CSF or middle ear effusion? Diagnostical dilemmas in a patient with temporal bone meningioma: A case report.

Introduction: Cerebrospinal fluid (CSF) fistulas are a rare phenomenon, that can lead to life-threatening complications if left untreated. Presenting as rhinorrhea or otorrhea, they can be difficult to diagnose due to admixture of other bodily fluids. Typically, CSF fistulas develop after trauma, but in rare instances, they can be diagnosed in patients with a neoplastic lesion. Objective: To discuss several steps in diagnosing CSF fistulas. Patient: A fifty-year-old female with an intra-osseous temporal bone meningioma. Interventions: For diagnosing CSF admixture in fluids, two tests are looked into: beta-2 transferrin (ß2T) and beta-trace protein (ßTP) testing. Conclusion: Testing for ßTP is a highly sensitive, quick and non-invasive method to assess CSF admixture in middle ear effusion. Because of its lower cost, faster results and easy sample collection, ßTP testing has in our clinic replaced ß2T testing. The current case illustrates a rare etiology of a CSF fistula, where ß2T testing presumably showed false-negative results and ßTP testing showed true-positive results.

A rare complication after vestibular schwannoma surgery: Neurogenic pulmonary edema.

Background: In our center, large vestibular schwannoma (VS) is typically managed by a planned partial resection through the translabyrinthine route. Here, we report on a rare complication of VS surgery and severe neurogenic pulmonary edema. Case Description: A 33-year-old male was referred to our skull-base center with a large VS. A planned partial resection was performed. The surgery was without complications and the patient showed good recovery without facial nerve dysfunction. In the evening of the 2nd day after surgery, the patient showed rapid neurological deterioration, accompanied by cardiac arrest. After the patient was resuscitated, a computed tomography (CT) was made, which showed generalized (infra- and supratentorial) brain edema and hematoma in the resection cavity. Despite rapid removal of the hematoma, there was no change in the neurological situation. The next CT scan showed a further increase of brain edema and the patient died eventually. Autopsy revealed generalized lung edema, brain edema, and Hashimoto's thyroiditis. The pathologist diagnosed neurogenic lung edema. Conclusion: Neurogenic lung edema can occur on the 2nd day after surgery and induce rapid deterioration of the patient with massive brain edema.

Clinical characteristics and an evaluation of predictors for a favourable outcome of Mycobacterium abscessus otomastoiditis: a systematic review and meta-analysis of individual participant data.

BACKGROUND: Otomastoiditis caused by Mycobacterium abscessus is rare, but its incidence has increased over the past decades and its optimal treatment remains unknown. This study aims to summarise the clinical and therapeutic features and find characteristics of patients with M. abscessus otomastoiditis associated with favourable treatment outcomes. METHODS: We searched MEDLINE, Embase and Web of Science to identify studies including patients with M. abscessus otomastoiditis. A 1-stage individual patient data (IPD) meta-analysis was conducted. A 2-level mixed-effects linear regression model was provided for antimycobacterial treatment duration. RESULTS: Twenty-three studies reported a total of 85 patients. Children possess a unique clinical profile including a history of ear infections, tympanostomy tube placement and antibiotic treatment. Antimycobacterial treatment was administered for 26 (interquartile range [IQR]: 15-35) weeks. Macrolides were prescribed in 98.8% of the cases. Surgery was performed in 80.5% of the cases, of which, 47.1% required revision surgery. Otalgia was a significant predictor (ß = 9.3; P = .049) of antimycobacterial treatment duration. CONCLUSIONS: Mastoid surgery (regularly requiring revision) and a multidrug regimen for a minimum of 6 months, including a minimum of 3 active agents, are most often needed to attain cure. The presence of otalgia significantly extends the treatment duration of M. abscessus otomastoiditis.

A closer look at the paralyzed face; a narrative review of the neurobiological basis for functional and aesthetic appreciation between patients with a left and a right peripheral facial palsy.

BACKGROUND: The facial nerve or n. facialis (NVII) is the seventh cranial nerve and it is responsible for the innervation of the mimic muscles, the gustatory organ, and the secretomotor function to the salivary, lacrimal, nasal and palatine glands. Clinical presentation of Facial Palsy (FP) is characterized by unilateral facial asymmetry and may present with a change in taste, decreased saliva production, and dysarthria. A facial palsy has a notable effect on the facial appreciation by both the patient and the environment and also affects quality of life and emotional processing. There appear to be differences in the appreciation of people with a left and right facial palsy. PURPOSE OF THIS REVIEW: The purpose of the review is to give an overview of the anatomy of the facial nerve, neuro-anatomy of face processing, and hemispheric specialization and lateralization. Further,an overview is given of the clinical studies that translated the neuro-anatomical and neurobiological basis of these concepts into clinical studies. What this review adds: This review emphasizes the neurobiological evidence of differences in face processing between the left and right cerebral hemisphere, wherein it seems that the right hemisphere is superior in emotional processing. Several theories are proposed; 1) a familiarity hypothesis and 2) a left-right hemispheric specialization hypothesis. In clinical studies, promising evidence might indicate that, in patients with FP, there is indeed a difference in how left and right FP are perceived. This might give differences in decreased quality of life and finally in occurrence of depression. Further research must aim to substantiate these findings and determine the need for altering the standard therapeutic advice given to patients.

