Jason A. Smith, MD * , Christopher J. Danner, MD Department of OtolaryngologyHead and Neck Surgery, University of Arkansas for Medical Sciences, 4301 West Markham Slot #543, Little Rock, AR 72205, USA Chronic otitis media (COM) is dened as persistent infection or inam- mation of the middle ear and mastoid air cells. This condition typically in- volves a perforation of the tympanic membrane, with intermittent or continuous otorrhea. As chronic otomastoiditis and eustachian tube dys- function persist, the tympanic membrane is weakened, which increases the likelihood of an atelectatic ear or cholesteatoma formation. The proximity of the middle ear cleft and mastoid air cells to the intra- temporal and intracranial compartments places structures located in these areas at increased risk of infectious complications. Acute otitis media (AOM) and its complications are more common in young children, whereas complications secondary to COM with and without cholesteatoma are more common in older children and adults. In a large series by Osma and col- leagues [1], 78% of subjects who had complications secondary to COM were found to have cholesteatoma. The complications of AOM and COM, dened using the same classica- tion system, are divided into intracranial and extracranial complications; extracranial complications are further divided into extratemporal and intra- temporal complications. The development and appropriate use of antibi- otics have led to a decrease in these potentially devastating complications. However, they continue to occur, and clinical vigilance is required for early detection and treatment. Furthermore, with the continued development of multidrug-resistant pathogens, these complications may again become more prevalent as our current antibiotics become less eective [2]. * Corresponding author. E-mail address: smithjasona@uams.edu (J.A. Smith). 0030-6665/06/$ - see front matter 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.otc.2006.09.001 oto.theclinics.com Otolaryngol Clin N Am 39 (2006) 12371255 Extratemporal (extracranial) complications Subperiosteal abscess Subperiosteal abscess is the most common extratemporal complication that occurs with COM. This abscess occurs over the mastoid cortex when the infectious process within the mastoid air cells extends into the subperios- teal space. This extension most commonly occurs as a result of erosion of the cortex secondary to acute or coalescent mastoiditis, but can also occur as a result of vascular extension secondary to phlebitis of the mastoid veins [3]. Subperiosteal abscesses are seen more commonly in young children with AOM, but are also found in chronic otitis with and without cholesteatoma. Cholesteatoma can block the aditus ad antrum, preventing communication of the infected contents of the mastoid with the middle ear space and the eu- stachian tube. This obstruction increases the possibility of infectious decom- pression through the mastoid cortex, presenting clinically as a subperiosteal abscess or Bezolds abscess. Diagnosis Often, the diagnosis of a subperiosteal abscess is made on clinical grounds. Commonly, the patient will present with systemic symptoms, in- cluding fever and malaise, along with local signs, including a protruding au- ricle that is laterally and inferiorly displaced, and the presence of a uctuant, erythematous, tender area behind the ear. When the diagnosis is not certain on clinical evaluation, a contrasted CT scan can demonstrate abscess and possibly the cortical defect in the mastoid (Fig. 1) [4]. A case can be made for a contrasted CT scan of the temporal bone in all patients presenting with these symptoms, to aid in therapeutic planning and to rule out other possible complications. Mastoiditis without abscess, lymphadenopathy, supercial abscess, and an infected sebaceous cyst are other possibilities that must be excluded. Fig. 1. An axial CT scan (A) of a 5-month-old child, demonstrating opacication of the left ear and mastoid with coalescence, and a coronal CT scan (B) of the left temporal bone in the same patient, demonstrating a subperiosteal abscess. 1238 SMITH & DANNER Management The management of a postauricular subperiosteal abscess from otitis me- dia without cholesteatoma is debatable. Conventional teaching and current texts advocate draining the abscess, in conjunction with a cortical mastoid- ectomy [3,4]. In recent years, other, less invasive treatment options have emerged. Patients have been treated with simple incision and drainage of the abscess in conjunction with intravenous (IV) antibiotics and a myringot- omy without sequelae [5]. One investigator advocates the use of IV antibi- otics, myringotomy, and needle aspiration of the abscess without formal drainage. In his experience, 14 of the 17 subjects treated in this manner re- solved their abscesses without the need for further intervention, and were discharged home signicantly sooner than those subjects who were managed with a mastoidectomy. The three subjects who failed needle aspiration went on to require cortical mastoidectomy [6]. An argument for conservative treatment modalities for subperiosteal abscesses from AOM is reasonable, because the process likely will be self-limited; however, there is not yet a con- sensus advocating these less aggressive approaches. The approach to a sub- periosteal abscess resulting from chronic otitis in the