Vestibular status in partial deafness / Condição vestibular na surdez parcial

Abstract Introduction The hair cells of the cochlea and the vestibulum are closely connected and may be susceptible to the same noxious factors. The relationship between their function has been a continuing field of investigation. The indications for cochlear implantation have been broadened and now include the patients with partial deafness. This raises the question of their vestibular status. Objective The aim of the study was to investigate whether there is any difference between the vestibular function of patients with low frequency residual hearing and those with totally deaf ears. Methods A total of 360 ears with profound sensorineural hearing loss were analysed before cochlear implantation. The patients were divided into four groups, according to their low frequency residual hearing (Group 1 ‒ normal or slightly elevated low frequency residual hearing; Group 2 ‒ elevated threshold but still usable hearing at low frequencies; Group 3 - non-functional residual hearing; Group 4 ‒ no detectable hearing threshold within the limits of the audiometer). The patients underwent vestibular tests: cervical vestibular evoked myogenic potential, ocular vestibular evoked myogenic potential, caloric test and video-head impulse test. Results The rates of elicited responses in cervical vestibular evoked myogenic potential were as follows: in Group 1 (59.3 %); Group 2 (57.5 %); Group 3 (35.2 %); Group 4 (7.7 %). For ocular vestibular evoked myogenic potential the percentage of correct outcomes was: Group 1 (70.8 %); Group 2 (56.0 %); Group 3 (40.0 %); Group 4 (14.3 %). For the caloric test we counted normal responses in 88.9 % of Group 1; 81.6 % of Group 2; 57.9 % of Group 3; 53.3 % of Group 4. For video-head impulse test we also found markedly better results in Group1, followed by Group 2, and much worse in Group 3 and 4. Conclusion Patients with partial deafness not only have a better cochlea but also better vestibular function, which needs to be protected. In summary, the better the low frequency residual hearing, the better the vestibular status.

Resumo Introdução As células ciliadas da cóclea e do vestíbulo estão intimamente ligadas e podem ser suscetíveis aos mesmos fatores nocivos. A relação entre suas funções tem sido um campo de investigação há muito tempo. As indicações para implante coclear foram ampliadas e agora incluem os pacientes com surdez parcial. Isso levanta a questão de sua condição vestibular. Objetivo Investigar se existe alguma diferença entre a função vestibular de pacientes com audição residual de baixa frequência e aqueles com surdez total. Método Foram analisadas antes do implante coclear 360 orelhas com perda auditiva neurossensorial profunda. Os pacientes foram divididos em quatro grupos, de acordo com a audição residual de baixa frequência (Grupo 1 - audição residual de baixa frequência normal ou levemente elevada; Grupo 2 - limiar auditivo elevado, mas ainda usável em baixas frequências; Grupo 3 - audição residual não funcional; Grupo 4 - sem limiar auditivo detectável dentro dos limites do audiômetro). Os pacientes foram submetidos a testes vestibulares: potencial evocado miogênico vestibular cervical, potencial evocado miogênico vestibular ocular, prova calórica e teste do impulso cefálico com vídeo. Resultados As taxas de respostas obtidas no potencial evocado miogênico vestibular cervical foram as seguintes: no Grupo 1 (59,3%); Grupo 2 (57,5%); Grupo 3 (35,2%); Grupo 4 (7,7%). Para o potencial evocado miogênico vestibular ocular, o percentual de resultados corretos foi: Grupo 1 (70,8%); Grupo 2 (56,0%); Grupo 3 (40,0%); Grupo 4 (14,3%). Para a prova calórica, contamos respostas normais em 88,9% do Grupo 1; 81,6% do grupo 2; 57,9% do Grupo 3; 53,3% do Grupo 4. Para o teste do impulso cefálico com vídeo, também encontramos resultados significativamente melhores no Grupo 1, seguidos pelo Grupo 2, e muito piores nos Grupos 3 e 4. Conclusão Pacientes com surdez parcial não só apresentam uma função coclear melhor, mas também melhor função vestibular, que precisa ser protegida. Em resumo, quanto melhor for a audição residual de baixa frequência, melhor a condição vestibular.

Vestibular status in partial deafness.

