Research Article
BibTex RIS Cite

Speech Perception Outcomes after Cochlear Implantation in Children with GJB2/DFNB1 associated Deafness

Year 2014, , 60 - 63, 07.08.2014
https://doi.org/10.5152/balkanmedj.2014.9535

Abstract

Background: Cochlear implants (CI) for the rehabilitation of patients with profound or total bilateral sensorineural hypoacusis represent the initial use of electrical fields to provide audibility in cases where the use of sound amplifiers does not provide satisfactory results. Aims: To compare speech perception performance after cochlear implantation in children with connexin 26-associated deafness with that of a control group of children with deafness of unknown etiology. Study Design: Retrospective comparative study. Methods: During the period from 2006 to , cochlear implantation was performed on 26 children. Eighteen of these children had undergone genetic tests for mutation of the Gap Junction Protein Beta 2 (GJB2) gene. Bi-allelic GJB2 mutations were confirmed in 7 out of 18 examined children. In order to confirm whether genetic factors have influence on speech perception after cochlear implantation, we compared the post-implantation speech performance of seven children with mutations of the GBJ2 (connexin 26) gene with seven other children who had the wild type version of this particular gene. The latter were carefully matched according to the age at cochlear implantation. Speech perception performance was measured before cochlear implantation, and one and two years after implantation. All the patients were arranged in line with the appropriate speech perception category (SPC). Non-parametric tests, Friedman ANOVA and Mann-Whitney's U test were used for statistical analysis. Results: Both groups showed similar improvements in speech perception scores after cochlear implantation. Statistical analysis did not confirm significant differences between the groups 12 and 24 months after cochlear implantation. Conclusion: The results obtained in this study showed an absence of apparent distinctions in the scores of speech perception between the two examined groups and therefore might have significant implications in selecting prognostic indicators of speech perception following cochlear implantation.

