Cochlear Implants in Children with Inner Ear Malformations, A Review of Current Literature
Main Article Content
Abstract
Cochlear implants (CIs) are a key option of hearing rehabilitation in certain children. Scientific, surgical and technological advances in CI technology have enabled implantation in a significant number of children with sensorineural hearing loss (SNHL). There are established criteria to characterize appropriate patients, improving successful performance post operatively by standard metrics of speech perception. Clinical outcomes are, however, modified by several patient-related. An increasing body of evidence in such domains has resulted in expansion of candidacy criteria. Approximately 20% of all congenital hearing loss is associated with inner ear malformations (IEMs). Implantation of such children was associated with uncertain surgical and clinical success, and hence was limited in the early years of CI surgery. To date, the literature regarding outcomes in this special population has varied. Studies have been mixed when comparing children with IEMs to those with normal anatomy regarding success with CI. Results may be related to the type and severity of IEM. There may also be differences in children with congenital deafness versus progressive hearing loss. The current data are limited as there is not a standardized testing paradigm for evaluation in children. The variability in the data suggests further research is required to fully understand the nuances in management of these complex children. The goal of this review is to discuss surgical management and outcomes of children who meet criteria for CI in the setting of an IEM.
Article Details
How to Cite
TEPLITZKY, Taylor B.; LEE, Kenneth H..
Cochlear Implants in Children with Inner Ear Malformations, A Review of Current Literature.
Medical Research Archives, [S.l.], v. 10, n. 5, june 2022.
ISSN 2375-1924.
Available at: <https://esmed.org/MRA/mra/article/view/2845>. Date accessed: 26 apr. 2024.
doi: https://doi.org/10.18103/mra.v10i5.2845.
Section
Review Articles
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
References
1. McConkey Robbins A, Koch DB, Osberger MJ, Zimmerman-Phillips S, Kishon-Rabin L. Effect of age at cochlear implantation on auditory skill development in infants and toddlers. Arch Otolaryngol Head Neck Surg. 2004;130(5):570-574. doi:10.1001/archotol.130.5.570
2. Nikolopoulos TP, O’Donoghue GM, Archbold S. Age at implantation: its importance in pediatric cochlear implantation. The Laryngoscope. 1999;109(4):595-599. doi:10.1097/00005537-199904000-00014
3. Papsin BC. Cochlear implantation in children with anomalous cochleovestibular anatomy. The Laryngoscope. 2005;115(1 Pt 2 Suppl 106):1-26. doi:10.1097/00005537-200501001-00001
4. Teagle HFB, Roush PA, Woodard JS, et al. Cochlear implantation in children with auditory neuropathy spectrum disorder. Ear Hear. 2010;31(3):325-335. doi:10.1097/AUD.0b013e3181ce693b
5. Berrettini S, Forli F, Genovese E, et al. Cochlear implantation in deaf children with associated disabilities: challenges and outcomes. Int J Audiol. 2008;47(4):199-208. doi:10.1080/14992020701870197
6. Spahn C, Richter B, Zschocke I, Löhle E, Wirsching M. The need for psychosocial support in parents with cochlear implanted children. Int J Pediatr Otorhinolaryngol. 2001;57(1):45-53. doi:10.1016/s0165-5876(00)00438-9
