There is no consensus on treatment of patients with congenital unilateral aural atresia. Currently, 3 intervention options are available, namely, surgical reconstruction, application of a bone-conduction device (BCD), or application of a middle ear implant.
Human non-hairy (glabrous) skin of the fingers, palms and soles wrinkles after prolonged exposure to water. Wrinkling is a sympathetic nervous system-dependent process but little is known about the physiology and potential functions of water-induced skin wrinkling. Here we investigated the idea that wrinkling might improve handling of wet objects by measuring the performance of a large cohort of human subjects (n?=?40) in a manual dexterity task. We also tested the idea that skin wrinkling has an impact on tactile acuity or vibrotactile sensation using two independent sensory tasks. We found that skin wrinkling did not improve dexterity in handling wet objects nor did it affect any aspect of touch sensitivity measured. Thus water-induced wrinkling appears to have no significant impact on tactile driven performance or dexterity in handling wet or dry objects.
Active middle ear implants (aMEI) are being increasingly used for hearing restoration in congenital aural atresia. The existing gradings used for CT findings do not meet the requirements for these implants. Some items are expendable, whereas other important imaging factors are missing. We aimed to create a new grading system that could describe the extent of the malformation and predict the viability and challenges of implanting an aMEI.
Silicone gel is one therapeutic approach in the treatment and prevention of excessive scarring. The likely mechanism of action is the hydration of the tissue. This should lead to reduced angiogenesis and capillary blood flow. The efficacy is still controversial and the evidence base, insufficient. The aim of this prospective and standardized study is to investigate silicone gel in the preventive treatment of scars.
The Vibrant Soundbridge offers an excellent audiologic rehabilitation for toddlers with microtia and atresia. It provides direct stimulation of the cochlea and straightforward adaption to the given anatomical structures. The posterior atresia incision preserves the physical integrity of the tissue layers around the ear remnant, which is essential for an aesthetic auricular reconstruction.
Active middle ear implants augment hearing in patients with sensorineural, conductive, and mixed hearing losses with great success. However, the application of active middle ear implants has been restricted to compromised ears in adults only. Recently, active middle ear implants have been successfully implanted in patients younger than 18 years of age with all types of hearing losses. The Vibrant Soundbridge (VSB) active middle ear implant has been implanted in more than 60 children and adolescents worldwide by the end of 2008. In October 2008, experts from the field with experience in this population met to discuss VSB implantation in patients below the age of 18.
Little is known about the psychosocial improvement of microtic patients following reconstruction with rib cartilage. Furthermore, no data exist on detailed follow-ups of patients who refused ear repair. To the best of our knowledge, this is the first report of a prospective evaluation of psychosocial outcomes with a validated instrument in ear reconstruction with rib cartilage. Twenty-one patients, who had undergone rib-cartilage reconstruction to treat a congenital auricular defect, were evaluated prospectively for psychosocial changes using a clinically validated questionnaire. In addition, patients were asked to judge the new auricle and thoracic scar. Twenty-three patients, who decided against an ear reconstruction following consultation, were analysed for the reasons behind their refusal. Almost 66% of the treated patients were able to integrate the new ear into their body concept. If faced with the same surgery decision again, 88% would still choose to undergo ear reconstruction with rib cartilage. There were strong postoperative improvements in psychosocial attitude (p=0.02). In our sample, patients who declined ear repair showed higher values of psychosocial attitude (p=0.006) compared with the preoperative results in treated patients. Our study shows that the clinically known improvement of psychosocial aspects can be documented by a validated psychological test. The patients expectations and surgical limits of the reconstruction with rib cartilage need detailed discussion prior to surgery to prevent dissatisfaction despite surgical success. Our data help to accept a childs denial as these patients have a good psychosocial standing even with an unrepaired microtia.
Somatic sensation relies on the transduction of physical stimuli into electrical signals by sensory neurons of the dorsal root ganglia. Little is known about how and when during development different types of sensory neurons acquire transduction competence. We directly investigated the emergence of electrical excitability and mechanosensitivity of embryonic and postnatal mouse sensory neurons. We show that sensory neurons acquire mechanotransduction competence coincident with peripheral target innervation. Mechanotransduction competence arises in different sensory lineages in waves, coordinated by distinct developmental mechanisms. Sensory neurons that are mechanoreceptors or proprioceptors acquire mature mechanotransduction indistinguishable from the adult already at E13. This process is independent of neurotrophin-3 and may be driven by a genetic program. In contrast, most nociceptive (pain sensing) sensory neurons acquire mechanosensitive competence as a result of exposure to target-derived nerve growth factor. The highly regulated process of mechanosensory acquisition unveiled here, reveals new strategies to identify molecules required for sensory neuron mechanotransduction.
Patients with high-grade atresia-microtia suffer from a combined malformation of the outer and middle ears, typically leading to a severe hearing impairment. Long-term results of middle ear reconstruction with tympanoplasty are often insufficient due to persistent air-bone gaps, and new techniques in hearing rehabilitation are required. The objective of this research is to evaluate the active middle ear implant, the Vibrant Soundbridge (VSB), for hearing rehabilitation of patients with unilateral osseous aural atresia.
Current strategies for functional rehabilitation of microtia-atresia patients with bone-anchored implants or surgical atresia repair have been extended by the feasibility of active middle ear implants. The aim of the present research is to evaluate a new flowchart of the treatment of these patients that considers active middle ear implants.
In all vertebrates hearing and touch represent two distinct sensory systems that both rely on the transformation of mechanical force into electrical signals. There is an extensive literature describing single gene mutations in humans that cause hearing impairment, but there are essentially none for touch. Here we first asked if touch sensitivity is a heritable trait and second whether there are common genes that influence different mechanosensory senses like hearing and touch in humans. Using a classical twin study design we demonstrate that touch sensitivity and touch acuity are highly heritable traits. Quantitative phenotypic measures of different mechanosensory systems revealed significant correlations between touch and hearing acuity in a healthy human population. Thus mutations in genes causing deafness genes could conceivably negatively influence touch sensitivity. In agreement with this hypothesis we found that a proportion of a cohort of congenitally deaf young adults display significantly impaired measures of touch sensitivity compared to controls. In contrast, blind individuals showed enhanced, not diminished touch acuity. Finally, by examining a cohort of patients with Usher syndrome, a genetically well-characterized deaf-blindness syndrome, we could show that recessive pathogenic mutations in the USH2A gene influence touch acuity. Control Usher syndrome cohorts lacking demonstrable pathogenic USH2A mutations showed no impairment in touch acuity. Our study thus provides comprehensive evidence that there are common genetic elements that contribute to touch and hearing and has identified one of these genes as USH2A.
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