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.

Health-related quality of life after otologic surgical treatment for chronic otitis media: systematic review.

Objective: This systematic review aims to describe the impact of otologic surgery as a treatment for chronic otitis media (COM) on the Health-Related Quality of Life (HRQoL) of adult patients. Methods: A literature search was performed in PubMed, Scopus, Embase, and Web of Science until May 2023. Prospective studies including adult patients with COM (cholesteatoma) who underwent canal wall up mastoidectomy, canal wall down mastoidectomy, or tympanoplasty without mastoidectomy, with pre- and postoperative HRQoL measurements, were considered eligible. Questionnaire validation studies were excluded. The risk of bias and study quality were evaluated with a Quality Assessment Tool (for before-after studies with no control group). To assess the change in HRQoL, pre- and postoperative HRQoL values and absolute changes were extracted, synthesized, and presented in tables. Standardized mean differences (SMD) were calculated to enhance comparisons. Results: Of the 720 studies identified, 16 met the inclusion criteria of this review. Different questionnaires were used throughout the studies. The CES and COMOT-15 were used in five studies and the ZCMEI-21 and COMQ-12 in three studies. All studies indicated statistically significant improvement in HRQoL from pre- to postoperative, measured with disease-specific HRQoL questionnaires. General HRQoL questionnaires did not show significant improvement. Calculated SMDs ranged from 0.24 to 6.99. Discussion and conclusion: Included studies had low (n = 10) to high (n = 6) risk of bias and poor (n = 4), fair (n = 7) or good (n = 5) study quality. Surgical treatment positively impacts the HRQoL of adult COM patients with and without cholesteatoma. However, the clinical relevance of the reported changes is unknown due to the lack of minimal clinically important differences (MCID) or cut-off values in each questionnaire. Therefore, further research regarding the MCIDs of each questionnaire is needed. Future research should also report preoperative chief symptoms and indications for surgery to improve individual patient counseling.

Subclassification of the Koos grade 2 vestibular schwannoma into 2a and 2b for individualized patient care: A validity and reliability study.

OBJECTIVE: Vestibular schwannoma (VS) growth of ≥2 mm during serial MRI observation, irrespective of size, is the benchmark for treatment initiation in almost all centers. Although the probability of less optimal outcomes significantly increases in VS closer to the brainstem, early intervention does not improve long-term quality of life. Moving beyond the recommendation of definitive treatment for all VS after detected growth, we subclassified Koos 2 tumors based on extrameatal extension and relation to the brainstem. The aim of the current study was to evaluate the Koos 2 subclassification's validity and the inter-and intra-rater reliability of the entire Koos classification. METHODS: Six experts, including neurosurgeons, otorhinolaryngologists and radiologists from two tertiary referral centers, classified 43 VS MRI scans. Validity of the Koos 2 subclassification was evaluated by the percentage agreement against the multidisciplinary skull base tumor board management advice. Inter- and intra-rater reliability were calculated using the intraclass correlation coefficient (ICC). RESULTS: Validity was almost perfect in Koos 2a VSs with a 100% agreement and 87.5% agreement for Koos 2b. Inter-rater reliability for all Koos grades was significantly excellent (ICC 0.91; 95%CI 0.866 to 0.944, p= <0.001). Five raters had an excellent intra-rater reliability (ICC > 0.90; p= <0.01) and one rater had a good intra-rater reliability (ICC 0.88; 95% CI 0.742 to 0.949). CONCLUSIONS: Although multiple factors influence decision-making, the classification of Koos 2a and 2b with excellent inter- and intra-rater reliability, can aid in recommending treatment initiation, moving beyond detected tumor growth, aiming to optimize patient centered care.

Characteristics and Clinical Management Strategy of Petrous Apex Cholesterol Granulomas.

