A rare cause of malignant otitis externa and skull base osteomyelitis.

An elderly male with type 2 diabetes presented with a 2-month history of otalgia and severe headaches. He was diagnosed with malignant otitis externa (MOE) and was commenced on empirical treatment with oral ciprofloxacin. Pseudomonas is the most common cause of MOE. A baseline CT scan was undertaken that demonstrated skull base osteomyelitis (SBO) due to findings of bone erosion at the mastoid tip and an infiltrating soft tissue mass eroding the clivus. Eight weeks later, he returned with worsening and bilateral symptoms of otitis externa, hearing loss, temporomandibular pain and dysfunction. Worsening and now bilateral malignant otitis externa were confirmed with an MRI scan that also demonstrated a small fluid collection in his left temporal region. The collection was aspirated and grew scedosporium apiospermum. He was diagnosed with fungal SBO and was commenced on treatment with the antifungal voriconazole, with significant improvement in symptoms and radiological findings. Fungal osteomyelitis is more likely in immunosuppressed patients, particularly those with type 2 diabetes. Fungal aetiology should be suspected in patients with progressive symptoms, despite treatment. A microbiology diagnosis of fungal SBO or MOE can be challenging to obtain and can lead to diagnostic delay. A sampling of the external auditory canal can aid in diagnosing MOE; however, scedosporium may also be isolated as a commensal organism. Aspirations from accessible fluid collections, infratemporal fossa needle sample and bone biopsy can provide material for diagnosis. Scedosporium is a rare cause of disease in humans, however, fungal infections are increasing in humans, due to an increase in susceptible populations. Scedosporium apiospermum is a rare cause of SBO and should be considered in patients not responding to standard treatment.

Granulomatous Inflammation of the Skull Base: A Rare Case of Mycobacterium tuberculosis in the United States.

The differential for an ill-defined, bone-destructive, granulomatous lesion of the skull base includes malignancy, as well as autoimmune and infectious processes. Suspicion for tuberculosis of the skull base in high-risk patients is particularly necessary given the difficulty to culture on standard cultures, need for specific and prolonged antibiotic therapy, and dire morbidity if not diagnosed and treated in a timely manner. Repeat biopsies and cultures were necessary to diagnose this case of Mycobacterium tuberculosis of the skull base after initial biopsy was non-diagnostic. Laryngoscope, 134:4023-4027, 2024.

Polymicrobial Skull Base Osteomyelitis Related to Chronic Sphenoiditis and Endoscopic Sinus Surgery.

Skull base osteomyelitis (SBO) secondary to endoscopic surgery for chronic sinusitis and post-operative sphenopalatine artery electrocautery has not been previously reported. This case report details an instance of Central SBO with an unusual microbiology profile and highlights the need to readily consider SBO should patients present with persistent headache and cranial nerve palsies following sinus surgery. Laryngoscope, 131:E1086-E1087, 2021.

Skull Base Fungal Osteomyelitis: A Case Report and Review of the Literature.

Skull base osteomyelitis (SBO) is an invasive infection refractory to therapy, closely linked with malignant otitis externa (MOE). It is characterized by a mild clinical presentation that can delay cross-sectional imaging considered as the key to revealing it. Skull base osteomyelitis typically affects elderly diabetics and immunocompromised patients (>70 years). It most commonly has an otogenic origin due to an extension of MOE. The prognosis can be very poor without the administration of adequate and timely therapy at an early disease stage. Nowadays, Pseudomonas aeruginosa remains the most common pathogen associated with SBO. Fungi are a rare cause of MOE. This report documents a rare case of otogenic SBO caused by Candida parapsilosis in a diabetic patient, with persistent otologic symptoms as clinical onset and resistance to medical treatment. Fungal MOE has more subtle symptoms and is more aggressive than its bacterial counterpart. When MOE is resistant to antibacterial drugs, this should raise the suspicion of a fungal etiology of MOE. The current guidelines do not exhaustively describe the diagnosis, antifungal drugs of choice, and optimum duration of treatment. The description of these rare clinical cases should help with the multidisciplinary management of this disease in order to optimize the diagnosis and therapeutic protocol.

