Hostname: page-component-586b7cd67f-dlnhk Total loading time: 0 Render date: 2024-11-22T19:51:49.344Z Has data issue: false hasContentIssue false

Cochlear implantation in children with Mondini dysplasia: our experience

Published online by Cambridge University Press:  11 February 2021

N M Suri*
Affiliation:
Department of Otorhinolaryngology, Gujarat Medical Education and Research Society (‘GMERS’) Medical College and Hospital, Gandhinagar, India
A R Prasad
Affiliation:
Department of Otorhinolaryngology, Gujarat Medical Education and Research Society (‘GMERS’) Medical College and Hospital, Gandhinagar, India
R K Sayani
Affiliation:
Department of Otorhinolaryngology, Gujarat Medical Education and Research Society (‘GMERS’) Medical College and Hospital, Gandhinagar, India
A Anand
Affiliation:
Asian Speech and Hearing Clinic, Ahmedabad, India
G Jaychandran
Affiliation:
Asian Speech and Hearing Clinic, Ahmedabad, India
*
Author for correspondence: Dr Neeraj M Suri, Department of Otorhinolaryngology, 2nd Floor, OPD Building, GMERS Medical College and Civil Hospital, Sect. 12b, near Pathikashram, Gandhinagar, Gujarat382016, India E-mail: [email protected]

Abstract

Objective

This study details the intra-operative complications, and compares auditory scales post-implantation of either profoundly deaf young children with radiologically normal inner ears (group A) or children with Mondini dysplasia (group B).

Methods

A retrospective survey was carried out of 338 patients with severe to profound sensorineural hearing loss who underwent cochlear implant surgery from February 2015 to May 2017. Patients were divided into 2 groups of 27 patients each. Both groups were followed up to three years post-implantation.

Results

Cerebrospinal fluid ooze developed in 12 patients, and 2 patients had a cerebrospinal fluid ‘gusher’, one of which had to be explored within 24 hours. After implant use for one year, both groups had similar speech perception scores.

Conclusion

The cerebrospinal fluid gusher in Mondini dysplasia should be anticipated and adequately managed intra-operatively. This study highlights the tailoring of a post-implantation rehabilitation programme according to individual needs.

Type
Main Articles
Copyright
Copyright © The Author(s), 2021. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Footnotes

