Conflicting reports and surgeon opinions have contributed to a long-standing debate regarding the merits of the intact canal wall versus canal wall down approach to cholesteatoma. The objective of this analysis was to identify and synthesize available data concerning rates of recidivism after the two primary types of cholesteatoma surgery. PubMed, Cochrane Collaboration, and Google Scholar searches were performed and articles filtered based on predetermined exclusion criteria. Individually reported rates of recurrent and residual disease were extracted and recorded. Meta-analysis demonstrated a relative risk of 2.87 with a confidence interval of 2.45-3.37, confirming a significantly increased incidence of postoperative cholesteatoma when using an intact canal wall approach rather than a canal wall down approach. Next, rates of recidivism following the typical two-stage intact canal wall operation were compared with a single-stage canal wall down operation and found to be similar. In conclusion, we advocate that greater consideration should be given to the canal wall down procedure in initial surgical management and identify the need for further exploration of rates of recidivism after staged or second-look procedures.

OBJECTIVE: A cholesteatoma is a mass of keratinising epithelium in the middle ear. It is a rare disorder that is associated with significant morbidity, and its causative risk factors are poorly understood; on a global scale up to a million people are affected by this each year. We have conducted a systematic literature review to identify reports about the heritability of cholesteatoma or any constitutional genetic factors that may be associated with its aetiology. DATA SOURCES: A systematic search of MEDLINE (EBSCO) and 2 databases of curated genetic research (OMIM and Phenopedia) was conducted. STUDY SELECTION: The participants and populations of interest for this review were people treated for cholesteatoma and their family members. The studies of interest reported evidence of heritability for the trait, or any association with congenital syndromes and particular genetic variants. DATA EXTRACTION: The searches identified 449 unique studies, of which 35 were included in the final narrative synthesis. DATA SYNTHESIS: A narrative synthesis was conducted and data were tabulated to record characteristics, including study design; genetic data; and author conclusions. Most of the studies identified in the literature search, and described here, are case reports and so represent the lowest level of evidence. In a few case-reports, congenital and acquired cholesteatoma have been shown to segregate within families in the pattern typical of a monogenic or oligogenic disorder with incomplete penetrance. Evidence from syndromic cases could suggest that genes controlling ear morphology may be risk factors for cholesteatoma formation. CONCLUSIONS: This is the first systematic review about the genetics of cholesteatoma; and we have identified a small body of relevant literature that provides evidence of a heritable component for its aetiology. Cholesteatoma is a complex and heterogeneous clinical phenotype, it is often associated with chronic otitis media and with some rare congenital syndromes known to affect ear morphology and related pathologies. This article is protected by copyright. All rights reserved.

OBJECTIVE: The purpose of this study was to assess the value of ear endoscopy in cholesteatoma surgery and to demonstrate its consequence in improving surgical outcome. MATERIALS AND METHODS: A total of 92 ears with acquired cholesteatoma (primary or secondary) were operated on. In this prospective study, 82 cases were operated on by using canal wall up (CWU) technique, and 10 cases were on operated on by using canal wall down (CWD) procedure. Endoscopically guided ear surgery was incorporated complementary to the microscope as a principal part in the procedure. Second-look endoscopic exploration was performed on some selected cases, depending on the finding during the primary surgery and the postoperative findings of clinical and computed tomographic studies. RESULTS: In the primary surgery after completion of microscopic cleaning, the overall incidence of intraoperative residuals detected with the endoscope was 22.8%. Sinus tympani was the most common site of intraoperative residuals in both CWU and CWD groups, followed by the facial recess and the undersurface of the scutum in the CWU cases. Reconstruction of the hearing mechanism was performed in the primary surgery in 86 cases (93.5%) and postponed to the second stage in only six cases (6.5%). Out of the 82 CWU cases, 35 second-look endoscopic explorations (42.7%) were performed. Three recurrences (8.6%) were identified. Two cases showed a tiny residual cholesteatoma pearl, and the third showed a larger open residual cholesteatoma filling the sinus tympani and extending to the aditus. In this series, no morbidity or complication was encountered secondary to the use of endoscopes in the mastoid or middle ear. CONCLUSION: Incorporating the endoscope into the surgical armamentarium in otology contributes much to the concept of minimally invasive surgery. Minimally invasive endoscopic ear surgery should be accepted as a new horizon in ear surgery. In this study, it became obvious that despite the use of the endoscope in conjunction with the operating microscope, 100% eradication of the disease still could not be achieved; however, the use of endoscopes did reduce the residual cholesteatoma rate.

