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Abstract Introduction Cochlear implantation is an effective treatment method for severe to profound hearing loss. Many factors that may influence cochlear implantation success have been explained in previous studies. Apart from those, minor differences in size of normal cochlear nerves may affect postoperative performance. Objective To investigate whether the minor differences in cochlear nerve size in normal cochlear nerves affect postoperative cochlear implant performance. Methods 30 pediatric prelingually deaf patients who were treated with cochlear implantation were included in this study. From the reconstructed parasagittal magnetic resonance images, the diameter and cross-sectional area of the cochlear nerve on the ipsilateral and contralateral side were measured. Auditory evaluations were performed 1, 3, 6 and 12 months following the first fitting. All the analysis was performed by using EARS®, evaluation of auditory responses to speech tool. Correlation between cochlear nerve diameter, cross-sectional area and postoperative auditory perception was analyzed to determine whether variation in cochlear nerve size contributes to postoperative auditory performance. Results The mean diameter of the cochlear nerve on the ipsilateral side was 718.4 μm (504.5 − 904.3 μm) and mean cross sectional area was 0.015 cm2 (0.012 − 0.018 cm2). On the contralateral side the mean cochlear nerve diameter was 714.4 μm (502.6 − 951.4 μm) and mean cross sectional area was 0.014 cm2 (0.011 − 0.019 cm2). The correlation between the diameter and cross-sectional area of the ipsilateral and contralateral cochlear nerve revealed no significance. Mean score at first month monosyllable-trochee-polysyllable test, MTP1, was 0.17 (0.08 − 0.33), at 6th month with 6 words test, 6th month MTP6 was 0.72 (0.39 − 1.0), at 6th month with 12 words, 6th month MTP 12 was 0.46 (0.17 − 0.75) and at 12th month with 12 words, 12th month MTP12 was 0.73 (0.25 − 1.0). There was no correlation between the monosyllable-trochee-polysyllable test, values at any time with the diameter of the ipsilateral cochlear nerve. However, the first month MTP, 6th month MTP6 and 12th month MTP12 correlated with the cross-sectional area of the ipsilateral cochlear nerve. Conclusion Measuring the cross sectional area of the normal- appearing cochlear nerve may give important prognostic knowledge on cochlear implant outcomes. In patients with a larger cross sectional area the auditory performance was better and faster. Although normal appearing, slight differences on cross sectional area of the cochlear nerve may affect performance. Measuring the size of the cochlear nerve on parasagittal magnetic resonance images may provide beneficial information on the postoperative rehabilitation process.
Resumo Introdução O implante coclear é um método de tratamento eficaz para a perda auditiva grave a profunda. Muitos fatores que podem influenciar o sucesso do implante coclear foram explicados em estudos anteriores. Além desses fatores, pequenas diferenças no tamanho dos nervos cocleares normais podem afetar o desempenho pós-operatório. Objetivo Investigar se pequenas diferenças no tamanho dos nervos cocleares normais afetam o desempenho pós-operatório do implante coclear. Método Foram incluídos neste estudo 30 pacientes pediátricos surdos pré-linguais, tratados com implante coclear. A partir de imagens de ressonância magnética parassagitais reconstruídas, foram medidos o diâmetro e a área de seção transversal do nervo coclear no lado ipsilateral e contralateral. As avaliações auditivas foram feitas 1, 3, 6 e 12 meses após a primeira adaptação. Todas as análises foram feitas com a ferramenta EARS® (do inglês evaluation of auditory responses to speech). A correlação entre o diâmetro do nervo coclear, a área transversal e a percepção auditiva pós-operatória foi analisada para determinar se a variação no tamanho do nervo coclear contribui para o desempenho auditivo pós-operatório. Resultados O diâmetro médio do nervo coclear no lado ipsilateral foi de 718,4 μm (504,5 a 904,3 μm) e a área da seção transversal média foi de 0,015 cm2 (0,012-0,018 cm2). No lado contralateral, o diâmetro médio do nervo coclear foi de 714,4 μm (502,6 a 951,4 μm) e a área da seção transversal média foi de 0,014 cm2 (0,011 a 0,019 cm2). A correlação entre o diâmetro e a área transversal do nervo coclear ipsilateral e contralateral não revelou qualquer significância. O escore médio do teste monosyllable-trochee-polysyllable no primeiro mês, denominado MTP1, foi de 0,17 (0,08-0,33), no sexto mês com teste de 6 palavras, MTP6, foi de 0,72 (0,39-1,0), no sexto mês com 12 palavras, MTP12, foi de 0,46 (0,17-0,75) e no 12° mês com 12 palavras, MTP12, foi de 0,73 (0,25-1,0). Não houve correlação entre os valores do teste monosyllable-trochee-polysyllable em qualquer momento com o diâmetro do nervo coclear ipsilateral. Entretanto, o teste monosyllable-trochee-polysyllable do primeiro mês, do 6° mês, e o do 12° mês correlacionaram-se com a área transversal do nervo coclear ipsilateral. Conclusão Medir a área da secção transversal do nervo coclear de aparência normal pode fornecer conhecimento prognóstico importante sobre os resultados do implante coclear. Em pacientes com área da secção transversal maior, o desempenho auditivo foi melhor e mais rápido. Embora o nervo coclear pareça normal, pequenas diferenças na área da secção transversal do nervo coclear podem afetar o desempenho, de forma que a medida do tamanho do nervo coclear nas imagens de ressonância magnética na projeção parassagital pode fornecer informações benéficas sobre o processo de reabilitação pós-operatória.
