Halláscsökkenést okozó etiológiai tényezők cochlearis implantáción átesett gyermekekben

Abstract

Introduction: Congenital sensorineural hearing loss is one of the most common sensory defects affecting 1–3 children per 1000 newborns. There are a lot of causes which result in congenital hearing loss, the most common is the genetic origin, but infection, cochlear malformation or other acquired causes can be reasons as well. Aim: The aim of this study was to establish the etiological factors of congenital profound sensorineural hearing loss in children who underwent cochlear implantation. Results: Our results show that the origin of the hearing loss was discovered in 62.9% of our patients. The most common etiological factor was the c.35delG mutation of the gap junction protein β-2 gene, the allele frequency was 38.7% in our cohort. Infection constituted to 10.1%, and meningitis and cytomegalovirus infection were the second most common cause. 79.9% of our patients received sufficient hearing rehabilitation before the end of the speech development’s period (6 years old), but 11.2% of our cases were still diagnosed late. Conclusions: Based on our data we can state that genetic evaluation is crucial in the diagnostic process of congenital profound sensorineural hearing loss. Sufficient hearing rehabilitation affects the whole life of the child, and by late cochlear implantation the speech development falls behind. We can decrease the ratio of the late implantation with the new protocol of newborn hearing screening, and with sufficient information provided to the colleagues, so the children may be referred to the proper center for rehabilitation without delay. Orv Hetil. 2019; 160(21): 822–828.

Vestibular Impairment and Postural Development in Children With Bilateral Profound Hearing Loss

“…However, the higher percentage could represent an overestimation of CBVL cases since the vestibular testing methods at the time of the study were less sensitive . Regarding the distribution of HL origin, it is difficult to compare the present results with those in the literature, partly due to the advancement in technologies, which now allow for better screening of CMV and genetic causes, but also because of differences in the size and characteristics of the populations studied, as most of the studies did not focus on children or on cochlear implant candidates with profound HL . In the present cohort, the main causes of profound HL in children were genetic and infectious.…”

“…1 Regarding the distribution of HL origin, it is difficult to compare the present results with those in the literature, partly due to the advancement in technologies, which now allow for better screening of CMV and genetic causes, but also because of differences in the size and characteristics of the populations studied, as most of the studies did not focus on children or on cochlear implant candidates with profound HL. [15][16][17][18][19][20][21][22][34][35][36] In the present cohort, the main causes of profound HL in children were genetic and infectious. Of note, the proportion of children with unidentified HL origin was high (55.1%), but it was close to the range of 33% to 48% reported in the meta-analysis by Petersen et al 35 This finding could be explained by the fact that, in France, genetic testing for HL in children cannot systematically be performed because the decision is left to the parents.…”

“…In the literature, the incidences of different HL causes are highly variable according to study cohort size and population characteristics. [15][16][17][18][19][20][21][22] The most common causes for bilateral HL in children are CMV infection and genetic causes. Genetic causes represent 50% of pediatric HL, with approximately 70% being nonsyndromic and 30% being syndromic genetic cases.…”

“…In the literature, the incidences of different HL causes are highly variable according to study cohort size and population characteristics . The most common causes for bilateral HL in children are CMV infection and genetic causes.…”

Umbilical Cord Mesenchymal Stromal Cell-Derived Exosomes Rescue the Loss of Outer Hair Cells and Repair Cochlear Damage in Cisplatin-Injected Mice

“…Sensorineural hearing loss (SNHL) results from the damage or loss of cochlear hair cells (HCs) or SGNs [22]. The risk factors that lead to the loss of inner ear HCs include exposure to loud noises, aging, genetic mutations, use of ototoxic drugs, and autoimmune diseases [23][24][25][26]. The chemotherapeutic drug cisplatin is one of the ototoxic drugs that cause many cancer patients to have the side effects of SNHL.…”