Non-invasive fungal sinusitis resulting in multiple cranial nerve neuropathies.

A 33-year-old man presented to the emergency department with a right-sided facial paralysis and maxillary division (V2, trigeminal nerve) paraesthesia. He had been suffering with upper respiratory tract symptoms in the preceding 2 months, including rhinorrhoea, fever and headache. The patient was otherwise fit and immunocompetent. Urgent radiological investigation revealed extensive fungal sinusitis with sphenoid sinus dehiscence and skull base osteitis. The patient underwent emergency endoscopic sinus surgery revealing concretions and debris in the ethmoid and sphenoid sinuses. He was commenced on systemic antifungal therapy and made a full recovery with resolution of his cranial neuropathies. The fungus Schizophyllum commune was isolated and is a rare cause of fungal sinusitis, but with the potential for invasive disease in immunosuppressed individuals.

Extensive Skull Base Osteomyelitis Secondary to Malignant Otitis Externa.

Skull base osteomyelitis is a severe complication of malignant otitis externa that affects the marrow of the temporal, sphenoid, and occipital bones. Skull base osteomyelitis is usually diagnosed based on clinical, microbiological, and radiological findings. Here, we present the imaging findings of a 76-year-old man who initially presented with right-sided malignant otitis externa, with the involvement of the otomastoid structures and ipsilateral temporal bone. Over the following 3 years, despite specific extended antibiotic therapy, the skull base osteomyelitis entirely involved the skull base, up to the contralateral petrous portion of the temporal bone, and it affected the cervical vertebral processes. This report describes an exceptional extent of unilateral malignant otitis externa with a severe involvement of the skull base on the contralateral side and the cervical spine.

Atypical Form of Cervicofacial Actinomycosis Involving the Skull Base and Temporal Bone.

BACKGROUND:: Cervicofacial actinomycosis is an uncommon indolent infection caused by Actinomyces spp that typically affects individuals with innate or adaptive immunodeficiencies. Soft tissues of the face and neck are most commonly involved. Actinomyces osteomyelitis is uncommon; involvement of the skull base and temporal bone is exceedingly rare. The authors present a unique case of refractory cervicofacial actinomycosis with development of skull base and temporal bone osteomyelitis in an otherwise healthy individual. METHODS:: Case report with literature review. RESULTS:: A 69-year-old man presented with a soft tissue infection, culture positive for Actinomyces, over the right maxilla. Previous unsuccessful treatment included local debridement and 6 weeks of intravenous ceftriaxone. He was subsequently treated with conservative debridement and a prolonged course of intravenous followed by oral antibiotic. However, he eventually required multiple procedures, including maxillectomy, pterygopalatine fossa debridement, and a radical mastoidectomy to clear his disease. Postoperatively he was gradually transitioned off intravenous antibiotics. CONCLUSIONS:: Cervicofacial actinomycosis involves soft tissue surrounding the facial skeleton and oral cavity and is typically associated with a history of mucosal trauma, surgery, or immunodeficiency. The patient was appropriately treated but experienced disease progression and escalation of therapy. Although actinomycosis is typically not an aggressive bacterial infection, this case illustrates the need for prompt recognition of persistent disease and earlier surgical intervention in cases of recalcitrant cervicofacial actinomycosis. Chronic actinomycosis has the potential for significant morbidity.

Skull base aspergillosis in an immunocompetent elderly man with early response to steroid.