presence of a cholesteatoma is not as controversial. In this setting, the cholesteatoma warrants surgical intervention, and therefore more conservative alternatives are not reasonable. The skin incision for the mastoidectomy should be mod- ied to incorporate the abscess cavity for adequate drainage. Once the ab- scess is drained, a mastoidectomy is performed and the cholesteatoma matrix is removed in the standard manner. Bezolds abscess A Bezolds abscess is a cervical abscess that develops from pathology sim- ilar to the subperiosteal abscess. In the presence of coalescent mastoiditis, if the mastoid cortex is violated at its tip, as opposed to its lateral cortex, an abscess will develop in the neck, deep to the sternocleidomastoid. This ab- scess will present as a tender, deep, poorly dened mass in level two of the neck. Because the abscess develops from air cells at the tip of the mas- toid, it is found in older children and adults, where pneumatization of the mastoid has extended to the tip. Most of these abscesses result from direct extension through the cortex, but transmission through an intact cortex by way of mastoid vein phlebitis is known to occur [1]. Although Bezolds ab- scess is more commonly a complication of AOM with mastoiditis in chil- dren, it is a known complication of COM with cholesteatoma [7]. Diagnosis A contrasted CT scan of the neck and mastoid is recommended to make the diagnosis of a Bezolds abscess [8]. The presentation of an enlarged, ten- der, deep neck mass must be dierentiated from inammatory cervical lymphadenopathy, which is dicult on clinical grounds alone. CT scans 1239 COMPLICATIONS OF CHRONIC OTITIS MEDIA AND CHOLESTEATOMA of Bezolds abscesses show a rim-enhancing abscess with surrounding in- ammation, may demonstrate the bony dehiscence in the tip of the mastoid, and can help in operative planning. Management The commonly recommended conventional approach for management of a Bezolds abscess is described as an open incision and drainage of the cer- vical abscess through a transcervical approach, combined with a cortical mastoidectomy to address the mastoiditis [3]. Abscess drainage combined with myringotomy and IV antibiotics has not been advocated for Bezolds abscesses in the literature, as it has been for the subperiosteal abscess. How- ever, time and investigation may prove that this complication can be man- aged by less aggressive surgical options. Intratemporal (extracranial) complications Labyrinthine stulae Labyrinthine stulae continue to be among the most common complica- tions of chronic otitis with associated cholesteatoma, and have been re- ported in approximately 7% of cases [911]. Few circumstances are more unsettling to an otologic surgeon than the presence of an open labyrinth found at the time of cholesteatoma surgery. The risk of signicant sensori- neural hearing loss as a result of surgical manipulation makes the open lab- yrinth and its management a highly controversial topic. As a result of its location near the antrum, the horizontal semicircular ca- nal is the most commonly involved portion of the labyrinth, and accounts for approximately 90% of these stulae (Fig. 2). Although the horizontal canal is usually involved, stulae have been described in both the superior canal and posterior canal, and in the cochlea itself. Cochlear stulae are as- sociated with a much higher incidence of hearing loss encountered during surgical manipulation than are labyrinthine stulae [10]. Fig. 2. Intraoperative picture of a horizontal canal stula. 1240 SMITH & DANNER Erosion of the bone of the otic capsule can occur through two distinct processes. In the presence of a cholesteatoma, activated mediators from the matrix, or pressure from the cholesteatoma itself, can lead to osteolysis and uncovering of the labyrinth. However, labyrinthine stulae can occur from resorption of the otic capsule due to inammatory mediators in the ab- sence of cholesteatoma, which typically occurs in COM with granulation [4]. One reason for the confusion and controversy in discussing these stulae is the lack of an accepted staging system. Multiple staging systems have been proposed [9,12]. The system introduced by Dornhoer and Milewski [9] is the classication used in the authors department, and is used in this article to discuss stulae and their management (Fig. 3). This system stages stulae with respect to the involvement of the underlying labyrinth. Fistulae with bony erosion and intact endosteum are classied as type I stulae. If the endosteum is violated, but the perilymphatic space is preserved, the stula is staged as type IIa. When the perilymph is violated by disease or inadver- tently suctioned, the stula is labeled as type IIb. Type III stula indicates that the membranous labyrinth and endolymph have been disrupted by dis- ease or surgical intervention [9]. Diagnosis Patients who have signicant erosion of the labyrinth classically present with subjective vertigo and a positive stula test on examination. Unfortu- nately, this classic picture is not sensitive in the preoperative identication of a stula. Periodic vertigo or signicant disequilibrium is found in 62% to 64% of patients who have stulae preoperatively. The stula test is positive in 32% to 50% of patients who are found ultimately to have stulae during surgical exploration. Although sensorineural hearing loss is found in most of these patients (68%), it is not a sensitive indicator of stula [9,10]. Although the presence of sensorineural hearing loss, vertigo, or a positive stula test in a patient who has a cholesteatoma should raise the suspicion for a stula, their absence does not guarantee an intact bony labyrinth. It is for this reason that a prudent surgical approach is to assume the presence of a stula in every cholesteatoma case, to prevent unexpected complications. Although universal imaging of all patients who have cholesteatoma has not been the standard, review of the literature demonstrates that the use Fig. 3. A stula staging system. (Adapted from Dornhoer JL, Milewski C. Management of the open labyrinth. Otolaryngol Head Neck Surg 1995;112(3):4104.) 