Aspergillus infections of lateral skull base: a case series.

PURPOSE: While extensive research with accurate classification has been done in mycoses of the paranasal sinuses and anterior skull base, a similar understanding of lateral skull base fungal pathologies is lacking due to relative rarity and diagnostic difficulties. We introduce a series of eleven cases and two different invasive entities of Aspergillus temporal bone diseases-fungal skull base osteomyelitis (SBO)/malignant otitis externa (MOE) and chronic invasive granulomatous fungal disease (CIGFD). METHODOLOGY: A retrospective observational study was conducted at the neuro-otology unit of a tertiary care referral center between July 2017 and November 2022. Diagnosed cases of lateral skull base osteomyelitis with atypical symptoms and lack of response to culture-directed antibiotics were evaluated for fungal origin. Patient data, including history, laboratory findings, serum galactomannan assay, CT and MRI imaging findings, clinical examination findings, and co-morbidities, were analyzed. The treatment course and response were assessed. RESULTS: A total of 11 cases were included in the study. Of these, 9 were cases of Aspergillus-induced skull base osteomyelitis (SBO) and 2 of Aspergillus-induced chronic invasive granulomatous fungal disease (CIGFD). CIGFD presented with persistent ear discharge and slowly progressive post-aural swelling, while all patients of fungal SBO had lower cranial nerve palsies. CIGFD responded to excision and antifungals, while SBO responded well to conservative anti-fungal treatment. CONCLUSION: In cases of lateral SBO not responding to antibiotic therapy, the possibility of fungal etiology should be considered. Aspergillus spp. seems to be the major fungal pathogen.

Neoplasms of the Ear Canal.

Primary EAC neoplasms include benign and malignant lesions of bony, glandular or cutaneous origin. Small, benign slow growing bony neoplasms are often asymptomatic, diagnosed incidentally and might not require intervention. Both malignant and benign neoplasms of cutaneous and glandular origin can present with symptoms of chronic otitis externa, leading to delays in diagnosis. Prompt biopsy of soft tissue lesions associated with non-resolving otitis externa are warranted. Local and regional imaging is helpful to understand disease extent and origin, but even early-stage malignant neoplasms require aggressive surgical treatment.

A Case of Adult Clival Osteomyelitis.

Clival osteomyelitis is a potentially life-threatening skull base infection. It is rare and generally challenging to diagnose and treat. Clival osteomyelitis is typically seen in the pediatric population and is very rare in the adult population. It occurs as a complication of recurring paranasal infections and malignant otitis externa. The exact pathophysiology of osteomyelitis of the clivus is relatively uncertain. Here, we describe a case of a 36-year-old man with medical history significant for hypertension and poorly controlled type 1 diabetes mellitus who experienced recurrent paranasal sinus infection for 2 years. He received multiple antibiotic treatments and underwent adenoidectomy without substantial improvement of symptoms. Ultimately, a diagnosis of the clival osteomyelitis through the help of a computed tomography (CT) scan of the paranasal sinus and neck was made. This diagnosis allowed for adequate intervention and treatment of our patient with subsequent resolution of his presenting symptoms. This case highlights the importance of high suspicion for clival osteomyelitis in patients with recurring sinus infections.

Acute otitis externa: Consensus definition, diagnostic criteria and core outcome set development.

OBJECTIVE: Evidence for the management of acute otitis externa (AOE) is limited, with unclear diagnostic criteria and variably reported outcome measures that may not reflect key stakeholder priorities. We aimed to develop 1) a definition, 2) diagnostic criteria and 3) a core outcome set (COS) for AOE. STUDY DESIGN: COS development according to Core Outcome Measures in Effectiveness Trials (COMET) methodology and parallel consensus selection of diagnostic criteria/definition. SETTING: Stakeholders from the United Kingdom. SUBJECTS AND METHODS: Comprehensive literature review identified candidate items for the COS, definition and diagnostic criteria. Nine individuals with past AOE generated further patient-centred candidate items. Candidate items were rated for importance by patient and professional (ENT doctors, general practitioners, microbiologists, nurses, audiologists) stakeholders in a three-round online Delphi exercise. Consensus items were grouped to form the COS, diagnostic criteria, and definition. RESULTS: Candidate COS items from patients (n = 28) and literature (n = 25) were deduplicated and amalgamated to a final candidate list (n = 46). Patients emphasised quality-of-life and the impact on daily activities/work. Via the Delphi process, stakeholders agreed on 31 candidate items. The final COS covered six outcomes: pain; disease severity; impact on quality-of-life and daily activities; patient satisfaction; treatment-related outcome; and microbiology. 14 candidate diagnostic criteria were identified, 8 reaching inclusion consensus. The final definition for AOE was 'diffuse inflammation of the ear canal skin of less than 6 weeks duration'. CONCLUSION: The development and adoption of a consensus definition, diagnostic criteria and a COS will help to standardise future research in AOE, facilitating meta-analysis. Consulting former patients throughout development highlighted deficiencies in the outcomes adopted previously, in particular concerning the impact of AOE on daily life.