We report the case of a previously well 80-year-old man who presented with subacute bilateral painful optic neuropathy with initial response to corticosteroids but ultimately progressed to a fatal skull base syndrome. Initial presentation of steroid-responsive painful bilateral posterior optic neuropathy, preliminary normal enhanced MRI, normal cerebrospinal fluid and inflammatory markers indicated atypical optic neuritis. However, this progressed to a bilateral orbital apex syndrome with ophthalmoplegia and evidence of abnormal skull base enhancement on subsequent MRI. Biopsy of radiologically abnormal dura was non-diagnostic and negative for fungal stains. He deteriorated and died 8 months after initial presentation. At postmortem, fungal skull base infection was diagnosed. This case demonstrates that chronic skull base fungal infection can: (1) present in elderly immunocompetent patients, (2) show initial improvement with corticosteroids and (3) evade diagnosis on biopsy. We encourage a high index of suspicion for fungal skull base infection in similar cases.

Severe destructive nasopharyngeal granulomatosis with polyangiitis with superimposed skull base Pseudomonas aeruginosa osteomyelitis.

Skull base osteomyelitis in the setting of granulomatosis with polyangiitis (GPA) is rare and entails significant diagnostic challenges. We present a case of a 65-year-old Caucasian man with a history of rheumatoid arthritis, off immunosuppression for 18 months, who presented with 2 years of chronic headaches, severe fatigue, saddle nose deformity and 20-kilogram unintentional weight loss. Maxillofacial CT revealed an extensive destructive sinonasal and erosive skull base process. Laboratory evaluation showed equivocal elevation of antiproteinase 3 antibodies with negative antineutrophil cytoplasmic antibody panel. Biopsy of the skull base/clivus revealed necrotising granulomatous inflammation with focal vasculitis consistent with GPA, and multiple bone cultures were positive for Pseudomonas aeruginosa This patient was diagnosed concurrently with GPA and P. aeruginosa skull base osteomyelitis. He was started on a 6-week course of cefepime intravenously and oral prednisone, with the plan to initiate rituximab infusion 2 weeks after initiation of antibiotic therapy.

A Treatment Protocol for Management of Bacterial and Fungal Malignant External Otitis: A Large Cohort in Tehran, Iran.

AIMS: High rates of negative microbiologic test results highlight the potential role of empiric antimicrobial agents in management of malignant otitis externa (MOE). This study investigates the clinical presentation, laboratory findings, and response to empiric treatment in a large group of patients admitted to a tertiary academic hospital in Tehran, Iran. METHODS AND MATERIALS: We recruited 224 patients diagnosed with MOE in a prospective observation from 2009 through 2015. All patients received a 2-agent antibacterial regimen at baseline (phase I). Patients with no improvement within 10 days and/or nonresponders to a second course of antibacterials were switched to antifungals (phase II). Response to treatment was observed and documented in both groups. RESULTS: All patients had physical symptoms for more than 12 weeks before admission. In total, 127 patients responded well to antibacterials. Eighty-seven out of 97 patients who were switched to antifungals had complete response to treatment; patients in the latter group had significantly higher A1C levels at baseline. CONCLUSION: Our findings provide evidence to develop clinical guidelines that accelerate diagnosis and treatment of MOE to improve patient outcomes.

Uncontrolled spread following radiosurgery for a skull base aspergilloma misdiagnosed as schwannoma: is radiosurgery responsible?

BACKGROUND: Little is known about the natural history of skull base fungal lesions in immunocompetent individuals and the effect of high-dose radiation on fungal lesions. METHOD AND RESULTS: We report a case where radiosurgery was given to a skull base aspergilloma, mistaking it to be a trigeminal schwannoma. There was dramatic spread of the lesion to periventricular region with significant increase in the skull base lesion. The patient never received steroids. A stereotactic biopsy established the diagnosis, and treatment with voriconazole helped. CONCLUSIONS: It is possible that radiosurgery decreases local immunity due to vascular sclerosis and aids in spread of the fungal lesion, though it is only speculative. This again highlights the importance of establishing the diagnosis prior to radiosurgery.

