Giant Cholesteatoma: A Case Report and Review of the Literature.

OBJECTIVE: To present a case of giant cholesteatoma and review of the contemporary literature. PATIENTS: A 61-year-old male presented with foul-smelling discharge from his right ear in the setting of a previous canal wall down mastoidectomy. Otomicroscopy demonstrated a mastoid cavity filled with infected keratinous debris. Neuro-imaging revealed soft tissue inflammatory changes in the mastoid cavity, with involvement of the temporomandibular joint (TMJ) and parotid gland and tegmen erosion. Biopsies showed evidence of desquamated epithelium consistent with cholesteatoma, with no malignant cells identified. Audiogram demonstrated a mean hearing loss of 65 dB on the right ear and an air-bone gap of 45 dB. Review of literature was also performed on giant cholesteatoma. INTERVENTIONS: He underwent a modified Fisch Type B infratemporal fossa approach to completely remove the cholesteatoma and a vascularized free flap was utilized to fill the surgical defect. MAIN OUTCOME MEASURES: Complete extirpation of choles-teatoma and resolution of otorrhoea. RESULTS: Repeat imaging showed complete removal of cholesteatoma and clinically there was resolution of the otorrhea. Review of the literature has shown a lack of consensus around the definition of giant cholesteatoma. Most cases in the literature demonstrated extensive involvement of anatomical spaces beyond the confines of the temporal bone to qualify for the diagnosis of giant cholesteatoma. CONCLUSIONS: Giant cholesteatoma should be suspected in cases of recurrent ear discharge following canal wall down mastoidectomy. The case we report add to the literature to benefit future patients in preoperative counseling and better inform management.

International Survey of Operative Practices for Otologists and Neurotologists During the COVID-19 Crisis.

OBJECTIVE: To investigate the influence of the COVID-19 pandemic on operative practices of otology and neurotology providers internationally. STUDY DESIGN: Cross-sectional survey. METHODS: A 78-question survey was distributed to otologists and neurotologists between May 12, 2020 and June 8, 2020 to assess the impact of the pandemic on surgical practices. Sections within the survey delineated time periods: prior to the crisis, onset of the crisis, during the crisis, postcrisis transition. RESULTS: Of 396 survey respondents, 284 participants from 38 countries met inclusion criteria.Respondents were 16.9% female and 82.4% male, with a most common age range of 40 to 49 years (36.3%). 69.8% of participants had been in practice for over 10 years and most respondents worked in an academic medical center (79.2%). The average operative weekly caseload was 5.3 (SD 3.9) per surgeon prior to the crisis, 0.7 (SD 1.2) during the COVID-19 crisis, and 3.5 (SD 3.3) for those who had begun a postcrisis transition at the time of survey administration (p < 0.001). 71.5% of providers did not perform an elective otologic or neurotologic operative procedure during the initial crisis period. 49.8% reported modifying their surgical technique due to the COVID-19 pandemic. Use of powered air-purifying respirators and filtering facepiece 2 or 3 (FFP2/FFP3) respirators were in minimal supply for 66.9% and 62.3% of respondents, respectively. CONCLUSION: The COVID-19 pandemic impacted the otology and neurotology community globally, resulting in significant changes in operative volume and case selection. Modification of surgical technique and shortages of personal protective equipment were frequently reported.

Endoscopic Stapedotomy: A Systematic Review.

OBJECTIVES: Stapes surgery has evolved from its origins in 1956. Microscopic assisted stapedotomy remains the most common technique but the introduction of endoscopic ear surgery has led to some units using this new approach. The endoscope delivers a wide angled, contextual view of the stapes, and associated pathology. This systematic review provides a critical analysis of the current published endoscopic data, allowing comparison to the established microscopic technique. DATA SOURCES: Six databases (PubMed, Medline, Cochrane database, AMED, EMBASE, and CINAHL) were searched for studies within the last 10 years. STUDY SELECTION AND DATA EXTRACTION: English language articles including 5 or more cases were included. Primary outcomes included audiological results and reported complications. Data was extracted according to PRISMA guidelines. RESULTS: Thirteen papers were identified comprising 361 endoscopic stapes surgeries. Postoperative temporary facial nerve weakness was reported in 3 patients (0.8%) which all resolved within 4 weeks. Chorda tympani injury occurred in 21 cases (5.8%) and vertigo in 61 (16.9%). The audiometric outcomes of endoscopic stapes surgery were available for 259 patients and showed air bone gap closure rates of 71.4% (0-10 dB), 25.9% (11-20 dB), 2.3% (21-30 dB), and 0.4% (>30 dB). CONCLUSIONS: Endoscopic stapes surgery has similar audiometric outcomes compared to the traditional microscopic approach with air bone gap closure values of <20 dB in 97.3% of cases. However, the complication rates of chorda tympani damage, postoperative dysguesia, and tympanic membrane perforation for endoscopic stapes surgery are high. Caution should therefore be taken before undertaking stapes surgery with the endoscopic technique. Further studies are required to prove superiority over well established existing microsurgical methods.

