A 31-year-old man presents with a 2-week history of a constant, dull ache and hearing loss in the right ear. He also complains of intermittent sharp pains that are usually followed by drainage through the external auditory canal. Another practitioner diagnosed acute otitis media with tympanic membrane perforation, for which he prescribed a 10-day course of amoxicillin(Drug information on amoxicillin). The patient completed the regimen but has obtained no relief. Since childhood, the patient has had chronic eustachian tube dysfunction and chronic otitis media with 16 tympanic membrane perforations in the right ear. An otolaryngologist was consulted after each perforation. A temporary pressure-equalization tube was placed in the affected ear at ages 27 years and 29 years. The patient also reports chronic conductive hearing loss in the right ear with periods of tinnitus and occasional vertigo.

Figure 1
Figure 1
Figure 2
Figure 2
This otherwise healthy patient has a low-grade fever, prominent submandibular lymphadenopathy on the right side, and mastoid tenderness to palpation. Otoscopic examination reveals a bulging tympanic membrane inferiorly, retraction superiorly, and a small amount of clear serous drainage from the perforation in the posterosuperior quadrant of the pars flaccida. A small, irregular mass within the middle ear is seen through the retracted portion of the membrane. No prior imaging studies are available. The history and clinical presentation point to a presumptive diagnosis of a cholesteatoma. An otolaryngologist concurs with the diagnosis, which is confirmed by microotoscopic examination and a CT scan of the temporal bones. The CT coronal view also shows extensive bone erosion within the middle ear space (Figure 1). The CT axial view demonstrates coalescing fluid in the right mastoid with sclerosis and hypoplasia of the air cells, which indicates that the fluid has occupied the mastoid air space for some time (Figure 2). A mastoidectomy, cholesteatoma resection, removal of the eroded head of the malleus and long process of the incus, and tympanoplasty with permanent pressure-equalization tube placement are performed. The patient tolerates the procedure well; there are no complications. In 6 months, a second surgical procedure will be done to resect any residual or recurrent cholesteatoma and to reconstruct the middle ear by implanting a prosthesis to partially restore the conductive hearing loss. COMPLICATIONS OF CHOLESTEATOMAS Cholesteatomas often result from chronic eustachian tube dysfunction.1 The negative pressure gradient within the middle ear causes retraction of the pars flaccida of the tympanic membrane and enables the formation of a cystic expansion of epithelial debris with erosive properties. Over time— usually months to years—the cholesteatoma erodes the bones of the middle ear and creates a hospitable environment for chronic otitis media. Although cholesteatomas rarely occur in adults, they are associated with life-threatening intracranial complications. Thus, clinicians need to maintain a high level of suspicion when evaluating patients with longstanding eustachian tube dysfunction or chronic otitis media.2 Bacterial meningitis. This is by far the most common intracranial complication of untreated cholesteatomas. A recent study demonstrated that cholesteatomas were directly responsible for more than 25% of all otologic infections that progressed to bacterial meningitis. Of the patients with cholesteatomas and bacterial meningitis, 41% had a history of corrective surgery for their chronic ear condition. Despite aggressive medical and surgical treatment, between 5% and 10% of patients in the study died of otogenic bacterial meningitis.3 Brain abscess and epidural empyema. These conditions are more serious intracranial sequelae of untreated cholesteatomas. Cholesteatomas are responsible for most of the brain abscesses and epidural empyemas that result from untreated otologic disease (from 59% to more than 95% in various studies).4-6 Despite undergoing aggressive medical and surgical treatment, 10% of patients with otologic infection–induced brain abscesses and epidural empyemas die of these conditions.5 TREATMENT When a cholesteatoma is suspected, immediate consultation with an otolaryngologist and a CT scan of the temporal bones are warranted. The scan can also be used to determine the extent of the cholesteatoma and to identify the location of critical surgical landmarks before the operation Surgical interventions include mastoidectomy, resection of the cholesteatoma, and tympanoplasty with permanent pressure-equalization tube placement. Once the diagnosis is confirmed, most procedures can be done on an elective basis; however, emergency surgery is indicated when:
  • The cholesteatoma is associated with coalescent mastoiditis, as in this patient.
  • Worsening neurologic and/or infectious complications are present.
  • Neurologic complications persist after surgical drainage and after 48 hours of appropriate high-dose antimicrobial therapy.7
After surgery, close follow-up is necessary to monitor the patient for potentially lethal intracranial complications.