1241 COMPLICATIONS OF CHRONIC OTITIS MEDIA AND CHOLESTEATOMA of preoperative CT imaging is increasing in this country [10]. Because of the inability to accurately diagnose stulae preoperatively on clinical grounds, the increase in imaging is likely an attempt to increase the detection of an exposed labyrinth, facial nerve, or dura, to aid in surgical planning (Fig. 4). Unfortunately, the ability to detect stulae accurately on preoper- ative CT has been reported as 57% to 60% [10,13]. In one of these reports, when subjects had stulae detected intraoperatively, their preoperative CT scan was reviewed retrospectively to look for radiologic evidence. Despite the knowledge of a conrmed stula, CT evidence could only be found in 60% of these cases [13]. In the labyrinth, where millimeters matter, CT scans of the temporal bone with 1 mm cuts can miss a thin layer of cortical bone, which falsely increases the concern for stula. Also, a small stula easily can be missed between cuts on the CT images. Although the debate regarding the need for preoperative imaging for cholesteatoma cases will continue, in current reports CT scans are no more sensitive than history and physical examination in detecting labyrinthine stulae. The denitive diagnosis for a stula is only made intraoperatively, which rearms the need to approach all cholesteatoma cases with caution. Management A tympanomastoidectomy is required for the treatment of the cholestea- toma, but the most appropriate management of the stula remains an ongo- ing debate. Some investigators believe that the most appropriate approach to the stula is to perform a canal wall down mastoidectomy, remove the bulk of the cholesteatoma, and leave the stula covered with the matrix ex- teriorizing it into the cavity [14,15]. Advocates of this approach argue that the complete removal of the matrix increases the risk of sensorineural hear- ing loss, and that by removing the sac itself, the pressure from the cholestea- toma is relieved and further bony erosion or infectious complications are unlikely. Other investigators advocate the complete removal of the choles- teatoma over the stula, with repair of the bony defect in all circumstances Fig. 4. A coronal CT scan of a postoperative right ear, revealing a horizontal canal stula. 1242 SMITH & DANNER [12,16]. These investigators feel that by removing the matrix in its entirety, the potential risk of continued bony erosion and infectious complications such as labyrinthitis can be prevented. Furthermore, they argue the risk of signicant sensorineural hearing loss with complete removal is minimal, and the long-term risk of sensorineural hearing loss is greater if the matrix is left intact. Complete removal can be performed in a single setting, or in a staged manner, with a second-look procedure. A recent review of the liter- ature found that hearing preservation for patients who underwent complete removal was equivalent to patients where the matrix was left over the stula [10]. It is impossible to know if the extent of disease was similar in these two groups because an accepted staging system was not used for comparison. The size, extent, and location of the stula should be considered when de- termining whether complete cholesteatoma removal should be attempted. Multiple studies have demonstrated that larger stulae have worse hearing results postoperatively [17,18]. Dornhoer evaluated hearing results after single-stage, complete cholesteatoma removal, and compared results, based on extent of disease [9]. No hearing loss resulted from the removal of type IIa stulae where the endosteum was violated but the perilymphatic space was not disrupted or suctioned signicantly. For type IIb and type III stu- lae, where the perilymph or endolymph was involved or violated signi- cantly, roughly one half of the subjects experienced signicant hearing loss postoperatively (8 of 17). Subjects who had stulae with signicant labyrinthine involvement (types IIb and III) who received intraoperative steroids experienced stable or improved hearing 90% of the time (9 of 10); and the one subject who had hearing loss sustained only moderate high fre- quency hearing loss. The anatomic location of the stula also impacts hearing results signicantly. A 35% rate of profound deafness has been reported with stulae that involve the promontory, compared with a 3% rate of deafness with semicircular canal stulae [10]. The debate over the most appropriate management of labyrinthine stu- lae continues, but a review of the literature can help with some recommen- dations. Small stulae involving the labyrinth can be removed safely in a primary