Comprehensive Etiologic Analyses in Pediatric Cochlear Implantees and the Clinical Implications
Abstract
:1. Introduction
2. Materials and Methods
2.1. Subject Recruitment and Clinical Evaluations
2.2. Genetic Examinations
2.3. Imaging Examinations
2.4. Assessments of the CI Outcomes
2.5. Statistical Analysis
3. Results
3.1. Demographic Data and Etiological Analyses
3.2. Outcome Analyses
4. Discussion
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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Characteristic | Overall Patients N = 160 |
---|---|
Age at implantation, y | Mean: 4.9, median: 3.3, range: 1.0~17.9 |
Sex | |
Male | 84 (52.5%) |
Female | 76 (47.5%) |
Laterality of implant | |
Unilateral | 96 (60.0%) |
Bilateral | 64 (40.0%) |
Genetic causes | 98 (61.3%) |
SLC26A4 | 36 |
GJB2 | 22 |
OTOF | 10 |
MYO15A | 10 |
ILDR1 | 1 |
MTRNR1 | 1 |
TECTA | 1 |
CHD7 (CHARGE syndrome) | 4 |
MYO7A (Usher IB syndrome) | 3 |
CDH23 (suspected Usher ID syndrome) | 1 |
MITF (Waardenburg syndrome type 2) | 1 |
PAX3 (Waardenburg syndrome type 3) | 1 |
PTPN11 (LEOPARD syndrome) | 2 |
WFS1 (suspected Wolfram syndrome) | 1 |
OPA1 (Dominant optic atrophy) | 1 |
FGFR3 (Crouzon syndrome) | 1 |
COL2A1 (Stickler syndrome) | 1 |
TBX1 (DiGeorge syndrome) | 1 |
Imaging diagnoses | 31 (19.4%) |
CND | 20 |
Other IEMs | 11 |
Common cavity | 2 |
Cochlear hypoplasia | 2 |
IP-I | 2 |
IP-II with EVA | 3 |
IP-III | 1 |
Isolated EVA | 1 |
cCMV infection | 4 (2.5%) |
Unknown etiologies | 27 (16.9%) |
Non-Syndromic SNHI N = 81 | Syndromic SNHI N = 17 | CND N = 20 | Other IEMs N = 11 | cCMV Infection N = 4 | Unknown Etiology N = 27 | ||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Mean | SD | Mean | SD | Mean | SD | Mean | SD | Mean | SD | Mean | SD | p Value | |
Number | % | Number | % | Number | % | Number | % | Number | % | Number | % | ||
OP age, y | 4.4 | 3.8 | 7.8 | 5.6 | 4.3 | 4.9 | 4.1 | 4.4 | 2.4 | 0.9 | 5.9 | 5.6 | 0.242 |
Gender | 0.176 | ||||||||||||
Female | 35 | 43.2 | 10 | 58.8 | 6 | 30.0 | 6 | 54.5 | 2 | 50.0 | 17 | 63.0 | |
Male | 46 | 56.8 | 7 | 41.2 | 14 | 70.0 | 5 | 45.5 | 2 | 50.0 | 10 | 37.0 | |
Laterality | 0.205 | ||||||||||||
Unilateral | 47 | 58.0 | 11 | 58.8 | 15 | 75.0 | 6 | 54.5 | 2 | 50.0 | 15 | 55.6 | |
Bilateral | 34 | 42.0 | 6 | 41.2 | 5 | 25.0 | 5 | 45.5 | 2 | 50.0 | 12 | 44.4 | |
F/U months | 27.8 | 22.0 | 20.5 | 12.3 | 19.6 | 15.0 | 25.6 | 21.4 | 39.5 | 31.9 | 32.0 | 29.9 | 0.337 |
CAP | 5.9 | 1.5 | 4.4 | 2.0 | 3.1 | 2.1 | 5.6 | 1.2 | 4.5 | 1.3 | 5.9 | 1.1 | <0.001 * |
SIR | 4.0 | 1.4 | 3.4 | 1.4 | 1.7 | 1.0 | 3.0 | 1.5 | 2.3 | 1.9 | 3.8 | 1.3 | <0.001 # |
SLC26A4 | GJB2 | MYO15A | OTOF | ||||||
---|---|---|---|---|---|---|---|---|---|
N = 36 | N = 22 | N = 10 | N = 10 | ||||||
Mean | SD | Mean | SD | Mean | SD | Mean | SD | ||
Number | % | Number | % | Number | % | Number | % | p Value | |
OP age, y | 5.4 | 3.7 | 3.8 | 4.1 | 2.9 | 1.1 | 2.4 | 1.3 | <0.001 * |
