ABSTRACT
Objective@#To investigate residual hearing of children severe and profound sensorineural deafness in whom wave V was not found in auditory brainstem response(ABR) testing, and to emphasize the importance of objective audiological tests.@*Methods@#Two hundred and fifty-two children who were admitted to the Second Affiliated Hospital of Zhengzhou University between January 2015 and April 2018, with an average age of 20 months from 72 days to 4 years, received a full battery of objective audiological tests consisting of distortion product otoacoustic emission(DPOAEs), tympanometry, auditory brainstem responses(ABRs), 40 Hz auditory event related potential(40 HzAERP) and auditory steady-state response(ASSRs).There were 159 males(318 ears) and 93 females(186 ears). Residual hearing obtained by 40 HzAERP、ASSR of 252 children with sensorineural deafness was studied in relation to the absence of wave V in click ABR. SPSS 16.0 software was used to analyze the data.@*Results@#Four hundred and forty-four ears of 504 ears have residual hearing of different degrees at different frequencies(88.1%),60 ears (11.9%) were found in whom responses was not found in 40 HzAERP、ASSR testing; Seventy-two ears(14.3%) in 38 patients were tested cochlear microphonic potentials (CMs).@*Conclusion@#In children hearing evaluations,a full battery of objective audiological tests could better investigate residual hearing; The CMs were tested could provide the Audiotery Neuropathy diagnosis in infants with OAEs and ABR absent.
ABSTRACT
OBJECTIVE@#To preliminarily determine the gene mutation frequency and the hotspots in Henan province, we analysed the deafness-related gene mutation in patients with non-syndromic hearing loss (NSHL).@*METHOD@#Genomic DNA samples of 100 patients with NSHL in Henan province were extracted from peripheral blood after clinical history inquiry and clinical examination, Four common deafness genes GJB2, SLC26A4, mitochondrial 12SrRNA, and GJB3 were detected by Sanger sequencing method,and then data analysis were conducted.@*RESULT@#Among 100 patients with NSHL. the gene mutation frequency was 44%. In these patients, 29 cases had GJB2 mutations, 13 cases had SLC26A4 gene mutations, and 3 cases had mitochondrial 12SrRNA mutations.@*CONCLUSION@#Among the patients with NSHL in Henan province, the most frequent mutation causing hereditary deafness was mutation in GJB2, followed by SLC26A4,and it will provide a theoretical basis to determine the etiology of deafness in Henan Province.
Subject(s)
Humans , China , Connexin 26 , Connexins , Genetics , DNA Mutational Analysis , DNA, Mitochondrial , Genetics , Deafness , Genetics , Gene Frequency , Membrane Transport Proteins , Genetics , Mutation , RNA, Ribosomal , Genetics , Sulfate TransportersABSTRACT
OBJECTIVE@#To observe the clinical features and the treatment effect of 32 cases of Ramsay Hunt syndrome and explore the relationship between different clinical manifestations and the prognosis.@*METHOD@#Using the integrated therapy including the medication and physiotherapy of ultrashort wave, the relationship between clinical manifestations and recovery time was analyzed.@*RESULT@#Twenty two cases were cured, 10 cases showed improvement of the symptoms, and no patients complained ineffectiveness. The effective rate was 100%. The shortest recovery time was 13 days, and the longest recovery time was 1 year and 7 months.@*CONCLUSION@#The key to cure Ramsay Hunt syndrome is comprehensive treatment in time. The different clinical manifestations have something to do with the prognosis.