Endoscopic Management of Early Stage Middle Ear Paragangliomas - An Australian Case Series.

OBJECTIVE: To analyze the outcomes of the endoscopic transcanal approach for removal of early stage middle ear paraganglioma tumors (MEPT). STUDY DESIGN: Cases series with chart review. SETTING: Two tertiary Australian Otology centers. PATIENTS: Adult patients with middle ear paraganglioma tumors treated with transcanal endoscopic approach from 2/2016 to 12/2019. Tumor staging was described using the Modified Fisch-Mattox (MFM). Inclusion criteria included patients with an MFM Class A or B. Exclusion criteria included higher staged or syndromic disease. INTERVENTION: All tumors were managed with transcanal endoscopic approach. MAIN OUTCOME MEASURES: Primary outcome measures included disease clearance and hearing measured according to the AAO-HNS guidelines. Secondary outcomes included complications, duration of surgery, and length of stay. RESULTS: Ten patients underwent totally endoscopic transcanal resection of MEPT (9 female, mean age of 45.5 years, 70% were left sided). Mean tumor size was 6.1 mm (SD 3.4 mm). Five cases (50%) were classified using the MFM system as class A1, two cases were class A2, and three cases were class B1. Three cases required canalplasty for access but were completed entirely endoscopically. Nine of the 10 cases had complete audiometric data. Pre- and postoperative mean air conduction remained stable with a decrease in mean air-bone gap of 2.84 dB. Postoperative complications include one pinhole perforation. There were no facial nerve complications. Mean follow-up period was 10 months (range 4-25 mo) with all cases having resolution of pulsatile tinnitus and no tumor recurrence. CONCLUSION: The transcanal endoscopic approach for early stage MEPT offers excellent visualization and permits safe and effective removal of disease with the advantages of a minimally invasive technique for patient recovery.

Novel Radiologic Approaches for Cholesteatoma Detection: Implications for Endoscopic Ear Surgery.

Technological advancement in computed tomography (CT) and MRI has improved cholesteatoma detection rates considerably in the past decade. Accurately predicting disease location and extension is essential for staging, planning, and preoperative counseling, in particular in the newer approach of endoscopic ear surgery. Improved sensitivity and specificity of these radiological methods may allow the surgeon to confidently monitor patients, therefore avoiding unnecessary surgery. This article outlines recent advances in CT and MRI technology and advantages and disadvantages of the newer techniques. Emphasis on improving the feedback loop between the radiologist and surgeon will increase the accuracy of these new technologies.

Endoscopic Stapedectomy: Collective Experience From a Large Australian Center.

INTRODUCTION: Endoscopic stapes surgery is a technically demanding procedure that is increasing in popularity. Surgical outcomes and complication rates have been demonstrated to be comparable to traditional microscopic techniques. The surgical outcomes for patients undergoing stapes surgery performed by the Sydney Endoscopic Ear Surgery Research Group are presented. STUDY DESIGN: Retrospective review from prospectively gathered database. SETTING: Tertiary referral centers. PATIENTS: A retrospective case review of patients undergoing endoscopic stapes surgery performed by four surgeons between February 2015 and July 2019 was carried out. Sixty nine patients were identified, and assessed for demographics, functional results, and postoperative complications. INTERVENTION: Endoscopic stapedotomy. MAIN OUTCOME MEASURE: Postoperative hearing results. RESULTS: Sixty eight of 69 patients (98.6%) achieved an air-bone gap (ABG) closure of less than 20 dB. Sixty of 69 patients (87%) achieved and ABG closure of less than 10 dB. The average improvement of the ABG over four frequencies achieved was 26.5 dB (range, 0-61). Postoperative complications were infrequent and self-limiting. Six patients experienced dysgeusia without obvious chorda tympani injury, four patients developed short lived vertigo, and two patients experienced tinnitus. CONCLUSIONS: Endoscopically assisted stapes surgery represents a safe alternative to traditional microscopic techniques, with similar morbidity and audiological outcomes.