INTRODUCTION: The hair cells of the cochlea and the vestibulum are closely connected and may be susceptible to the same noxious factors. The relationship between their function has been a continuing field of investigation. The indications for cochlear implantation have been broadened and now include the patients with partial deafness. This raises the question of their vestibular status. OBJECTIVE: The aim of the study was to investigate whether there is any difference between the vestibular function of patients with low frequency residual hearing and those with totally deaf ears. METHODS: A total of 360 ears with profound sensorineural hearing loss were analysed before cochlear implantation. The patients were divided into four groups, according to their low frequency residual hearing (Group 1 ‒ normal or slightly elevated low frequency residual hearing; Group 2 ‒ elevated threshold but still usable hearing at low frequencies; Group 3 - non-functional residual hearing; Group 4 ‒ no detectable hearing threshold within the limits of the audiometer). The patients underwent vestibular tests: cervical vestibular evoked myogenic potential, ocular vestibular evoked myogenic potential, caloric test and video-head impulse test. RESULTS: The rates of elicited responses in cervical vestibular evoked myogenic potential were as follows: in Group 1 (59.3 %); Group 2 (57.5 %); Group 3 (35.2 %); Group 4 (7.7 %). For ocular vestibular evoked myogenic potential the percentage of correct outcomes was: Group 1 (70.8 %); Group 2 (56.0 %); Group 3 (40.0 %); Group 4 (14.3 %). For the caloric test we counted normal responses in 88.9 % of Group 1; 81.6 % of Group 2; 57.9 % of Group 3; 53.3 % of Group 4. For video-head impulse test we also found markedly better results in Group1, followed by Group 2, and much worse in Group 3 and 4. CONCLUSION: Patients with partial deafness not only have a better cochlea but also better vestibular function, which needs to be protected. In summary, the better the low frequency residual hearing, the better the vestibular status.

Does cochlear implantation influence postural stability in patients with hearing loss?

BACKGROUND: Cochlear implantation (CI) procedure carries the potential risk for vestibular system insult or stimulation with resultant dysfunction due to its proximity to the cochlea. The vestibular system plays an essential role in crucial tasks such as postural control, gaze stabilization and spatial orientation. RESEARCH QUESTION: How does standard cochlear implantation influence postural stability in patients with hearing loss? METHODS: The study included 21 individuals (age 51 ± 18 years) qualified to undergo CI due to severe or profound hearing loss. Participants were qualified for both groups by a physician based on an interview, an otoneurological examination and vestibular tests. The first group included patients without vestibular dysfunction, whereas the other group consisted of persons with vestibular dysfunction. The research methodology included medical examinations, anthropometric measurements and stabilometry on the Biodex Balance System SD (BBS) platform. The examinations were carried out twice, i.e. prior to and 3 months post implantation. The recorded data was compared between the first and the second examination using a non-parametric Wilcoxon test. The analysis of variance (ANOVA) and Tukey's post-hoc HSD unequal sample sizes were performed for patients with and without vestibular dysfunction. RESULTS AND SIGNIFICANCE: Study showed that 52.4% of the participants obtained results within the norm, while 47.6% scored below it. The comparison of stability indices of the examined individuals, with and without vestibular dysfunction, did not reveal statistically significant differences. The only difference was the anterior-posterior stability index assessed in static conditions. Three months after the implantation, no changes in the majority of indices were noted, with the exception of anterior-posterior stability index, which improved following the implantation. CI does not affect postural stability changes in the study participants.

Effect on vestibular function of cochlear implantation by partial deafness treatment-electro acoustic stimulation (PDT-EAS).

PURPOSE: Although the cochlear implantation procedure does not interfere with vestibular structures directly, both the vestibulum and the cochlea share the same inner ear fluid space, and this fluid may be responsible for transferring possibly damaging forces from one to the other. The purpose of the study is to assess postoperative vestibular function after partial deafness treatment-electro-acoustic stimulation (PDT-EAS) cochlear implantation. METHODS: Fifty-five patients were included in the study (30 females, 25 males, age 11-80, mean 41.8 ± 19.35). cVEMP and oVEMP were performed preoperatively and 1-3 months after cochlear implantation. Caloric and vHIT tests were conducted preoperatively and 4-6 months after cochlear implantation. RESULTS: Our study shows that, based on a wide range of electrodes, use of PDT-EAS is protective in terms of preserving vestibular function. It gives a rate of saccular damage of 15.79%, utricular damage of 19.04%, and a horizontal semicircular canal response reduction of 15.79%. CONCLUSIONS: PDT-EAS is protective in terms of preserving vestibular function. Nevertheless, it should be emphasized that the risk of vestibular damage cannot be totally eliminated even when hearing preservation techniques are adopted.