Dr N M Suri takes responsibility for the integrity of the content of the paper

References

Jensen, J. Malformations of the inner ear in deaf children. A tomographic and clinical study. Acta Radiol Diagn (Stockh) 1968;286:197Google Scholar
Lo, WW. What is a ‘Mondini’ and what difference does a name make? AJNR Am J Neuroradiol 1999;20:1442–4Google Scholar
Nomura, Y. Anomalies. In: Nomura, Y, eds. Morphological Aspects of Inner Ear Disease. Tokyo: Springer Japan, 2013;205–29Google Scholar
Silverstein, H, Smouha, E, Morgan, N. Multichannel cochlear implantation in a patient with bilateral Mondini deformities. Am J Otol 1988;9:451–5Google Scholar
Chen, X, Yan, F, Liu, B, Liu, S, Kong, Y, Zheng, J et al. The development of auditory skills in young children with Mondini dysplasia after cochlear implantation. PLoS One 2014;9:e108079CrossRefGoogle ScholarPubMed
Fishman, AJ, Holliday, RA. Principles of cochlear implant imaging. In: Waltzman, SB, Cohen, NL, eds. Cochlear Implants. New York: Thieme, 2000;79107Google Scholar
Papsin, BC. Cochlear implantation in children with anomalous cochleovestibular anatomy. Laryngoscope 2005;115:126CrossRefGoogle ScholarPubMed
Luntz, M, Balkany, T, Hodges, AV, Telischi, FF. Cochlear implants in children with congenital inner ear malformations. Arch Otolaryngol Head Neck Surg 1997;123:974–7CrossRefGoogle ScholarPubMed
Daneshi, A, Hassanzadeh, S, Abasalipour, P, Emamdjomeh, H, Farhadi, M. Cochlear implantation in Mondini dysplasia. ORL J Otorhinolaryngol Relat Spec 2003;65:3944CrossRefGoogle ScholarPubMed
Buchman, CA, Copeland, BJ, Yu, KK, Brown, CJ, Carrasco, VN, Pillsbury, HC. Cochlear implantation in children with congenital inner ear malformations. Laryngoscope 2004;114:309–16CrossRefGoogle ScholarPubMed
Sennaroglu, L. Cochlear implantation in inner ear malformations–a review article. Cochlear Implants Int 2010;11:441CrossRefGoogle Scholar
Sennaroglu, L. Histopathology of inner ear malformations: do we have enough evidence to explain pathophysiology? Cochlear Implants Int 2016;17:320CrossRefGoogle ScholarPubMed
Sennaroglu, L, Bajin, MD. Classification and current management of inner ear malformations. Balkan Med J 2017;34:397411CrossRefGoogle ScholarPubMed
Graham, JM, Phelps, PD, Michaels, L. Congenital malformations of the ear and cochlear implantation in children: review and temporal bone report of common cavity. J Laryngol Otol 2000;114:114CrossRefGoogle Scholar
Jackler, RK, Luxford, WM, House, WF. Sound detection with the cochlear implant in five ears of four children with congenital malformations of the cochlea. Laryngoscope 1987;97:1517CrossRefGoogle ScholarPubMed
Paparella, MM. Mondini's deafness. A review of histopathology. Ann Otol Rhinol Laryngol Suppl 1980;89:110CrossRefGoogle ScholarPubMed
Ohlms, LA, Edwards, MS, Mason, EO, Igarashi, M, Alford, BR, Smith, RJ. Recurrent meningitis and Mondini dysplasia. Arch Otolaryngol Head Neck Surg 1990;116:608–12CrossRefGoogle ScholarPubMed
Phelps, PD, King, A, Michaels, L. Cochlear dysplasia and meningitis. Am J Otol 1994;15:551–7Google ScholarPubMed
Weber, BP, Dillo, W, Dietrich, B, Maneke, I, Bertram, B, Lenarz, T. Pediatric cochlear implantation in cochlear malformations. Am J Otol 1998;19:747–53Google ScholarPubMed
Page, EL, Eby, TL. Meningitis after cochlear implantation in Mondini malformation. Otolaryngol Head Neck Surg 1997;116:104–6CrossRefGoogle ScholarPubMed
Nikolopoulos, TP, Archbold, SM, Gregory, S. Young deaf children with hearing aids or cochlear implants: early assessment package for monitoring progress. Int J Pediatr Otorhinolaryngol 2005;69:175–86CrossRefGoogle ScholarPubMed
Otte, J, Schuknecht, H, Kerr, A. Ganglion cell populations in normal and pathological human cochleae. Implications for cochlear implantation. Laryngoscope 1978;8:1231–47Google Scholar
Schmidt, JM. Cochlear neuronal populations in developmental defects of the inner ear. Implications for cochlear implantation. Acta Otolaryngol 1985;99:1420CrossRefGoogle ScholarPubMed
Linthicum, FH Jr, Fayad, JN. Spiral ganglion cell loss is unrelated to segmental cochlear sensory system degeneration in humans. Otol Neurotol 2009;30:418–22CrossRefGoogle ScholarPubMed
Wang, B, Cao, KL, Wang, Y, Li, H. Comparison of hearing rehabilitation for patients with Mondini malformation after cochlear implantation with different electrodes [in Chinese]. Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2017;31:284–9Google Scholar
Bille, J, Fink-Jensen, V, Ovesen, T. Outcome of cochlear implantation in children with cochlear malformations. Eur Arch Otorhinolaryngol 2015;272:583–9CrossRefGoogle ScholarPubMed
Munro, KJ, George, CR, Haacke, NP. Audiological findings after multichannel cochlear implantation in patients with Mondini dysplasia. Br J Audiol 1996;30:369–79CrossRefGoogle ScholarPubMed