Congenital sensorineural hearing loss in consanguineous marriages – Does the cochlear length vary

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Rohit Aggarwal
Kavitha Yellur
Upendra Kumar Joish
Raju Augustine George
Himanshu Swami
Sabarigirish Kanjully

Abstract

Objective: There is an increased prevalence of congenital sensorineural hearing loss (SNHL) among children born out of consanguineous wedlocks, and congenital deafness is associated with increased prevalence of structural inner-ear malformations. This study is done to evaluate whether consanguinity affects the cochlear length, which in turn will influence the type of cochlear implant and depth of electrode insertion during surgery in these patients.
Methods: Children presenting with congenital SNHL and born out of consanguineous marriages (Group A) were compared with children presenting with SNHL and born out of nonconsanguineous marriages(Group B). Patients in both groups were evaluated with magnetic resonance imaging as a routine pretreatment workup. A high-resolution three-dimensional T2-weighted sampling perfection with application-optimized contrasts using different flip-angle evolution imaging of the inner ears was performed. Curved multiplanar
reconstruction module was used to deconvolute the membranous cochlea and measure its length. The cochlear lengths among both the groups were compared using analysis of variance test.
Results: A total of seven patients were included in both Groups A and B each. The mean length of membranous cochlea in Group A was 22.6 mm and Group B was 22.5 mm. There was no statistically significant variation in the cochlear lengths of both the groups.
Conclusion: Consanguinity is unlikely to produce any significant variation in the length of the cochlea.

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Aggarwal, R., Yellur, K., Joish, U. K., George, R. A., Swami, H., & Kanjully, S. (2019). Congenital sensorineural hearing loss in consanguineous marriages – Does the cochlear length vary. West African Journal of Radiology, 26(2), 117-120. https://doi.org/10.4103/wajr.wajr_40_17

References

1. Rutherford KD, Lerer TS, Schoem SR, Valdez TA. Evaluation of pediatric sensorineural hearing loss: A survey of pediatric otolaryngologists. Ann Otol Rhinol Laryngol 2011;120:674‑81.

2. Parry DA, Booth T, Roland PS. Advantages of magnetic resonance imaging over computed tomography in preoperative evaluation of pediatric cochlear implant candidates. Otol Neurotol 2005;26:976‑82.

3. Fishman AJ. Imaging and anatomy for cochlear implants. Otolaryngol Clin North Am 2012;45:1‑24.

4. Zakzouk S. Consanguinity and hearing impairment in developing countries: Acustom to be discouraged. J Laryngol Otol 2002;116:811‑6.

5. Tayebi N, Yazdani K, Naghshin N. The prevalence of congenital malformations and its correlation with consanguineous marriages. Oman Med J 2010;25:37‑40.

6. Shrikrishna BH, Deepa G. Study of association of family history and consanguinity with congenital hearing loss. Int J Otorhinolaryngol Head Neck Surg 2016;2:61‑5.

7. Alsmadi O, Meyer BF, Alkuraya F, Wakil S, Alkayal F, Al‑Saud H, et al. Syndromic congenital sensorineural deafness, microtia and microdontia resulting from a novel homoallelic mutation in fibroblast growth factor 3 (FGF3). Eur J Hum Genet 2009;17:14‑21.

8. Tekin M, Oztürkmen Akay H, Fitoz S, Birnbaum S, Cengiz FB, Sennaroğlu L, et al. Homozygous FGF3 mutations result in congenital deafness with inner ear agenesis, microtia, and microdontia. Clin Genet 2008;73:554‑65.

9. Sensi A, Ceruti S, Trevisi P, Gualandi F, Busi M, Donati I, et al. LAMM syndrome with middle ear dysplasia associated with compound heterozygosity for FGF3 mutations. Am J Med Genet A 2011;155A:1096‑101.

10. Nair G, Vadivu S, Sampathkumar R, Pradhananga RB, Kameswaran M. A study of anomalies of cochlea and cochlear nerve in children with congenital profound hearing loss – An Indian perspective. J Sci Res Rep 2017;13:1‑11.

11. Sampaio AL, Araújo MF, Oliveira CA. New criteria of indication and selection of patients to cochlear implant. Int J Otolaryngol 2011;2011:573968.

12. Bener A, Eihakeem AA, Abdulhadi K. Is there any association between consanguinity and hearing loss. Int J Pediatr Otorhinolaryngol 2005;69:327‑33.

13. Reddy MV, Bindu LH, Reddy PP, Rani PU. Role of consanguinity in congenital neurosensory deafness. Int J Hum Genet 2006;6:357‑8.

14. Selvarajan HG, Arunachalam RK, Bellur R, Mandke K, Nagarajan R. Association of family history and consanguinity with permanent hearing impairment. Indian J Otol 2013;19:62‑5.

15. Rabionet R, Zelante L, López‑Bigas N, D’Agruma L, Melchionda S, Restagno G, et al. Molecular basis of childhood deafness resulting from mutations in the GJB2 (connexin 26) gene. Hum Genet 2000;106:40‑4.

16. Agha M, Eid M, Eid AF, Abu‑Samra M. Congenital hearing loss. Is CT enough? Alexandria J Med 2014;50:113‑21.

17. Connor SE, Bell DJ, O’Gorman R, Fitzgerald‑O’Connor A. CT and MR imaging cochlear distance measurements may predict cochlear implant length required for a 360 degrees insertion. AJNR Am J Neuroradiol 2009;30:1425‑30.

18. Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: A classification based on embryogenesis. Laryngoscope 1987;97:2‑14.

19. Pochini Sobrinho F, Lazarini PR, Yoo HJ, Abreu Júnior LD, Meira Ade S. A method for measuring the length of the cochlea through magnetic resonance imaging. Braz J Otorhinolaryngol 2009;75:261‑7.

20. Ketten DR, Skinner MW, Wang G, Vannier MW, Gates GA, Neely JG, et al. In vivo measures of cochlear length and insertion depth of nucleus cochlear implant electrode arrays. Ann Otol Rhinol Laryngol Suppl 1998;175:1‑6.

21. Korver AM, Konings S, Dekker FW, Beers M, Wever CC, Frijns JH, et al. Newborn hearing screening vs. later hearing screening and developmental outcomes in children with permanent childhood hearing impairment. JAMA 2010;304:1701‑8.

22. Mehra S, Eavey RD, Keamy DG Jr. The epidemiology of hearing impairment in the United States: Newborns, children, and adolescents. Otolaryngol Head Neck Surg 2009;140:461‑72.

23. Morton CC, Nance WE. Newborn hearing screening – A silent revolution. N Engl J Med 2006;354:2151‑64.

24. Reddy M, Vardhan V. Perinatal risk factors for congenital sensorineural hearing loss. Indian J Otol 2006;12:7‑12.

25. Schrijver I. Hereditary non‑syndromic sensorineural hearing loss. J Mol Diagn 2004;6:275‑84.

26. Smith RJ, Bale JF Jr., White KR. Sensorineural hearing loss in children. Lancet 2005;365:879‑90.

27. Alford RL, Arnos KS, Fox M, Lin JW, Palmer CG, Pandya A, et al. American college of medical genetics and genomics guideline for the clinical evaluation and etiologic diagnosis of hearing loss. Genet Med 2014;16:347‑55.

28. World Population Prospects: The 2017 Revision. (Custom data acquired via website). United Nations Department of Economic and Social Affairs, Population Division. Available from: http://www.ESA.UN.org. [Last accessed on 2018 Apr 10].

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