setting. The use of corticosteroids at the time of cholesteatoma removal from stulae may have a protective eect on hearing. Large stulae involving the labyrinth can be treated by carefully removing the cholestea- toma matrix and covering the defect with bone pa te and fascia (Fig. 5). However, stulae involving the cochlea should be approached with more caution because of the greater risk for iatrogenic sensorineural hearing loss, and exteriorization of matrix remaining on the stula may be the best option. Coalescent mastoiditis Mastoiditis is a spectrum of disease that must be dened appropriately to be treated adequately. Mastoiditis, dened as mucosal thickening or 1243 COMPLICATIONS OF CHRONIC OTITIS MEDIA AND CHOLESTEATOMA a mastoid eusion, is common in the face of an acute or chronic otitis, and is appreciated routinely on a CT scan performed in this setting. This entity is of little clinical signicance. Clinical mastoiditis presenting with postauricu- lar erythema, tenderness, and edema, with an inferiorly and posteriorly displaced auricle, is a distinctly dierent scenario. In this setting, further workup is indicated to determine the most appropriate treatment. Fig. 6 demonstrates the dierence between coalescent mastoiditis and a mastoid eusion, as seen on CT scan. Diagnosis In the presence of clinical mastoiditis, a CT scan should be performed to evaluate for an unappreciated subperiosteal abscess or coalescent mastoid- itis (see Fig. 1). Coalescent mastoiditis is an acute, infectious process of the mastoid bone, with characteristic loss of trabecular bone. It is a rare complication, and is seen usually in young children with AOM. Classically, coalescent mastoiditis is described as occurring in a well pneumatized mas- toid with a signicant, incompletely treated AOM, whereas chronic otitis and cholesteatoma occur in a sclerotic temporal bone. However, as many as 25% of cases of coalescent mastoiditis have been reported to occur in a sclerotic temporal bone with COM and cholesteatoma [19]. Fig. 5. The repair of a labyrinthine stula after removal of cholesteatoma. Fig. 6. Coalescent mastoiditis in a sclerotic temporal bone with COM (A), as compared with a mastoid eusion in a well-aerated temporal bone with AOM (B). 1244 SMITH & DANNER Management Coalescent mastoiditis is a serious medical problem that requires aggres- sive treatment, either surgical or medical. Classically, treatment included IV antibiotics and mastoidectomy with removal of necrotic, devitalized bone. In recent years, the use of myringotomy and IV antibiotics has been advo- cated as an alternative [3,20]. This medical management requires a CT scan to conrm resolution of the infection and aeration of the mastoid. The pres- ence of a cholesteatoma is a surgical indication, and therefore coalescent mastoiditis in this setting is a surgical disease. In this case, a tympanomastoi- dectomy is performed to remove devitalized bone and the cholesteatoma, and to re-establish aeration to the mastoid and middle ear. Petrous apicitis The petrous apex comprises the anterior, medial portion of the temporal bone, and has been reported to be pneumatized in 30% of individuals [3]. These air cells, when present, are in continuity with the middle ear and mas- toid through well-described cell tracts around the labyrinth, allowing for in- fection involving the mastoid and middle ear cleft to extend into the petrous apex. Petrous apicitis is a spectrum of disease much like mastoiditis, and can involve anything from an asymptomatic eusion to coalescence and abscess formation. Infection of the petrous apex is a dangerous entity because of its proximity to the middle and posterior cranial fossae and their contents. Diagnosis The classic symptomatology associated with petrous apicitis is a triad of deep retro-orbital pain, aural discharge, and sixth nerve palsy, also known as Gradenigos syndrome. Although these symptoms can be associated with apicitis, they are by no means pathognomonic of this condition. Retro-orbital pain and abducens palsy have been reported to occur in 50% and 25% of reported cases, respectively [21]. Petrous apicitis becomes evident only after failure to control chronic suppurative otomastoiditis with prolonged medical and surgical management. When apicitis is suspected, a CT scan should be performed to make the diagnosis and to evaluate sur- rounding anatomy. A CT scan will also aid in the diagnosis of intracranial complications that often accompany this condition. Some believe that once the diagnosis is made with clinical evaluation and CT, an MRI of the brain or a lumbar puncture should be performed to evaluate for intracranial com- plications [4]. Management Early in the twentieth century, before the widespread use of antibiotics, surgical intervention for petrous apex abscesses and petrous apicitis was rel- atively common. However, as the use of antibiotics has increased, the prev- alence of apicitis has decreased signicantly. The petrous apex is an area that is not easily approached surgically because of its relationship with 1245 COMPLICATIONS OF CHRONIC OTITIS MEDIA AND CHOLESTEATOMA the otic capsule and carotid artery. Because of the dicult surgical approach and the response rate to antibiotics, IV antibiotics are often the rst-line treatment of petrous apicitis. IV antibiotics require a long duration of treat- ment. Serial C-reactive