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Objectives/hypothesis: Currently, it is possible to assess in vivo the morphology of each compartment of the endolymphatic spaces 4 hours after an intravenous administration of gadolinium on magnetic resonance imaging (MRI). The aim of this study was to assess the correlation between otolithic and ampullar functions (cervical vestibular evoked myogenic potential [cVEMP], ocular vestibular evoked myogenic potential [oVEMP], video head impulse test [VHIT]) and delayed inner ear MRI based on a compartmental, anatomically based classification that included the cochlea, the saccule, the utricle, and the ampullas., Study Design: Retrospective case-control study., Methods: In this retrospective study, we performed three-dimensional fluid-attenuated inversion recovery sequences with delayed acquisition in 26 healthy subjects and 31 definite Menière's disease (MD) patients. Each subject was then graded on MRI on the basis on cochlear, saccular, utricular, and ampullar hydrops in MD patients. All patients underwent pure-tone audiometry, VHIT, cVEMP, and oVEMP testing., Results: Cochlear, saccular, utricular, and ampullar hydrops were found on MRI in 88%, 91%, 50%, and 8.5% respectively. We found no significant correlation between the presence of saccular hydrops versus cVEMP, utricular hydrops versus oVEMP, and ampullar hydrops versus VHIT. However, the severity of endolymphatic hydrops on MRI was correlated to the degree of hearing loss., Conclusions: We proposed a compartmental, anatomically based classification for endolymphatic hydrops on MRI, which included the whole vestibular compartment. Using this classification, we observed increasing morphological changes as the disease evolved, affecting first the saccule, then the utricle, and finally the ampullas. The severity of vestibular endolymphatic hydrops is only correlated to hearing loss severity., Level of Evidence: 3 Laryngoscope, 130:E444-E452, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Previous studies showed that various types of cerebral lesions, as assessed on MRI, largely contribute to the clinical severity of CADASIL. However, the clinical impact of longitudinal changes of classical markers of small vessel disease on conventional MRI has been only poorly investigated. One hundred sixty NOTCH3 mutation carriers (mean age ± SD, 49.8 ± 10.9 years) were followed over three years. Validated methods were used to determine the percent brain volume change (PBVC), number of incident lacunes, change of volume of white matter hyperintensities and change of number of cerebral microbleeds. Multivariable logistic regression analyses were performed to assess the independent association between changes of these MRI markers and incident clinical events. Mixed-effect multiple linear regression analyses were used to assess their association with changes of clinical scales. Over a mean period of 3.1 ± 0.2 years, incident lacunes are found independently associated with incident stroke and change of Trail Making Test Part B. PBVC is independently associated with all incident events and clinical scale changes except the modified Rankin Scale at three years. Our results suggest that, on conventional MRI, PBVC and the number of incident lacunes are the most sensitive and independent correlates of clinical worsening over three years in CADASIL.

Objectives/hypothesis: Because delayed post-contrast three-dimensional fluid-attenuated inversion recovery imaging sequences enable the distinction between the utricle and the saccule, we raised the hypothesis that patients with vestibular atelectasis (VA) could show unilateral collapse of the utricle and the ampullas on imaging., Study Design: Retrospective case series., Methods: We retrospectively reviewed 200 patients who underwent 3 T magnetic resonance imaging (MRI) after intravenous administration of gadolinium. MRI scans were assessed for the presence of VA. The endolymphatic space was considered as collapsed when the utricle and at least two ampullas were not visible or were barely visible., Results: We reported four patients with VA on MRI responsible for atypical clinical presentations of acute vestibular deficit. All patients presented a specific involvement of the pars superior sensory captors (utricle, ampullas), preserving the pars inferior sensory captors (cochlea and saccule). This was confirmed both clinically and on MRI., Conclusions: Our study is the first to describe in vivo unilateral collapse of the pars superior on delayed postcontrast MRI in patients with a clinical unilateral vestibular loss., Level of Evidence: 4 Laryngoscope, 129:1689-1695, 2019., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Most patients with suspicion of hydrops do not have access to MRI with 3D reconstruction of the endolymphatic space. Our main objective was to show that measurements of the saccule on a non-enhanced 3D-T2 MRI could show hydrops and help diagnose Menière disease. We conducted a prospective study from 2015 to 2016 to compare consecutive patients consulting for Menière's disease to a control group (patients with unilateral non-hydrops disorders and contralateral healthy ears). They all received full auditory and vestibular testing. They also underwent a 3-Tesla 3D-T2 MRI using CISS sequence (0.4 mm thick slices), which were blindly evaluated by two independent neuroradiologists. The saccular height and width were measured in a coronal plane and Menière's disease patients' symptomatic ears were compared to asymptomatic and control ears. 36 patients with definite Menière's disease and 36 control patients were studied, including 42 symptomatic Menière, 30 asymptomatic Menière and 72 control ears. Saccular measurements were significantly different between symptomatic Menière ears compared to healthy ears (1.59 vs 1.32 mm, p < 0.001 for height; 1.13 vs 0.90 mm, p < 0.001 for width). Symptomatic and asymptomatic Menière ears' measurements were not significantly different (p = 0.307 and p = 0.109). Using ROC curve, we found cut-off values for saccular height 1.51 mm, Se = 63%, Sp = 95% and width 1.05 mm, Se = 41%, Sp = 95%. Routine 3D-T2 MRI, which patients must undergo for differential diagnosis, could help diagnose hydrops with high specificity using saccular measurements.

Background and Purpose: Unruptured anterior inferior cerebellar artery (AICA) aneurysms are rare but potentially lethal cerebellopontine angle (CPA) lesions that may be misdiagnosed as vestibular schwannomas when they present with vestibulo-cochlear symptoms., Methods: We report two cases of unruptured but symptomatic AICA aneurysms initially referred to us as atypical vestibular schwannomas requiring surgery. Two discriminant MR features are described., Results: One patient refused treatment. The other was successfully treated by coil occlusion., Conclusions: Caution is advised before suspecting a CPA mass to be a purely extra-canalicular schwannoma, given its extreme rarity. Deafness and cerebellar ischemia may be prevented if AICA aneurysms are correctly identified preoperatively. In the absence of specific arterial imaging, two MR features may distinguish them from vestibular schwannomas: (1) the absence of internal auditory canal enlargement and (2) the "blurry dot sign," representing blood flow artefacts on pre- and postcontrast studies., (Copyright © 2013 by the American Society of Neuroimaging.)

Background and Purpose: The extent of white matter hyperintensities (WMH) is associated with cerebral atrophy in elderly people. WMH is a radiological hallmark of cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), but their relationship with brain volume remains poorly understood. The association between WMH and brain volume was analyzed in a large population of patients with CADASIL., Methods: Demographic and MRI data of 278 patients recruited from a prospective cohort study were analyzed. Volumes of WMH and lacunar infarcts, number of cerebral microbleeds, and brain parenchymal fraction were measured. Multivariate analysis was used to study the impact of WMH on brain volume at baseline., Results: In univariate analyses, brain parenchymal fraction was negatively associated with age, male sex, and all MRI markers. Multiple regression modeling showed that brain parenchymal fraction was inversely related to age, number of cerebral microbleeds, and normalized volume of lacunar infarcts but positively related to normalized volume of WMH (P<0.001). This positive relationship was independent of the presence/absence of lacunar infarcts or of cerebral microbleeds. Subgroup analysis showed that this association was significant in subjects having normalized volume of WMH ≥6.13 or brain parenchymal fraction ≥86.37% (median values, both P≤0.001)., Conclusions: The results of the present study suggest that extensive WMH may be associated with increase of brain volume in CADASIL. In this disorder, WMH may be related not only to loss of white matter components, but also to a global increase of water content in the cerebral tissue.

CADASIL is an arteriopathy caused by mutations of the Notch3 gene. White matter hyperintensities (WMH), lacunar lesions (LL), cerebral microhemorrhages (CM), brain atrophy and tissue microstructural changes are detected on MRI. Using an integrated multi-modal approach, we examined the relative impact of lesion burden and location of these MRI markers on cognitive impairment and disability. Multi-modal imaging was performed on 147 patients from a two-center cohort study. Volume of LL, WMH and number of CM was determined. Whole brain mean apparent diffusion coefficient (mean-ADC) and brain parenchymal fraction (BPF) were measured. In multivariate models accounting for lesion burden and location, volume of LL, mean-ADC, and BPF each had an independent influence on global cognitive function and disability. BPF explained the largest portion of the variation in cognitive and disability scores (35-38%). Brain atrophy has the strongest independent influence on clinical impairment in CADASIL when all MRI markers in the disease are considered together. The results suggest that the clinical impact of cerebral tissue loss plays a principal role in this genetic model of subcortical ischemic vascular dementia., (Copyright 2008 Elsevier Inc. All rights reserved.)

Background and Purpose: Three-dimensional MRI segmentation may be useful to better understand the physiopathology of lacunar infarctions. Using this technique, the distribution of lacunar infarctions volumes has been recently reported in patients with cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Whether the volume of each lacune (individual lacunar volume [ILV]) is associated with the patients' other MRI lesions or vascular risk factors has never been investigated. The purpose of this study was to study the impact of age, vascular risk factors, and MRI markers on the ILV in a large cohort of patients with CADASIL., Methods: Of 113 patients with CADASIL, 1568 lacunes were detected and ILV was estimated after automatic segmentation on 3-dimensional T1-weighted imaging. Relationships between ILV and age, blood pressure, cholesterol, diabetes, white matter hyperintensities load, number of cerebral microbleeds, apparent diffusion coefficient, brain parenchymal fraction, and mean and median of distribution of lacunes volumes at the patient level were investigated. We used random effect models to take into account intraindividual correlations., Results: The ILV varied from 4.28 to 1619 mm(3). ILV was not significantly correlated with age, vascular risk factors, or different MRI markers (white matter hyperintensity volume, cerebral microbleed number, mean apparent diffusion coefficient or brain parenchymal fraction). In contrast, ILV was positively correlated with the patients' mean and median of lacunar volume distribution (P=0.0001)., Conclusions: These results suggest that the ILV is not related to the associated cerebral lesions or to vascular risk factors in CADASIL, but that an individual predisposition may explain predominating small or predominating large lacunes among patients. Local anatomic factors or genetic factors may be involved in these variations.

Brain atrophy represents a key marker of disease progression in cerebrovascular disorders. The 3D changes of cortex morphology occurring during the course of small vessel diseases of the brain (SVDB) remain poorly understood. The objective of this study was to assess the changes affecting depth and surface area of cortical sulci and their clinical and radiological correlates in a cohort of patients with cerebral autosomal dominant arteriolopathy with subcortical infarcts and leukoencephalopathy (CADASIL), a genetic SVDB. Data were obtained from a series of 69 CADASIL patients. Validated methods were used to determine depth and surface area of four cortical sulci. The ratio of brain to intracranial cavity volumes (brain parenchymal fraction--BPF), volume of lacunar lesions (LL) and of white matter hyperintensities, number of cerebral microhaemorrhages, and mean apparent diffusion coefficient were also measured. Association between depth and surface area of the cortical sulci and BPF, clinical status and subcortical MRI lesions were tested. Depth and surface area of cortical sulci obtained in 54 patients were strongly correlated with both cognitive score and disability scales. Depth was related to the extent of subcortical lesions, surface area was related only to age. In additional analyses, the depth of the cingular sulcus was independently associated with the volume of LL (P = 0.001), and that of the superior frontal sulcus with the mean apparent diffusion coefficient (P = 0.003). In CADASIL, important morphological changes of cortical sulci occur in association with clinical worsening, extension of subcortical tissue damage and progression of global cerebral atrophy. These results suggest that the examination of cortical morphology may be of high clinical relevance in SVDB.

Objective: We report the initial results of a pilot clinical trial exploring the safety and feasibility of the first real-time magnetic resonance-guided laser-induced thermal therapy of treatment-resistant focal metastatic intracranial tumors., Methods: Patients with resistant metastatic intracranial tumors who had previously undergone chemotherapy, whole-brain radiation therapy, and radiosurgery and who were recused from surgery were eligible for this trial. Under local anesthesia, a Leksell stereotactic head frame was used to insert a water-cooled interstitial fiberoptic laser applicator inside the cranium. In the bore of a magnetic resonance imaging (MRI) scanner, laser energy was delivered to heat the tumor while continuous MRI was performed. A computer workstation extracted temperature-sensitive information to display images of laser heating and computed estimates of the thermal damage zone. Posttreatment MRI scans were used to confirm the zone of thermal necrosis, and follow-up was performed at 7, 15, 30, and 90 days after treatment., Results: In all cases, the procedure was well tolerated without secondary effect, and patients were discharged to home within 14 hours after the procedure. Follow-up imaging showed an acute increase in apparent lesion volume followed by a gradual and steady decrease. No tumor recurrence within thermal ablation zones was noted., Conclusion: In this ongoing trial, a total of four patients have had six metastatic tumors treated with laser thermal ablations. Magnetic resonance-guided laser-induced thermal therapy appears to provide a new, efficient treatment for recurrent focal metastatic brain disease. This therapy is a prelude to the future development of closed-head interventional MRI techniques in neurosurgery.

Background: Transpetrosal approaches are technically complex and require a complete understanding of surgical and radiological anatomy. A careful evaluation of pre-operative magnetic resonance imaging and computed tomography scan is mandatory, because anatomical or pathological variations are common and may increase the risk of complications related with the approach. Methods: Pre-operative characteristics of venous and petrous bone anatomy were analysed and correlated with intraoperative findings, using injected magnetic resonance imaging and thin-slices computed tomography scan. These data regularly checked before each transpetrosal approach were progressively included in the presented checklist. Results: Transpetrosal approaches have been used in 101 patients. Items included in the checklist were petrous bone pneumatization, angle between petrous apex and clivus, dehiscence of petrous carotid artery, dehiscence of geniculate ganglion, distance between superior semicircular canal and middle fossa floor, distance between cochlea and middle fossa floor, sigmoid sinus dominance, transverse sigmoid sinus junction depth to the outer cortical bone, jugular bulb height (high or low), location of the vein of Labbé, characteristics of superior petrosal vein complex. Conclusion: The presented checklist provides a systematic scheme of consultation of characteristic of venous and petrous bone anatomy for transpetrosal approaches. In our experience, the use of this checklist reduces the risk of complications related with approach, by minimizing the neglect of crucial information. [ABSTRACT FROM AUTHOR]

Purpose: Patients with superior semicircular canal dehiscence syndrome, which can only be treated by surgery, present cochleo-vestibular symptoms related to a third-mobile window but also endolymphatic hydrops. Since cVEMP and oVEMP are disturbed by the presence of the dehiscence, the aim of the study is to assess the value of MRI for the diagnosis of endolymphatic hydrops in patients with superior semicircular canal dehiscence syndrome in comparison with cVEMP and oVEMP. Methods: In this retrospective cohort study we enrolled 33 ears in 24 patients with superior semicircular dehiscence syndrome who underwent a 4-h delayed intravenous Gd-enhanced 3D-FLAIR MRI and pure tone audiometry, cVEMP and oVEMP. For each patient MRI images were evaluated by two radiologists who used a compartmental endolymphatic hydrops grading system in comparison with cVEMP and oVEMP. Results: Endolymphatic hydrops was found on MRI in 9 out of 33 SCDS ears (27.3%). We found no significant correlation between the presence of endolymphatic hydrops on MRI and cVEMP and oVEMP (p = 0.36 and p = 0.7, respectively). However, there was a significant correlation between the presence of endolymphatic hydrops on MRI and the degree of sensorineural hearing loss, Air Conduction-Pure Tone Average level (p = 0.012) and Bone Conduction-Pure Tone Average level (p = 0.09), respectively. Conclusion: We demonstrated that EH might be observed in 27.3% of superior semicircular dehiscence syndrome ears. The role of inner ear MRI is important to detect endolymphatic hydrops, since cVEMP and oVEMP are disturbed by the presence of the dehiscence, because these patients could benefit from a medical treatment. Level of evidence: Level 3. [ABSTRACT FROM AUTHOR]

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Background Intraosseous arachnoid cysts are rare and difficult to diagnose. The purpose of this study was to describe the clinical and radiological semiology of petrous and sphenoid arachnoid cysts and to propose a specific management strategy. Methods This was a retrospective, descriptive study of patients with arachnoid cysts, which utilized CT, MRI, and the patients' medical histories. Results Ten patients were included in this study. On CT, the lesions were lytic with bony delineation. On MRI, the lesions exhibited the same signals as cerebrospinal fluid and were not enhanced after contrast. On fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted sequences, the arachnoid cysts' signal disappeared, which is a key feature for eliminating the diagnosis of cholesteatoma. Two patients underwent surgery because of misdiagnosis, either with a meningocele or a cholesteatoma. Conclusion MRI FLAIR and diffusion-weighted sequences, together with osseous CT scans, help to distinguish arachnoid cysts from meningoceles and avoid unnecessary surgeries with potential complications. © 2014 Wiley Periodicals, Inc. Head Neck 37: 823-828, 2015 [ABSTRACT FROM AUTHOR]

Diffusion tensor imaging (DTI) histogram metrics are correlated with clinical parameters in cerebral small vessel diseases (cSVD). Whether ADC histogram parameters derived from simple diffusion weighted imaging (DWI) can provide relevant markers for long term studies of cSVD remains unknown. CADASIL patients were evaluated by DWI and DTI in a large cohort study overa6-year period. ADC histogram parameters were compared to those derived from mean diffusivity (MD) histograms in 280 patients using intra-class correlation and Bland-Altman plots. Impact of image corrections applied to ADC maps was assessed and a mixed effect model was used for analyzing the effects of scanner upgrades. The results showed that ADC histogram parameters are strongly correlated to MD histogram parameters and that image corrections have only limited influence on these results. Unexpectedly, scanner upgrades were found to have major effects on diffusion measures with DWI or DTI that can be even larger than those related to patients’ characteristics. These data support that ADC histograms from daily used DWI can provide relevant parameters for assessing cSVD, but the variability related to scanner upgrades as regularly performed in clinical centers should be determined precisely for longitudinal and multicentric studies using diffusion MRI in cSVD. [ABSTRACT FROM AUTHOR]

