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Cochlear implantation in a profoundly deaf child with cystic leukoencephalopathy without megalencephaly

Published online by Cambridge University Press:  18 August 2022

A Gopalakrishnan Open the ORCID record for A Gopalakrishnan [Opens in a new window] ,
S Thangavel Open the ORCID record for S Thangavel [Opens in a new window] ,
S Chowdhary  and
A Alexander Open the ORCID record for A Alexander [Opens in a new window]
A Gopalakrishnan
Affiliation:
Department of ENT, Jawaharlal Institute of Post Graduate Medical Education and Research, Pondicherry, India
S Thangavel*
Affiliation:
Department of ENT, Jawaharlal Institute of Post Graduate Medical Education and Research, Pondicherry, India
S Chowdhary
Affiliation:
Department of ENT, Jawaharlal Institute of Post Graduate Medical Education and Research, Pondicherry, India
A Alexander
Affiliation:
Department of ENT, Jawaharlal Institute of Post Graduate Medical Education and Research, Pondicherry, India
*
Author for correspondence: Dr S Thangavel, Department of ENT, Jawaharlal Institute of Postgraduate Medical Education and Research, Dhanvanthri Nagar, Pondicherry 605006, India E-mail: [email protected]
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Abstract

Background

Cochlear implantation candidacy criteria have continued to evolve over the years, and cochlear implantation is possible with many inner-ear and brain anomalies with good hearing and linguistic outcomes. Cystic leukoencephalopathy without megalencephaly is a rare disease in children, with only 30 cases reported in the literature, but it is associated with hearing loss in only three cases. Radiological investigations can help in diagnosing this rare entity before proceeding with cochlear implantation.

Case Report

A four-year-old female child born out of consanguinity with normal psychomotor development, bilateral sensorineural hearing loss and an incidental magnetic resonance imaging finding of cystic leukoencephalopathy without megalencephaly underwent successful cochlear implantation. Her post-operative period was uneventful with successful mapping of the cochlear implant.

Conclusion

This is the first reported case of cystic leukoencephalopathy without megalencephaly and with sensorineural hearing loss in which cochlear implantation was performed successfully. White matter and temporal lobe abnormalities should not deter paediatric cochlear implantation.

Keywords

Cochlear ImplantationRare DiseasesLeukoencephalopathiesTemporal LobeMegalencephaly
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 137 , Issue 5 , May 2023 , pp. 584 - 587
Copyright
Copyright © The Author(s), 2022. Published by Cambridge University Press on behalf of J.L.O. (1984) LIMITED

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Footnotes

Dr S Thangavel takes responsibility for the integrity of the content of the paper

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