The Endoscopic Relationship of the Stapedius Muscle to the Facial Nerve: Implications for Retrotympanic Surgery.

OBJECTIVES: The stapedius muscle, tendon, and pyramidal eminence are structures within the retrotympanum. In cholesteatoma surgery, the retrotympanum is a common site of residual disease. The removal of the pyramidal eminence during surgery is sometimes necessary to obtain better visualization of the superior retrotympanum during surgery. Understanding the relational anatomy of structures in the region to the facial nerve allows the surgeon to safely access regional disease. This study aims to better understand the anatomical relationship between the mastoid portion of the facial nerve, the pyramidal eminence, and the stapedius muscle. A secondary aim is to demonstrate that removal of the stapedius muscle in the cadaver model, can increase exposure to the retrotympanic space. STUDY DESIGN: Anatomical cadaveric observation study. METHODS: Endoscopic dissection of cadaveric heads was undertaken. Classification of the superior and inferior retrotympanic area was performed. The anatomy of the stapedius muscle was described including relationships, depth, course, and angle with respect to the facial nerve. The pyramidal eminence and stapedius muscle were removed in all specimens and the exposure of the retrotympanum re-evaluated to determine if exposure of the region was increased. RESULTS: In all cases (11 ears), the stapedius muscle was located medial and anterior to the mastoid portion of the facial nerve, with the second genu superior. The mean antero-posterior distance from the apex of the pyramidal eminence, which the stapedius tendon enters, to the stapes itself was 4.10 mm (range, 2.92-5.73 mm; standard deviation [SD] 0.90 mm). In all cases, irrespective of sinus tympani conformation, removal of the pyramidal eminence and stapedial bony crest in proximity to the facial nerve allowed exposure of the whole retrotympanic region, using a 0-degree endoscope. CONCLUSIONS: The pyramidal eminence and stapedius muscle have a relatively constant relationship to the facial nerve. Removal of the stapedius muscle in the human cadaver model increases the exposure of the sinus tympani and subpyramidal space. Increased visualization in this region, may reduce risk of residual cholesteatoma in patients.

Radiological prevalence of superior and posterior semicircular canal dehiscence in children.

OBJECTIVE: Establishing the prevalence of semicircular canal dehiscence in a pediatric population using temporal bone CT imaging. STUDY DESIGN: Retrospective analysis of all temporal bone CT scans during a 5-year period (2007-2012). METHODS: CT scan images were reformatted in the plane of the canals and assessed by two independent reviewers with a third to resolve disagreement. Detailed chart review was performed for those found to have dehiscence. Superior and posterior canals were classified as "dehiscent", "possibly dehiscent", "thin" or "normal" for each case. RESULTS: 649 temporal bones were assessed from 334 children (under 18 years of age). The prevalence rate of superior canal dehiscence (SCD) was 1.7% (3.3% of individuals). Posterior canal dehiscence (PCD) was present in 1.2% (2.1% of individuals). There were no cases of bilateral SCD, and one case of bilateral PCD. Age under 3 years was associated with a higher prevalence of thinning but not dehiscence. Congenital inner ear malformation was not related to a higher probability of dehiscence. The superior petrosal sinus was associated with the SCD in three cases (27.3%). Retrospective chart review highlighted possible vestibular symptoms in 3/11 patients with SCD (27.3%). CONCLUSIONS: This forms the largest pediatric study of canal dehiscence to date. This study's prevalence rate is significantly lower than previous reports. The identified association with overlying venous structures may reflect the etiological process involved. The occurrence in children supports the hypothesis of a congenital predisposition for development of canal dehiscence syndrome.