ABSTRACT
The broad spectrum of causal variants in the newly discovered GIPC3 gene is well reflected in worldwide studies. Except for one missense variant, none of the reported variants had reoccurred, thus reflecting the intragenic heterogeneity. We screened all the six coding exons of GIPC3 gene in a large cohort of 177 unrelated prelingual hearing impaired after excluding the common GJB2, GJB6 nuclear and A1555G mitochondrial variants. We observed a single homozygous pathogenic frameshift variant c.685dupG (p.A229GfsX10), accounting for a low incidence (0.56%) of GIPC3 variants in south Indian population. GIPC3 being a rare gene as a causative for deafness, the allelic spectra perhaps became much more diverse from population to population, thus resulting in a minimal recurrence of the variants in our study, that were reported by authors from other parts of the globe.
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Abstract Introduction Cervical vestibular-evoked myogenic potentials (cVEMPs) are biphasic, short latency potentials, which represent the inhibition of the contraction of the sternocleidomastoid muscle (SCM) mediated by the saccule, the inferior vestibular nerve, the vestibular nuclei and the medial vestibular spinal tract. Objective To evaluate the response of cVEMPs in individuals with profound prelingual bilateral cochlear hearing loss. Methods A prospective case-control study. A total of 64 volunteers, divided into a study group (31 patients with profound prelingual sensorineural hearing loss) and a control group (33 subjectsmatched for age and gender with psychoacoustic thresholds of ≤ 25 dB HL between 500 and 8,000 Hz) were submitted to the cVEMP exam. The causes of hearing loss were grouped by etiology and the involved period. Results The subjects of the study group aremore likely to present changes in cVEMPs compared to the control group (35.5% versus 6.1% respectively; p = 0.003), with an odds ratio (OR) of 8.52 (p = 0.009). Itmeans that they had 8.52-fold higher propensity of presenting altered cVEMP results. There were no statistically significant differences between the latencies, the interamplitude and the asymmetry index. Regarding the etiology, there was a statistically significant difference when the cause was infectious, with an OR of 15.50 (p = 0.005), and when the impairment occurred in the prenatal period, with an OR of 9.86 (p = 0.009). Conclusion The present study showed abnormalities in the sacculocolic pathway in a considerable portion of individuals with profound prelingual sensorineural hearing loss due to infectious and congenital causes, as revealed by the cVEMP results. (AU)
Subject(s)
Humans , Male , Female , Adolescent , Adult , Vestibular Evoked Myogenic Potentials , Hearing Loss, Sensorineural/etiology , Vestibulocochlear Nerve/physiopathology , Communicable Diseases/complications , Prospective Studies , Deafness/etiology , Genetic Diseases, Inborn/complicationsABSTRACT
Objective To investigate the development of auditory and speech skills and the safety and stability among the prelingually deaf children with Nurotron Venus cochlear implants.Methods A total of 78 cochlear implant subjects were recruited from the first of affiliated hospital of Zhengzhou university.They were divided into 5 groups according to the ages at the time of implantation: group A(between 13 and 24 months), group B(between 25 and 36 months),group C(between 37 and 48 months),group D(between 49 and 72 months),group E(between 73 and 96 months).Children were evaluated by IT-MAIS(group A and B) and MAIS(group C,D and E) before the surgery and 1 month,3 months,6 months, and 12 months after surgery.All children were evaluated by MUSS questionnaires 1 months, 3 months,6 months,and 12 months after surgery.Post-operative cochlear radiographs determine the position of the CIs.The complications and usage of The CIs were followed up.Results The auditory and speech ability of children with CIs improved constantly.The auditory and speech of different groups were statistically significant.All the surgeries of 78 cases Nurotron-Venus cochlear implants were successful.Post-operative cochlear radiographs showed electrodes in the normal position.2 cases had processor failures.The remaining CIs were implanted completely.Conclusion Within 1 year, the ability of auditory and speech improved gradually.The scores of auditory and speech about the small age groups are lower than older children in the early days.There is no influence on the audition in 12 months.Small children's scores of speech are lower than older children in 12 months.The better ability to integrate auditory information is , the better the ability to speak is.The Nurotron CIs work safety and effectively.