Gender | 0.017 | ||||||||
Female | 17 | 47.2 | 6 | 27.3 | 8 | 80.0 | 2 | 20.0 | |
Male | 19 | 52.8 | 16 | 72.7 | 2 | 20.0 | 8 | 80.0 | |
Laterality | 0.070 | ||||||||
Unilateral | 26 | 72.2 | 9 | 40.9 | 5 | 50.0 | 4 | 40.0 | |
Bilateral | 10 | 27.8 | 13 | 59.1 | 5 | 50.0 | 6 | 60.0 | |
F/U months | 31.2 | 27.4 | 29.3 | 17.1 | 17.9 | 11.5 | 24.1 | 18.9 | 0.274 |
CAP | 6.0 | 1.5 | 5.7 | 1.8 | 6.1 | 1.0 | 6.2 | 0.8 | 0.891 |
SIR | 4.4 | 1.3 | 3.6 | 1.5 | 4.2 | 1.3 | 3.7 | 0.9 | 0.081 |
Variables | Unfavorable Outcomes N = 20 | Favorable Outcomes N = 140 | p Value | ||
---|---|---|---|---|---|
Mean/ | SD | Mean/ | SD | ||
Number | % | Number | % | ||
OP age, y | 3.4 | 3.4 | 5.1 | 4.7 | 0.065 |
Gender | 0.031 | ||||
Female | 5 | 25.0 | 71 | 50.7 | |
Male | 15 | 75.0 | 69 | 49.3 | |
Laterality | 0.196 | ||||
Unilateral | 14 | 70.0 | 82 | 58.6 | |
Bilateral | 6 | 30.0 | 58 | 41.4 | |
F/U months | 28.2 | 14.6 | 26.5 | 23.0 | 0.155 |
CAP | 2.9 | 1.5 | 5.7 | 1.6 | <0.001 |
SIR | 1.5 | 0.8 | 3.8 | 1.4 | <0.001 |
Four predominant non-syndromic SNHI genes | 5 | 25 | 73 | 52.1 | 0.023 |
SLC26A4 | 3 | 60 | 33 | 45.2 | |
GJB2 | 2 | 40 | 20 | 27.4 | |
MYO15A | 0 | 0 | 10 | 13.7 | |
OTOF | 0 | 0 | 10 | 13.7 | |
Syndromic SNHI | 3 | 15.0 | 14 | 10.0 | 0.387 |
CND | 9 | 45.0 | 11 | 7.9 | <0.001 |
Other IEMs | 0 | 0 | 11 | 7.9 | |
cCMV infection | 1 | 5.0 | 3 | 2.1 | 0.351 |
Unknown etiology | 1 | 5.0 | 26 | 18.6 | 0.079 |
Univariate Analyses | Multivariate Analyses | |||
---|---|---|---|---|
Variables | OR (95%CI) | p Value | OR (95%CI) | p Value |
OP age, y | 1.13 (0.96–1.33) | 0.130 | ||
Gender (male) | 0.32 (0.11–0.94) | 0.038 | 0.30 (0.09–0.95) | 0.041 |
Laterality (bilateral) | 2.00 (0.69–5.81) | 0.203 | ||
F/U months | 1.00 (0.98–1.02) | 0.758 | ||
Four predominant non-syndromic SNHI genes | 3.27 (1.13–9.48) | 0.029 | 1.78 (0.51–6.27) | 0.368 |
CND | 0.08 (0.03–0.26) | <0.001 | 0.11 (0.03–0.40) | <0.001 |
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Lee, C.-Y.; Lin, P.-H.; Tsai, C.-Y.; Chiang, Y.-T.; Chiou, H.-P.; Chiang, K.-Y.; Chen, P.-L.; Hsu, J.S.-J.; Liu, T.-C.; Wu, H.-P.; et al. Comprehensive Etiologic Analyses in Pediatric Cochlear Implantees and the Clinical Implications. Biomedicines 2022, 10, 1846. https://doi.org/10.3390/biomedicines10081846
Lee C-Y, Lin P-H, Tsai C-Y, Chiang Y-T, Chiou H-P, Chiang K-Y, Chen P-L, Hsu JS-J, Liu T-C, Wu H-P, et al. Comprehensive Etiologic Analyses in Pediatric Cochlear Implantees and the Clinical Implications. Biomedicines. 2022; 10(8):1846. https://doi.org/10.3390/biomedicines10081846
Chicago/Turabian StyleLee, Chee-Yee, Pei-Hsuan Lin, Cheng-Yu Tsai, Yu-Ting Chiang, Hong-Ping Chiou, Ko-Yin Chiang, Pei-Lung Chen, Jacob Shu-Jui Hsu, Tien-Chen Liu, Hung-Pin Wu, and et al. 2022. "Comprehensive Etiologic Analyses in Pediatric Cochlear Implantees and the Clinical Implications" Biomedicines 10, no. 8: 1846. https://doi.org/10.3390/biomedicines10081846