Surgical Treatment for Epstein-Barr Virus Otomastoiditis Complicated by Facial Nerve Paralysis: A Case Report of Two Young Brothers and Review of Literature.

We report the case of two young brothers with Epstein-Barr virus (EBV) otomastoiditis complicated by a facial nerve paralysis. The boys, aged 7 months (patient A) and 2 years and 8 months (patient B), were diagnosed with a facial nerve paralysis House-Brackmann (HB) grade IV (A) and V (B). After unsuccessful pharmacological treatment, patient A underwent mastoidectomy and atticoantrotomy and patient B underwent a transmastoidal surgical decompression of the facial nerve. They recovered to HB grades I and II facial nerve palsy (FNP), respectively. Although rare and relatively unknown, EBV should be considered in the differential diagnosis of children with FNP of unknown cause. Surgical intervention may be a viable therapy with good recovery.

Repeated Postoperative Follow-up Diffusion-weighted Magnetic Resonance Imaging to Detect Residual or Recurrent Cholesteatoma.

OBJECTIVE: In our institution, follow-up diffusion-weighted imaging (DWI) after cholesteatoma surgery is performed at least twice. The aim of this study was to determine the yield of the second follow-up DWI (D-W MRI-2) in patients in whom the first postoperative DWI (D-W MRI-1) was negative for residual or recurrent cholesteatoma. STUDY DESIGN: A retrospective analysis. SETTING: Tertiary referral center. PATIENTS: Patients were included if 1) they had at least two postoperative DWI examinations after a canal wall up procedure with apparently complete cholesteatoma resection; 2) D-W MRI-1 was performed between 6 and 24 months after surgery and D-W MRI-2 performed at least 6 months after D-W MRI-1; 3) both DWI examinations were of good quality and covering the whole mastoid-middle ear region; 4) D-W MRI-1 was unequivocally negative for cholesteatoma; and 5) there was no clinical suspicion on otoscopy of recurrent cholesteatoma nor a surgical intervention between these two postoperative DWI examinations. In total, 45 separate ears in 44 patients were included. RESULTS: In 14 ears (31%) D-W MRI-2 was positive (n = 8) or equivocal (n = 6) for cholesteatoma. In six of eight patients with positive D-W MRI-2, follow-up surgery was performed. Cholesteatoma was found in five of them. None of the patients with equivocal findings on D-W MRI-2 was operated on. Patients with positive D-W MRI-2 were of young age. There were no observable differences for sex, side, time between surgery and D-W MRI-1, time between surgery and D-W MRI-2, or time between D-W MRI-1 and D-W MRI-2, or for the location of cholesteatoma at surgery. In the study period there was a trend to perform D-W MRI-1 and D-W MRI-2 earlier after initial surgery. In the same period, there was an evident decrease in average age of the patient population. CONCLUSION: Despite cholesteatoma surgery without macroscopic residue, clinical follow-up and routine first follow-up DWI without any signs of residual or recurrent disease, repeat follow-up DWI showed evidence of cholesteatoma in 31% of patients. On the basis of the findings in this study, repeated follow-up DWI is recommended.

Correlation between in vivo 18F-FDG PET and immunohistochemical markers of glucose uptake and metabolism in pheochromocytoma and paraganglioma.