Evaluation of electrocardiographic parameters in patients with hearing loss genotyped for the connexin 26 gene (GJB2) mutations / Avaliação dos parâmetros eletrocardiográficos em pacientes com perda auditiva genotipada para mutações do gene conexina 26 (GJB2)

Abstract Introduction: Several studies have associated congenital sensorineural hearing loss in children with prolongation of the cardiac parameter QTc. The cause of this association is unknown. At the same time, mutations in GJB2, which encodes connexin 26, are the most common cause of congenital hearing impairment. Objective: To compare electrocardiographic parameters (PR interval, QRS complex, and QTc interval) in patients with hearing loss who were tested for mutations in GJB2 and GJB6 to investigate whether these mutations affect electrical activity of the heart. Methods: 346 patients (176 males, 170 females) with sensorineural hearing loss of 30 dB HL or more, aged 21.8 ± 19.9 years (including 147 children <14 years), underwent both genetic study for GJB2 and GJB6 mutations and electrocardiography. Results: Mutations in GJB2, including homozygotes and heterozygotes, were found in 112 (32%) patients. There were no significant differences in ECG parameters between groups of patients with and without mutations in GJB2. No differences were observed either in men (mean PR with mutation: 155 ± 16.6 vs. 153.6 ± 30.1 without; QRS: 99.9 ± 9.9 vs. 101.1 ± 15.4; QTc: 414.9 ± 29.9 vs. 412.4 ± 25.7) or women (mean PR with: 148.7 ± 21 vs. 143.8 ± 22.8 without; QRS: 94.8 ± 7.6 vs. 92.9 ± 9.6; QTc: 416.8 ± 20.6 vs. 424.9 ± 22.8). In similar fashion, we did we find any significant differences between groups of children with and without GJB2 mutations (mean PR with: 126.3 ± 19.6 vs. 127 ± 19.7 without; QRS: 80.7 ± 9.5 vs. 79.4 ± 11.6; QTc: 419.7 ± 23.5 vs. 419.8 ± 24.8). Conclusion: No association was found between the presence of GJB2 mutations encoding connexin 26 in patients with hearing loss and their ECG parameters (PR, QRS, QTc).

Resumo Introdução: Vários estudos têm associado a perda auditiva neurossensorial congênita em crianças ao prolongamento do parâmetro cardíaco QTc. A causa dessa associação é desconhecida. Ao mesmo tempo, as mutações no GJB2, que codifica a conexina 26, são a causa mais comum de deficiência auditiva congênita. Objetivo: Comparar parâmetros eletrocardiográficos (intervalo PR, complexos QRS e intervalo QTc) em pacientes com perda auditiva que foram testados para mutações no GJB2 e GJB6 para investigar se essas mutações afetam a atividade elétrica do coração. Método: Foram submetidos a estudo genético para mutações de GJB2 e GJB6 e eletrocardiograma 346 pacientes (176 homens, 170 mulheres) com perda auditiva neurossensorial de 30 dB ou mais, com média de 21,8 ± 19,9 anos (incluindo 147 crianças <14 anos). Resultados: Mutações no GJB2, inclusive homozigóticos e heterozigóticos, foram encontradas em 112 (32%) pacientes. Não houve diferenças significativas nos parâmetros de ECG entre grupos de pacientes com e sem mutações no GJB2. Não foram observadas diferenças em homens (PR médio com mutação: 155 ± 16,6 vs. 153,6 ± 30,1 sem mutação; QRS: 99,9 ± 9,9 vs. 101,1 ± 15,4; QTc: 414,9 ± 29,9 vs. 412,4 ± 25,7) nem em mulheres (PR médio com: 148,7 ± 21 vs. 143,8 ± 22,8, sem; QRS: 94,8 ± 7,6 vs. 92,9 ± 9,6; QTc: 416,8 ± 20,6 vs. 424,9 ± 22,8). Da mesma forma, encontramos diferenças significativas entre os grupos de crianças com e sem mutações de GJB2 (PR médio com: 126,3 ± 19,6 vs. 127 ± 19,7, sem; QRS: 80,7 ± 9,5 vs. 79,4 ± 11,6; QTc: 419,7 ± 23,5 vs. 419,8 ± 24,8). Conclusão: Não foi encontrada associação entre a presença de mutações de GJB2 que codificam conexina 26 em pacientes com perda auditiva e seus parâmetros de ECG (PR, QRS, QTc).