A Case Series of Malignant Otitis Externa Mimicking Malignancy.

Malignant otitis externa is an inflammation of the external auditory canal with preceding osteomyelitis of the temporal bone and the adjacent structures that could be potentially lethal. Malignant otitis externa may present with cranial nerve involvements and massive spread of disease mimicking nasopharyngeal carcinoma or any other malignancies on imaging. Two elderly patients who presented with severe otalgia and significant facial nerve palsy and lower cranial nerve palsies showing extensive spread of disease are reported in this case series. They both had resolution of disease after a prolonged course of antibiotics and cortical mastoidectomy for disease clearance in one of them.

Prognosticating patients with necrotising otitis externa based on response to treatment.

INTRODUCTION: Necrotising otitis externa (NOE) is a severe infection of the temporal bone. The traditional severity based staging system does not fully prognosticate all patients with NOE. We hypothesise that a patient response staging system would more accurately capture the disease process and guide prognosis. METHODS: We carried out a retrospective notes review of patients diagnosed with NOE from January 2017 to December 2018 in a regional tertiary referral centre. Patient outcomes from our proposed patient response staging system were compared to a modified previously published severity based Gleeson staging system with patients requiring prolonged treatment classified as having a poor outcome. RESULTS: A total of 34 patients were treated for NOE. The majority were male (n=24) and had diabetes (n=25). Patients with the most severe Gleeson staging did not have the worst outcome. Daily delay in resolution of otorrhoea was associated with an increased need for more than six weeks of treatment. Rapid responders are patients who had resolution of otalgia, otorrhoea and C-reactive protein normalisation within 14 days, and all were cured following standard 6 weeks of treatment. CONCLUSIONS: The Gleeson staging system was valuable in assessing the extent of disease and all early Gleeson staged patients had good outcomes. However, patients with higher severity staging on the Gleeson system did not necessarily require prolonged treatment. There is a role for a joint approach in staging patients based on both modified Gleeson and treatment response, which would subsequently guide prognosis, duration of treatment and early diagnosis of potential fungal NOE.

A retrospective review and multi-specialty, evidence-based guideline for the management of necrotising otitis externa.

BACKGROUND: Necrotising otitis externa is a progressive infection of the external auditory canal which extends to affect the temporal bone and adjacent structures. Progression of the disease process can result in serious sequelae, including cranial nerve palsies and death. There is currently no formal published treatment guideline. OBJECTIVE: This study aimed to integrate current evidence and data from our own retrospective case series in order to develop a guideline to optimise necrotising otitis externa patient management. METHODS: A retrospective review of necrotising otitis externa cases within NHS Lothian, Scotland, between 2013 and 2018, was performed, along with a PubMed review. RESULTS: Prevalent presenting signs, symptoms and patient demographic data were established. Furthermore, features of cases associated with adverse outcomes were defined. A key feature of the guideline is defining at-risk patients with initial intensive treatment. Investigations and outcomes are assessed and treatment adjusted appropriately. CONCLUSION: This multi-departmental approach has facilitated the development of a succinct, systematic guideline for the management of necrotising otitis externa. Initial patient outcomes appear promising.

Aspergillus necrotizing otitis externa with temporomandibulozygomatic involvement.