protein levels and erythrocyte sedimentation rates have been used to monitor for response of bony infections to medical man- agement elsewhere in the body [22], and are a reasonable option for follow- ing a patient who has petrous apicitis to assess for response. In the presence of abscess, necrotic bone, or persistent infection despite medical therapy, surgical drainage is required. Air cell tracts extend to the apex below, above, and anterior to the labyrinth. In a hearing ear, these three air cell tracts al- low several possibilities for surgical approach including: infracochlear, infralabyrinthine, retrolabyrinthine, subarcuate, and even middle fossa [4]. In a nonhearing ear, the translabyrinthine or transcochlear approaches are reasonable and give wide exposure to the aected area. One disadvan- tage of these two approaches is that they could potentially expose the cere- brospinal uid (CSF) to the infectious process. Facial paralysis Otogenic causes of facial nerve paralysis include AOM, COM without cholesteatoma, and cholesteatoma. The rst usually occurs with a dehiscent fallopian canal within its tympanic segment, allowing direct contact of in- ammatory mediators with the facial nerve itself. COM with or without cholesteatoma can result in facial paralysis through involvement of a dehis- cent nerve, or through bony erosion. Facial paralysis secondary to AOM of- ten presents in children with incomplete paresis that comes on abruptly and is usually short-lived with appropriate treatment. On the other hand, paral- ysis secondary to COM or cholesteatoma often presents with slowly pro- gressive facial paralysis and has a worse prognosis [3]. Diagnosis The diagnosis of otogenic facial paralysis is made on clinical grounds. Facial paresis or paralysis in the presence of AOM, COM, or cholesteatoma is not a dicult diagnosis to make by examination alone. The role of diag- nostic CT imaging is questionable. Although a CT scan is not required, it can be useful in therapeutic planning and patient counseling. When choles- teatoma involves the fallopian canal, it may also erode structures such as the labyrinth or tegmen. Furthermore, the extent of bony erosion of the fallo- pian canal and degree of involvement is better appreciated on CT [23]. Management Although facial paralysis secondary to AOM usually is treated with ap- propriate antibiotics and myringotomy, the treatment of paralysis with COM with or without cholesteatoma requires surgical intervention. When facial paralysis is associated with cholesteatoma, a mastoidectomy is 1246 SMITH & DANNER performed to remove the cholesteatoma or granulation tissue that is contact- ing the facial nerve. The nerve is approached on both sides of the involve- ment, and a diamond burr is used to expose the epineurium on either side of the diseased segment. Once the proximal and distal segments of the fallo- pian canal have been opened, blunt dissection is used to remove the disease from the epineurium. The nerve sheath does not have to be incised unless the cholesteatoma has invaded the nerve itself [4]. Although cholesteatoma can involve the facial nerve at any point through its intratemporal course, the tympanic segment and second genu are involved most commonly [24]. Intracranial complications Meningitis Meningitis is the most common intracranial complication of acute and COM; conversely, AOM is the most common secondary cause of meningitis [25,26]. In a recent series of COM complications, meningitis occurred in ap- proximately 0.1% of subjects [1,4]. Although this remains a signicant com- plication, the mortality rate from otitic meningitis has declined signicantly, from 35% in the preantibiotic era to 5% in the postantibiotic era [27]. Men- ingitis can arise from three distinct otogenic routes: hematogenous seeding of the meninges and subarachnoid space; spread from the middle ear or mastoid through preformed channels (Hyrtls ssures); or through bony ero- sion and direct extension. Of these three possibilities, otogenic meningitis most commonly results from hematogenous seeding [3,4]. Diagnosis The prompt diagnosis of meningitis relies on the recognition of warning signs by an astute clinician. Signs that should increase the suspicion of an intracranial complication include persistent or intermittent fever; nausea and vomiting; irritability; lethargy; or persistent headache. Ominous signs virtually diagnostic of an intracranial process include visual changes; new onset seizures; nuchal rigidity; ataxia; or decreased mental status [3]. If any of these suspicious or ominous signs occur, immediate treatment and further workup are critical. Broad-spectrum antibiotics, such as third-gener- ation cephalosporins, should be administered while diagnostic tests are ordered and arranged. A contrasted CT scan or MRI will show character- istic meningeal enhancement and rule out additional intracranial complica- tions known to occur in up to 50% of these cases [28]. In the absence of a signicant mass eect on imaging, a lumbar puncture should be performed to conrm the diagnosis and to allow for culture and sensitivity. Management The presence of symptoms suspicious for otogenic intracranial complica- tions warrants the use of broad-spectrum IV antibiotic therapy while the 1247 COMPLICATIONS OF CHRONIC OTITIS MEDIA AND CHOLESTEATOMA workup is being performed. If meningitis is conrmed by