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Fibrous dysplasia of bone is a benign, uncommon, sporadic, congenital skeletal disorder resulting in deformity. This disease arises from activating somatic mutation in GNAS which encodes the α subunit of the G stimulatory protein associated with proliferation of undifferentiated osteogenic cells resulting in marrow fibrosis, abnormal matrix production, and stimulation of osteoclastic resorption upon overproduction of IL-6 observed in dysplastic cells. Fibrous dysplasia may be monostotic or polyostotic. This mutation affecting many tissues, cafe au lait skin macules and endocrinopathies (precocious puberty, hyperthyroidism, growth hormone excess, Cushing syndrome) may be associated in McCune-Albright syndrome, but also myxoma in Mazabraud syndrome or phosphate diabetes. Diagnosis of craniofacial fibrous dysplasia should be considered in the presence of headache, neuralgia, sensory disorders (vision, hearing, balance, smelling), functional disorders (nasal obstruction, nasolacrimal duct obstruction, non-matching occlusion), infectious complications (sinusitis, otitis, mastoiditis). Such symptoms should lead to perform craniofacial CT scan completed with MRI. Bone biopsy is not systematic. Surgical treatment is discussed in cases of nervous complication, facial deformity or active lesions. In case of pain resistant to conventional analgesics, intravenous bisphosphonates can be proposed. In non-responder patients, several case reports suggest the efficacy of a monoclonal antibody directed against the IL-6 receptor which requires to be confirmed by randomized studies.

Fibrous dysplasia of bone is a benign, uncommon, sporadic, congenital skeletal disorder resulting in deformity. This disease arises from activating somatic mutation in GNAS which encodes the α subunit of the G stimulatory protein associated with proliferation of undifferentiated osteogenic cells resulting in marrow fibrosis, abnormal matrix production, and stimulation of osteoclastic resorption upon overproduction of IL-6 observed in dysplastic cells. Fibrous dysplasia may be monostotic or polyostotic. This mutation affecting many tissues, café au lait skin macules and endocrinopathies (precocious puberty, hyperthyroidism, growth hormone excess, Cushing syndrome) may be associated in McCune-Albright syndrome, but also myxoma in Mazabraud syndrome or phosphate diabetes. Diagnosis of craniofacial fibrous dysplasia should be considered in the presence of headache, neuralgia, sensory disorders (vision, hearing, balance, smelling), functional disorders (nasal obstruction, nasolacrimal duct obstruction, non-matching occlusion), infectious complications (sinusitis, otitis, mastoiditis). Such symptoms should lead to perform craniofacial CT scan completed with MRI. Bone biopsy is not systematic. Surgical treatment is discussed in cases of nervous complication, facial deformity or active lesions. In case of pain resistant to conventional analgesics, intravenous bisphosphonates can be proposed. In non-responder patients, several case reports suggest the efficacy of a monoclonal antibody directed against the IL-6 receptor which requires to be confirmed by randomized studies.

Resume Propos. – Les surdites brusques idiopathiques sont des surdites neurosensorielles sans cause declenchante reconnue au moment de leur installation. L’atteinte neurosensorielle est habituellement cochleaire, mais certaines lesions retrocochleaires (tumorales de l’angle pontocerebelleux, degeneratives, ou ischemiques du nevraxe) peuvent se reveler ainsi. La prise en charge vise a rechercher une cause et a instaurer un traitement en urgence. Lorsqu’aucune cause n’est trouvee, on pose le diagnostic de surdite brusque idiopathique. Habituellement l’atteinte est cochleaire. La physiopathologie de cette atteinte sensorielle est encore inconnue. Il est tres probable que plusieurs causes soient possibles, leur point commun etant une alteration de la boucle de retrocontrole de l’organe de Corti. Actualites et points forts. – Il est fort probable que parmi les causes, une reactivation de virus neurotropes et/ou une ischemie cochleaire soient frequentes. Quelle que soit la cause, le traitement doit etre instaure en urgence et comprendre une corticotherapie a forte dose. Tout autre traitement n’a jamais prouve formellement son efficacite. On cherche ensuite, dans un second temps moins urgent, a eliminer une atteinte retrocochleaire, telle qu’une tumeur de l’angle pontocerebelleux, notamment chez le sujet jeune. Perspectives et projets. – Un des objectifs actuels est de pouvoir determiner les cas d’ischemie cochleaire de facon mini-invasive, notamment par velocimetrie laser doppler, afin d’optimiser le traitement. Sur le plan therapeutique, la protection acoustique precoce est benefique en cas d’ischemie cochleaire, au moins chez l’animal. Son efficacite en cas de surdite brusque, toute etiologie confondue, est en cours d’evaluation, dans un projet multicentrique.

Resume Objectif Le but de cette etude etait d'evaluer la place de la tomodensitometrie dans le suivi des patients chez lesquels se developpent des lesions pleuropulmonaires au cours d'une granulomatose de Wegener. Materiels et methodes Il s'agissait d'une etude retrospective de dix patients dont le suivi a ete en moyenne de 23 mois, avec des extremes de 4 a 61 mois. Resultats Au stade initial, les lesions qui dominaient etaient les nodules (90% des patients) et les condensations alveolaires (100% des patients). Les nodules ont disparu en 6 mois chez 60% des patients ayant beneficie d'une tomodensitometrie de controle et avaient completement disparu chez tous les patients en 12 mois. Les nodules sous-pleuraux ont regresse en laissant des opacites lineaires de traction cicatricielle. Ces nodules sous-pleuraux pouvaient, lorsqu'ils etaient excaves, se compliquer de pneumothorax. Les condensations alveolaires ont disparu dans 44% des cas le plus souvent dans les 4 mois. Lorsqu'elles persistaient, elles laissaient des infiltrats alveolaires associes a des bronchectasies et des opacites lineaires. Le mode d'expression des rechutes etait similaire a celui des lesions initiales dans 40% des cas. Conclusion Cette etude a montre une disparition sans sequelle de tous les nodules medullaires et corticaux sous traitement. Les nodules sous-pleuraux qui apparaissaient frequents ont ete remplaces par des epaississements cicatriciels. Les condensations alveolaires ont eu une evolution plus variable. Lorsque leur regression etait incomplete, une image de condensation retractile pouvait eventuellement permettre de les differencier d'une complication pulmonaire infectieuse ou d'une rechute de la granulomatose de Wegener dont l'aspect tomodensitometrique le plus evocateur etait nodulaire. La tomodensitometrie a permis de depister des complications souvent meconnues en radiographie standard comme le pneumothorax.