Purpose: To evaluate the clinical characteristics of petrous apex cholesterol granulomas (PACG) and assess outcomes after different treatment strategies. Method: A consecutive case series of 34 patients with a PACG. Main outcomes were PACG growth, symptoms, and the outcomes of different treatment strategies: wait-and-scan (WS) and surgical drainage. Results: Thirty-four patients were analyzed; mean follow-up time was 7.1 years. Twenty-one patients (61.7%) showed symptoms, mostly more than one. Most symptoms reported were cranial nerve palsy (58.8%) and headache (35.3%). Twenty-one patients (61.8%) received solely wait-and-scan (WS), and thirteen patients (38.2%) underwent surgery, five of whom (38.5%) after an initial WS period. In the solely WS group, one (4.8%) developed new symptoms, and two (9.5%) reported symptom progression despite a stable granuloma size. Two (9.5%) showed granuloma growth on follow-up scans without symptom progression. Surgery consisted of drainage. Eleven (84.6%) of these thirteen patients reported partial recovery; one (7.7%) reported no recovery; and one (7.7%) reported full recovery of reported symptoms related to PACG. Among the patients with cranial nerve involvement, 7.7% showed full recovery after surgery; 84.6% showed partial recovery; and 7.7% did not recover. Adverse events occurred in five out of 13 patients who underwent surgery, all with full recovery. Conclusions: This study confirms that PACG are slow-growing lesions with a low risk of adverse events. Solely using wait-and-scan strategy is a safe option for patients without symptoms, with acceptable symptoms without symptom progression, and with asymptomatic growth. Surgical treatment can be considered in patients with symptom progression or symptomatic growth.

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.

The association between radiological spreading pattern and clinical outcomes in necrotizing external otitis.

Objectives: Necrotizing external otitis (NEO) is a rare infectious disease of the skull base. The purpose of this study was to determine whether clinical outcomes of NEO can be correlated to different infectious spread patterns. Methods: Retrospective chart review from 2010 to 2019 with NEO patients, who were divided into two cohorts: single spreading patterns (group A) or complex spreading patterns (group B) as diagnosed by CT. Clinical symptoms, diagnostic and treatment delay, course of disease, complications, and duration of antibiotic exposure were retrospectively collected from patient records. Results: 41 NEO patients were included, of which 27 patients belonged to group A (66%). The disease-related mortality rate was 12.2% among the entire cohort, no differences were found between group A and B. Higher rates of N.VII (42.9% vs 14.8% P = 0.047) and N. IX palsies were found in group B compared to group A (28.6% vs 3.7%, P = 0.039). The median duration of antibiotic use was significantly different for a complex spreading pattern, clinical recovery and hospitalizations. Complications were associated with higher diagnostic delay and with a complex spread pattern. The median duration of follow-up was 12.0 (IQR 6.0-19.5) months. Conclusion: NEO is a severe disease, with significant mortality and morbidity (cranial nerve palsies). The radiological spread pattern may assist in predicting clinical outcome. Furthermore, complex spread patterns are associated with higher rates of clinical nerve palsies (N. VII and N.IX), complications, surgery rates and longer duration of antibiotic use. Diagnostic delay was associated with mortality, complications and facial palsies. Level of evidence: Level IV.

Diagnosis, treatment and prognosis of otomastoiditis induced by Fusobacterium necrophorum: A retrospective multicentre cohort study.

OBJECTIVES: Otomastoiditis caused by the anaerobic Fusobacterium necrophorum (F. necrophorum) often induces severe complications, such as meningitis and sinus thrombosis. Early diagnosis is difficult, partly because little is known about specific early signs. Comprehensive research about clinically chosen antimicrobial therapy has not been done yet and prognostic information about otomastoiditis caused by F. necrophorum is scarce. More knowledge about this subject is required. METHODS: In this retrospective cohort study, we included all cases of otomastoiditis caused by F. necrophorum treated in two university medical centres in the Netherlands during the past 10 years. Data was gathered from patient records and analysed using independent sample T-tests and Chi2-tests. RESULTS: This study reveals that otomastoiditis caused by F. necrophorum potentially induces neurological sequelae. Thereby, 80% of all included patients (n = 16) needed readmission within six months due to recurrence or complications of otomastoiditis caused by F. necrophorum. Mean (range) of age, CRP and temperature were 4.5 years (0.9-29.3), 243 mg/L (113-423) and 40 °C (37-41). All patients were hospitalized and treated with antibiotics, mostly metronidazole (n = 13/16) and a ß -lactam (n = 15/16). Additional treatment contained low molecular weight heparin (83%, n = 10/12), dexamethasone (78%, n = 7/9) and/or surgery (80%, n = 12/16, whereof 9/12 mastoidectomy). CONCLUSIONS: Patients and/or their parents need to be informed about this potential unfortunate prognosis when otomastoiditis caused by F. necrophorum is diagnosed. To improve early diagnosis, otomastoiditis caused by F. necrophorum should be suspected and therefore immediately cultured when a) young children present with otomastoiditis, with b) high CRP values, and/or c) vomiting and decreased consciousness.