Evaluation of electrocardiographic parameters in patients with hearing loss genotyped for the connexin 26 gene (GJB2) mutations.

INTRODUCTION: Several studies have associated congenital sensorineural hearing loss in children with prolongation of the cardiac parameter QTc. The cause of this association is unknown. At the same time, mutations in GJB2, which encodes connexin 26, are the most common cause of congenital hearing impairment. OBJECTIVE: To compare electrocardiographic parameters (PR interval, QRS complex, and QTc interval) in patients with hearing loss who were tested for mutations in GJB2 and GJB6 to investigate whether these mutations affect electrical activity of the heart. METHODS: 346 patients (176 males, 170 females) with sensorineural hearing loss of 30dB HL or more, aged 21.8±19.9 years (including 147 children <14 years), underwent both genetic study for GJB2 and GJB6 mutations and electrocardiography. RESULTS: Mutations in GJB2, including homozygotes and heterozygotes, were found in 112 (32%) patients. There were no significant differences in ECG parameters between groups of patients with and without mutations in GJB2. No differences were observed either in men (mean PR with mutation: 155±16.6 vs. 153.6±30.1 without; QRS: 99.9±9.9 vs. 101.1±15.4; QTc: 414.9±29.9 vs. 412.4±25.7) or women (mean PR with: 148.7±21 vs. 143.8±22.8 without; QRS: 94.8±7.6 vs. 92.9±9.6; QTc: 416.8±20.6 vs. 424.9±22.8). In similar fashion, we did we find any significant differences between groups of children with and without GJB2 mutations (mean PR with: 126.3±19.6 vs. 127±19.7 without; QRS: 80.7±9.5 vs. 79.4±11.6; QTc: 419.7±23.5 vs. 419.8±24.8). CONCLUSION: No association was found between the presence of GJB2 mutations encoding connexin 26 in patients with hearing loss and their ECG parameters (PR, QRS, QTc).

QTc prolongation in patients with hearing loss: Electrocardiographic and genetic study.

BACKGROUND: The aim of the study was to determine, whether electrocardiogram (ECG) screening could reduce the risk of sudden cardiac death in patients with hearing loss through the early diagnosis of Jervell and Lange-Nielsen syndrome and the introduction of the therapy. METHODS: One thousand and eighty patients with hearing loss (aged 21.8 ± 19.9 years) underwent ECG. Additionally, all subjects were asked to complete a 3-question survey. Those who met, at least, one of the high-risk criteria underwent further cardiac assessment and genetic testing. RESULTS: QTc assessment was possible in 1,027 patients. Mean QTc measured 422.8 ± 23.7 ms in 313 women, 414.9 ± 27.7 ms in 273 men and 421.1 ± 21.5 ms in 441 children (individuals younger than 14 years). Abnormal QTc was found in 13 (4.1%) women, 20 (7.3%) men, and 72 (16.3%) children. In the studied group, no recessive mutation of KNCQ1 or KCNE1 was found. In 6 patients, other mutations were found: in KCNQ1 (n = 1), in KCNH2 (n = 3) and in SCN5A (n = 1), which were pathogenic for long-QT-syndromes (LQTS), and 2 mutations of unknown clinical significance in SCN5A. Overall, out of these 6 patients LQTS was diagnosed in 3 asymptomatic patients, but with abnormal QTc and in 2 patients with normal QTc, but who were previously treated for epilepsy. CONCLUSIONS: Jervell and Lange-Nielsen syndrome is a very rare condition even in a population with hearing loss. In this population, the prevalence of prolonged QT interval is increased over the general population. Further investigations are necessary.