References

  • Morton NE. Genetic epidemiology of hearing impairment. Ann NY Acad Sci 1991;630:16-31. [CrossRef]
  • Mason JA, Herrmann KR. Universal hearing screening by automated auditory brainstem response measurement. Pediatrics 1998;101:221-8. [CrossRef]
  • Van Camp G, Willems PJ, Smith RJ. Non-syndromic hearing impairment: unparalleled heterogeneity. Am J Hum Genet 1997;60:758-64.
  • Van Camp, Smith RJH. Hereditary Hearing Loss. Available from: http:// webhost.ua.ac.be/hhh/
  • Zelenate L, Gasparini P, Estivill X, Melchionda S, D’Agruma I, Govea N, et al. Connexin 26 mutations associated with the common form of non-syndromic neurosensory autosomal recessive deafness (DFNB1) in Mediterranean. Hum Mol Gent 1997;6:1605-9. [CrossRef]
  • Cohn ES, Kelley PM. Clinical phenotype and mutations in connexin 26 (DFNB1/GJB2), the most common cause of childhood hearing loss. Am J Genet 1999;89:130-6. [CrossRef]
  • Estivill X, Fortina P, Surrey S, Rabionet R, Meichionda S, D’Agruma L, et al. Connexin-26 mutations in sporadic and inherited sensorineural hearing deafness. Lancet 1998;351:394-8. [CrossRef]
  • Schrijver I. Hereditary Non-Syndromic Sensorineural Hearing Loss. J Mol Diagn 2004;6:275-84. [CrossRef]
  • Sukarova-Stefanovska E, Momirovska A, Davcheva-Chakar M, Efremov G. GJB2 mutation in non-syndromic hearing loss in the Republic of Macedonia. BJMG 2009;12:11-6.
  • Sukarova-Stefanovska E, Cakar M, Filipce I, Plaseska-Karanfilska D. Genetics of non-syndromic hearing loss in Republic of Macedonia. BJMG 2012;Suppl 15:57-9.
  • Clark GM. Cochlear implants. Bologna: Monduzzi Editore, 1977.
  • Francis HW, Pulsifer MB, Chinnici J, Nutt R, Venick HS, Yeagle JD, et al. Effects of central nervous system residua on cochlear implant results in children deafened by meningitis. Arch Otolaryngol Head Neck Surg 2004;130:604-11. [CrossRef]
  • Taitelbaum-Swead R, Brownstein Z, Muchnik C, Kishon-Rabin L, Kronenberg J, Megirov L, et al. Connexin associated deafness and speech perception after cochlear implantation. Arch Otolaryngol Head Neck Surg 2006;132:495-500. [CrossRef]
  • Black J, Hickson L, Black B, Perry C. Prognostic indicators in pediatric cochlear implant surgery: a systematic literature review. Cochlear implans Int 2011;12:67-93. [CrossRef]
  • Wiley S, Choo D, Meinzen-Deer J, Hilbert L, Greinwald J. GJB2 mutations and additional disabilities in a pediatric cochlear Implant population. Int J Pediatr Otorhinolaryngol 2006;70:493-500. [CrossRef]
  • Geers AE, Moog JS. Predicting spoken language acquisition of profoundly hearing -impaired children. J Speech Hear Disorders 1987;52:84-94.
  • Dahl HH, Wake M, Sarant J, Poulakis, Siemering K, Blamey P. Language and Speech perception outcomes in hearing -impaired children with and without connexin 26 mutations. Audiol Neurootol 2003;8:263-8. [CrossRef]
  • Cullen RD, Buchman CA, Brown CJ, Copeland BJ, Zdanski C, Pillsbury HC 3rd, et al. Cochlear implantation for children with GJB2- related deafness. Laryngoscope 2004;114:1415-9. [CrossRef]
  • Dalamon V, Lotersztein V, Lipovsek M, Beheran A, Mondino ME, Diamante F, et al. Performance of the speech perception after cochlear implantation in DFNB1 patients. Acta Otolaryngol 2009;129:395-8. [CrossRef]
  • Wrobel M, Magierska-Krzyszton M, Szyfter K, Mietkiewska D, Szyfter W, Rydzanicz M, et al. Comparison of rehabilitation results in deaf patients with and without genetically related hearing loss. Cochlear Implants Int 2008;9:132-42. [CrossRef]
  • Bauer PW, Geers AE, Brenner C, Moog JS, Smith RJ. The Effect of GJB2 allele variants on performance after cochlear implantation. Laryngoscope 2003;113:2135-40.[CrossRef]
  • Liu J, Yu F, Dai P, Han DY, Yang SM, Wang GJ, et al. Mutation of Gap junction protein beta 2 gene and treatment outcome of cochlear implantation in Cochlear implantation recipients. Zhonghua Yi Xue Za Zhi 2009;89:433-7.
  • Yoshida H, Takahashi H, Kanda Y, Usami S. Long term speech perception after cochlear implant in pediatric patients with GJB2 mutations. Auris Nasus Larynx 2013;40:435-9. [CrossRef]
  • Fukushima K, Sugata K, Kasai N, Fukuda S, Nagayasu R, Toida N, et al. Better speech performance in cochlear implant patients with GBJ2-related deafness. Int J Pediatr Otorinolaryngol 2002;62:151-7. [CrossRef]
  • Connell SS, Angeli SI, Suarez H, Hodges AV, Balkany TJ, Liu HZ, et al. Performance after cochlear implantation in DFNB1 patients. Otolaryngol Head Neck Surgery 2007;137:596-602. [CrossRef]
  • Sinnathuray AR, Toner JG, Clarke-Lytlle J, Geddis A, Patterson CC, Hughs AE, et al. Connexin 26 (GBJ2) gene-related deafness and speech intelligibility after cochlear implantation. Otol Neurotol 2004;25:935-42. [CrossRef]
  • Chora JR, Matos TD, Martins JH, Alves MC, Andrade SM, Silva LF, et al. DFNB1-associared deafness in Portuguese cochlear implant users: prevalence and impact on oral communication. Int J Pediatr Otorhinolaryngol 2010;74:1136-9. [CrossRef]

Speech Perception Outcomes after Cochlear Implantation in Children with GJB2/DFNB1 associated Deafness

