology, Washington, DC; and †Department of Pathology, Presbyterian University Hospital, Pittsburgh, PA.	The American Journal of Surgical Pathology : Volume 28(3) March 2004 pp 308-318 Abstract quote Background: Ceruminous gland neoplasms are rare neoplasms. To date, a large clinicopathologic study of benign ceruminous gland neoplasms has not been reported. Design: Forty-one cases of ceruminous gland adenomas diagnosed between 1970 and 2000 were retrieved from the files of the Armed Forces Institute of Pathology. Histologic features were reviewed, immunohistochemical analysis was performed (n = 21), and patient follow-up was obtained (n = 40). Results: The patients included 22 men and 19 women, 24 to 85 years of age (mean, 54.2 years). Patients presented clinically with a painless mass of the outer half of the external auditory canal (n = 33) or with hearing changes (n = 11). Symptoms were present for an average of 16.3 months. The polypoid masses affected the external auditory canal only and ranged in size from 0.4 to 2 cm in greatest dimension (mean, 1.1 cm). Histologically, the tumors demonstrated glands and small cysts lined by a tubuloglandular proliferation of inner ceruminous cells (cerumen-secreting epithelium with decapitation secretion) subtended by a spindled to cuboidal myoepithelial layer. A hyalinized stroma created an infiltrative pattern of growth; surface involvement (n = 8) was seen. Tumors were divided into ceruminous adenoma (n = 36), ceruminous pleomorphic adenoma (n = 4), and syringocystadenoma papilliferum (n = 1) types. The luminal cells were strongly and diffusely immunoreactive with CK7, while the basal cells were highlighted with CK5/6, S-100 protein, and p63. CD117 highlighted the luminal cells preferentially. The proliferation markers revealed a low index. Adenocarcinoma and middle ear adenoma are the principal differential consideration. Surgical excision was used in all patients. Four patients developed a recurrence due to incomplete excision. All patients were without evidence of disease at the last follow-up: alive (n = 28, mean 16.3 years) or dead (n = 12, mean 11.8 years). Conclusion: Ceruminous gland adenomas are the most common external auditory canal tumors. They demonstrate a dual cell population of basal myoepithelial-type cells and luminal ceruminous (ceruminal) cells. Cerumen pigment, CK7, and p63 can help to distinguish this tumor from other neoplasms that occur in the region. Complete surgical excision results in an excellent long-term clinical outcome. Tumors arising from the ceruminal glands of the external ear canal can present a diagnostic dilemma because of their varied clinical and histologic manifestations. While well described in cats and dogs, tumors of this type are rare in humans and therefore are seldom seen by general surgical pathologists.4,8,27 Further adding to the confusion for pathologists and clinicians alike is the variable nomenclature used to describe tumors of ceruminal gland origin ( Table 1 ). These terms in general do not suggest a specific diagnosis, clinical behavior, treatment alternatives, or long-term clinical outcome. Likewise, many reports in the literature, when critically reviewed, are examples of neuroendocrine adenoma of the middle ear (middle ear adenoma), endolymphatic sac tumors (Heffner tumor), paraganglioma, or most likely represent parotid gland neoplasms that have extended into the external auditory canal.5,7,10,13,16,17,20,22,25,29,33-38 Many cases are not illustrated or incompletely described histologically, further preventing a critical review. Limited mostly to case reports and small series ( Table 2 ), the English literature is devoid of a large comprehensive evaluation of ceruminous adenomas with respect to their histomorphology, immunohistochemical reactivity, treatment outcomes, and clinical behavior. We undertook this study in an attempt to identify the histologic and immunohistochemical features that can be used to separate benign from malignant ceruminous tumors and to determine the best nomenclature to yield a meaningful clinical management impact.

 

PROGNOSIS AND TREATMENT

CHARACTERIZATION

Mucosal Biofilm Formation on Middle-Ear Mucosa in the Chinchilla Model of Otitis Media Garth D. Ehrlich, PhD; Richard Veeh, PhD; Xue Wang, MD; J. William Costerton, PhD; Jay D. Hayes; Fen Ze Hu, MS; Bernie J. Daigle; Miles D. Ehrlich; J. Christopher Post, MD, PhD

JAMA. 2002;287:1710-1715 Abstract quote Context Chronic otitis media with effusion (OME) has long been considered to be a sterile inflammatory process. The previous application of molecular diagnostic technologies to OME suggests that viable bacteria are present in complex communities known as mucosal biofilms; however, direct imaging evidence of mucosal biofilms associated with OM is lacking. Objective To determine whether biofilm formation occurs in middle-ear mucosa in an experimental model of otitis media. Design and Materials A total of 48 research-grade, young adult chinchillas weighing 500 g were used for 2 series of animal experiments: one to obtain specimens for scanning electron microscopy and the other to obtain specimens for confocal laser scanning microscopy using vital dyes. In each series, 21 animals were bilaterally injected with viable Haemophilus influenzae bacteria and 1 was inoculated to account for expected mortality. Three served as negative controls. Effusions and mucosal specimens were collected from 2 infected animals that were euthanized at 3, 6, 12, and 24 hours and at days 2, 4, 5, 10, 16, and 22 after inoculation. Main Outcome Measures Images were analyzed for biofilm morphology, including presence of microcolony formation and for presence of bacteria on tissue surfaces. Results Scanning electron microscopy demonstrated that biofilm formation was evident in all specimens from animals beginning 1 day after infection and was present through 21 days. Confocal laser scanning microscopy indicated that bacteria within the biofilms are viable. Conclusion These preliminary findings provide evidence that mucosal biofilms form in an experimental model of otitis media and suggest that biofilm formation may be an important factor in the pathogenesis of chronic otitis media with effusion.

Rosai J. Ackerman's Surgical Pathology. Eight Edition. Mosby 1996.
Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.
Weedon D. Weedon's Skin Pathology. Churchill Livingstone. 1997.
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.

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Last Updated 3/15/2004

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