INTRODUCTION: Aspergillus necrotizing otitis externa (NOE) is a rare disease, often associated with delayed diagnosis, the management of which is poorly defined. SUMMARY: The authors report a case of Aspergillus flavus necrotizing otitis externa with temporomandibular arthritis and temporozygomatic osteomyelitis with Staphylococcal coinfection in a diabetic patient. The diagnosis and discontinuation of treatment were guided by PET-CT scan. A favourable course without sequelae was observed after repeated surgical curettage and 3 months of antifungal therapy. DISCUSSION: Aspergillus flavus is the agent most commonly incriminated in NOE. Indirect diagnostic tests (serology) may be negative. The diagnosis is based on imaging-guided surgical biopsy with histological examination and standard and fungal microbiological culture. Treatment requires a combination of surgery and antifungal therapy. The duration of antifungal therapy is poorly defined and discontinuation of therapy can be guided by PET-CT scan.

Thirty-Day Readmission and Prolonged Length of Stay in Malignant Otitis Externa.

OBJECTIVES: To determine independent risk factors for 30-day readmission, prolonged length of stay (PLOS), and discharge to a rehabilitation facility for those with malignant otitis externa. METHODS: Retrospective cohort study of patients hospitalized with malignant otitis externa (International Classification of Diseases, 9th edition, code 380.14) in the Nationwide Readmissions Database (2013-2014). Overall and disease-specific complication and mortality data were analyzed using chi-squared and multivariate analysis. RESULTS: There were 1267 cases of malignant otitis externa extracted. A PLOS of ≥8 days (90th percentile) was found in 14.2% (n = 180) of patients, and 13.7% (n = 174) were discharged to a facility. Patients were readmitted within 30 days at a rate of 12.5% (n = 159). The overall rates of uncomplicated and complicated diabetes were found to be 42.1% and 17.8%, respectively. Factors independently associated with PLOS included undergoing a surgical procedure (odds ratio [OR] 2.08, P < .001), and having central nervous system complications (OR 3.21, P < .001). Independent risk factors for disposition to a facility included nutritional deficiency (OR 1.91, P = .029), PLOS (OR 4.61, P < .001), and age 65-79 years (OR 6.57, P = .001). Readmission was independently linked to PLOS (OR 3.14, P < .001). Diabetes was not an independent risk factor for any outcome. CONCLUSIONS: Thirty-day readmission, PLOS, and ultimate discharge to a rehabilitation facility were common and closely intertwined. Despite the classic association between diabetes and malignant otitis externa, diabetes was not an independent risk factor for any of our outcomes. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:2220-2228, 2020.

A Persistent Foramen of Huschke: A Small Road to Misery in Necrotizing External Otitis.

BACKGROUND AND PURPOSE: Necrotizing external otitis is a serious complication of external otitis with different spreading patterns. A persistent foramen of Huschke is a dehiscence located antero-inferior in the osseous external ear canal and posterior-medial to the temporomandibular joint. This dehiscence can facilitate extension of infection in an anterior pattern next to classic spread along the fissures of Santorini. The aim of this study was to define the prevalence and size of a persistent foramen of Huschke in patients with necrotizing external otitis. MATERIALS AND METHODS: We retrospectively examined 78 CT temporal bone studies (39 patients with necrotizing external otitis, 39 control subjects). The side and presence of the foramen were noted, and its prevalence was calculated. The maximal width of the foramen of Huschke was measured in the axial plane and classified as subtle, mild, moderate, or extensive. RESULTS: A persistent foramen of Huschke was present in 21 patients (26 ears) and 7 control subjects (9 ears). Prevalence was 50% (20/40) and 11.5% (9/78) in affected ears of patients with necrotizing external otitis and control subjects, respectively. Almost all affected ears showed an anterior distribution pattern of necrotizing external otitis. The extensive dehiscence was most common in affected ears. CONCLUSIONS: An anterior necrotizing external otitis spreading pattern is associated with the presence and increased size of a persistent foramen of Huschke. These findings facilitate the theory that a persistent foramen of Huschke is an additional risk factor the development of necrotizing external otitis.

Malignant (necrotizing) externa otitis: the experience of a single hyperbaric centre.