imaging and lum- bar puncture, IV antibiotics should be continued. In the presence of COM with or without cholesteatoma, gram-negative and anaerobe coverage is necessary. In addition to IV antibiotics, the use of systemic corticosteroids is benecial because they have been shown to decrease auditory and neuro- logic sequelae [29]. Corticosteroids should be administered as early in the course as possible to maximize their ecacy. The role of mastoidectomy in this diagnosis is not completely clear. Indications for mastoidectomy in- clude the presence of cholesteatoma; coalescent mastoiditis; bony erosion with direct extension of disease; or persistence of symptoms despite maximal medical therapy [3]. Although debated, a trial of IV antibiotics, steroids, and myringotomy is a reasonable treatment option for meningitis secondary to AOM or COM without cholesteatoma [3]. Brain abscess Brain abscess is the second most common intracranial complication of otitis media after meningitis, but it is perhaps the most lethal. In contrast to meningitis, which is caused more frequently by AOM, brain abscesses al- most exclusively result from COM [30]. The temporal lobe and cerebellum are aected most often. These abscesses develop as a result of hematogenous extension secondary to thrombophlebitis in virtually all cases, but tegmen erosion with epidural abscess can lead to temporal lobe abscess. Cultures of these abscesses are often sterile, and, when positive, usually reveal mixed ora; however Proteus is cultured more frequently than any other pathogen [30]. The clinical progression seen in these patients occurs in three named stages. The rst stage is described as the encephalitic stage, and includes the u-like symptoms of fever, rigors, nausea, vomiting, headache, and men- tal status changes or seizures. This stage is followed by the quiescent, or latent, stage, in which acute symptoms abate, but general fatigue and list- lessness persist. The third and nal stage marks the return of acute symp- toms, including severe headaches, vomiting, fevers, mental status changes, hemodynamic changes, and increased intracranial pressure. This third stage is attributable to rupture or expansion of the abscess cavity [3]. Diagnosis As with meningitis, the presence of any symptoms that might indicate in- tracranial involvement requires prompt action. In the presence of these symptoms, a contrasted CT scan or MRI should be ordered while IV anti- microbial therapy is initiated. For brain abscesses, MRI is superior (Fig. 7). Although the MRI gives better detail regarding the abscess itself, a CT scan gives valuable information about bony erosion of the mastoid, and can help in determining the cause of the abscess and the most appropriate treatment options. The imaging itself is diagnostic of a signicant parenchymal abscess, and a thorough evaluation of the imaging is required to rule out 1248 SMITH & DANNER concomitant intracranial complications, or evidence of increased intracra- nial pressure. Management Immediate initiation of broad-spectrum antibiotics that cover gram pos- itives, gram negatives, and anaerobes is necessary because of the severity of this infection and its polymicrobial nature. As in otitic meningitis, these an- tibiotics should be initiated while further workup is being performed. Once the diagnosis of a brain abscess is made, surgical intervention is required. Drainage of the abscess requires neurosurgical intervention, but the patient must be stabilized from a neurologic standpoint. IV steroids are often given to decrease brain edema, and anticonvulsants are given to prophylax against seizures. When the patient is stable, neurosurgical drainage is performed, either through an open craniotomy with drainage or excision, or by stereo- tactic aspiration through a burr hole. This procedure not only drains the abscess, but provides a culture, enabling antibiotic therapy to be tailored. Drainage of the brain abscess is paramount, and should be performed within 24 hours of presentation, if the patient is stable [3]. However, the most appropriate way to treat the otologic component is somewhat controversial. In the setting of AOM, myringotomy with evacu- ation of the purulent eusion is sucient. In the presence of COM with or without cholesteatoma, a mastoidectomy is required to eradicate the source of infection. The most appropriate time to perform the mastoidec- tomy is controversial. It has been conventional teaching that a mastoidec- tomy is performed in a delayed manner after the patient recovers from the abscess and neurosurgical drainage. Current recommendations, how- ever, are to perform a mastoidectomy at the time of abscess drainage to Fig. 7. An axial T1-weighted MRI (A) and a coronal T2-weighted MRI (B) demonstrating a large right cerebellar brain abscess with resulting hydrocephalus. 