Resume Introduction De nombreuses localisations extrapulmonaires de la tuberculose ont ete decrites. La localisation linguale est rare et le plus frequemment secondaire a une atteinte pulmonaire ou plus rarement ganglionnaire. Observation Un homme de 64 ans, tabagique chronique souffrait d'une ulceration linguale chronique s'accompagnant d'une anorexie et d'une perte de poids importante. Une biopsie profonde de cette lesion a mis en evidence un granulome epithelioide et gigantocellulaire sans necrose caseeuse, sans bacille acido-alcooloresistant a l'examen direct. Les examens complementaires (radiographie thoracique, scanner thoracoabdominopelvien) montraient des signes de tuberculose disseminee confirmee par la positivite des cultures des prelevements pulmonaires, sanguins et urinaires. L'introduction d'un traitement anti-tuberculeux a permis la disparition rapide de l'ulceration linguale, evitant ainsi une evolution defavorable, voire fatale. Conclusion Une ulceration linguale d'origine tuberculeuse doit etre differenciee d'une lesion cancereuse, traumatique, d'un aphte ou d'un chancre syphilitique. Le diagnostic de l'origine tuberculeuse est difficile mais important a poser du fait de l'amelioration generalement rapide de la lesion apres introduction d'un traitement antituberculeux.

Tuberculous lesions of the oral cavity are uncommon. Most of cases are secondary to pulmonary disease and the primary form is rare.We report the case of a 64 year-old man, smoker, presenting a chronic ulcer of the tongue, with anorexia and important weight loss. The biopsy of this ulcer showed granulomatous inflammation and Langhans type giant cells, without necrosis. Ziehl-Nielsen stain was negative. Pulmonary lesions were subsequently detected (chest X-ray, CT-scan) and the disseminated tuberculosis was confirmed by a positive culture with acid-fast bacilli in urine, blood, and pulmonary sample. Antituberculosis treatment resulted in the complete resolution of the oral lesion.Biopsy for histopathological diagnosis, acid-fast stains and culture, is essential to determine the exact nature of chronic oral ulceration to distinguish between oral malignancy, infectious (syphilis), traumatic, or aphthous ulcers. Tuberculosis of the tongue is a difficult diagnosis. However it should be searched for because treatment usually results in a rapid recovery.

Sudden idiopathic deafness is a sensorineural hearing loss with no recognized causes at the time of onset. The impairment site is usually localized in the cochlea, but some cases of retrocochlear lesions (e.g., cerebellopontine angle tumors, degenerative neural diseases, neuraxial ischemic lesions) can induce sensorineural deafness. The medical management of patients presenting with sudden deafness aims at detecting a causal mechanism, and at administering emergency therapeutic drugs. The diagnosis of idiopathic sudden deafness can be definitely made when no causes are found. Usually, the impairing mechanism involves the cochlea. The pathophysiology of this sensorineural alteration is still unknown. It is most likely that several mechanisms are associated together, their common point being an impairment to the feedback loop of the organ of Corti.It is very likely that reactivation of neurotropic viruses and/or cochlear ischemia are frequent etiologies. Whatever the cause, the treatment is to be administered urgently, and consists of a high-dose corticotherapy at the least. Other treatments have never really proven to be effective. It is secondarily checked that no retrocochlear pathological processes, such as a cerebellopontine angle tumor, is present, in particular in young people.One of the current objectives is to determine when cochlear ischemia is involved, in a mini-invasive manner, such as with laser Doppler flowmetry, so that the treatment can be optimized. From a therapeutic point of view, early acoustic protection has been proven to be effective in cases of cochlear ischemia in small laboratory animals. Its efficacy in case of sudden deafness, non-exclusive of other causes than ischemia, is being assessed in a multicentric project.

The aim of this study was to determine CT scan value for the follow-up of patients with pleuropulmonary lesions related to Wegener's granulomatosis.Retrospective study of ten patients who were diagnosed with diffuse Wegener's granulomatosis including lung involvement and for whom mean follow-up was 23 months (range 4-61).During early stages of the disease, the most common finding was the existence of either nodules (90% of the patients) or consolidations (100% of the patients). CT scan controls showed that nodules disappeared within 6 months in 60% of patients and had completely resolved after 12 months. Linear opacities relating to traction developed, replacing subpleural nodules. A pneumothorax occurred in two patients who had excavated subpleural nodules. Consolidations disappeared in 44% of the patients, most frequently within 4 months. When consolidations persisted, they were replaced by alveolar infiltrates accompanied by bronchiolectasies and linear opacities. Regarding either nodules or consolidations, CT features related to relapse were similar to those of initial lesions in 40% of the cases.The main features of pleuropulmonary lesions relating to Wegener's granulomatosis were subpleural nodules that either disappeared with, however, subsequent linear opacities sequelae, or were complicated by the occurrence of pneumothorax.