The higher cultural and social level of people affected by hypoacusis, leads to a growth of the request of high quality hearing aid systems: to achieve this quality enhancement, the evolution of technologies in the hearing aid and audiometric fields is moving towards the digital world. Even if digital audio prosthesis are not new, the algorithms for the enhancement of speech quality and intelli...

OBJECTIVES Isolated complex IV (cytochrome c oxidase) deficiency is one of the most frequent respiratory chain defects in mitochondrial disorders (MIDs) and usually occurs together with severe pediatric or rarely adult multisystem disease. Here we report an adult with isolated complex IV deficiency with unusually mild clinical manifestations. CASE REPORT A 50-year-old man had developed genera...

THE AIM The aim of the research work is the evaluation of auditory function in children with rare, genetically determined Brachmann-de Lange syndrome. METHODS Test material came from 18 children (7 girls and 11 boys) between 11 months and 18 years of age with Brachmann-de Lange syndrome who have been diagnosed and treated at ENT Department and Department of Paediatrics, Haematology, Oncology ...

A woman aged 73 presented with a four-week history of hypoacusis and low-density clear discharge from her right ear, with no history of traumatic or surgical events. Physical examination showed right middle ear invasion and discharge of a clear liquid, which was suggestive of CSF caused by tympani defects. Left middle ear invasion presented with no other condition. Computerised tomography (CT) ...

BACKGROUND Eosinophilia has not been reported as a manifestation of a mitochondrial disorder (MID). Here, we report a patient with clinical features suggesting a MID and permanent eosinophilia, multisystem disease, and progressive hyper-creatine-kinase (CK)-emia for at least 10 years. MATERIALS AND METHODS Methods applied included a clinical exam, blood chemical investigations, electrophysiol...

Objective: To evaluate the hearing status of patients being treated for pulmonary tuberculosis at referral hospitals in Brazil. Methods: This was a descriptive study involving 97 male and female inpatients/outpatients between 18 and 60 years of age who were undergoing treatment for active pulmonary tuberculosis at one of two referral hospitals in the state of Rio de Janeiro. After being intervi...

INTRODUCTION Hearing is foundamental for human social life. Secretory Otitis Media (SOM) is the most important cause of trasmissive hypoacusis in early childhood. METHODS The Department of Physiopathology, Experimental Medicine and Public Health of Siena University in collaboration with the Thermal Baths "Bagni delle Galleraie" proposed a prevention and cure campaign of rhinogenous deafness i...

The objective of this study was verification, through suitable hematochemical tests, of the supposition that central-systemic microtoxicosis plays a role either in the etiopathogenesis of Ménière's syndrome or in labyrinthine pathological processes or hypoacusis. We did not, therefore, exclude other well-known hypotheses in the causality of these pathologies. Nonetheless, one finds, particularl...

With interest we read Finsterer et al.'s response [1] to our manuscript ‘Case Report: 5 Year Follow-Up of Adult Late-OnsetMitochondrial Encephalomyopathy with Lactic Acid and Stroke-Like Episodes (MELAS)’ [2]. We have the following responses: Regarding the lack of theMRIfigure, theMRIwas performed at an outside hospital. As a result an MRI figure was not available despite efforts to the contrar...