Progeria is a rare syndrome, with an estimated incidence of 1 per 250,000 births. Although children with progeria have the appearance of premature aging or senility, the term is misleading because reported cases of progeria have not manifested most physical or biochemical aspects of old age. Many children with progeria appear normal at birth and then progressively, and rather rapidly, develop t...

UNLABELLED Oculo-auriculo-vertebral spectrum (OAVS) is a rare condition characterized by the involvement of the first branchial arches. PURPOSE To investigate the ear abnormalities of a sample of patients with OAVS. MATERIALS AND METHODS The sample consisted of 12 patients with OAVS seen at the Clinical Genetics Unit, UFCSPA/CHSCPA. The study included only patients who underwent mastoid com...

In nine dogs submitted to a severe myocardial ischaemic challenge, multiple prosthetic implants running from the descending aorta to myocardial tunnels were successful in sustaining effective ventricular contraction for a mean period of 27 minutes. A control series of five dogs and five animals, in which the prostheses clotted, developed ventricular fibrillation within six minutes of the ligati...

Introduction Titanium implants can be regarded as the current gold standard for restoration of sound transmission in the middle ear following destruction of the ossicular chain by chronic inflammation. Many efforts have been made to improve prosthesis design, while less attention had been given to the role of the interface. We present a study on chemical nanocoating on microstructured titanium ...

Background: Pigmented villonodular synovitis is a rare condition typically affecting the major joints and the involvement of the temporomandibular joint is rare. Although, it is histologically benign, it can be locally aggressive, capable of extensive bone destruction. The disease has aggressive behavior and a high incidence of recurrence (40% 60%). Case report: A 35 years old male presented wi...

BACKGROUND AND PURPOSE Congenital causes of hearing loss in children commonly are encountered, and imaging aids in diagnosis as well as presurgical evaluation. Atresia of the oval window not associated with atresia of the external auditory canal (EAC) is a rare cause of congenital hearing loss in children. We present the clinical and imaging findings in children with isolated oval-window atresi...

Due to the simplicity of their manufacture and placement, middle-ear prostheses are generally constructed using only a single 'ossicle'. The variety of pathologies frequently make it necessary to devise individual solutions and the results are often unsatisfactory. Many methods of improving the prostheses have been tried, with very mixed results. Even until recently, the fundamental assumptions...