7. Geers A, Brenner C, Nicholas J, Uchanski R, Tye-Murray N, Tobey E. Rehabilitation factors contributing to implant benefit in children. Ann Otol Rhinol Laryngol Suppl. 2002;189:127-130. doi:10.1177/00034894021110s525
8. Park AH, Kou B, Hotaling A, Azar-Kia B, Leonetti J, Papsin B. Clinical course of pediatric congenital inner ear malformations. The Laryngoscope. 2000;110(10 Pt 1):1715-1719. doi:10.1097/00005537-200010000-00029
9. Luxford WM, House WF. Cochlear implants in children: medical and surgical considerations. Ear Hear. 1985;6(3 Suppl):20S-23S. doi:10.1097/00003446-198505001-00005
10. Clark GM, Blamey PJ, Busby PA, et al. A multiple-electrode intracochlear implant for children. Arch Otolaryngol Head Neck Surg. 1987;113(8):825-828. doi:10.1001/archotol.1987.01860080031010
11. Buchman CA, Copeland BJ, Yu KK, Brown CJ, Carrasco VN, Pillsbury HC. Cochlear implantation in children with congenital inner ear malformations. The Laryngoscope. 2004;114(2):309-316. doi:10.1097/00005537-200402000-00025
12. Aldhafeeri AM, Alsanosi AA. Management of surgical difficulties during cochlear implant with inner ear anomalies. Int J Pediatr Otorhinolaryngol. 2017;92:45-49. doi:10.1016/j.ijporl.2016.11.001
13. Pakdaman MN, Herrmann BS, Curtin HD, Van Beek-King J, Lee DJ. Cochlear implantation in children with anomalous cochleovestibular anatomy: a systematic review. Otolaryngol--Head Neck Surg Off J Am Acad Otolaryngol-Head Neck Surg. 2012;146(2):180-190. doi:10.1177/0194599811429244
14. Isaiah A, Lee D, Lenes-Voit F, et al. Clinical outcomes following cochlear implantation in children with inner ear anomalies. Int J Pediatr Otorhinolaryngol. 2017;93:1-6. doi:10.1016/j.ijporl.2016.12.001
15. Slattery WH, Luxford WM. Cochlear implantation in the congenital malformed cochlea. The Laryngoscope. 1995;105(11):1184-1187. doi:10.1288/00005537-199511000-00008
16. Hoffman RA, Downey LL, Waltzman SB, Cohen NL. Cochlear implantation in children with cochlear malformations. Am J Otol. 1997;18(2):184-187.
17. Weber BP, Dillo W, Dietrich B, Maneke I, Bertram B, Lenarz T. Pediatric cochlear implantation in cochlear malformations. Am J Otol. 1998;19(6):747-753.
18. Hashemi SB, Bozorgi H, Kazemi T, Babaei A. Cerebrospinal fluid gusher in cochlear implant and its associated factors. Acta Otolaryngol (Stockh). 2020;140(8):621-625. doi:10.1080/00016489.2020.1751276
19. Sennaroglu L. Cochlear implantation in inner ear malformations--a review article. Cochlear Implants Int. 2010;11(1):4-41. doi:10.1002/cii.416
20. Wootten CT, Backous DD, Haynes DS. Management of cerebrospinal fluid leakage from cochleostomy during cochlear implant surgery. The Laryngoscope. 2006;116(11):2055-2059. doi:10.1097/01.mlg.0000240286.43289.87
21. Ying YLM, Lin JW, Oghalai JS, Williamson RA. Cochlear Implant Electrode Misplacement: Incidence, Evaluation, and Management. The Laryngoscope. 2013;123(3):757-766. doi:10.1002/lary.23665
22. Al-Mahboob A, Alhabib SF, Abdelsamad Y, Alzhrani F. Cochlear implantation in common cavity deformity: a systematic review. Eur Arch Oto-Rhino-Laryngol Off J Eur Fed Oto-Rhino-Laryngol Soc EUFOS Affil Ger Soc Oto-Rhino-Laryngol - Head Neck Surg. 2022;279(1):37-48. doi:10.1007/s00405-021-06884-5
23. Sharma SD, Cushing SL, Papsin BC, Gordon KA. Hearing and speech benefits of cochlear implantation in children: A review of the literature. Int J Pediatr Otorhinolaryngol. 2020;133:109984. doi:10.1016/j.ijporl.2020.109984