OBJECTIVES: Microscopic postauricular tympanomastoidectomy provides a limited exposure to the attic, especially anteriorly. In contrast, the endoscope offers wide transcanal access to the attic, allowing for complete removal of limited attic disease, possibly without interrupting the ossicular chain. This report evaluates 8 years of experience with transcanal endoscopic management of limited attic cholesteatoma. STUDY DESIGN: Case series. METHODS: Seventy-three ears with limited attic cholesteatoma underwent endoscopic transcanal tympanotomy and extended atticotomy to access and completely remove the sac. Disease was dissected off the tegmen, the medial and lateral attic walls, and the ossicles. Appropriate ossicular reconstruction was performed. The defect was reconstructed with a composite tragal graft. RESULTS: A transcanal endoscopic approach was adequate for removal of disease in all cases. There were no iatrogenic facial nerve injuries. Bone thresholds were stable. Disease was dissected off the head of the malleus and the body of incus with preservation of both in 24 ears. Mean follow-up was 43 months. Five ears required revision for recurrent disease, and eight were revised for failed ossicular reconstruction or persistent perforation. Moderate to severe retraction in other areas of the tympanic membrane was evident in 28 cases; none of these required further intervention. CONCLUSION: An endoscopic technique allows transcanal, minimally invasive, eradication of limited attic cholesteatoma. Preservation of the ossicles coupled with complete removal of disease is more likely with the endoscope.

OBJECTIVES: The purpose of this study was to examine the utility of using an endoscope in cholesteatoma surgery and to demonstrate how it allows a reduction in the incidence of residual disease. MATERIAL AND METHODS: Thirty-two ears with acquired cholesteatoma (primary) were resected. Twenty cases were resected using a canal wall up (CWU) technique and six cases using a canal wall down (CWD) technique, and in six cases, a transcanal tympanotomy-atticotomy was performed. All of the patients in our study group underwent explorative and operative endoscopic ear surgery complementary to use of the operating microscope to uncover and remove residual cholesteatoma. RESULTS: In the primary surgery after completion of microscopic cleaning, the overall incidence of intraoperative residual disease detected with the endoscope was 37.5%. The sinus tympani was the most common site of intraoperative residual disease, followed by the anterior epitympanic recess and protympanum. Of the 20 CWU cases, 12 second-look endoscopies were performed. Two residuals were identified, both in the sinus tympani. No significant complications were associated with the 32 endoscopic procedures. CONCLUSION: The endoscope allowed a better understanding of cholesteatoma and improved eradication of residual disease from hidden areas such as the anterior epitympanic recess, retrotympanum, and hypotympanum not yet controllable by the operating microscope.

A series of 221 ears with chronic suppurative otitis media without cholesteatoma is presented--84% of the cases were treated using one-stage tympanomastoidectomy and 15% underwent cortical mastoidectomy with planned second-stage tympanoplasty. Mean follow-up period was 6.3 years. Control of infection succeeded in 92% after the primary operation. Failures were most common in ears infected with Pseudomonas aeruginosa. Postoperative cholesteatoma developed in 5 ears (2.2%). Hearing results were unsatisfactory; a postoperative air-bone gap within 20 dB was achieved in only 62%. In revision operations, retained mastoid air cells were found in 64% of ears with recurrent or persistent discharge. Thirty-seven percent of patients with unsuccessful outcome were observed to have a possible underlying or concomitant disease. The importance of intensive preoperative conservative treatment and careful surgical technique is stressed.

OBJECTIVES: Diffusion-weighted (DW) magnetic resonance imaging (MRI) is emerging as an alternative to second-look surgery in ruling out residual or recurrent disease after cholesteatoma eradication. However, the DW MRI appearances of postoperative or inflammatory mucosal changes have not been well investigated, thus rendering the interpretation of postoperative DW MRI difficult in the presence of mucosal reactions. We investigated the turbo-spin echo (TSE) DW MRI changes of the middle ear and mastoid mucosa after cholesteatoma surgery and compared these with the TSE DW MRI features of cholesteatoma with an aim to identify a rapid and cost-effective purely DW MRI sequence that can be used to screen for cholesteatoma. STUDY DESIGN: A prospective comparative study. SETTING: A tertiary referral center in Western Australia. PATIENTS: Patients undergoing revision or second-look cholesteatoma surgery. INTERVENTION: Patients underwent 3 to 6 monthly half-Fourier-acquisition single-shot turbo-spin-echo TSE DW MRI before their second surgery. The MRI findings were then correlated with the intraoperative findings at second-look surgery 6 to 17 months after primary surgery or of revision surgery in the cases that were referred from other centers. MAIN OUTCOME MEASURE: Detection of cholesteatoma and noncholesteatoma mucosal changes on TSE DW MRI, compared with the gold standard of findings at second surgery. RESULTS: Twenty-two patients underwent 23 second-look or revision procedures. All patients had DW MRI before their "second-look" or revision surgery. TSE DW MRI detected cholesteatomas in 7 patients whom all had disease confirmed at second-look or revision surgery. In 16 cases shown to be negative on DW MRI for cholesteatoma, all were confirmed to be disease-free on second-look surgery. Cholesteatomas were shown to produce a TSE DW MRI signal clearly distinct from the spectrum of imaging findings encountered in postoperative mucosal changes. CONCLUSION: TSE DW MRI holds great promise in screening for cholesteatoma as an alternative to exploratory second-look surgery.

