Man With Chronic Eustachian Tube Dysfunction, Otitis Media, and Hearing Loss

A 31-year-old man presents with a2-week history of a constant, dull acheand hearing loss in the right ear. Healso complains of intermittent sharppains that are usually followed bydrainage through the external auditorycanal. Another practitioner diagnosedacute otitis media with tympanic membraneperforation, for which he prescribeda 10-day course of amoxicillin.The patient completed the regimen buthas obtained no relief.Since childhood, the patient hashad chronic eustachian tube dysfunctionand chronic otitis media with16 tympanic membrane perforationsin the right ear. An otolaryngologistwas consulted after each perforation.A temporary pressure-equalizationtube was placed in the affected ear atages 27 years and 29 years. The patientalso reports chronic conductivehearing loss in the right ear withperiods of tinnitus and occasionalvertigo.

This otherwise healthy patient hasa low-grade fever, prominent submandibularlymphadenopathy on the rightside, and mastoid tenderness to palpation.Otoscopic examination reveals abulging tympanic membrane inferiorly,retraction superiorly, and a smallamount of clear serous drainage fromthe perforation in the posterosuperiorquadrant of the pars flaccida.A small, irregular mass within themiddle ear is seen through the retractedportion of the membrane. No priorimaging studies are available.The history and clinical presentationpoint to a presumptive diagnosisof a cholesteatoma. An otolaryngologistconcurs with the diagnosis, which isconfirmed by microotoscopic examinationand a CT scan of the temporalbones. The CT coronal view also showsextensive bone erosion within the middleear space (Figure 1). The CT axialview demonstrates coalescing fluid inthe right mastoid with sclerosis andhypoplasia of the air cells, which indicatesthat the fluid has occupiedthe mastoid air space for some time(Figure 2).A mastoidectomy, cholesteatomaresection, removal of the eroded headof the malleus and long process of theincus, and tympanoplasty with permanentpressure-equalization tube placementare performed. The patient toleratesthe procedure well; there are nocomplications. In 6 months, a secondsurgical procedure will be done to resectany residual or recurrent cholesteatomaand to reconstruct the middle ear byimplanting a prosthesis to partially restorethe conductive hearing loss.COMPLICATIONS OFCHOLESTEATOMASCholesteatomas often result fromchronic eustachian tube dysfunction.¹The negative pressure gradient withinthe middle ear causes retraction ofthe pars flaccida of the tympanic membraneand enables the formation ofa cystic expansion of epithelial debriswith erosive properties. Over time--usually months to years--the cholesteatomaerodes the bones of the middleear and creates a hospitable environmentfor chronic otitis media.Although cholesteatomas rarelyoccur in adults, they are associatedwith life-threatening intracranialcomplications. Thus, clinicians needto maintain a high level of suspicionwhen evaluating patients with longstandingeustachian tube dysfunctionor chronic otitis media.2Bacterial meningitis. This is byfar the most common intracranial complicationof untreated cholesteatomas.A recent study demonstrated thatcholesteatomas were directly responsiblefor more than 25% of all otologicinfections that progressed to bacterialmeningitis. Of the patients with cholesteatomasand bacterial meningitis,41% had a history of corrective surgeryfor their chronic ear condition.Despite aggressive medical and surgicaltreatment, between 5% and 10% ofpatients in the study died of otogenicbacterial meningitis.³Brain abscess and epiduralempyema. These conditions aremore serious intracranial sequelae ofuntreated cholesteatomas. Cholesteatomasare responsible for mostof the brain abscesses and epiduralempyemas that result from untreatedotologic disease (from 59% to morethan 95% in various studies).^4-6 Despiteundergoing aggressive medicaland surgical treatment, 10% of patientswith otologic infection-inducedbrain abscesses and epidural empyemasdie of these conditions.⁵TREATMENTWhen a cholesteatoma is suspected,immediate consultation with anotolaryngologist and a CT scan of thetemporal bones are warranted. Thescan can also be used to determinethe extent of the cholesteatoma and toidentify the location of critical surgicallandmarks before the operationSurgical interventions includemastoidectomy, resection of thecholesteatoma, and tympanoplastywith permanent pressure-equalizationtube placement. Once the diagnosisis confirmed, most procedures canbe done on an elective basis; however,emergency surgery is indicatedwhen:

• The cholesteatoma is associatedwith coalescent mastoiditis, as in thispatient.
• Worsening neurologic and/or infectiouscomplications are present.
• Neurologic complications persistafter surgical drainage and after48 hours of appropriate high-dose antimicrobialtherapy.⁷

After surgery, close follow-upis necessary to monitor the patientfor potentially lethal intracranialcomplications.

References:

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Chao WY, Tseng HZ, Chang SJ. Eustachian tubedysfunction in the pathogenesis of cholesteatoma:clinical considerations. J Otolaryngol. 1996;25:334-338.

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Noble J, ed. Textbook of Primary Care Medicine.3rd ed. St Louis: Mosby; 2001:1733-1736.

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Barry B, Delattre J, Vie F, et al. Otogenic intracranialinfections in adults. Laryngoscope. 1999;109:483-487.

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Singh B, Maharaj TJ. Radical mastoidectomy: itsplace in otitic intracranial complications. J LaryngolOtol. 1993;107:1113-1118.

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Kangsanarak J, Navacharoen N, Fooanant S,Ruckphaopunt K. Intracranial complications of suppurativeotitis media: 13 years’ experience. Am JOtol. 1995;16:104-109.

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Sennaroglu L, Sozeri B. Otogenic brain abscess:review of 41 cases. Otolaryngol Head Neck Surg. 2000;123:751-755.

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Gower D, McGuirt WF. Intracranial complicationsof acute and chronic infectious ear disease: a problemstill with us. Laryngoscope. 1983;93:1028-1033.