Aneurysmal bone cyst in the sphenoid bone: treatment with minimally invasive surgery.

Aneurysmal bone cysts are vascular lesions that destroy and expand bone. We report a recently treated case of an aneurysmal cyst of the sphenoid bone. A 14-year-old girl presented with frontal headaches, bouts of nausea, and vomiting. Computed tomography and magnetic resonance imaging showed typical features of an aneurysmal bone cyst. Arterial embolization was undertaken before surgery. The endoscopic transnasal procedure used allowed the complete removal of the aneurysmal bone cyst. This use of minimally invasive surgery makes this case of interest to surgeons of the skull base and sinuses.

[Is sudden sensorineural hearing loss a therapeutic emergency? Early results in a prospective cohort of 136 patients]. / Les surdités brusques sont-elles une urgence thérapeutique?

Idiopathic sudden sensorineural hearing loss is one of the most controversial issues in otology. A prospective cohort study including 136 sudden hearing losses seen at the ENT emergency referral center of Lariboisiere Hospital during an 18-month period was conducted. Patients were hospitalized and a corticotherapy course was administered, eventually associated with an antiviral treatment, low molecular weight dextran or sedative. At the end of the 5-day hospitalization, 74% of the patients had recovered, at least partially, with a mean gain of 22 dB. The outcome was considered satisfactory, i.e. superior to 50% of the initial loss, in 30% of the patients. After 1 month, 86% of the patients recovered at least partially with a mean gain of 30 dB. The outcome was considered satisfactory in 44% of the patients. A prognosis analysis showed that vertigo, age of the patient and anacusis are pejorative factors. Ascending audiogram shapes seem to involve a better prognosis. The therapeutic delay is not correlated with the quality of the outcome, which questions the classical notion of therapeutic emergency.

[Hemangiomas and superficial vascular malformations of the face and neck]. / Hemangioma y malformaciones vasculares superficiales de la cara y del cuello.

Hemangiomas and superficial vascular anomalies of the head and neck form what usually calls the angiomas. Many terms exist in the literature for that reason the classification of the ISSVA, admitted by most of the doctors, helps us in naming the different anomalies. The processing of this pathology needs a multidisciplinary collaboration with doctors of different specialties. The evolution of the radiological, surgical techniques of laser, and who knows, of the research, allows to hope us to cure some of the particularly dangerous anomalies like the arteriovenous malformations. We presented here the different technics used in 2001 in the management of hemangioma and superficial vascular anomalies insisting on a multidisciplinary approach.

[Endolymphatic sac tumor and von Hippel-Lindau disease. Review of the literature]. / Tumor del saco endolinfático y enfermedad de Von Hippel-Lindau. Revision de la literatura.

This report describes a patient with Von Hippel-Lindau disease revealed by an endolymphatic sac tumor. Endolymphatic sac tumor (EST) was only recently recognized as a manifestation of Von Hippel-Lindau (VHL) disease. EST are vascular lesions that destroy and expand bone. We report a recently treated case of an EST. A 30-year-old woman presented with otalgia and hearing loss. Computed tomography and magnetic resonance imaging showed typical features of an EST. We checked for VHL and found this disease in the patient. VHL disease is a hereditary cancer syndrome caused by germline mutations of the VHL tumor suppressor gene. A molecular diagnosis of VHL is nowadays available, and this has change the clinical management of patients and their families. Diagnosis of VHL has to be suspected in patients with a VHL-related tumor without familial history and especially in those cases of hemangioblastoma or endolymphatic sac tumors. Such patients should be systematically investigated for clinical and molecular evidence of VHL disease.

beta2-adrenergic but not vasopressin V2 receptor mRNAs are expressed in the stria vascularis of the rat inner ear.

Activation of beta2-adrenergic receptors (beta2-AR) and of V2-vasopressin receptors (V2-VR) has been shown to stimulate cAMP production in the stria vascularis. Expression of these receptors in this epithelial structure has never been investigated at the mRNA level. A quantitative assay based on the reverse transcriptase-polymerase chain reaction (RT-PCR) method was developed to study the expression of beta2-AR, vasopressin (V1a,V1b, V2) and oxytocin receptors in rat microdissected stria vascularis fragments. Steady-state mRNA levels were measured using mutant cRNAs as internal standards. We consistently found evidence of the expression of beta2-AR transcripts in the stria vascularis; however, no such evidence of V2-VR mRNA expression was found in studies of the same samples. As our method is sensitive enough to detect 200 mRNA molecules, V2-VR mRNA is thought to be present, if at all, at a concentration that is 40 times lower than that of the beta2-AR transcripts. V1a-VR mRNA was found to be present in trace amounts and is the only subtype of the vasopressin-oxytocin receptor family expressed in this epithelium. These data demonstrate, at the mRNA level, the expression of beta2-AR in the stria vascularis, and indicate that V2-VR transcripts are not present in this structure.