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Objective To investigate hearing and speech rehabilitation effects of music therapy for prelingual-ly deaf children with cochlear implantation.Methods A total of 107 cases of prelingual deaf children with cochlear implantation were selected and divided into control group (n=50)and observation group (n= 57 ).The control group was given conventional hearing and speech rehabilitation training while the observation group was given more music therapy.Before and 3 months,6 months and 12 months after rehabilitation training,the auditory behavior level (CAP),speech understand the grading (SIR)and the auditory speech recognition ability from the 2 groups were compared.ResuIts Before training,CAP of the observation group was 2.85±0.02 level,SIR was 2.92±0.03 and hearing and speech recognition ability was 59.51±5.41,while the control group yielded levels of 2.92±0.03, 1.05±0.02 and 58.99±4.31 respectively.The difference between the two groups was statistically insignificant (P>0.05).After training for 3,6,and 12 months,the two groups had different degrees of improvement in hearing and speech rehabilitation:the observation group in the CAP was up to 6.95±0.34 level,SIR up to 4.37±0.09 level,and hearing and speech recognition capacity up to 92.03 ± 11.32,showing significantly more improvements than those of in control group (P<0.05).ConcIusion Music therapy can significantly improve the effects of the hearing and speech rehabilitation training in cochlear implantation in prelingually deafened patients.The auditory behavior after rehabilitation training classification,speech intelligibility rating and auditory speech recognition ability can also be improved.
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Objective To evaluate auditory and speech development longitudinally over the first year after co-chlear implantation in prelingual deaf pediatric cochlear implant (CI) recipients .Methods Meaningful auditory inte-gration scale (MAIS) and infant-toddler meaningful auditory integration scale (ITMAIS) were used to evaluate 34 prelingual deaf children between 14 month and 5 years and 10 month before CI and 3 ,6 ,and 12 months after im-plantation .The effects of genders ,age at implantation ,and duration of post -implantation time were evaluated .Re-sults Three MAIS scores were displayed :total ,detection ,and perception .Each score from all the subjects was significantly improved than that before cochlear implantation ,and still getting further better with CI use(P0 .05) .Conclusion After cochlear im-plantation ,a significant increase in auditory capability occurred ,especially in the first 3 months .The factor of gen-der was no influence on auditory development .These results provide preliminary validation of MAIS/IT -MAIS for evaluation of pre -lingual deaf pediatric CI recipients .
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Limiares auditivos de crianças surdas pré-linguais usuárias de implante coclear foram avaliados com estimulação elétrica em um dos eletrodos mediais. A avaliação empregou um procedimento operante do tipo go/no go para ensinar uma discriminação simples, evidenciada por uma resposta motora, entre presença e ausência do estímulo auditivo. Estabelecida a linha de base, a manipulação na intensidade do estímulo foi implementada de acordo com o método psicofísico de escada modificado, começando por uma seqüência descendente. Os sete participantes do estudo mostraram perda da precisão no responder sob controle do estímulo quando a intensidade diminuía além de um certo valor e a precisão era recuperada quando a intensidade era novamente aumentada, o que permitiu a identificação de limiares individuais. Os resultados sugerem que o método psicofísico combinado com o procedimento operante pode ser uma alternativa viável para avaliar limiar auditivo de pessoas sem linguagem em situação clínica de regulagem do implante coclear.