[CHRONIC OSTHEOMYELITIS OF THE FACIAL MIDDLE ZONE WITH SPREADING ON THE BONES OF CRANIAL BASE ON BACKGROUND OF NARCOTIC DEPENDENCE].

Own experience of treatment of a narcotic dependence patients, suffering osteomyelitis of jaws, was analyzed. In those patients, who have had exposed themselves towards toxic impact of narcotic substances (even after arrest of the consumption) for a long period of time, the inflammation spreading on the bones of facial middle zone and the skull base with occurrence of a life3threatening purulent3septic, including intracerebral, complications (cerebral аbscess, meningitis, sepsis) were observed. Additional division of cerebral abscess on open and closed forms for optimization of diagnosis of іntracranial complications was proposed.

Central skull base osteomyelitis: a rare but life-threatening disease.

We present the case of a 70-year-old non-diabetic patient who presented to the emergency department with unrelenting otalgia. A severe otitis externa (OE) and mastoiditis were treated with broad spectrum antibiotics and surgical drainage. No bacteria was isolated from surgical samples. Because the otalgia persisted, a magnetic resonance (MR) was performed and showed an infiltrating process at the skull base. Biopsies failed to prove malignancy or granulomatosis. The patient's neurological state deteriorated. The suspicion of a skull base osteomyelitis (SBO) was raised and proven by CT-guided biopsies that grew Pseudomonas aeruginosa. Meropenem and ciprofloxacin, given for 8 weeks, lead to a fast clinical improvement and a full recovery. SBO is uncommon, often complicating severe OE. Pseudomonas aeruginosa is the main pathogen. Prompt diagnosis and adequate antibiotherapy are required to lower mortality and morbidity. The diagnosis may be delayed because of unawareness and large differential diagnosis including solid neoplasic tumours, malignant hemopathies and granulomatosis.

Th17 cytokine deficiency in patients with Aspergillus skull base osteomyelitis.

BACKGROUND: Fungal skull base osteomyelitis (SBO) is a severe complication of otitis externa or sinonasal infection, and is mainly caused by Aspergillus species. Here we investigate innate and adaptive immune responses in patients with Aspergillus SBO to identify defects in the immune response that could explain the susceptibility to this devastating disease. METHODS: Peripheral blood mononuclear cells isolated from six patients with Aspergillus SBO and healthy volunteers were stimulated with various microbial stimuli, among which also the fungal pathogens Candida albicans and Aspergillus fumigatus. The proinflammatory cytokines IL-6, TNFα and IL-1ß, and the T-helper cell-derived cytokines IFNγ, IL-17 and IL-22 were measured in cell culture supernatants by ELISA. RESULTS: Proinflammatory cytokine responses did not differ between SBO patients and healthy volunteers. The Candida- and Aspergillus-specific Th17 response (production of IL-17 and IL-22) was significantly decreased in the SBO patients compared to healthy individuals, while Th1 cytokine response (IFNγ production) did not differ between the two groups. CONCLUSIONS: We show that patients with Aspergillus skull base osteomyelitis infection have specific defects in Th17 responses. Since IL-17 and IL-22 are important for stimulating antifungal host defense, we hypothesize that strategies that have the ability to improve IL-17 and IL-22 production may be useful as adjuvant immunotherapy in patients with Aspergillus SBO.

[Two cases of allergic fungal rhinosinusitis with bone destruction].

We report herein two cases of allergic fungal rhinosinusitis accompanied by bone destruction of the adjacent nasal sinuses. The first case involved a 21-year-old man who presented with left exophthalmos. Computed tomography (CT) showed soft tissue lesions in the left paranasal sinuses and destruction of the left lamina papyraceae, as well as infiltration of the lesion into the orbit. In the second case, a 39-year-old man, CT showed bone destruction of the skull base and medial wall of the left orbit. In both cases, total serum immunoglobulin (Ig)E level was >1000 IU/mL and fungus-specific IgEs were increased. Fungal hyphae were identified within the mucus on histopathological examination in both cases; however, no fungal invasion of the mucosa was apparent. Final diagnosis was allergic fungal rhinosinusitis (AFRS) in both cases. AFRS is a relatively new disease concept that was proposed in the early 1980s, with disease characteristics very similar to eosinophilic rhinosinusitis. Occasionally, AFRS must be differentiated from malignant disease or invasive fungal rhinosinusitis, so an understanding of the clinical features is important.