24. Park LR, Perkins EL, Woodard JS, Brown KD. Delaying Cochlear Implantation Impacts Postoperative Speech Perception of Nontraditional Pediatric Candidates. Audiol Neurootol. 2021;26(3):182-187. doi:10.1159/000510693
25. Kocabay AP, Cinar BC, Batuk MO, Yarali M, Sennaroglu G. Pediatric cochlear implant fitting parameters in inner ear malformation: Is it same with normal cochlea? Int J Pediatr Otorhinolaryngol. 2022;155:111084. doi:10.1016/j.ijporl.2022.111084
26. Dowell RC, Dettman SJ, Blamey PJ, Barker EJ, Clark GM. Speech perception in children using cochlear implants: prediction of long-term outcomes. Cochlear Implants Int. 2002;3(1):1-18. doi:10.1179/cim.2002.3.1.1
27. Waltzman SB, Roland JT, Cohen NL. Delayed implantation in congenitally deaf children and adults. Otol Neurotol Off Publ Am Otol Soc Am Neurotol Soc Eur Acad Otol Neurotol. 2002;23(3):333-340. doi:10.1097/00129492-200205000-00018
28. Peixoto MC, Spratley J, Oliveira G, Martins J, Bastos J, Ribeiro C. Effectiveness of cochlear implants in children: long term results. Int J Pediatr Otorhinolaryngol. 2013;77(4):462-468. doi:10.1016/j.ijporl.2012.12.005
29. Eisenman DJ, Ashbaugh C, Zwolan TA, Arts HA, Telian SA. Implantation of the malformed cochlea. Otol Neurotol Off Publ Am Otol Soc Am Neurotol Soc Eur Acad Otol Neurotol. 2001;22(6):834-841. doi:10.1097/00129492-200111000-00020
30. Loundon N, Rouillon I, Munier N, Marlin S, Roger G, Garabedian EN. Cochlear implantation in children with internal ear malformations. Otol Neurotol Off Publ Am Otol Soc Am Neurotol Soc Eur Acad Otol Neurotol. 2005;26(4):668-673. doi:10.1097/01.mao.0000178126.58859.a9
31. Rachovitsas D, Psillas G, Chatzigiannakidou V, Triaridis S, Constantinidis J, Vital V. Speech perception and production in children with inner ear malformations after cochlear implantation. Int J Pediatr Otorhinolaryngol. 2012;76(9):1370-1374. doi:10.1016/j.ijporl.2012.06.009
32. Melo AS, Martins J, Silva J, Quadros J, Paiva A. Cochlear implantation in children with anomalous cochleovestibular anatomy. Auris Nasus Larynx. 2017;44(5):509-516. doi:10.1016/j.anl.2017.02.003
33. Celik M, Karatas E, Kanlikama M. Outcomes of cochlear implantation in children with and without inner ear malformations. Pak J Med Sci. 2018;34(2):380-384. doi:10.12669/pjms.342.14066
34. Kamogashira T, Akamatsu Y, Kashio A, et al. Development of auditory skills after cochlear implantation in children with inner ear malformations. Acta Otolaryngol (Stockh). 2016;136(1):78-82. doi:10.3109/00016489.2015.1087047
35. Demir B, Cesur S, Sahin A, Binnetoglu A, Ciprut A, Batman C. Outcomes of cochlear implantation in children with inner ear malformations. Eur Arch Oto-Rhino-Laryngol Off J Eur Fed Oto-Rhino-Laryngol Soc EUFOS Affil Ger Soc Oto-Rhino-Laryngol - Head Neck Surg. 2019;276(9):2397-2403. doi:10.1007/s00405-019-05475-9
36. Dettman S, Sadeghi-Barzalighi A, Ambett R, Dowell R, Trotter M, Briggs R. Cochlear implants in forty-eight children with cochlear and/or vestibular abnormality. Audiol Neurootol. 2011;16(4):222-232. doi:10.1159/000320608
37. Catli T, Uckan B, Olgun L. Speech and language development after cochlear implantation in children with bony labyrinth malformations: long-term results. Eur Arch Oto-Rhino-Laryngol Off J Eur Fed Oto-Rhino-Laryngol Soc EUFOS Affil Ger Soc Oto-Rhino-Laryngol - Head Neck Surg. 2015;272(11):3131-3136. doi:10.1007/s00405-014-3319-5