Fibrous dysplasia of the temporal bone: systematic review of management and hearing outcomes.

OBJECTIVES: The management options for otologic symptoms (i.e., hearing loss, otorrhea) in patients with fibrous dysplasia of the temporal bone (FDTB) include either observation or otologic surgery. The objective of this review is to describe the hearing outcomes in patients with FDTB to compare surgical intervention and conservative management to determine an evidence-based approach to patient management. DATA SOURCES: Cochrane Central Register of Controlled Trials (1995-April 5, 2013), MEDLINE (January 1948-April 5, 2013), EMBASE (January 1974-April 5, 2013), conference proceedings, and the reference lists of articles. STUDY SELECTION: Studies describing interventions or no intervention in patients with FDTB were included. Studies failing to report hearing outcomes were excluded. DATA EXTRACTION: Two authors independently reviewed titles and abstracts, read full-text articles, assessed quality, and extracted data. The Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) guidelines were followed. DATA SYNTHESIS: Seventy-two studies involving 215 patients were included. Thirty-one studies (n = 49 patients), including three case series and 28 case reports, had sufficient information for analysis of hearing outcomes (Level 4 evidence). The surgical intervention showing the best hearing outcomes was canaloplasty, with improvement in 84% of patients (n = 16 patients) and stable hearing thresholds in 18% (n = 3 patients). CONCLUSION: The literature lacks consistent reporting of hearing outcomes after otologic surgery in FDTB. Level 4 evidence suggests that an improvement in hearing thresholds is achievable in most patients when the disease involves the external ear canal. A management algorithm is proposed using the available evidence.

Surgical management of benign idiopathic osteonecrosis of the external auditory canal.

BACKGROUND/AIMS: Benign idiopathic osteonecrosis of the external ear canal remains a challenging management problem, with many patients experiencing a prolonged chronic course requiring frequent debridement. The technique of bony sequestrectomy, followed by rotation of a vascularized fascial flap and then a free full-thickness skin graft is presented. METHODS: Success with this technique is described in a retrospective case series format in addition to a review of the existing literature on surgical techniques for the treatment of this condition. RESULTS: The described technique was performed on 5 patients following prolonged periods of failed medical therapy (minimum 8 months). All 5 patients achieved a healed external auditory canal, with no ongoing skin ulceration or bony sequestration. Follow-up data (median 22 months) suggests that the technique provides a robust and long-lasting therapy. CONCLUSION: Symptomatic benign idiopathic osteonecrosis of the tympanic bone is well treated by provision of vascularized tissue to maximize the chance of bone healing and subsequent cure. It should be considered early in the management protocol to avoid lengthy symptomatic periods, the symptoms of which ultimately may not heal with medical therapy alone.

The rhinological manifestations of women's health.

OBJECTIVE: To systematically review the literature and appraise the evidence reporting the effects of women's health, including pregnancy, postpartum, menstruation, oral contraception, menopause, and hormone replacement therapy, on common rhinological pathologies and nasal physiology. DATA SOURCES: Systematic search strategy using MEDLINE (1966-2012) and EMBASE (1980-2012) databases. REVIEW METHODS: Title review, abstract screening, and then full paper analysis were undertaken by 2 authors independently. Level of evidence was graded according to the Oxford Centre of Evidence Based Medicine 2011 criteria and risk of bias assessment using the Jadad scale for randomized controlled trials and Newcastle-Ottawa Scale for cohort and case-controlled studies. RESULTS: Over the 46 years analyzed, the search strategy produced 2904 titles. In total, 314 abstracts were screened, from which 192 full-text articles were evaluated, and 145 research papers met all the criteria for inclusion in the study. Overall, the available evidence was of low quality. Seventy percent of studies (102 of 145) were case reports or case series from which only limited conclusions can be drawn. Only 3% of the included papers (4 of 145) were randomized controlled studies. The remaining data were mainly of a prospective cohort design. Study heterogeneity in design and measured outcomes resulted in data synthesis being limited to a descriptive/exploratory review. Study findings are presented by women's health category and then by rhinological manifestation with important clinical correlations highlighted. CONCLUSION: Physiological and hormonal changes occurring as a normal part of women's health have an important influence on rhinological function and disease.