INTRODUCTION: Malignant otitis externa (MOE) is a potentially life-threatening infection of the soft tissues of the external ear, quickly spreading to involve the periosteum and bone of the skull base. Treatment includes antibiotics and eventually surgery. Hyperbaric oxygen treatment (HBOT) has been proposed as an adjunctive therapy. However, in the tenth consensus conference, this disease was considered as a non-indication for HBOT. The aim of this study was to evaluate the effectiveness of HBOT in MOE treatment. METHODS: Retrospective and observational study was conducted of patients with MOE treated in our centre. Staging of the disease was made according to the clinicopathological classification system. RESULTS: From March 1998 to November 2016, 16 patients were referred. 6% patients were on stage 1 of the disease at the time they were referred, 20% in stage 2, 7% in stage 3a, 13% in stage 3b and 53% in stage 4. Seven (43.75%) patients had VII nerve palsy and three (18.75%) patients had multiple nerve palsy. Average length of symptoms of disease was 5 months (maximum 11 months). Average number of sessions was 33 and the length of hospitalization prior to HBOT (median 90 days) was significantly longer than the time between beginning HBOT and cure (p = 0.028, Wilcoxon signed rank test). There were no fatalities due to MOE and all patients were considered free of disease after HBOT. CONCLUSION: HBOT was well tolerated and revealed to be a helpful adjuvant treatment in MOE. According to our data, HBOT should be considered for patients who failed conventional treatments and in severe cases.

Effective Inexpensive Management of Necrotizing Otitis Externa Is Possible in Resource-Poor Settings.

INTRODUCTION: Necrotizing otitis externa resolves best with antimicrobial treatment. How to care for these patients and monitor their resolution remains a problem. Our protocol in Bangalore can manage these patients inexpensively and well. MATERIALS AND METHODS: Patients who were referred to our patients became the subjects for this paper. They were managed through our protocol, which consists of IV ciprofloxacin and meropenem, weekly labs, weekly examinations, and photodocumention. RESULTS: Fifty-one people presented with necrotizing otitis externa (NOE) between October 2015 and November 2017 and completed our entire protocol. Forty-six had complete resolution of their disease, while 5 had to undergo surgical removal of necrotic bone. Six of 8 patients with facial weakness had improvement in their House-Brackmann scores. Reduction of self-reported nocturnal pain, dissolution of ear canal granulations, and normalization of the erythrocyte sedimentation rate (ESR) proved to be the most accurate indicators of disease regression. CONCLUSION: Patients are monitored closely with review of their otalgia, examination of their canal, repeated ESRs, effective control of their diabetes, and radiological imaging. All this can be done in a resource-poor country, which in turn serves as a model for the wealthier nations.

Fungal Malignant Otitis Externa Involves a Cascade of Complications Culminating in Pseudoaneurysm of Internal Maxillary Artery: A Case Report.

BACKGROUND Pseudomonal infection is the most common cause of malignant otitis externa (MOE), which typically affects elderly diabetic patients. Fungi are a rare cause of MOE. MOE can be life-threatening if not recognized and treated promptly. It can result in a wide spectrum of complications, including skull-base osteomyelitis, cranial nerve palsy, cerebral venous thrombosis, and brain abscess. Pseudoaneurysm formation of the intracranial vessels is a life-threatening complication of MOE that is seldom reported in the literature. CASE REPORT We report the case of a 66-year-old diabetic man with MOE who was initially treated with antipseudomonal antibiotics after negative initial culture results. His MOE resulted in a cascade of complications, including facial nerve palsy, skull base osteomyelitis, and sigmoid sinus thrombosis, and culminated in left maxillary artery pseudoaneurysm formation resulting in massive epistaxis and hemodynamic instability. Endovascular embolization resulted in a successful obliteration of the pseudoaneurysm. A subsequent functional endoscopic sinus surgical (FESS) tissue biopsy confirmed Candida glabrata as the etiological agent. The patient was successfully treated with antibiotics and antifungal and anticoagulation therapy, and was discharged home in good condition. CONCLUSIONS A high index of suspicion for the diagnosis of fungal MOE, particularly in intractable cases of MOE with negative initial cultures, should be maintained. Pseudoaneurysm formation is a life-threatening complication of MOE that is seldom reported in the literature and should be suspected in any patient with MOE who presents with epistaxis or intracranial bleeding.

Cotton bud in external ear canal causing necrotising otitis externa and subdural abscess.