38. Daneshi A, Farhadi M, Ajalloueyan M, et al. Cochlear implantation in children with inner ear malformation: A multicenter study on auditory performance and speech production outcomes. Int J Pediatr Otorhinolaryngol. 2020;132:109901. doi:10.1016/j.ijporl.2020.109901
39. Farhood Z, Nguyen SA, Miller SC, Holcomb MA, Meyer TA, Rizk HG. Cochlear Implantation in Inner Ear Malformations: Systematic Review of Speech Perception Outcomes and Intraoperative Findings. Otolaryngol--Head Neck Surg Off J Am Acad Otolaryngol-Head Neck Surg. 2017;156(5):783-793. doi:10.1177/0194599817696502
2. Nikolopoulos TP, O’Donoghue GM, Archbold S. Age at implantation: its importance in pediatric cochlear implantation. The Laryngoscope. 1999;109(4):595-599. doi:10.1097/00005537-199904000-00014
3. Papsin BC. Cochlear implantation in children with anomalous cochleovestibular anatomy. The Laryngoscope. 2005;115(1 Pt 2 Suppl 106):1-26. doi:10.1097/00005537-200501001-00001
4. Teagle HFB, Roush PA, Woodard JS, et al. Cochlear implantation in children with auditory neuropathy spectrum disorder. Ear Hear. 2010;31(3):325-335. doi:10.1097/AUD.0b013e3181ce693b
5. Berrettini S, Forli F, Genovese E, et al. Cochlear implantation in deaf children with associated disabilities: challenges and outcomes. Int J Audiol. 2008;47(4):199-208. doi:10.1080/14992020701870197
6. Spahn C, Richter B, Zschocke I, Löhle E, Wirsching M. The need for psychosocial support in parents with cochlear implanted children. Int J Pediatr Otorhinolaryngol. 2001;57(1):45-53. doi:10.1016/s0165-5876(00)00438-9
7. Geers A, Brenner C, Nicholas J, Uchanski R, Tye-Murray N, Tobey E. Rehabilitation factors contributing to implant benefit in children. Ann Otol Rhinol Laryngol Suppl. 2002;189:127-130. doi:10.1177/00034894021110s525
8. Park AH, Kou B, Hotaling A, Azar-Kia B, Leonetti J, Papsin B. Clinical course of pediatric congenital inner ear malformations. The Laryngoscope. 2000;110(10 Pt 1):1715-1719. doi:10.1097/00005537-200010000-00029
9. Luxford WM, House WF. Cochlear implants in children: medical and surgical considerations. Ear Hear. 1985;6(3 Suppl):20S-23S. doi:10.1097/00003446-198505001-00005
10. Clark GM, Blamey PJ, Busby PA, et al. A multiple-electrode intracochlear implant for children. Arch Otolaryngol Head Neck Surg. 1987;113(8):825-828. doi:10.1001/archotol.1987.01860080031010
11. Buchman CA, Copeland BJ, Yu KK, Brown CJ, Carrasco VN, Pillsbury HC. Cochlear implantation in children with congenital inner ear malformations. The Laryngoscope. 2004;114(2):309-316. doi:10.1097/00005537-200402000-00025
12. Aldhafeeri AM, Alsanosi AA. Management of surgical difficulties during cochlear implant with inner ear anomalies. Int J Pediatr Otorhinolaryngol. 2017;92:45-49. doi:10.1016/j.ijporl.2016.11.001
13. Pakdaman MN, Herrmann BS, Curtin HD, Van Beek-King J, Lee DJ. Cochlear implantation in children with anomalous cochleovestibular anatomy: a systematic review. Otolaryngol--Head Neck Surg Off J Am Acad Otolaryngol-Head Neck Surg. 2012;146(2):180-190. doi:10.1177/0194599811429244
14. Isaiah A, Lee D, Lenes-Voit F, et al. Clinical outcomes following cochlear implantation in children with inner ear anomalies. Int J Pediatr Otorhinolaryngol. 2017;93:1-6. doi:10.1016/j.ijporl.2016.12.001
15. Slattery WH, Luxford WM. Cochlear implantation in the congenital malformed cochlea. The Laryngoscope. 1995;105(11):1184-1187. doi:10.1288/00005537-199511000-00008
16. Hoffman RA, Downey LL, Waltzman SB, Cohen NL. Cochlear implantation in children with cochlear malformations. Am J Otol. 1997;18(2):184-187.