Year 2014, , 60 - 63, 07.08.2014
https://doi.org/10.5152/balkanmedj.2014.9535

Abstract

References

  • Morton NE. Genetic epidemiology of hearing impairment. Ann NY Acad Sci 1991;630:16-31. [CrossRef]
  • Mason JA, Herrmann KR. Universal hearing screening by automated auditory brainstem response measurement. Pediatrics 1998;101:221-8. [CrossRef]
  • Van Camp G, Willems PJ, Smith RJ. Non-syndromic hearing impairment: unparalleled heterogeneity. Am J Hum Genet 1997;60:758-64.
  • Van Camp, Smith RJH. Hereditary Hearing Loss. Available from: http:// webhost.ua.ac.be/hhh/
  • Zelenate L, Gasparini P, Estivill X, Melchionda S, D’Agruma I, Govea N, et al. Connexin 26 mutations associated with the common form of non-syndromic neurosensory autosomal recessive deafness (DFNB1) in Mediterranean. Hum Mol Gent 1997;6:1605-9. [CrossRef]
  • Cohn ES, Kelley PM. Clinical phenotype and mutations in connexin 26 (DFNB1/GJB2), the most common cause of childhood hearing loss. Am J Genet 1999;89:130-6. [CrossRef]
  • Estivill X, Fortina P, Surrey S, Rabionet R, Meichionda S, D’Agruma L, et al. Connexin-26 mutations in sporadic and inherited sensorineural hearing deafness. Lancet 1998;351:394-8. [CrossRef]
  • Schrijver I. Hereditary Non-Syndromic Sensorineural Hearing Loss. J Mol Diagn 2004;6:275-84. [CrossRef]
  • Sukarova-Stefanovska E, Momirovska A, Davcheva-Chakar M, Efremov G. GJB2 mutation in non-syndromic hearing loss in the Republic of Macedonia. BJMG 2009;12:11-6.
  • Sukarova-Stefanovska E, Cakar M, Filipce I, Plaseska-Karanfilska D. Genetics of non-syndromic hearing loss in Republic of Macedonia. BJMG 2012;Suppl 15:57-9.
  • Clark GM. Cochlear implants. Bologna: Monduzzi Editore, 1977.
  • Francis HW, Pulsifer MB, Chinnici J, Nutt R, Venick HS, Yeagle JD, et al. Effects of central nervous system residua on cochlear implant results in children deafened by meningitis. Arch Otolaryngol Head Neck Surg 2004;130:604-11. [CrossRef]
  • Taitelbaum-Swead R, Brownstein Z, Muchnik C, Kishon-Rabin L, Kronenberg J, Megirov L, et al. Connexin associated deafness and speech perception after cochlear implantation. Arch Otolaryngol Head Neck Surg 2006;132:495-500. [CrossRef]
  • Black J, Hickson L, Black B, Perry C. Prognostic indicators in pediatric cochlear implant surgery: a systematic literature review. Cochlear implans Int 2011;12:67-93. [CrossRef]
  • Wiley S, Choo D, Meinzen-Deer J, Hilbert L, Greinwald J. GJB2 mutations and additional disabilities in a pediatric cochlear Implant population. Int J Pediatr Otorhinolaryngol 2006;70:493-500. [CrossRef]
  • Geers AE, Moog JS. Predicting spoken language acquisition of profoundly hearing -impaired children. J Speech Hear Disorders 1987;52:84-94.
  • Dahl HH, Wake M, Sarant J, Poulakis, Siemering K, Blamey P. Language and Speech perception outcomes in hearing -impaired children with and without connexin 26 mutations. Audiol Neurootol 2003;8:263-8. [CrossRef]
  • Cullen RD, Buchman CA, Brown CJ, Copeland BJ, Zdanski C, Pillsbury HC 3rd, et al. Cochlear implantation for children with GJB2- related deafness. Laryngoscope 2004;114:1415-9. [CrossRef]
  • Dalamon V, Lotersztein V, Lipovsek M, Beheran A, Mondino ME, Diamante F, et al. Performance of the speech perception after cochlear implantation in DFNB1 patients. Acta Otolaryngol 2009;129:395-8. [CrossRef]
  • Wrobel M, Magierska-Krzyszton M, Szyfter K, Mietkiewska D, Szyfter W, Rydzanicz M, et al. Comparison of rehabilitation results in deaf patients with and without genetically related hearing loss. Cochlear Implants Int 2008;9:132-42. [CrossRef]
  • Bauer PW, Geers AE, Brenner C, Moog JS, Smith RJ. The Effect of GJB2 allele variants on performance after cochlear implantation. Laryngoscope 2003;113:2135-40.[CrossRef]
  • Liu J, Yu F, Dai P, Han DY, Yang SM, Wang GJ, et al. Mutation of Gap junction protein beta 2 gene and treatment outcome of cochlear implantation in Cochlear implantation recipients. Zhonghua Yi Xue Za Zhi 2009;89:433-7.
  • Yoshida H, Takahashi H, Kanda Y, Usami S. Long term speech perception after cochlear implant in pediatric patients with GJB2 mutations. Auris Nasus Larynx 2013;40:435-9. [CrossRef]
  • Fukushima K, Sugata K, Kasai N, Fukuda S, Nagayasu R, Toida N, et al. Better speech performance in cochlear implant patients with GBJ2-related deafness. Int J Pediatr Otorinolaryngol 2002;62:151-7. [CrossRef]
  • Connell SS, Angeli SI, Suarez H, Hodges AV, Balkany TJ, Liu HZ, et al. Performance after cochlear implantation in DFNB1 patients. Otolaryngol Head Neck Surgery 2007;137:596-602. [CrossRef]
  • Sinnathuray AR, Toner JG, Clarke-Lytlle J, Geddis A, Patterson CC, Hughs AE, et al. Connexin 26 (GBJ2) gene-related deafness and speech intelligibility after cochlear implantation. Otol Neurotol 2004;25:935-42. [CrossRef]
  • Chora JR, Matos TD, Martins JH, Alves MC, Andrade SM, Silva LF, et al. DFNB1-associared deafness in Portuguese cochlear implant users: prevalence and impact on oral communication. Int J Pediatr Otorhinolaryngol 2010;74:1136-9. [CrossRef]
There are 27 citations in total.