Diagnosing necrotizing external otitis on CT and MRI: assessment of pattern of extension.

BACKGROUND AND PURPOSE: Necrotizing external otitis (NEO) is a serious complication of external otitis. NEO can be classified according to-anterior, medial, posterior, intracranial, and contralateral-extension patterns. Currently there is no consensus on the optimal imaging modality for the identification of disease extension. This study compares NEO extension patterns on MR and CT to evaluate diagnostic comparability. METHODS: Patients who received a CT and MR within a 3-month interval were retrospectively examined. Involvement of subsites and subsequent spreading patterns were assessed on both modalities by a radiologist in training and by a senior head and neck radiologist. The prevalence of extension patterns on CT and MR were calculated and compared. RESULTS: All 21 included NEO cases showed an anterior extension pattern on CT and MR. Contrary to MR, medial extension was not recognized on CT in two out of six patients, and intracranial extension in five out of eight patients. The posterior extension pattern was not recognized on MR. Overall, single anterior extension pattern (62%) is more prevalent than multiple extension patterns (38%). CONCLUSION: All anterior NEO extension pattern were identified on CT as well as MR. However, the medial and intracranial spreading patterns as seen on MR could only be identified on CT in a small number of patients. The posterior spreading pattern can be overlooked on MR. Thus, CT and MR are complimentary for the initial diagnosis and work-up of NEO as to correctly delineate disease extent through the skull base.

Spontaneous Regression of a Middle Ear Melanoma.

OBJECTIVE: To describe a case of complete spontaneous regression of a middle ear melanoma. PATIENT: We present a case of a 68-year-old man with complaints of unilateral hearing loss and an ipsilateral facial nerve paresis. Radiological and histopathological examination revealed a cT4bN0M0 mucosal melanoma of the middle ear. INTERVENTIONS: The patient underwent a subtotal petrosectomy and postoperative radiotherapy. MAIN OUTCOME MEASURE: Computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography/computed tomography with 2-[fluorine-18]-fluoro-2-deoxy-D-glucose (FDG-PET-CT), and histopathological examination. RESULTS: After subtotal petrosectomy, histopathological examination of the resection specimen showed only fibrosis and a histiocytic and clonal T-cell infiltration, but no residual melanoma at the primary tumor site, consistent with spontaneous tumor regression. Follow-up MRI scanning 6 and 12 months after radiotherapy showed no signs of tumor recurrence. CONCLUSIONS: This case describes the concept of spontaneous regression of a mucosal melanoma of the middle ear. Spontaneous tumor regression at this location has not been described before.

Proposal of a timing strategy for cholesteatoma surgery during the COVID-19 pandemic.

PURPOSE: The COVID-19 infection is an aggressive viral illness with high risk of transmission during otolaryngology examination and surgery. Cholesteatoma is known for its potential to cause complications and scheduling of surgery during the pandemic must be done carefully. The majority of otological surgeries may be classified as elective and postponed at this time (e.g., stapedotomy, tympanoplasty); whereas, others are emergencies (e.g., complicated acute otitis media, complicated cholesteatoma with cerebral or Bezold's abscess, meningitis, sinus thrombosis) and require immediate intervention. What is the ideal time for the surgical management of Cholesteatoma during the COVID-19 pandemic? METHODS: Senior otologic surgeons from six teaching hospitals from various countries affected by the COVID-19 from around the world met remotely to make recommendations on reorganizing schedules for the treatment of cholesteatoma which has a risk of severe morbidity and mortality. The recommendations are based on their experiences and on available literature. RESULTS: Due to the high risk of infecting the surgical staff it is prudent to stop all elective ear surgeries and plan cholesteatoma surgery after careful selection of patients, based on the extent of the disease and available resources. Specific precautions including use of appropriate personal protection equipment should be followed when operating on all patients during the pandemic. To facilitate the decision-making in the management of cholesteatoma, timing for surgery can be divided into two categories with 3 and 2 sub-groups based on disease severity. CONCLUSIONS: Evidence on the timing of surgery of patients with cholesteatoma during the COVID-19 pandemic is lacking. This manuscript contains practical tips on how cholesteatoma surgery can be reorganized during this pandemic.

Successful Systemic and Topical Treatment of Mycobacterium abscessus Otomastoiditis.