17. Weber BP, Dillo W, Dietrich B, Maneke I, Bertram B, Lenarz T. Pediatric cochlear implantation in cochlear malformations. Am J Otol. 1998;19(6):747-753.
18. Hashemi SB, Bozorgi H, Kazemi T, Babaei A. Cerebrospinal fluid gusher in cochlear implant and its associated factors. Acta Otolaryngol (Stockh). 2020;140(8):621-625. doi:10.1080/00016489.2020.1751276
19. Sennaroglu L. Cochlear implantation in inner ear malformations--a review article. Cochlear Implants Int. 2010;11(1):4-41. doi:10.1002/cii.416
20. Wootten CT, Backous DD, Haynes DS. Management of cerebrospinal fluid leakage from cochleostomy during cochlear implant surgery. The Laryngoscope. 2006;116(11):2055-2059. doi:10.1097/01.mlg.0000240286.43289.87
21. Ying YLM, Lin JW, Oghalai JS, Williamson RA. Cochlear Implant Electrode Misplacement: Incidence, Evaluation, and Management. The Laryngoscope. 2013;123(3):757-766. doi:10.1002/lary.23665
22. Al-Mahboob A, Alhabib SF, Abdelsamad Y, Alzhrani F. Cochlear implantation in common cavity deformity: a systematic review. Eur Arch Oto-Rhino-Laryngol Off J Eur Fed Oto-Rhino-Laryngol Soc EUFOS Affil Ger Soc Oto-Rhino-Laryngol - Head Neck Surg. 2022;279(1):37-48. doi:10.1007/s00405-021-06884-5
23. Sharma SD, Cushing SL, Papsin BC, Gordon KA. Hearing and speech benefits of cochlear implantation in children: A review of the literature. Int J Pediatr Otorhinolaryngol. 2020;133:109984. doi:10.1016/j.ijporl.2020.109984
24. Park LR, Perkins EL, Woodard JS, Brown KD. Delaying Cochlear Implantation Impacts Postoperative Speech Perception of Nontraditional Pediatric Candidates. Audiol Neurootol. 2021;26(3):182-187. doi:10.1159/000510693
25. Kocabay AP, Cinar BC, Batuk MO, Yarali M, Sennaroglu G. Pediatric cochlear implant fitting parameters in inner ear malformation: Is it same with normal cochlea? Int J Pediatr Otorhinolaryngol. 2022;155:111084. doi:10.1016/j.ijporl.2022.111084
26. Dowell RC, Dettman SJ, Blamey PJ, Barker EJ, Clark GM. Speech perception in children using cochlear implants: prediction of long-term outcomes. Cochlear Implants Int. 2002;3(1):1-18. doi:10.1179/cim.2002.3.1.1
27. Waltzman SB, Roland JT, Cohen NL. Delayed implantation in congenitally deaf children and adults. Otol Neurotol Off Publ Am Otol Soc Am Neurotol Soc Eur Acad Otol Neurotol. 2002;23(3):333-340. doi:10.1097/00129492-200205000-00018
28. Peixoto MC, Spratley J, Oliveira G, Martins J, Bastos J, Ribeiro C. Effectiveness of cochlear implants in children: long term results. Int J Pediatr Otorhinolaryngol. 2013;77(4):462-468. doi:10.1016/j.ijporl.2012.12.005
29. Eisenman DJ, Ashbaugh C, Zwolan TA, Arts HA, Telian SA. Implantation of the malformed cochlea. Otol Neurotol Off Publ Am Otol Soc Am Neurotol Soc Eur Acad Otol Neurotol. 2001;22(6):834-841. doi:10.1097/00129492-200111000-00020