Auditory thresholds of prelingually deaf children who received cochlear implants were evaluated for the electrical stimulation to one of the medial electrodes. A go/no go operant procedure was used to teach a simple discrimination, indicated by a motor response, between the presence versus the absence of an auditory stimulus. Simple discrimination was used as a baseline upon which the electrical stimulus's intensity was manipulated in a decreasing sequence followed by an increasing sequence, according to a modified psychophysical staircase method. The accuracy of responding in the presence of the electrical stimulus was reduced for all seven participants when the stimulus intensity decreased bellow a certain value and was recovered when the stimulus intensity was increased to the previous level. The experimental design allowed reliable identification of individual thresholds. The results suggest that the psychophysical paradigm in combination with the operant procedure might be useful for the evaluation of the auditory threshold for purposes of cochlear implant fitting in clinical settings.
Subject(s)
Humans , Male , Female , Child , Auditory Threshold , Conditioning, Operant , Deafness , Cochlear Implantation/psychologyABSTRACT
Objective To determine the prevalence and characteristics of the del(GJB6-D13S1830) in Connexin30(Cx30) gene in children with prelingual deafness.Methods Forty-six prelingual deaf children and 30 children with normal comprehension were obtained,and the del(GJB6-D13S1830)in the Cx30 gene were screened by polymerase chain reaction(PCR) in 2 groups.Results Three cases of 46 deaf children were found to have heterozygous del(GJB6-D13S1830) in Cx30 gene.The rest deaf children and the normal controls did not harbor this deletion.Conclusion The heterozygous del(GJB6-D13S1830) in Cx30 gene is one of causes of prelingual deafness.
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BACKGROUND: As the population of children received a cochlear implant at an early age is increasing, there is a need to test speech perception ability for the young children. OBJECTIVES: The purpose of this study is to evaluate speech perception ability for young children using a cochlear implant with Vowel and Consonant Imitation test. MATERIAL AND METHODS: Subjects were nine children with prelingual deafness who had at least 6 months of experience with Nucleus 22-channel cochlear implant. The age at implantation ranged from 3 years and 5 months to 10 years and 11 months. A 14-set vowel imitation test in a "hVd" environment and a 18-set consonant imitation test in a "aCa" environment(Vowel and Consonant Imitation test) and Peabody Picture Vocabulary Test(PPVT) were administered with audition alone at 6 month intervals from initial stimulation. RESULTS: Six out of 9 children performed above chance on the Vowel and Consonant Imitation test at 6 months from initial stimulation. The average scores for vowel and consonant were 34%(range: 0-79) and 22%(range: 0-47) correct, respectively. Generally, the Vowel and Consonant Imitation test scores and PPVT equivalent ages were increased over time. CONCLUSION: The Vowel and Consonant Imitation test appears to be a useful tool for assessment of speech perception ability of young children with a cochlear implant.
Subject(s)
Child , Humans , Cochlear Implants , Deafness , Hearing , Speech Perception , VocabularyABSTRACT
Objective To investigate the molecular epidemiology of GJB2 mutations as a causative effect of prelingual deafness in northwestern China. Methods The medical history of 274 deaf-mute students was collected. Blood samples were obtained from them with informed consent. GJB2 gene sequences of genomic DNAs were amplified by polymerase chain reaction (PCR) with a pair of primers, and bidirectional sequencing of PCR products was performed and analyzed with DNAStar Software. Results A total number of 274 deaf-mute students were diagnosed as non-syndromic hearing impairment, and profound prelingual deafness. Two kinds of polymorphism, seven pathologic mutations and one novel mutation were revealed in the GJB2 screenings of them, and 79G→A and 341A→G were polymorphism with high frequency. Conclusion GJB2 gene mutation is the causative gene in the prelingual deafness with a high incidence of 10.95% in northwestern China. Based on the investigation, it is clear that screening of GJB2 gene mutation should play a significant role in early diagnosis of deaf-mutism in this region.