Mycobacterium abscessus is an extensively drug-resistant opportunistic pathogen that can cause chronic otomastoiditis. There are no evidence-based treatment regimens for this severe infection. We treated four children with M. abscessus otomastoiditis with a structured regimen of topical imipenem and tigecycline, intravenous imipenem and tigecycline, and oral clofazimine and azithromycin and adjunctive surgery. This structured approach led to cure, with 1 year of follow-up after treatment. Adverse events were frequent, mostly caused by tigecycline.

Clinical Approach After Complicated Ear Mold Fitting: A Case Series of Six Patients and Evaluation of Literature.

OBJECTIVES: Insertion of mold material into the middle ear is a complication of molding procedure for ear impression. These cases are referred to an ENT specialist. There is no standardized approach to this problem. Literature shows different clinical strategies. The aim of this study is to share our experience and to analyze the adverse outcome of different clinical approaches. METHODS: A case series of six patients with molding material inside the middle ear after complicated molding procedure for swimming earplugs are described. Additionally, available literature was reviewed to analyze results of the clinical approach after iatrogenic molding procedures. Forty-nine ears were included. RESULTS: In-office removal of the material is associated with a significant risk of adverse outcome if the eardrum cannot be examined. This also accounts for ossicular involvement. CONCLUSIONS: Temporal bone CT is advised in patients after complicated ear mold fitting if the tympanic membrane cannot be examined completely or the middle ear is involved. Blind removal should be avoided. Retroauricular transcanal tympanotomy or transmastoidal tympanotomy with facial recess approach is best practice in case the mold material has entered the middle ear. A clinical treatment algorithm is presented.

Measuring growth of residual cholesteatoma in subtotal petrosectomy.

BACKGROUND: Little is known about the growth rate of cholesteatoma in patients. OBJECTIVE: Investigate the growth of residual cholesteatoma in subtotal petrosectomy based on volume measured in MRI scans. MATERIALS AND METHODS: Retrospective case series in a Tertiary Medical Centre. Thirteen residual cholesteatomas were identified in 10 patients after subtotal petrosectomy for which a wait-and-scan policy was adopted. Volume of the residual cholesteatoma was calculated by manual segmentation as well as the 'box method'. RESULTS: Mean growth rate was 27.9 mm3/month (SD 22.8), with a large individual variation ranging from 2.2 to 69.8 mm3/month. No complications were reported in 10 patients with a wait-and-scan policy for residual cholesteatoma in subtotal petrosectomy. The box method overestimates growth rate compared to the reference method manual segmentation and a linear increase of this systematic error was seen with increasing size of the cholesteatoma. CONCLUSIONS: Residual cholesteatoma growth rate shows a large individual variation. A wait-and-scan policy could be considered in case of a (small) residual in subtotal petrosectomy with ample room to grow before destroying any remaining structures. Furthermore, the clinically more applicable and less time-consuming box method can be used to accurately measure volumes of small cholesteatomasup to a volume of 500 mm3.

What is the Required Frequency of MRI Scanning in the Wait and Scan Management?

The wait and scan policy is being increasingly used as the first measure after the diagnosis of a vestibular schwannoma (VS) using magnetic resonance imaging (MRI). As part of the European Academy of Otology and Neuro-Otology (EAONO) position statement on VS, the frequency of imaging has been studied in the literature. Among 163 studies, 29 fulfilled the inclusion criteria and were scored using the Grading of Recommendations, Assessment, Development, and Evaluation system. Because tumor growth rate during the first 5 years of follow-up is predictive of further growth during the upcoming years, a protocol for wait and scan is useful for centers dealing with this condition. The EAONO proposal is that after the initial diagnosis by MRI, a first new MRI would take place after 6 months, annually for 5 years, and then every other year for 4 years, followed by a lifelong MRI follow-up every 5 years. The first early MRI is to screen for fast-growing tumors, and the lifelong follow-up with tapered intervals is to detect late repeated growth.

EAONO position statement on Vestibular Schwannoma: Imaging Assessment Question: How should growth of Vestibular Schwannoma be defined?

The relevance of defining the growth of vestibular schwannoma (VS) is that any significant VS growth may impact treatment strategy. A conservative treatment strategy is often proposed as a primary treatment option in the management of VS. Several authors have demonstrated that a significant proportion of VSs do not grow, and those that do, usually grow slowly. Surgical and/or radiosurgical treatment options may be offered to the patient according to the VS growth. Therefore, defining the VS growth is a determinant in managing treatment strategies. A comprehensive literature search was performed to examine the definition of tumor growth for VS. The literature review was conducted using PubMed and Embase databases dated back to 20 years (1995-2015) and was updated until February 2015. VS growth should be measured on contrast-enhanced T1-weighted images. Although there the overall quality of the present studies is low, all highlight a significant VS growth of > 2 mm, and/or 1.2 cm3, and/or 20% change in volume, and/or the square of the product of the 2 orthogonal diameters. We suggest that VS growth should instead change management strategies when a 3-mm increase in diameter on two consecutive MRI scans are performed 1 year apart.

