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.

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.

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.

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.

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.

Bone metabolic activity in hyperostosis cranialis interna measured with 18F-fluoride PET.

PURPOSE: (18)F-Fluoride PET/CT is a relatively undervalued diagnostic test to measure bone metabolism in bone diseases. Hyperostosis cranialis interna (HCI) is a (hereditary) bone disease characterised by endosteal hyperostosis and osteosclerosis of the skull and the skull base. Bone overgrowth causes entrapment and dysfunction of several cranial nerves. The aim of this study is to compare standardised uptake values (SUVs) at different sites in order to quantify bone metabolism in the affected anatomical regions in HCI patients. METHODS: Nine affected family members, seven non-affected family members and nine non-HCI non-family members underwent (18)F-fluoride PET/CT scans. SUVs were systematically measured in the different regions of interest: frontal bone, sphenoid bone, petrous bone and clivus. Moreover, the average (18)F-fluoride uptake in the entire skull was measured by assessing the uptake in axial slides. Visual assessment of the PET scans of affected individuals was performed to discover the process of disturbed bone metabolism in HCI. RESULTS: (18)F-Fluoride uptake is statistically significantly higher in the sphenoid bone and clivus regions of affected family members. Visual assessment of the scans of HCI patients is relevant in detecting disease severity and the pattern of disturbed bone metabolism throughout life. CONCLUSION: (18)F-Fluoride PET/CT is useful in quantifying the metabolic activity in HCI and provides information about the process of disturbed bone metabolism in this specific disorder. Limitations are a narrow window between normal and pathological activity and the influence of age. This study emphasises that (18)F-fluoride PET/CT may also be a promising diagnostic tool for other metabolic bone disorders, even those with an indolent course.

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.

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.

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

18F-fluoride PET/CT scan for quantification of bone metabolism in the inner ear in patients with otosclerosis--a pilot study.

BACKGROUND: In otosclerosis, CT of the temporal bone is used to confirm the diagnosis. Whereas CT is a static diagnostic tool displaying the demineralization caused by otosclerosis, imaging of bone metabolism by (18)F-fluoride PET may provide quantitative information. This could be useful for prognosis and stratification of patients. The aim of this study was to assess (18)F-fluoride activity in patients with otosclerosis and to evaluate its use as a complementary diagnostic tool. METHODS: All patients with otosclerosis underwent a PET/CT scan. Audiometric data were collected. The severity of otosclerosis was assessed using a recognized radiological classification. The control group consisted of patients who had undergone (18)F-fluoride PET/CT scan for orthopedic purpose. Regions of interest were drawn on PET scans which corresponded to standardized anatomical sites as defined on CT, to measure bone metabolism using standardized uptake values (SUV(max) and SUV(mean)). RESULTS: Group 1 consisted of 11 otosclerosis patients (16 eligible temporal bones) and group 2 consisted of 5 control patients (10 temporal bones). On PET scan, visual assessment of temporal bones with otosclerosis showed increased metabolic activity in the otic capsule in 11/16 cases. The SUV(max) in the entire otic capsule was significantly higher in otosclerosis patients compared to control subjects. Significant differences in SUV(mean) were found between otosclerosis and control subjects in the fenestral and saccule area. Moreover, metabolic activity in these regions significantly correlated with hearing loss and CT classification. CONCLUSIONS: (18)F-Fluoride PET scanning using SUV measurements has the potential to be a diagnostic tool in otosclerosis.