History
Painless otorrhea
The hallmark symptom of cholesteatoma is a painless otorrhea, either unremitting or frequently recurrent. When the cholesteatoma becomes infected, it may be extremely difficult to eradicate. Owing to a lack of blood supply to the cholesteatoma, systemic antibiotics cannot be delivered to its center. Topical antibiotics can often surround the cholesteatoma, suppress infection, and penetrate a few millimeters toward the center of the mass; however, large infected cholesteatomas are resistant to any type of antimicrobial therapy. Consequently, otorrhea either persists or recurs despite frequent and aggressive treatment with antibiotics.
Hearing loss
Hearing loss is also a common symptom of cholesteatomas. Large cholesteatomas interfere with sound transmission by filling the middle ear space with desquamated epithelium, with or without associated mucopurulent discharge. Ossicular damage frequently occurs and can also produce or may magnify a conductive loss. (See the image below.)
A large epitympanic (attic) cholesteatoma that is much more advanced than the lesion in the previous image.
A study by Yehudai et al of 124 pediatric patients with chronic otitis media, without or without cholesteatoma, found a significant association between the presence of cholesteatoma in this disease and the degree of sensorineural hearing loss at 2000 Hz. [17]
Vertigo
Vertigo, a progressive symptom of cholesteatoma, occurs infrequently due to early diagnosis of the disease. It can result when the middle ear ossicles are eroded and produce a labyrinthine fistula or when the cholesteatoma is lying directly on the stapes footplate. Vertigo is a worrisome symptom, as it may presage the development of more serious complications.
Facial nerve palsy
Rarely, cholesteatoma presents with facial nerve paralysis. Please see the Complications subsection below for further details.
Central nervous system
Occasionally, cholesteatoma initially presents with symptoms of CNS complications, sigmoid sinus thrombosis, [18] epidural abscess, or meningitis.
Craniofacial abnormalities
A Danish study indicated that in persons with a cleft palate, the risk of cholesteatoma is 20-fold that of the general population; siblings of these individuals were also determined to be at somewhat increased risk. Persons with a cleft lip, however, were found not to have a higher cholesteatoma risk. The historical cohort study included 8593 persons with a nonsyndromic orofacial cleft, as well as 6989 siblings. [19]
Physical Examination
On physical examination, the most common signs of cholesteatoma formation are drainage and granulation tissue in the ear canal and middle ear space. Quite frequently, the only finding on physical examination is an ear canal filled with mucoid drainage and granulation tissue; sometimes, eliminating the infection and resolving the granulation tissue with either systemic antibiotics or ototopical antibiotic drops is impossible. When ototopical therapy is successful, a deep tympanic retraction pocket may be seen in the pars flaccida (the portion of the tympanic membrane that is superior to the short process of the malleus) or in the posterior quadrant of the tympanic membrane.
In more than 90% of cases, a tympanic membrane perforation is also present. Congenital cholesteatomas are an exception to this, with the eardrum often remaining intact until the middle ear component is quite large.
Occasionally, cholesteatoma is produced by surgical implantation of squamous epithelium that manifests prior to disruption of the tympanic membrane, but even in such cases, the cholesteatoma eventually produces a tympanic membrane perforation. [20]
Very seldom is cholesteatoma first identified based on one of its complications; however, this occasionally happens in children. An infection associated with the cholesteatoma can erupt through the inferior mastoid cortex and manifest as a neck abscess. Rarely, cholesteatoma can manifest with signs and symptoms of CNS complications, sigmoid sinus thrombosis, epidural abscess, or meningitis.
Complications
Mastoiditis
Mastoiditis develops due to infection of the mastoid cells. Symptomatic coalescent mastoiditis (which is a separate entity from simple mastoid effusion) is a rare complication of both acute otitis media and chronic otitis media, with or without cholesteatoma. Nonetheless, mastoiditis can be serious due to its proximity to the posterior cranial fossa, lateral sinuses, facial nerve canal, semicircular canals, and the petrous tip of the temporal bone. A study that reviewed almost 3000 cases of chronic suppurative otitis media (CSOM) in a 9-year period in Turkey reported 25 cases of mastoid abscess. [21] Clinical mastoiditis may present with fever, posterior ear pain and/or local erythema over the mastoid bone, edema of the pinna, or a posteriorly and downward displaced auricle. In coalescent mastoiditis, CT scanning demonstrates characteristic loss of trabecular bone [22] .
Antibiotics for the treatment of mastoiditis should include coverage for Staphylococcus aureus, Pseudomonas, and enteric gram-negative rods, as well as Streptococcus pneumoniae and Haemophilus influenzae. If patients do not respond to conservative therapy with intravenous (IV) antibiotics, further intervention is warranted. When cholesteatoma is present, tympanomastoidectomy should be considered to remove necrotic bone in the mastoid and cholesteatoma deposits.
Facial nerve palsy
The facial nerve is vulnerable to infection during chronic otitis media, and facial palsy may occur as a complication of cholesteatoma, as well as tympanomastoid surgery. It typically arises in association with more advanced lesions of chronic ear infections. CSOM, which is characterized by persistent otorrhea through the tympanic perforation and can occur with or without cholesteatoma, can be complicated by facial nerve palsy, owing to involvement of the dehiscent facial nerve or direct bony erosion of the facial canal. [22, 23] The onset is usually gradual. In one case series of 70 patients with complicated CSOM, 14% of the patients had accompanying facial palsy. [24] Treatment of facial paralysis in the setting of CSOM, with or without cholesteatoma, requires surgical intervention. [22]
Intracranial complications
Intracranial complications—such as suppurative thrombophlebitis of the lateral and/or cavernous sinuses, meningitis, and intracranial abscesses—are potentially life-threatening and require immediate intervention. [21] These complications are rare, however.
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Epitympanic (attic) cholesteatoma. This is a typical primary acquired cholesteatoma in its earliest stages.
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A large epitympanic (attic) cholesteatoma that is much more advanced than the lesion in the previous image.
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A large cholesteatoma. No landmarks are visible, which typically is the case with advanced cholesteatomas.
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A cholesteatoma with a white mass can be seen behind an intact drum.
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An unenhanced computed tomography (CT) scan demonstrating that the posterior canal wall has been eroded and the external auditory canal has filled with cholesteatoma, pus, and debris. Surprisingly, the middle ear appears relatively free of disease, a characteristic of primary acquired cholesteatomas.
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The photo exhibits a large meatoplasty performed as part of an open cavity (canal wall–down) mastoidectomy. A similar meatoplasty usually is necessary if a clean, dry, problem-free cavity is to be maintained.
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A typical audiogram demonstrating bilateral conductive hearing loss, which may be observed in an individual with a cholesteatoma.
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Advanced cholesteatoma with exposure of posterior cranial fossa dura.