Necrotising otitis externa (NOE) is an infection originating in the soft tissues of the external auditory canal (EAC) spreading to the surrounding bone and rarely causing intracranial complications. It is usually caused by Pseudomonas aeruginosa and has historically occurred in elderly patients with diabetes or immunodeficiency. EAC foreign body is a risk factor for otitis externa but has not been described in NOE. A healthy 31-year-old man presented with new-onset seizures and worsening left-sided otalgia and otorrhoea. Brain imaging revealed left temporal subdural abscesses superior to the petrous bone. A retained cotton bud was identified in the left EAC, along with osseocartilaginous junction and mastoid granulation tissue. The foreign body was removed; a cortical mastoidectomy performed and intravenous antibiotic administered. At 10 weeks, the patient remained well, with no neurological deficit and no residual ear symptoms, and CT demonstrated complete resolution of the intracranial abscesses.

Necrotizing Otitis Externa-Analysis of 83 Cases: Clinical Findings and Course of Disease.

OBJECTIVES: Evaluate the relationship between clinical findings and course of disease among patients with necrotizing otitis externa (NOE). STUDY DESIGN: Retrospective case series review. SETTING: Tertiary referral center. PATIENTS: Adult patients with no previous history of chronic ear disease, hospitalized due to NOE between the years 1990 to 2015. MAIN OUTCOME MEASURES: 1) Duration of hospitalization and 2) necessity for surgery. RESULTS: Eighty-one patients were included in the study, corresponding to 83 effected ears. Thirty-two patients (38.5%) were hospitalized longer than 20 days and 20 patients (24.0%) underwent surgery. Otalgia was the most common complaint (n = 71, 85.5%). Pseudomonas Aeruginosa (PA) was the most common isolated bacteria (n = 40, 48.1%). Shifting incidence of culture results was noted, as rates of PA NOE decreased and rates of sterile culture and fungal NOE increased. Duration of complaints and presence of aural discharge at admission were associated with prolonged hospitalization (p = 0.010, p = 0.011, respectively). Advanced age, duration of hospitalization, and rates of readmission were associated with surgery (p = 0.037, p < 0.001, p < 0.001 respectively). CONCLUSIONS: Duration of complaints and presence of aural discharge may indicate advanced NOE and require longer in-hospital treatment. Elderly patients are at increased risk for conservative treatment failure and are more likely to require surgery. With shifting incidence of pathogens, a wider empirical treatment covering nontraditional pathogens should be considered.

FDG-PET/CT for diagnosis and follow-up of necrotizing (malignant) external otitis.

OBJECTIVE: Imaging is important for the diagnosis and follow-up of necrotizing external otitis (NEO). The best imaging modality is controversy. To suggest 2-deoxy-2-[fluorine-18] fluoro-D-glucose-positron emission tomography/computed tomography (18F-FDG-PET/CT) as an alternative to technetium-99m and gallium-67 scans for diagnosis and assessment of response to treatment for patients with NEO. STUDY DESIGN: Case series. METHODS: Tertiary referral center. From 2013 through 2017, 12 patients were diagnosed with NEO based on clinical features and positive FDG uptake within the temporal bone on PET/CT. Mean age was 74 ± 11.5; 83% of the patients were male; and 83% had diabetes. RESULTS: PET/CT scans were reviewed independently by two nuclear medicine specialists. Imaging located osteomyelitis in external ear canal, mastoid, temporomandibular joint, and nasopharyngeal region (100%, 50%, 16%, 8%, respectively). Mean follow-up was 16 months. Eight patients (67%) underwent a second PET/CT scan after active otitis resolved and after at least 6 weeks of antibiotic treatment. The scan demonstrated no or substantially reduced FDG uptake and treatment was stopped. The patients had no NEO symptoms at the end of follow-up. One patient had significant uptake, and antibiotic treatment was continued until a third scan demonstrated no FDG uptake. Two patients died before the second PET/CT, and two were lost to follow-up. CONCLUSION: 18F-FDG-PET/CT is a reliable imaging modality for diagnosis, disease localization, and decision making regarding treatment cessation. 18F-FDG-PET/CT should be considered as the imaging modality of choice for initial diagnosis and follow-up in NEO patients. Larger, controlled studies are warranted. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:961-966, 2019.