34 research outputs found
Pediatric cochlear implantation: an update
Deafness in pediatric age can adversely impact language acquisition as well as educational and social-emotional
development. Once diagnosed, hearing loss should be rehabilitated early; the goal is to provide the child with
maximum access to the acoustic features of speech within a listening range that is safe and comfortable. In presence
of severe to profound deafness, benefit from auditory amplification cannot be enough to allow a proper language
development. Cochlear implants are partially implantable electronic devices designed to provide profoundly deafened
patients with hearing sensitivity within the speech range. Since their introduction more than 30 years ago, cochlear
implants have improved their performance to the extent that are now considered to be standard of care in the
treatment of children with severe to profound deafness. Over the years patient candidacy has been expanded and
the criteria for implantation continue to evolve within the paediatric population. The minimum age for implantation
has progressively reduced; it has been recognized that implantation at a very early age (12–18 months) provides
children with the best outcomes, taking advantage of sensitive periods of auditory development. Bilateral implantation
offers a better sound localization, as well as a superior ability to understand speech in noisy environments than unilateral
cochlear implant. Deafened children with special clinical situations, including inner ear malformation, cochlear nerve
deficiency, cochlear ossification, and additional disabilities can be successfully treated, even thogh they require
an individualized candidacy evaluation and a complex post-implantation rehabilitation. Benefits from cochlear
implantation include not only better abilities to hear and to develop speech and language skills, but also improved
academic attainment, improved quality of life, and better employment status. Cochlear implants permit deaf people
to hear, but they have a long way to go before their performance being comparable to that of the intact human ear;
researchers are looking for more sophisticated speech processing strategies as well as a more efficient coupling
between the electrodes and the cochlear nerve with the goal of dramatically improving the quality of sound of
the next generation of implants
Long-term microdystrophin gene therapy is effective in a canine model of Duchenne muscular dystrophy
Duchenne muscular dystrophy (DMD) is an incurable X-linked muscle-wasting disease caused by mutations in the dystrophin gene. Gene therapy using highly functional microdystrophin genes and recombinant adeno-associated virus (rAAV) vectors is an attractive strategy to treat DMD. Here we show that locoregional and systemic delivery of a rAAV2/8 vector expressing a canine microdystrophin (cMD1) is effective in restoring dystrophin expression and stabilizing clinical symptoms in studies performed on a total of 12 treated golden retriever muscular dystrophy (GRMD) dogs. Locoregional delivery induces high levels of microdystrophin expression in limb musculature and significant amelioration of histological and functional parameters. Systemic intravenous administration without immunosuppression results in significant and sustained levels of microdystrophin in skeletal muscles and reduces dystrophic symptoms for over 2 years. No toxicity or adverse immune consequences of vector administration are observed. These studies indicate safety and efficacy of systemic rAAV-cMD1 delivery in a large animal model of DMD, and pave the way towards clinical trials of rAAV-microdystrophin gene therapy in DMD patients
Importance des fausses routes respiratoires chez 50 résidents testés aléatoirement en établissement d'hébergement pour personnes âgées dépendantes (EHPAD).
International audienc
The economics of cochlear implant management in France: a multicentre analysis
Cochlear implantation, Clinical management, Economic analysis,