OBJECTIVE: A systematic review to determine whether the diffusion-weighted (DW) magnetic resonance imaging scan can reliably detect residual or recurrent cholesteatoma after mastoid surgery. DESIGN: A systematic review. DATA SOURCES: Databases including EMBASE, MEDLINE, CINAHL, Web of Science, and Cochrane Review were searched for studies published without language restriction from the start of the databases. Additional studies were identified from cited references. SELECTION CRITERIA: Initial search identified 402 publications, of which 16 studies met the inclusion criteria for the systematic review. The DW imaging (DWI) scan was used to detect residual or recurrent cholesteatoma and subsequent second-look surgery was performed to correlate the findings. REVIEW METHODS: Studies were assessed for their selection of patients for radiologic investigations, imaging parameters, and subsequent surgery. Outcome measures included sensitivity, specificity, positive and negative predictive values of the DWI, and the incidence and size of residual or recurrent cholesteatoma. RESULTS: Two different modalities of DWI sequences have been described. Eight studies with 225 patients analyzed echo-planar imaging (EPI) and 8 studies with 207 patients described the "non-EPI" scanning techniques. Non-EPI parameters are more reliable in identifying residual or recurrent cholesteatoma with sensitivity, specificity, and positive and negative predictive values of 91%, 96%, 97%, and 85%, respectively. CONCLUSION: The available evidence suggests that non-EPI such as half-Fourier acquisition single-shot turbo spin echo sequences are more reliable in identifying residual or recurrent cholesteatoma. This is a promising radiologic investigation; however, we think further studies are required with more patients and long-term results to establish its place as an alternative to a second-stage surgery after canal wall up surgery.

Traditional surgery for cholesteatoma of the middle ear is performed by microscopic approaches. However, in recent years endoscopic instrumentation, techniques and knowledge have greatly improved, and in our opinion endoscopic surgical techniques will gain increasing importance in otologic surgery in the future. The aim of this study was to focus on outcomes obtained using endoscopic surgery for the treatment of middle ear cholesteatoma. A systematic review of the literature was performed. A total of 7 articles comprising 515 patients treated exclusively with endoscope or with a combined technique were found. During post-surgical follow-up, 48 (9.3%) patients showed a residual or recurrent pathology. Despite the small number of patients analyzed in our review, the outcomes of this technique appear to be promising. In particular, concerning the rates of recurrences and residual disease, endoscopic middle ear surgery appears to guarantee similar results in comparison to classic microscopic approaches with the advantage of performing minimally invasive surgery.

High recurrence rate of the middle ear cholesteatoma requires regular postoperative follow-up. This study evaluated data from the patients investigated with DW MRI to ascertain (1) the strength of the technique in detecting primary, and residual recurrent cholesteatoma, and (2) its accuracy in differentiating cholesteatoma from postoperative tissue changes. The diagnostic accuracy of two different DW imaging (EPI and non-EPI) techniques was evaluated. The data have been collected prospectively from 33 consecutive patients with either primary cholesteatoma, or with suspicious symptoms for potential cholesteatoma recurrence. The findings from non-EPI (HASTE) DW MR and EPI DW MR images were blindly compared with those obtained during a primary or secondary surgery. Preoperative non-EPI (HASTE) DWI pointed to a cholesteatoma in 25 out of 33 patients. In this subgroup, cholesteatoma were confirmed also by the surgery. In five cases, the non-EPI (HASTE) DWI did not show a cholesteatoma in the temporal bone, which agreed with the surgical findings. Three misclassifications were made by non-EPI (HASTE) DWI, all in the subgroup of patients indicated for primary surgery. The resulting pooled sensitivity of non-EPI (HASTE) DW imaging for diagnosing cholesteatoma in our study amounted to 96.15% (95% confidence interval (CI) 80.36-99.9), specificity was 71.43% (95% CI 29.04-96.33). Positive predictive value was 92.59% (95% CI 75.71-99.09) and negative predictive value 83.33% (95% CI 35.88-99.58). In conclusion, we recommend the non-EPI (HASTE) DW MRI as a valid method for diagnosing cholesteatoma and follow-up after cholesteatoma surgery.