UNLABELLED: Pheochromocytomas and paragangliomas (PPGLs) can be localized by (18)F-FDG PET. The uptake is particularly high in tumors with an underlying succinate dehydrogenase (SDH) mutation. SDHx-related PPGLs are characterized by compromised oxidative phosphorylation and a pseudohypoxic response, which mediates an increase in aerobic glycolysis, also known as the Warburg effect. The aim of this study was to explore the hypothesis that increased uptake of (18)F-FDG in SDHx-related PPGLs is reflective of increased glycolytic activity and is correlated with expression of different proteins involved in glucose uptake and metabolism through the glycolytic pathway. METHODS: Twenty-seven PPGLs collected from patients with hereditary mutations in SDHB (n = 2), SDHD (n = 3), RET (n = 5), neurofibromatosis 1 (n = 1), and myc-associated factor X (n = 1) and sporadic patients (n = 15) were investigated. Preoperative (18)F-FDG PET/CT studies were analyzed; mean and maximum standardized uptake values (SUVs) in manually drawn regions of interest were calculated. The expression of proteins involved in glucose uptake (glucose transporters types 1 and 3 [GLUT-1 and -3, respectively]), phosphorylation (hexokinases 1, 2, and 3 [HK-1, -2, and -3, respectively]), glycolysis (monocarboxylate transporter type 4 [MCT-4]), and angiogenesis (vascular endothelial growth factor [VEGF], CD34) were examined in paraffin-embedded tumor tissues using immunohistochemical staining with peroxidase-catalyzed polymerization of diaminobenzidine as a read-out. The expression was correlated with corresponding SUVs. RESULTS: Both maximum and mean SUVs for SDHx-related tumors were significantly higher than those for sporadic and other hereditary tumors (P < 0.01). The expression of HK-2 and HK-3 was significantly higher in SDHx-related PPGLs than in sporadic PPGLs (P = 0.022 and 0.025, respectively). The expression of HK-2 and VEGF was significantly higher in SDHx-related PPGLs than in other hereditary PPGLs (P = 0.039 and 0.008, respectively). No statistical differences in the expression were observed for GLUT-1, GLUT-3, and MCT-4. The percentage anti-CD 34 staining and mean vessel perimeter were significantly higher in SDHx-related PPGLs than in sporadic tumors (P = 0.050 and 0.010, respectively). Mean SUVs significantly correlated with the expression of HK-2 (P = 0.027), HK-3 (P = 0.013), VEGF (P = 0.049), and MCT-4 (P = 0.020). CONCLUSION: The activation of aerobic glycolysis in SDHx-related PPGLs is associated with increased (18)F-FDG accumulation due to accelerated glucose phosphorylation by hexokinases rather than increased expression of glucose transporters.

Standardized multidisciplinary evaluation yields significant previously undiagnosed morbidity in adult women with Turner syndrome.

CONTEXT: Besides short stature and gonadal dysgenesis, Turner syndrome (TS) is associated with various abnormalities. Adults with TS have a reduced life expectancy, mainly related to structural abnormalities of the heart and aorta, and an increased risk of atherosclerosis. OBJECTIVE: Our objective was to investigate the yield of an initial standardized multidisciplinary screening in adult TS patients. DESIGN AND SETTING: This was an observational study at a multidisciplinary care unit for adult women with TS. PARTICIPANTS: Participants were adult women with TS (n = 150). Mean age was 31.0 ± 10.4 yr, with 47% karyotype 45,X. INTERVENTIONS: All women were consulted by an endocrinologist, a gynecologist, a cardiologist, an otorhinolaryngologist, and when indicated, a psychologist. The screening included magnetic resonance imaging of the heart and aorta, echocardiography, electrocardiogram, dual-energy x-ray absorptiometry, renal ultrasound, audiogram, and laboratory investigations according to international expert recommendations. MAIN OUTCOME MEASURES: New diagnoses and prevalence of TS-associated morbidity were evaluated. RESULTS: Thirty percent of patients currently lacked medical follow-up, and 15% lacked estrogen replacement therapy in the recent last years. The following disorders were newly diagnosed: bicuspid aortic valve (n = 13), coarctation of the aorta (n = 9), elongation of the transverse aortic arch (n = 27), dilation of the aorta (n = 34), osteoporosis (n = 8), osteopenia (n = 56), renal abnormalities (n = 7), subclinical hypothyroidism (n = 33), celiac disease (n = 3), glucose intolerance (n = 12), dyslipidemia (n = 52), hypertension (n = 39), and hearing loss warranting a hearing aid (n = 8). Psychological consultation was needed in 23 cases. CONCLUSIONS: Standardized multidisciplinary evaluation of adult women with TS as advocated by expert opinion is effective and identifies significant morbidity. Girls with TS benefit from a careful transition to ongoing adult medical care.

Endolymphatic sac tumors: clinical outcome and management in a series of 9 cases.

OBJECTIVES: Endolymphatic sac tumors (ELSTs) are rare adenomatous tumors arising in the petrous bone, either spontaneously (isolated) or in association with von Hippel-Lindau (VHL) disease. For 9 patients, this study describes the typical features and management of ELST and the clinical outcome of treatment. Similarities and differences are identified for ELST in isolation and in association with VHL disease. STUDY DESIGN: Case series. SETTING: Tertiary referral center. PATIENTS: The patient database was searched for patients diagnosed with ELST. All other tertiary referral centers in The Netherlands were invited to do likewise. INTERVENTIONS: The patient records were screened for presenting symptoms, hearing, imaging, treatment, and treatment outcome. Histopathologic finding was reviewed by a senior pathologist. RESULTS: Six patients with an isolated ELST and 3 patients with an ELST in association with VHL disease were identified. In the isolated ELST group, 5 patients were treated surgically and 1 patient was administered primary radiotherapy. In the VHL group, 1 patient underwent surgery and 2 patients were treated conservatively after a wait-and-magnetic resonance imaging-scan protocol. All individual treatment outcomes are scrupulously presented in this article. CONCLUSION: This study compiles and describes 9 cases of ELST. In 8 of 9 patients, the ELST could be controlled by surgery, radiotherapy, or a wait-and-scan protocol. The large interpatient differences make it difficult to standardize the management of this disease. The management is strongly influenced by comorbidity and tumor stage.