30. Loundon N, Rouillon I, Munier N, Marlin S, Roger G, Garabedian EN. Cochlear implantation in children with internal ear malformations. Otol Neurotol Off Publ Am Otol Soc Am Neurotol Soc Eur Acad Otol Neurotol. 2005;26(4):668-673. doi:10.1097/01.mao.0000178126.58859.a9
31. Rachovitsas D, Psillas G, Chatzigiannakidou V, Triaridis S, Constantinidis J, Vital V. Speech perception and production in children with inner ear malformations after cochlear implantation. Int J Pediatr Otorhinolaryngol. 2012;76(9):1370-1374. doi:10.1016/j.ijporl.2012.06.009
32. Melo AS, Martins J, Silva J, Quadros J, Paiva A. Cochlear implantation in children with anomalous cochleovestibular anatomy. Auris Nasus Larynx. 2017;44(5):509-516. doi:10.1016/j.anl.2017.02.003
33. Celik M, Karatas E, Kanlikama M. Outcomes of cochlear implantation in children with and without inner ear malformations. Pak J Med Sci. 2018;34(2):380-384. doi:10.12669/pjms.342.14066
34. Kamogashira T, Akamatsu Y, Kashio A, et al. Development of auditory skills after cochlear implantation in children with inner ear malformations. Acta Otolaryngol (Stockh). 2016;136(1):78-82. doi:10.3109/00016489.2015.1087047
35. Demir B, Cesur S, Sahin A, Binnetoglu A, Ciprut A, Batman C. Outcomes of cochlear implantation in children with inner ear malformations. Eur Arch Oto-Rhino-Laryngol Off J Eur Fed Oto-Rhino-Laryngol Soc EUFOS Affil Ger Soc Oto-Rhino-Laryngol - Head Neck Surg. 2019;276(9):2397-2403. doi:10.1007/s00405-019-05475-9
36. Dettman S, Sadeghi-Barzalighi A, Ambett R, Dowell R, Trotter M, Briggs R. Cochlear implants in forty-eight children with cochlear and/or vestibular abnormality. Audiol Neurootol. 2011;16(4):222-232. doi:10.1159/000320608
37. Catli T, Uckan B, Olgun L. Speech and language development after cochlear implantation in children with bony labyrinth malformations: long-term results. Eur Arch Oto-Rhino-Laryngol Off J Eur Fed Oto-Rhino-Laryngol Soc EUFOS Affil Ger Soc Oto-Rhino-Laryngol - Head Neck Surg. 2015;272(11):3131-3136. doi:10.1007/s00405-014-3319-5
38. Daneshi A, Farhadi M, Ajalloueyan M, et al. Cochlear implantation in children with inner ear malformation: A multicenter study on auditory performance and speech production outcomes. Int J Pediatr Otorhinolaryngol. 2020;132:109901. doi:10.1016/j.ijporl.2020.109901
39. Farhood Z, Nguyen SA, Miller SC, Holcomb MA, Meyer TA, Rizk HG. Cochlear Implantation in Inner Ear Malformations: Systematic Review of Speech Perception Outcomes and Intraoperative Findings. Otolaryngol--Head Neck Surg Off J Am Acad Otolaryngol-Head Neck Surg. 2017;156(5):783-793. doi:10.1177/0194599817696502