Cochlear implantation in a profoundly deaf child with cystic leukoencephalopathy without megalencephaly.
Cochlear Implantation
Leukoencephalopathies
Megalencephaly
Rare Diseases
Temporal Lobe
Journal
The Journal of laryngology and otology
ISSN: 1748-5460
Titre abrégé: J Laryngol Otol
Pays: England
ID NLM: 8706896
Informations de publication
Date de publication:
May 2023
May 2023
Historique:
medline:
20
4
2023
pubmed:
19
8
2022
entrez:
18
8
2022
Statut:
ppublish
Résumé
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. 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. 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.
Sections du résumé
BACKGROUND
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
METHODS
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
CONCLUSIONS
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.
Identifiants
pubmed: 35979751
doi: 10.1017/S0022215122001931
pii: S0022215122001931
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM