A case of spontaneous intracranial hypotension: from Ménière-like syndrome to cerebral involvement.

INTRODUCTION: Spontaneous intracranial hypotension (SIH) is a rare pathology caused by unexplained and variably localized leakage of cerebrospinal fluid (CSF). The prime symptom is orthostatic headache, although other less specific clinical signs may predominate, and mislead diagnosis. CASE REPORT: A 47-year-old man presented with Ménière-like symptoms of sudden onset. Secondary orthostatic headache led to the performance of cerebral MRI, which found signs suggestive of intracranial hypotension. A blood-patch was immediately carried out, and was followed by consciousness disorder associated with onset of bilateral subdural hematoma, which required iterative neurosurgical drainage. Myelo-CT confirmed CSF leakage facing the right 12th dorsal nerve root sheath. Radio-guided sealing with biologic glue provided complete regression of all symptoms. DISCUSSION/CONCLUSION: Auditory signs may predominate in the clinical presentation of SIH. Their orthostatic character is suggestive. The present case is of a rare severe form. The role of neurosurgery in such cases remains to be defined.

[Sarcoidosis of the upper respiratory tract]. / Sarcoïdose des voies aérodigestives supérieures.

INTRODUCTION: Sarcoidosis is a non-caseating granulomatous disease of unknown origin, principally affecting the respiratory tract. BACKGROUND: Sarcoidosis of the upper respiratory tract (SURT) includes sino-nasal sarcoidosis (SNS) and pharyngo-laryngeal sarcoidosis (PLS). SURT may be isolated or, more often, part of multisystemic sarcoidosis. Its clinical symptomatology is protean and non specific. The natural history, course and prognosis are poorly understood and unpredictable. The treatment has not yet been standardised and the long-term therapeutic results are often disappointing. VIEWPOINT: In this work, we try to make a synthesis of our experience and publications, and the data in the existing international literature, to improve the diagnosis and therapeutic management of SURT. The usefulness of both morphological and functional imaging techniques, in particular 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET/CT), is evaluated for use in the management of the severe phenotypes of sarcoidosis such as SURT. CONCLUSIONS: Even if guided biopsy remains necessary for confirmation of SURT, medical imaging plays an important role in the management of this disease: CT imaging allows the description of SNS and classification into two stages that correlate well with the severity, reversibility and course of the sino-nasal involvement, 18F-FDG PET/CT, providing a complete morpho-functional mapping of active inflammatory lesions, could be a useful technique in patients with biopsy-proven SURT, for both diagnosis and follow up of medical treatment.

Interest of diffusion-weighted and gadolinium-enhanced dynamic MR sequences for the diagnosis of parotid gland tumors.

PURPOSE: This study aimed to evaluate the value of diffusion-weighted imaging (DWI) and gadolinium-enhanced dynamic magnetic resonance imaging (MRI) for differentiating benign and malignant parotid gland tumors, and for characterizing the various histological types (pleomorphic adenoma, and Warthin's and malignant tumors). PATIENTS AND METHODS: This retrospective study involved 60 patients with suspected parotid gland tumors (mean age: 59.4 years), and was carried out from April 2005 to February 2008. All had undergone pathological examination. All MRI examinations were performed using the Siemens Magnetom Avanto 1.5T MRI system. Non-enhanced T1-weighted (T1W), gadolinium-enhanced fat-suppressed T1W and T2-weighted (T2W) images were obtained for all 60 patients, with diffusion-weighted echoplanar imaging (DW-EPI) and apparent diffusion coefficient (ADC) evaluation in 59 patients, and gadolinium-enhanced dynamic MRI sequences in 51 patients. Interpretation was carried out by two experienced radiologists (the first evaluation used T1W, gadolinium-enhanced fat-suppressed T1W and T2W images; the second evaluation used T1W, T2W, DWI and dynamic MRI) and, for each case, the benign/malignant nature of the tumor and its histological type were determined. RESULTS: After the second reading, increases were noted in sensitivity, specificity, malignant positive predictive value (PPV) and negative predictive value (NPV), as well as in accuracy (90-100% for the first observer, and 90-97% for the second observer). Interobserver reliability also showed a significant increase from the first to the second reading (kappa=0.63 to 0.87, respectively). CONCLUSION: Gadolinium-enhanced dynamic MRI and DW-EPI with ADC evaluation improved the performance of MRI in distinguishing between benign and malignant parotid gland tumors, and characterizing the different histological types of benign tumors (pleomorphic adenoma and Warthin's), thus leading to greater consensus in interpretation of the images.

Lateral semicircular canal plugging: a simple and effective surgical treatment against incapacitating Menière's disease.

OBJECTIVES: To present lateral semicircular canal plugging (LCP) technique and late results dedicated to patients with incapacitating Menière's disease. METHOD: In a prospective case-controlled baseline study, patients with unilateral incapacitating Menière's disease were treated either by LCP (n = 11) or by vestibular neurotomy (VN) (n = 11). LCP was performed by retro auricular approach, vestibular neurotomy by retro sigmoid approach. According to Menière's disease therapeutic evaluation guidelines, disability and hearing loss were assessed before and after both surgical treatments, with a 2 years followup at least. After LCP, a CT and MRI scan analyse was performed on the inner ear RESULTS: LCP was effective (A or B class) in 82% of cases (n = 9/11), 91% after VN (n = 10/11). When treatment was effective, 100% of patients regained a normal life after LCP (postoperative functional level I or 2, n = 9/9), versus 50% after VN (n = 5/10). After LCP, postoperative hearing level was unchanged in 82% of cases, n = 9/11 (73% after VN, n = 8/11), and decreased of about 30 dB in 18% (n = 2/11). There was no surgical complication. The endolymph interruption area was well visualized on MRI, allowing determining the optimal plugging area to be as far as possible from the ampulla. CONCLUSION: LCP is a simple and safe new treatment that could be very useful to control vertigo in Menière's disease. Early and late tolerance are excellent. This prospective study will go on to evaluate the potential of this innovating treatment: LCP could be recommended as an alternative to VN or chemical labyrinthectomy, except in case of drop attacks.

[Reconstruction of the anterior mandibule using a porous titanium implant: a case report]. / Reconstruction de la symphyse mandibulaire par implant de titane poreux: à propos d'un cas.

OBJECTIVE: Anterior mandibular arch reconstruction. PATIENT AND METHOD: A 55-year-old immuno-depressed female underwent resection of the lower third of the face subsequent to extensive mucormycosis-related necrosis. Reconstruction of the anterior part of the mandible and adjacent soft tissue was carried out with a mandibular prosthesis and a latissimus dorsi flap. The mandibular prosthesis was made of titanium T40 micro-beads, consolidated by two parallel plates of titanium. The porous structure is intended to enhance cellular and bone integration. RESULTS: The tolerance of the prosthesis was still excellent after 36 months. Labial continence was restored in a second procedure. Unrestricted diet was allowed from the third month. Radiological investigations confirm the good stability of the prosthesis. CONCLUSION: This original prosthesis design offers an alternative to the reconstruction of an anterior mandibular arch by plate or by vascularised free osseous tissue transfer that is sometimes associated with significant morbidity.

[Recovery of saccular function after vestibular rehabilitation]. / Récupération de la fonction sacculaire après rééducation vestibulaire.

OBJECTIVE: The authors present a case of recovery of saccular function after vestibular rehabilitation. MATERIAL AND METHOD: Vestibular rehabilitation is proposed to a patient presenting a left lateral body deviation as well as postural disorders at the time of the vertical movements and in car, associated with an unilateral abolition of vestibular evoked myogenic potentials (VEMP). RESULTS: We observe a recovery of VEMP and a disappearance of symptomatology after vestibular rehabilitation. CONCLUSION: A loss of saccular function can involve a postural disorder in some circumstances. The best treatment is vestibular rehabilitation. We insist on the need for knowing otolithic semiology in order to propose suitable explorations and therapy.

[Primary ciliary dyskinesia. Clinical presentation and diagnosis]. / La dyskinésie ciliaire primitive. Etude clinique et diagnostic.

UNLABELLED: Primary ciliary dyskinesia (PCD) is a genetic disease characterized by abnormalities in ciliary structure/function. OBJECTIVE: We analyzed the main clinical features and test results of PCD in order to evaluate their usefulness for diagnosis. PATIENTS AND METHODS: Retrospective study of 35 cases of PCD evaluated by the same team, with nasal brushings in all cases (special light microscopy) and electron microscopy and/or by isotopic mucociliary clearance study in some. RESULTS: In a cohort of 145 patients with suspected PCD, the diagnosis of PCD was established in 35 cases using a combination of compatible clinical features coupled with the study of nasal brushings: 13 females and 22 males, average age at time of diagnosis 25 years, situs inversus in 12 patients (34%). CONCLUSION: In the absence of consensus in the literature for diagnosis of PCD, we propose the association of the following diagnostic criteria: upper airway and bronchopulmonary infections beginning often early in the life, more inconstantly situs inversus, familial cases of PCD, consanguinity, infertility and permanent and ubiquitous abnormalities of ciliary structure/function. Nasal brushing with ciliary study (special light microscopy) seems to be an easy and reliable diagnostic criterion. Electron microscopy is necessary for proving ultrastructural abnormalities.

[The respiratory mucociliary system and its exploration in primary ciliary dyskinesia]. / Le système mucociliaire respiratoire et son exploration dans le cadre de la dyskinésie ciliaire primitive.

BACKGROUND: We analyzed the main characteristic features of the respiratory epithelium mucociliary system and the different tests of ciliary beat and mucociliary transport (mucociliary clearance). This knowledge is necessary for an often interdisciplinary diagnosis and treatment of primary ciliary dyskinesia. METHODS: Review of the literature and personal experience of the different tests of ciliary structure and function. RESULTS: This disease is characterized by abnormalities in ciliary structure/function. The genetic mechanisms and the ultrastructural abnormalities that are involved are heterogenous compared to the relative homogeneity of the clinical presentation. CONCLUSION: The diagnostic criteria are compatible clinical features (chronic upper airway and bronchopulmonary infections, situs inversus...) coupled with tests of ciliary structure and function.

[Surgical treatment of malignant sinonasal tumors with orbital extension: 20 patients]. / Traitement des tumeurs malignes des cavités nasosinusiennes à extension orbitaire. A propos de 20 patients.

OBJECTIVE: The purpose of this work was to assess changes in surgical treatment of malignant sinonasal tumors with orbital extension, with or without orbital exenteration, on the basis of clinical, radiological and preoperative data. MATERIAL AND METHODS: We conducted a retrospective analysis of twenty patients with malignant sinonasal tumors with orbital extension operated on from 1995 to 2002 in the ENT and head and neck department of the Strasbourg University Hospital. CT-scan and MRI were performed before surgery. When periorbital invasion was observed without orbital extension and confirmed by histological examination in the operative theatre, the orbit was preserved during the surgical procedure. Orbital exenteration was necessary for the other patients. Adjunct postoperative radiotherapy was performed in all patients (n=20). Clinical, radiological and surgical data were compared with patient follow-up to search for correlations between radiological signs and the difficult surgical decision for orbital exenteration. RESULTS: For orbital extension, the histological examination performed in the operative theatre did not confirm the results of the preoperative CT-scan in 15% of patients and the preoperative MRI in 11%. In a first group of nine patients with bony wall extension (78%, 7/9) or periorbital extension (22%, 2/9), there were no cases of recurrence at six years follow-up. In a second group of eleven patients with intraorbital extension, orbital exenteration was not performed in two (18%) because of surgically unresectable massive extension. Four of the nine other patients who underwent orbital exenteration were free of local recurrence at ten months follow-up and five were considered to have achieved oncological cure at four years follow-up. CONCLUSION: Preoperative CT and MRI are essential but the decision for orbital exenteration must be made on the basis of the histological examination performed during the operative procedure because imaging often underestimates tumor extension.

Treatment of acute mastoiditis: report of 31 cases over a ten year period.

OBJECTIVE: With possible extracranial and intracranial complications, acute mastoiditis is the leading complication of acute otitis media (AOM). The goal of this study is to assess the clinical features, pathogens, complications and especially management of acute mastoiditis in the ENT service, University Hospital of Strasbourg, France. METHODS: Systematic review of all medical records of patients who were admitted with acute mastoiditis from January 1993 to April 2003. RESULTS: 31 patients, 18 male (58%) and 13 female (42%) fulfilled inclusion criteria. The average age was 16, going from 6 months to 70 years, with 55% between 0 to 5 years. Most common symptom was otalgia (84%), 58% of patients had history of past AOM and 61% were under antibiotic therapy during admission. Twenty-three patients (74%) presented retroauricular swelling and erythema. 18 (58%) had a displaced pinna. Cultures taken from pus isolated Streptococcus pneumoniae in 12 cases (38.7%), Pseudomonas aeruginosa in 2 cases (6.4%), Streptococcus beta-haemolyticuis 1 case, Staphylococcus coagulase-positive 1 case and Mycobacterium tuberculosis hominis 1 case (3%). Complications of acute mastoiditis occurred in 3 cases (10%): Meningitis 2 cases and facial nerve paralysis 1 case. Surgery therapy was periformed in 84% of cases (mastoidectomy only or in combination with myringotomy with tube insertion) and medical therapy only in 16% of cases. CONCLUSION: Despite use of antibiotics, acute mastoiditis remains still a threat for patients with AOM, especially for children under 5 years of age. Great care is required from clinicians to make an early diagnosis in order to promote adequate management and prevent complications.

Bilateral latero-pontine infarction: a rare syndrome, an unusual imaging.

The authors present one case of bilateral infarction of the brain stem, in the territory of the antero-inferior cerebellar artery. Besides the fact that such bilateral ischaemic lesions are rare, the initial clinical presentation mimicking a vestibular neuronitis indicates that this latero-pontine syndrome may appear as a peripheral disorder. It also reminds clinicians that careful and repeated examinations should always be performed in all patients suffering from vertigo. An unusual iconography is also presented.

[Allergic fungal sinusitis: ten cases]. / La Sinusite Fongique Allergique (SFA). A propos de 10 cas.

OBJECTIVES: Diagnosis, nosological individualization, and treatment of allergic fungal sinusitis remain difficult and controversial despite the increasing number of publications. We present ten cases of allergic fungal sinusitis and review the literature to highlight the main clinical, radiological, biological, immunoallergological, mycological, and therapeutics features. MATERIAL AND METHODS: This retrospective study included ten patients (six men and four women, mean age 45 years) with allergic fungal sinusitis diagnosed on the basis of all diagnostic criteria reported in the literature. RESULTS: Six patients had isolated allergic fungal sinusitis which was associated with allergic bronchopulmonary aspergillosis in the four others. Treatment combined endoscopic sinus surgery and corticosteroids, which provided good results in six patients and average results in three. Treatment failure was observed in one patient. CONCLUSION: As in the case of allergic bronchopulmonary aspergillosis, a set of clinical, radiological, histopathological, immunoallergological and mycological criteria is necessary for precise diagnosis and to avoid fungal drift. The most appropriate endoscopic sinus surgery and the best corticosteroid regimen remain to be determined.

[ENT Resident Activity in the Strasbourg Hospital ENT Emergency Clinic]. / Activité de l'interne d'ORL durant la garde au CHU de Strasbourg.

OBJECTIVES: We present an original study about ENT resident activity in an emergency ENT clinic. MATERIALS AND METHODS: The emergency unit's activity was analyzed over a seven-Month period from December 1, 1999 to June 30, 2000. ENT residents reported patient age, gender, duration of consultation, symptoms, and care delivered as well as the level of emergency and referral (general practitioner, emergency unit, other clinic, direct). RESULTS: A total of 147 emergency days were analyzed. During the study period, 1237 patients were examined, representing 8.4 patients per day and 26.5% of the ENT clinic outpatient activity. Mean patient age was 31.8 Years; 58% of the patient were male. Most of the cases did not correspond to true emergencies (53%). Half of the patients were not referred by a general practitioner. Emergency care was more justified when the patients were referred by a general practitioner or another emergency unit. The predominant pathological situations managed were acute external and middle ear otitis, epistaxis, angina, vertigo, and facial injuries. CONCLUSION: Over half of the patients came to the emergency clinic spontaneously, presenting minor problems. These data can be explained by the fact that residents are permanently present at the ENT emergency clinic. It would be interesting to conduct a similar study in ENT emergency care units where the resident is not permanently present.

[Nasosinusal sarcoidosis: 13 cases]. / La sarcoïdose nasosinusienne. A propos de 13 cas.

We report 13 cases of nasosinusal sarcoidosis. Sarcoidosis is a chronic, non caseating granulomatous disease. Nasosinusal involment is rare or exceptional and may be isolated (1 case in our series) or associated (12 cases in our series) with other lesions of the chest, skin, liver, spleen, bone, eyes, salivary glands, peripheral lymph nodes or with neurosarcoidosis. The clinical and CT features are various and often non specific. Nasal biopsy guided by physical examination is easy and constitute the main diagnostic criterion. The course of nasosinusal sarcoidosis is variable and no standard treatment has been established. Response to local or systemic corticosteroid therapy is also variable. The recurrence is frequent after tapering off or interrupting corticosteroids that also have important side effects.

[Surgical treatment for squamous-cell carcinoma of the posterior pharyngeal wall: reconstruction with a free forearm flap]. / Traitement chirurgical des carcinomes épidermoïdes de la paroi pharyngée postérieure: reconstruction par un transplant antébrachial à pédicule radial.

Squamous-cell carcinoma of the posterior pharyngeal wall is a rare finding. Surgical treatment with preservation of laryngeal function is usually associated with relatively poor functional outcome. We report the cases of 5 patients treated by pharyngectomy with preservation of the laryngeal function. The posterior pharyngeal wall was reconstructed with a free forearm flap. This reconstruction method provided satisfactory functional results. Swallowing disorders resolved within 2 to 6 weeks following the reintroduction of oral food intake. Mean follow-up of nearly 3 years did not evidence any local tumor recurrence.

Intra-laryngeal endoprosthesis: an alternative therapeutic approach to surgical procedures of laryngeal exclusion.

The uncertain results of aryepiglottopexy in our personal experience led us to develop, for patients with aspirations associated with the risk of severe pneumonitis, three types of intralaryngeal endoprostheses (ILEP) under the systematic cover of a tracheostomy. From September 1997 to May 1999, seven protheses were implanted in six patients. It was not possible to restore the full range of laryngeal functions, i.e. deglutition, phonation and respiration. However, our results, that were obtained with an intracricoidal prothesis closed at its upper end or equipped with a phonatory valve, are quite encouraging, even more so when in association with early alimentary re-education and support continuation of our trial. These ILEPs are simple to place, well tolerated, efficacious in preventing deglutition pneumonitis and easy to remove. Furthermore, the reversibility of this bloodless procedure facilitates the monitoring of a possible recovery of the laryngeal functions.

Pseudorandom rotational stimuli of the vestibulo-ocular reflex in humans: normal values of the transfer function.

Pseudorandom binary rotatory stimulation is used to study the vestibulo-ocular reflex (VeOR) frequency response (from 0.01 to 0.64 Hz). Fifty-two normal subjects volunteered for this test. The transfer function's gain and phase, and the corresponding coherence function between slow phase eye velocity and head velocity were computed. According to the linearity hypothesis, the results match the sinusoidal stimulation studies. In both cases, the variability of the gain was higher than that of the phase shift. Identification of some parameters of the VeOR's mathematical model were performed, using the experimental results.

[Caseous sinusitis. Clinical, x-ray computed, surgical, histopathological, biological, biochemical and myco-bacteriological aspects. Apropos of 33 cases]. / Les sinusites caséeuses aspects cliniques, TDM, opératoires, anatomopathologiques, biologiques, biochimiques et myco-bactériologiques. A propos de 33 cas.

We analyse the most important clinical, CT, surgical, histopathological, biological, biochemical, mycological and bacteriological features of caseous sinusitis, i.e. chronic, poorly symptomatic sinusitis which are resistant to usual treatment. CT shows an opacity of the maxillary sinus with often a hyperdense foreign body (dental overfilling) but no valid predictive criteria for fungal etiology. The endoscopic or surgical procedures point out a caseous mass often called fungal mass or aspergilloma. The biochemical composition of this mass (water, proteins, lipids) is similar to that of tissues surrounding the sinus. The calcium value is variable and shows no correlation with CT imaging. The qualitative and quantitative analysis of the metals (zinc, lead, silver, copper, iron) is of relevance for exogenic origin (dental overfilling). The fungal etiology is inconstant (20 cases) after the mycological investigations. We discuss the nosologic and diagnostic features of caseous sinusitis, fungal or not, and which are often or too often called aspergillosis sinusitis.

[Rotatory impulse test does not replace caloric tests]. / L'épreuve rotatoire impulsionnelle ne remplace pas les tests caloriques.

We sought to determine whether the rotatory impulsional test was capable of exploring the canalar function with sufficient precision to replace the caloric test, as it has been recently affirmed. We first compared the observed preponderance from this test with that measured during a sinusoidal (20 and 4 s) test. We observed that, in the case of a significant preponderance for a given test, there was complete redondance with the preponderance observed with any other test. The rotatory impulsional test does not present any specific advantage compared to other kinetic test as far as the observation of the preponderance phenomenon. We then compared the preponderance with the results of the caloric tests and came to the following conclusions i) the absence of preponderance does not allow us to predict the absence of vestibular deficit, due to the fact that 37% of the deficits were compensated for including acoustic neuroma; ii) the presence of a preponderance does not allow a priori to say whether it is of vestibular, cervical, or central origin and systematic caloric tests shows that almost one fourth of preponderance observed is not associated with unilateral weakness iii) supposing that a clinical argument allow us to conclude as to the probable vestibular origin of a vestibular preponderance, the direction of this preponderance does not allow us to determine which side is involved. In fact, if the undercompensated deficits are 3 times more frequent than overcompensated deficits, the proportion of preponderance not linked to a significant deficit indicates that the probability of encountering a preponderance related to a specific undercompensated deficit is approximately 50%. We thus did not find in the rotatory impulsional test any specific advantage allowing us to predict the laterality of a vestibular lesion.