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OBJECTIVES To identify the clinical and anatomical presentations and to discuss the guidelines for surgical management of anomalies of the first branchial cleft. DESIGN Retrospective study. SETTING Three tertiary care centers. PATIENTS Thirty-nine patients with first branchial cleft anomalies operated on between 1980 and 1996. INTERVENTION All(More)
Prelingual non-syndromic (isolated) deafness is the most frequent hereditary sensory defect. In >80% of the cases, the mode of transmission is autosomal recessive. To date, 14 loci have been identified for the recessive forms (DFNB loci). For two of them, DFNB1 and DFNB2, the genes responsible have been characterized; they encode connexin 26 and myosin(More)
BACKGROUND DFNB1, the locus of an autosomal recessive form of deafness due to mutations in the connexin-26 gene (CX26 or GJB2) is one of the most frequent hereditary defects in human beings. To date, no clinical characterisation of the DFNB1 inner-ear defects has been reported, which precludes the provision of prognostic information and genetic counselling.(More)
OBJECTIVE To evaluate difficulties encountered in genetic counseling in deaf children carrying connexin 26 gene (CX26 or GJB2) mutations. DESIGN Prospective study. SETTING Outpatients, tertiary referral center. PATIENTS Ninety-six unrelated deaf children in whom CX26 mutations had been detected consecutively. Children were recruited to a center for(More)
The development of surgically implantable hearing aids that are placed directly in the cochlea where they send electrical impulses to the cochlear nerve is a major break-through for patients whose hearing loss is so severe as to make conventional electroacoustic hearing aids ineffectual. Initially used only in adults, this method has gradually been extended(More)
Between 1987 and 1993, 115 children were operated on for severe forms of laryngomalacia in two pediatric ear, nose, and throat (ENT) departments. The criteria used to determine the severity of the illness were selected following short hospitalization periods during which the children received both pediatric and ENT checkups. Based on clinical manifestations(More)
BACKGROUND Numerous complications have been described following cochlear implantation. Most of them are post-operative and benign, but some are intra-operative and can be more difficult to manage. METHODS Case report of a pediatric case of misplaced cochlear electrode in the carotid canal and literature review. RESULTS AND CONCLUSIONS Post-operative(More)
The carbon dioxide (CO2) laser has been used to treat tracheobronchial pathology for more than 20 years. It was initially employed in adults and now has been found to be useful in the treatment of certain lesions in children. We present our experience with this technique in 30 patients treated with a CO2 bronchoscopic laser from 1987 to 1995 in the Ear,(More)
Nineteen children with asthma underwent bilateral inferior turbinectomy from 1990 to 1995. Mean age was 11.5 years (range 8-17). All had obstructive allergic rhinitis and were resistant to long-term local corticosteroids. All operations were done under general anesthesia and endoscopic control. Mean hospital stay was 3 days. The postoperative period was(More)
OBJECTIVE The purpose of the study is to present the results of cochlear implantation in case of deafness involving mutations in the OTOF gene. This form of deafness is characterized by the presence of transient evoked otoacoustic emissions (TEOAE). In cases of profound deafness with preserved TEOAE, two main etiologies should be considered: either an(More)