Background and Aim: Dilated perivascular spaces (dPVS) have previously been associated with aging and hypertension-related cerebral microangiopathy. However, their risk factors, radiological features and clinical relevance have been poorly evaluated in CADASIL (cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy), a unique model to investigate the pathophysiology of ischemic small-vessel disease. The purpose of this study was to investigate these different aspects in a large cohort of patients with this disorder. Methods: Demographic and MRI data of 344 patients from a prospective cohort study were analyzed. The severity of dPVS was evaluated separately in the anterior temporal lobes, subinsular areas, basal ganglia and white matter, using validated semiquantitative scales. Logistic and multiple linear regression models were used to determine the risk factors associated with the severity of dPVS in these different regions and their relationships with cognition, disability and the MRI markers of the disease (white matter hyperintensities (WMH) lacunar infarcts, microbleeds and brain parenchymal fraction (BPF)). Results: The severity of dPVS was found to increase with age regardless of cerebral area (p < 0.001). In contrast with dPVS in other locations, the severity of dPVS in the temporal lobes or subinsular areas was also found strongly and specifically related to the extent of WMH (p < 0.001). Conversely, no significant association was detected with lacunar volume, number of microbleeds or BPF. A high degree of dPVS in the white matter was associated with lower cognitive performances independently of age and other MRI markers of the disease including BPF (p ≤ 0.04). Conclusions: In CADASIL, the progression of the hereditary microangiopathy with aging may promote the dilation of perivascular spaces throughout the whole brain but with variable extent according to cerebral location. In temporal lobes and subinsular areas, dPVS are common MRI features and may share a similar pathogenesis with the extension of WMH during the course of the disease. dPVS may also participate in the development of cognitive decline in this model of small-vessel disease, and their large number in white matter may alert clinicians to a higher risk of cognitive decline in CADASIL. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background: CADASIL is responsible for diffuse hyperintensities in the white matter on FLAIR images. These lesions are often associated with focal lesions in the basal ganglia such as lacunar infarctions. The prevalence and significance of diffuse or confluent thalamic hyperintensities (CTH) remain unknown. Methods: The frequency of hyperintensities on FLAIR images in the thalamus was assessed in 147 CADASIL patients, and signal abnormalities on both FLAIR and T1-weighted images were categorized as focal/punctuate or diffuse/confluent by the same reader. The areas of increased diffusion were also analyzed on apparent diffusion coefficient maps. The association of CTH with vascular risk factors, the main clinical manifestations of the disease and MRI markers (brain parenchymal fraction, volume of white matter hyperintensities, volume of lacunar infarcts and number of microbleeds) was analyzed with generalized linear regression models. Results: CTH were detected in 12% of the CADASIL subjects in association with hypointensities on T1-weighted images. CTH corresponded to areas of increased diffusion on apparent diffusion coefficient maps. CTH were found significantly associated with age and independently related to the volume of white matter hyperintensities but not to that of lacunar infarctions or to cerebral atrophy after adjustment for age and sex. No significant association was found between CTH and global cognitive performances. Conclusion: CTH are observed on FLAIR images in a sizeable proportion of CADASIL patients. They are mainly related to the extent of white matter hyperintensities and do not correlate with cognitive decline. Demyelination and/or loss of glial cells appear to be the most plausible cause of these confluent signal changes in the thalamus. Copyright © 2010 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Simple Summary: Pretreatment assessment of local extension in sinonasal cancer is essential for prognostic evaluation and surgical planning. It essentially relies on CT and MRI imaging whose performance is not accurately described in the scientific literature. The aim of this study was to assess the diagnostic performance of CT and MRI for the diagnosis of skull base and orbital invasion in sinonasal cancer by comparing imaging findings to histopathological data. A total of 176 patients were included. Objective data about the diagnostic value of pretreatment imaging in patients with sinonasal cancer were obtained: they suggest that pretreatment assessment of orbital invasion is difficult, even with the combination of CT and MRI. Background: Pretreatment assessment of local extension in sinonasal cancer is essential for prognostic evaluation and surgical planning. The aim of this study was to assess the diagnostic performance of two common imaging techniques (CT and MRI) for the diagnosis of skull base and orbital invasion by comparing imaging findings to histopathological data. Methods: This was a retrospective two-center study including patients with sinonasal cancer involving the skull base and/or the orbit operated on between 2000 and 2019. Patients were included only if pre-operative CT and/or MRI, operative and histopathologic reports were available. A double prospective blinded imaging review was conducted according to predefined radiological parameters. Radiologic tumor extension was compared to histopathological reports, which were considered the gold standard. The predictive positive value (PPV) for the diagnosis of skull base/orbital invasion was calculated for each parameter. Results: A total of 176 patients were included. Ethmoidal intestinal-type adenocarcinoma was the most common type of cancer (41%). The PPV for major modification of the bony skull base was 78% on the CT scan, and 89% on MRI. MRI signs of dural invasion with the highest PPVs were: contact angle over 45° between tumor and dura (86%), irregular deformation of dura adjacent to tumor (87%) and nodular dural enhancement over 2 mm in thickness (87%). Signs of orbital invasion had low PPVs (<50%). Conclusions: This retrospective study provides objective data about the diagnostic value of pretreatment imaging in patients with sinonasal cancer. [ABSTRACT FROM AUTHOR]

This is an answer to the Letter to the Editor regarding our previously published article « Saccular measurements in routine MRI can predict hydrops in Menière's disease ». We thank the authors for their interest in our article and their insightful comments. We would like to emphasise that our article was a preliminary study and to our knowledge the first published series establishing a correlation between measurements of saccular morphology in T2-weighed 3D CISS images and clinical definite Menière's disease. Therefore, we agree with the authors of the Letter that verifying reproducibility is paramount for the technique to be widely used. Further studies should be conducted to investigate the risk of artefacts reducing the reliability of saccular width measurements and to confirm the clinical implications. We recommend the use of saccular height measurements which have higher reliability. Also, the goal of the study was to present a possible radiological alternative to the more established methods of endolymphatic hydrops visualisation. If accessible, we agree that the latter techniques should be preferred, but we find that they are unfortunately too often unavailable in routine clinical practice to ENT physicians. [ABSTRACT FROM AUTHOR]