1,721,079 research outputs found
Preservation of residual hearing in cochlear implantation: comparison between three different surgical techniques
No full textThe preservation of residual hearing is becoming a high priority in cochlear implant surgery. It allows
better speech understanding and ensures long-lasting and stable performance; it also allows the
possibility, in selected cases, of combining electro-acoustic stimulation in the same ear.
We present the results of a retrospective study of the conservation of residual hearing in three different
groups of patients who had undergone cochlear implantation using three different cochlear implant
electrode arrays, combined with three different surgical techniques for the cochleostomy. The study
aimed to evaluate which approach allowed greater preservation of residual hearing.
The best residual hearing preservation results (i.e. preservation in 81.8 per cent of patients) were
achieved with the Contour Advance electrode array, using the Advance Off-Stylet technique and
performing a modified anterior inferior cochleostomy; this combination enabled reduced trauma to the
lateral wall of the cochlea during electrode insertion
Lo screening audiologico neonatale e il percorso diagnostico in età precoce nella Regione Toscana.
No full textLo screening audiologico neonatale e il percorso diagnostico in età precoce nella Regione Toscan
A rare association between Neurofibromatosis type I and Cogan Syndrome: case report
No full textSensorineural hearing loss (SNHL) has been rarely reported in neurofibromatosis type 1 (NF type 1) and literature data concerning its pathogenesis are lacking. We report a patient affected by NF type 1, who several years later developed Cogan's syndrome, with a quickly progressive bilateral SNHL, leading to total deafness. Cochlear implantation was carried out in a partially ossified cochlea, with a good outcome. On the basis of our findings, we underscore the importance of comprehensive evaluation of patients with NF type 1 who develop SNHL, to accurately evaluate the pathogenesis of the hearing loss and explore other possible causes of hearing impairment especially with the emergence of new symptoms. If a retrocochlear lesion is excluded, a cochlear implantation is a viable option for those NF type 1 patients who develop a severe to profound SNHL. We also highlight the importance of a prompt and accurate radiological study of the petrous bone in patients with Cogan's syndrome considered for cochlear implantation, in order to plan a proper surgical approach and the importance of a thorough post-implant medical follow up
Exploring the genetic landscape of otosclerosis: current understanding and future perspectives
No full textOtosclerosis is characterised by abnormal bone remodelling in the otic capsule, leading to progressive hearing loss. Unlike many genetic disorders, the causative genes for otosclerosis remain largely unidentified despite extensive research using linkage analysis and genome-wide association studies (GWAS). Inheritance patterns in otosclerosis suggest a multifactorial model involving genetic predisposition and environmental triggers, a model applied to other common diseases, such as age-related hearing loss, coronary artery disease, and Alzheimer’s disease. Linkage analysis has identified nine loci associated with monogenic forms of otosclerosis, yet the specific causative genes and variants remain elusive. Promising insights have emerged from GWAS, with strong associations identified for novel candidate regions, including the RELN gene. Recent studies using next generation sequencing have identified several candidate genes such as SERPINF1, ACAN, and MEPE. SERPINF1, encoding pigment epithelium-derived growth factor, is linked to regulation of angiogenesis in bone remodelling. ACAN, associated with the OTSC1 locus, encodes aggrecan a crucial component of the extracellular matrix in cartilage, showing a range of variants with varied effect sizes and frequencies. MEPE, involved in bone homeostasis, has been significantly associated with otosclerosis in large family-based and case-control cohorts. While considerable progress has been made in identifying potential genetic contributors, the precise genetic architecture of otosclerosis remains to be fully elucidated. An integrated approach combining genetic data and clinical information, such as audiometric testing and temporal bone imaging, is essential for a comprehensive understanding of otosclerosis
Cochlear implant in children: rational, indications and cost/efficacy
Full text linkA cochlear implant (CI) is a partially implanted
electronic device that can help to provide a
sense of sound and support speech to severely
to profoundly hearing impaired patients.
It is constituted by an external portion, that
usually sits behind the ear and an internal
portion surgically placed under the skin. The
external components include a microphone
connected to a speech processor that selects
and arranges sounds pucked up by the microphone.
This is connected to a transmitter coil,
worn on the side of the head, which transmits
data to an internal receiver coil placed under
the skin. The received data are delivered
to an array of electrodes that are surgically
implanted within the cochlea. The primary
neural targets of the electrodes are the spiral
ganglion cells which innervate fibers of the
auditory nerve. When the electrodes are activated
by the signal, they send a current along
the auditory nerve and auditory pathways to
the auditory cortex. Children and adults who
are profoundly or severely hearing impaired
can be fitted with cochlear implants. According
to the Food and Drug Administration, approximately
188,000 people worldwide have
received implants. In Italy it is extimated that
there are about 6-7000 implanted patients,
with an average of 700 CI surgeries per year.
Cochlear implantation, followed by intensive
postimplantation speech therapy, can help
young children to acquire speech, language,
and social skills. Early implantation providesexposure to sounds that can be helpful during
the critical period when children learn
speech and language skills. In 2000, the
Food and Drug Administration lowered the
age of eligibility to 12 months for one type
of CI. With regard to the results after cochlear
implantation in relation to early implantation,
better linguistic results are reported
in children implanted before 12 months of
life, even if no sufficient data exist regarding
the relation between this advantage and the
duration of implant use and how long this
advantage persists in the subsequent years.
With regard to cochlear implantation in children
older than 12 months the studies show
better hearing and linguistic results in children
implanted at earlier ages. A sensitive period
under 24-36 months has been identified
over which cochlear implantation is reported
to be less effective in terms of improvement
in speech and hearing results. With regard to
clinical effectiveness of bilateral cochlear implantation,
greater benefits from bilateral implants
compared to monolateral ones when
assessing hearing in quiet and in noise and
in sound localization abilities are reported to
be present in both case of simultaneous or
sequential bilateral implantation. However,
with regard to the delay between the surgeries
in sequential bilateral implantation, although
benefit is reported to be present evenafter very long delays, on average long delays
between surgeries seems to negatively affect
the outcome with the second implant. With
regard to benefits after cochlear implantation
in children with multiple disabilities, benefits
in terms of speech perception and communication
as well as in quality of the daily
life are reported even if benefits are slower
and lower in comparison to those generally
attained by implanted children without additional
disabilities. Regarding the costs/efficacy
ratio, the CI is expensive, in particular
because of the cost of the high technological
device, long life support, but even if healthcare
costs are high, the savings in terms of indirect
costs and quality of life are important.
The CI, in fact, has a positive impact in terms
of quality of life
Database Informatizzato per il registro impianti cocleari: usabilità e validità
No full textDatabase Informatizzato per il registro impianti cocleari: usabilità e validita
- …