1249 COMPLICATIONS OF CHRONIC OTITIS MEDIA AND CHOLESTEATOMA remove the infectious focus, assuming the patient is stable enough to toler- ate this additional surgery. However, specic parameters dictating staged versus simultaneous surgery have not yet been reported or established. One recent report has advocated primary mastoidectomy with needle aspi- ration of the abscess through the dura exposed in the mastoidectomy cavity [30]. Regardless of the type of surgical intervention, when it has been com- pleted, IV antibiotics should be continued for several weeks and serial CT scans with contrast followed to assure resolution of the abscess [3]. Lateral sinus thrombosis Sigmoid sinus or lateral sinus thrombosis is a well-known complication of otitis media that compromises 17% to 19% of intracranial complications [26,31]. The proximity of the middle ear and mastoid air cells to the dural venous sinuses predisposes them to thrombosis and thrombophlebitis sec- ondary to infection and inammation in the middle ear and mastoid. In- volvement of the sigmoid or lateral sinus can result from bony erosion secondary to COM and cholesteatoma, with direct extension of the infec- tious process to the perisinus space, or from the seeding of the space from thrombophlebitis of mastoid emissary veins. Once the sinuses have become involved, and an intramural thrombus develops, any number of serious complications can result. Otitic hydrocephalus is known to complicate a sig- nicant number of these cases. The infected clot can propagate proximally to involve the conuence of sinuses (torcular herophili) and sagittal sinus, causing life-threatening hydrocephalus, or propagate distally to involve the internal jugular vein [3]. Involvement of the internal jugular vein in- creases the risk of septic pulmonary emboli. Diagnosis The classic presentation of sigmoid or lateral sinus thrombosis is the pres- ence of high spiking fevers in a picket fence pattern, often seen with head- aches and general malaise [2]. Like many of these complications, a high degree of suspicion is required because the spiking fevers may be blunted by concurrent antibiotic use. With the presence of high spiking fevers, or concern for increased intracranial pressure, a contrasted CTscan should be performed to screen for thrombophlebitis. The sinus wall will enhance brightly with con- trast and produce the characteristic delta sign associated with sinus thrombo- sis (Fig. 8). With the presence of signicant sinus thrombosis, an MRI and magnetic resonance venogram (MRV) are warranted, because they can be used serially to evaluate for clot propagation or resolution. Management Dural venous thrombosis in the presence of chronic otomastoiditis with or without cholesteatoma is a surgical disease. At a minimum, a mastoidec- tomy with removal of chronic infection, granulation, and cholesteatoma is 1250 SMITH & DANNER required. The sigmoid sinus is exposed and the surrounding epidural abscess or granulation is removed. The best way to manage the sinus itself is a point of contention in the otology literature. Classically, most texts recommend a diagnostic needle aspiration of the aected sinus, once it is exposed surgi- cally. If the aspiration reveals normal blood return, then the sinus is left in- tact; but if the aspiration is negative or reveals frank pus, the sinus is opened and at least a portion of the infected clot is evacuated. However, recent re- ports have challenged this dictum, and demonstrated that if the surrounding granulation tissue and inammation are removed through a mastoidectomy, the sinus will recannalize without clot evacuation [32]. One report demon- strated that with sinus thrombosis in the presence of AOM, myringotomy and IV antibiotics resolved the infection, and the sinus was shown to recan- nulate in three subjects without mastoidectomy [33]. Ligation of the internal jugular vein is not necessary unless there is evidence of continued septic em- bolization after surgical intervention and IV antibiotics. After surgical inter- vention, the patient should remain on IV antibiotics for at least 2 weeks, at which point a repeat MRI and MRV should be performed to rule out the development of a secondary intracranial complication such as brain abscess, or propagation of the thrombus into the superior sagittal sinus. [2] Systemic anticoagulation is not necessary unless the clot is shown to involve the sag- ittal sinus, or signs of increased intracranial pressure persist despite medical management. Epidural abscess The presence of an epidural abscess can often be insidious in develop- ment. These abscesses develop as a result of bony destruction from Fig. 8. A contrasted axial CT scan demonstrating right sigmoid sinus thrombosis with an empty delta sign. 1251 COMPLICATIONS OF CHRONIC OTITIS MEDIA AND CHOLESTEATOMA cholesteatoma or from coalescent mastoiditis. The signs and symptoms do not dier signicantly from those found in COM. Occasionally, dural irrita- tion can result in increased otalgia or headaches that serve as a concerning sign in the background of COM. Because this complication can be subtle in presentation, it is often found incidentally at the time of cholesteatoma sur- gery or CT scan for other purposes. Diagnosis Unlike other intracranial complications, there are no sensitive or specic symptoms suggestive of this disease process. A high degree of clinical suspi- cion is required to diagnose an epidural abscess preoperatively. The presence of increased otalgia or headache should raise the suspicion for an intracra- nial complication, and warrants imaging. A contrasted CT scan or MRI is sucient to diagnose this abscess. Even with a careful evaluation, this diag- nosis is often made at the time of surgery. Management When an epidural abscess is appreciated intraoperatively or on CT scan, surgical drainage is necessary. A mastoidectomy is performed to treat the underlying pathology, paying