Conditional mouse models support the role of SLC39A14 (ZIP14) in Hyperostosis Cranialis Interna and in bone homeostasis.

Hyperostosis Cranialis Interna (HCI) is a rare bone disorder characterized by progressive intracranial bone overgrowth at the skull. Here we identified by whole-exome sequencing a dominant mutation (L441R) in SLC39A14 (ZIP14). We show that L441R ZIP14 is no longer trafficked towards the plasma membrane and excessively accumulates intracellular zinc, resulting in hyper-activation of cAMP-CREB and NFAT signaling. Conditional knock-in mice overexpressing L438R Zip14 in osteoblasts have a severe skeletal phenotype marked by a drastic increase in cortical thickness due to an enhanced endosteal bone formation, resembling the underlying pathology in HCI patients. Remarkably, L438R Zip14 also generates an osteoporotic trabecular bone phenotype. The effects of osteoblastic overexpression of L438R Zip14 therefore mimic the disparate actions of estrogen on cortical and trabecular bone through osteoblasts. Collectively, we reveal ZIP14 as a novel regulator of bone homeostasis, and that manipulating ZIP14 might be a therapeutic strategy for bone diseases.

The effects of alterations in the osseous external auditory canal on perceived sound quality.

OBJECTIVES/HYPOTHESIS: To evaluate the perceptual effect of the altered shape of the osseous external auditory canal (OEAC) on sound quality. STUDY DESIGN: Prospective study. METHODS: Twenty subjects with normal hearing were presented with six simulated sound conditions representing the acoustic properties of six different ear canals (three normal ears and three cavities). The six different real ear unaided responses of these ear canals were used to filter Dutch sentences, resulting in six simulated sound conditions. A seventh unfiltered reference condition was used for comparison. Sound quality was evaluated using paired comparison ratings and a visual analog scale (VAS). RESULTS: Significant differences in sound quality were found between the normal and cavity conditions (all P < .001) using both the seven-point paired comparison rating and the VAS. No significant differences were found between the reference and normal conditions. Sound quality deteriorates when the OEAC is altered into a cavity. CONCLUSIONS: This proof of concept study shows that the altered acoustic quality of the OEAC after radical cavity surgery may lead to a clearly perceived deterioration in sound quality. Nevertheless, some questions remain about the extent to which these changes are affected by habituation and by other changes in middle ear anatomy and functionality. LEVEL OF EVIDENCE: 4

Treatment of traumatic stapediovestibular luxation: case report with the introduction of a new technique and review of literature.

OBJECTIVE: Stapediovestibular luxations are rare lesions that are most commonly caused by direct, penetrating trauma to the external ear canal. In this type of ossicular dislocation, disruption of the annular ligament or footplate fracture may lead to a perilymphatic fistula (PLF) presenting with cochleovestibular symptoms including (progressive) sensorineural hearing loss, tinnitus, and vestibular symptoms. The objective of this article is to define the optimal treatment of stapediovestibular luxations and review the literature on this topic. PATIENT: We present a case of internal stapediovestibular dislocation and pneumolabyrinth after penetrating trauma with predominantly conductive hearing loss and incapacitating vertigo. INTERVENTION: Middle ear inspection with removal of the luxated incus, repositioning of the stapes with a "stapedial strut" and closure of the tympanic membrane. MAIN OUTCOME MEASURES: Hearing outcome and vestibular symptoms of this patient are presented, and all comparable cases in the literature are reviewed. RESULTS: Complete resolution of the vestibular symptoms and stabilization of the bone conduction thresholds. CONCLUSION: In patients with pneumolabyrinth after middle ear trauma with mild symptoms, we recommend initial conservative treatment with close monitoring of hearing. In patients with severe, persisting, or progressive vestibulocochlear symptoms, exploratory tympanotomy should be performed to check for and treat PLF. Oval window fistula repair is reported to have variable hearing outcomes but offers complete resolution of vestibular symptoms in most cases. The stapedial strut is one of the possible surgical techniques in case of an internally luxated stapes.