Pseudotumoral allergic fungal sinusitis with skull base involvement.

Here we report a case of pseudotumoral recurrence of allergic fungal sinusitis with involvement of the skull base that was successfully treated with systemic corticosteroids and itraconazole without surgery. This report discusses the sometimes misleading radiological and clinical features as well as the diagnostic and therapeutic challenges of a condition that should be recognized by ENT specialists, neurosurgeons, ophtalmologists and radiologists.

Central skull base osteomyelitis: an emerging clinical entity.

OBJECTIVES/HYPOTHESIS: Skull base osteomyelitis (SBO) most commonly results as a complication of otitis externa in diabetic patients. Central or atypical presentations, unrelated to aural pathology, have been documented, though restricted to small patient series or case reports. The current study systematically analyzed the literature to construct the clinical profile of this rare entity. STUDY DESIGN: Systematic review of case series. METHODS: The MEDLINE database was searched for all published cases of central SBO. Four additional cases were included from the University of Texas Southwestern Medical Center. Statistical analysis was performed to identify trends affecting overall outcomes or differences in treatment. RESULTS: A total of 42 cases were included with mean age of 52 years and male:female ratio of 2.2:1. The most common presenting symptoms were headaches and cranial nerve palsies, including VI (31%), IX (29%), and X (29%). Staphylococcus aureus (21%) and Pseudomonas aeruginosa (19%) were the two most common causative pathogens. The mean duration of antimicrobial therapy was 21 weeks, with 55% requiring intravenous antibiotics for a mean duration of 6.9 weeks. Twenty of 42 patients (48%) received surgical biopsy for diagnosis, whereas 18 patients (43%) required adjunct surgical debridement. Women were more likely than men to require multiple courses of therapy (46% vs. 7%, P = .01). The overall mortality was 9.5%, with an additional 31% experiencing residual neurological dysfunction. CONCLUSIONS: Osteomyelitis of the central skull base poses significant challenges for timely and accurate diagnosis. Aggressive management with antimicrobials, coupled with surgical debridement in select cases, may avoid serious neurologic morbidity and mortality.

Allergic fungal rhinosinusitis infiltrating anterior skull base and clivus.

Bone erosion and skull base invasion are often suggestive of a malignant mass in paranasal and nasal cavities. Nevertheless, forms of chronic rhinosinusitis, such as allergic fungal rhinosinusitis (AFRS), could mimic malignant features. Here, we report AFRS patient with orbital, anterior cranial fossa, Turkish saddle and clivus erosion. A 48-year-old Caucasian female with history of drug-resistant headache, nasal obstruction and anosmia was referred to our institution. Imaging showed hyperdense featureless tissue with signs of medial orbital wall, cribiform lamina and clivus erosions and encasement of right internal carotid artery. Massive amounts of thick and grayish mucoid material were evacuated during surgery. In case of bony erosion, malignancy should always be excluded. Often the correct diagnosis will be obtained only by operative specimens. AFRS could usually be managed endoscopically. Appropriate medical management of the AFRS should be administered in order to prevent relapses.

Clinical-scintigraphy discordance in a case of malignant external otitis.

We report the case of an immunocompromised female patient with external otitis, who developed a picture of external otitis with poor response to conventional treatment and subsequent isolation of Pseudomona Aeruginosa in the ear secretion culture. The final diagnosis was malignant external otitis. Our objective is to describe the scintigraphic findings as well as to analyze their correlation with the clinical pattern and to evaluate the possible causes of the discordance found.