careful attention to thin the bony tegmen and bone overlying the posterior fossa dura as much as possible, so epidural pus or granulation can be appreciated. The bone overlying the dura is re- moved to evacuate the pus and granulation until normal dura is encountered [4]. Postoperative antibiotics are continued at least until the symptoms of the abscess and otitis have resolved. Otitic hydrocephalus Otitic hydrocephalus is described as signs and symptoms indicative of in- creased intracranial pressure with normal CSF studies on lumbar puncture, which can present as a complication of AOM, COM, or otologic surgery. Otitic hydrocephalus is somewhat of a misnomer, and its pathophysiol- ogy is not understood completely. It is a misnomer because this condition can be found in the absence of otitis, and patients do not have dilated ven- tricles indicative of true hydrocephalus. Symonds [34], who coined the term otitic hydrocephalus, felt that this condition developed from infection of the lateral (transverse) sinus, with extension of thrombophlebitis into the con- uence of sinuses to involve the superior sagittal sinus. Inammation or in- fection of the superior sagittal sinus prevents CSF absorption through the arachnoid villi, resulting in increased intracranial pressure. This infectious thrombophlebitis usually occurs as a result of otologic infection, but multi- ple cases have been described in the absence of otitis or otologic surgery [34,35]. Furthermore, although lateral sinus thrombosis is found usually in the presence of otitic hydrocephalus, cases have been reported without thrombosis of the dural sinuses [36]. 1252 SMITH & DANNER Diagnosis The diagnosis of otitic hydrocephalus is one of exclusion, and requires a high degree of suspicion to recognize the suggestive symptoms. The symp- toms found in these patients are a result of increased intracranial pressure and include diuse headaches, nausea, vomiting, visual changes, and leth- argy. The presence of these symptoms requires a thorough examination and imaging. A dilated fundoscopic examination should be conducted to evaluate for papilledema as evidence of increased intracranial pressure. An MRI and MRV should be performed to evaluate for ventricular enlarge- ment, or coexisting intracranial complications, such as signicant sinus thrombosis with obstruction. Increased intracranial pressure with clinical symptoms and papilledema in the absence of ventricular dilation or menin- gitis is enough to make this diagnosis. MRV will conrm the presence and extent of dural sinus thrombosis, but is not required to make a diagnosis of otitic hydrocephalus. Management The goal in the treatment of otitic hydrocephalus is to treat any underly- ing infectious otitis, decrease intracranial pressure, and prevent the poten- tially devastating complication of optic nerve atrophy. With COM (with or without cholesteatoma) in the presence of dural sinus thrombosis, a mas- toidectomy should be performed to remove the infectious process and the cholesteatoma, and to address the dural venous sinus. In the absence of a surgical indication such as cholesteatoma or tumor, the treatment is med- ical in nature, and should include acetazolamide, uid restriction, and cor- ticosteroids to decrease intracranial pressure and cerebral edema. Systemic anticoagulation is not required unless an MRV shows sinus thrombophlebi- tis involves the sagittal sinus. In these cases, the risk of neurologic sequelae and death are signicant enough to warrant anticoagulation [35]. If aggres- sive medical management does not normalize the intracranial pressure, lum- bar drainage of CSF can be performed serially or by a lumbar drain. If prolonged drainage is required because of recalcitrant papilledema, a shunt may be required [4,35]. Summary The incidence of extracranial and intracranial complications of COM and cholesteatoma has decreased since the proliferation of antibiotics early in the twentieth century. However, these complications continue to occur, and can be lethal if they are not identied and treated properly. Therapy for the complications associated with COM, unlike those of AOM, usually includes surgical intervention, in addition to medical therapy. As medical (antibiotic) therapy continues to improve, and new imaging techniques are introduced, less invasive treatment modalities may be shown to be as eec- tive as the classic, time-tested, surgical options. 1253 COMPLICATIONS OF CHRONIC OTITIS MEDIA AND CHOLESTEATOMA References [1] Osma U, Cureoglu S, Hosoglu S. The complications of chronic otitis media: report of 93 cases. J Laryngol Otol 2000;114(2):97100. [2] Agrawal S, Husein M, MacRae D. Complications of otitis media: an evolving state. J Oto- laryngol 2005;34(Suppl 1):S339. [3] Harker LA, Shelton C. Complications of temporal bone infections. In: Cummings CW, editor. Cummings otolaryngology head and neck surgery. 4th edition. Philadelphia: Elsevier Mosby; 2005. p. 301338. [4] Hashisaki GT. Complications of chronic otitis media. In: Canalis RF, Lambert PR, editors. The ear: comprehensive otology. Philadelphia: Lippincott Williams & Wilkins, 2000. p. 4336. [5] Bauer PW, Brown KR, Jones DT. 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Ezekiel J. Emanuel, Christine Grady, Robert A. Crouch, Reidar Lie, Franklin Miller, David Wendler - The Oxford Textbook of Clinical Research Ethics (2008)