Details

Primary Language English
Subjects Health Care Administration
Journal Section Articles
Authors

Marina Davcheva-chakar This is me

Emilija Sukarova-stefanovska This is me

Valentina Ivanovska This is me

Vesna Lazarevska This is me

İlija Filipche This is me

Beti Zafirovska This is me

Publication Date August 7, 2014
Published in Issue Year 2014

Cite

APA Davcheva-chakar, M., Sukarova-stefanovska, E., Ivanovska, V., Lazarevska, V., et al. (2014). Speech Perception Outcomes after Cochlear Implantation in Children with GJB2/DFNB1 associated Deafness. Balkan Medical Journal, 2014(1), 60-63. https://doi.org/10.5152/balkanmedj.2014.9535
AMA Davcheva-chakar M, Sukarova-stefanovska E, Ivanovska V, Lazarevska V, Filipche İ, Zafirovska B. Speech Perception Outcomes after Cochlear Implantation in Children with GJB2/DFNB1 associated Deafness. Balkan Medical Journal. January 2014;2014(1):60-63. doi:10.5152/balkanmedj.2014.9535
Chicago Davcheva-chakar, Marina, Emilija Sukarova-stefanovska, Valentina Ivanovska, Vesna Lazarevska, İlija Filipche, and Beti Zafirovska. “Speech Perception Outcomes After Cochlear Implantation in Children With GJB2/DFNB1 Associated Deafness”. Balkan Medical Journal 2014, no. 1 (January 2014): 60-63. https://doi.org/10.5152/balkanmedj.2014.9535.
EndNote Davcheva-chakar M, Sukarova-stefanovska E, Ivanovska V, Lazarevska V, Filipche İ, Zafirovska B (January 1, 2014) Speech Perception Outcomes after Cochlear Implantation in Children with GJB2/DFNB1 associated Deafness. Balkan Medical Journal 2014 1 60–63.
IEEE M. Davcheva-chakar, E. Sukarova-stefanovska, V. Ivanovska, V. Lazarevska, İ. Filipche, and B. Zafirovska, “Speech Perception Outcomes after Cochlear Implantation in Children with GJB2/DFNB1 associated Deafness”, Balkan Medical Journal, vol. 2014, no. 1, pp. 60–63, 2014, doi: 10.5152/balkanmedj.2014.9535.
ISNAD Davcheva-chakar, Marina et al. “Speech Perception Outcomes After Cochlear Implantation in Children With GJB2/DFNB1 Associated Deafness”. Balkan Medical Journal 2014/1 (January 2014), 60-63. https://doi.org/10.5152/balkanmedj.2014.9535.
JAMA Davcheva-chakar M, Sukarova-stefanovska E, Ivanovska V, Lazarevska V, Filipche İ, Zafirovska B. Speech Perception Outcomes after Cochlear Implantation in Children with GJB2/DFNB1 associated Deafness. Balkan Medical Journal. 2014;2014:60–63.
MLA Davcheva-chakar, Marina et al. “Speech Perception Outcomes After Cochlear Implantation in Children With GJB2/DFNB1 Associated Deafness”. Balkan Medical Journal, vol. 2014, no. 1, 2014, pp. 60-63, doi:10.5152/balkanmedj.2014.9535.
Vancouver Davcheva-chakar M, Sukarova-stefanovska E, Ivanovska V, Lazarevska V, Filipche İ, Zafirovska B. Speech Perception Outcomes after Cochlear Implantation in Children with GJB2/DFNB1 associated Deafness. Balkan Medical Journal. 2014;2014(1):60-3.