Biomaterial Database

Cochlear implantation in a profoundly deaf child with cystic leukoencephalopathy without megalencephaly. 2023

A Gopalakrishnan, and S Thangavel, and S Chowdhary, and A Alexander

Department of ENT, Jawaharlal Institute of Post Graduate Medical Education and Research, Pondicherry, India.

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. 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. 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.

UI	MeSH Term	Description	Entries
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D003054	Cochlear Implants	Electronic hearing devices typically used for patients with normal outer and middle ear function, but defective inner ear function. In the COCHLEA, the hair cells (HAIR CELLS, VESTIBULAR) may be absent or damaged but there are residual nerve fibers. The device electrically stimulates the COCHLEAR NERVE to create sound sensation.	Auditory Prosthesis,Cochlear Prosthesis,Implants, Cochlear,Auditory Prostheses,Cochlear Implant,Cochlear Prostheses,Implant, Cochlear,Prostheses, Auditory,Prostheses, Cochlear,Prosthesis, Auditory,Prosthesis, Cochlear
D005260	Female		Females
D006319	Hearing Loss, Sensorineural	Hearing loss resulting from damage to the COCHLEA and the sensorineural elements which lie internally beyond the oval and round windows. These elements include the AUDITORY NERVE and its connections in the BRAINSTEM.	Deafness Neurosensory,Deafness, Neurosensory,Deafness, Sensoryneural,Neurosensory Deafness,Sensorineural Hearing Loss,Sensoryneural Deafness,Cochlear Hearing Loss,Hearing Loss, Cochlear,Deafnesses, Neurosensory,Deafnesses, Sensoryneural,Neurosensory Deafnesses,Sensoryneural Deafness,Sensoryneural Deafnesses
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D056784	Leukoencephalopathies	Any of various diseases affecting the white matter of the central nervous system.	Childhood Ataxia with Diffuse Central Nervous System Hypomyelination,Leukoencephalopathy,Leukoencephalopathy with Vanishing White Matter,Vanishing White Matter Disease,CACH Syndrome,CACH VWM Syndrome,Childhood Ataxia with Central Nervous System Hypomyelination,Childhood Ataxia with Central Nervous System Hypomyelinization,Cree Leukoencephalopathy,Myelinosis Centralis Diffusa,Vanishing White Matter Leukodystrophy,White Matter Diseases,CACH Syndromes,CACH VWM Syndromes,Centralis Diffusa, Myelinosis,Cree Leukoencephalopathies,Diffusa, Myelinosis Centralis,Diffusas, Myelinosis Centralis,Leukoencephalopathy, Cree,Myelinosis Centralis Diffusas,Syndrome, CACH VWM,VWM Syndrome, CACH,White Matter Disease
D058627	Megalencephaly	A congenital abnormality in which the occipitofrontal circumference is greater than two standard deviations above the mean for a given age. It is associated with HYDROCEPHALUS; SUBDURAL EFFUSION; ARACHNOID CYSTS; or is part of a genetic condition (e.g., ALEXANDER DISEASE; SOTOS SYNDROME).	Macrocephaly,Megacephaly,Megalocephaly,Macrocephalies,Megacephalies,Megalencephalies,Megalocephalies
D019929	Cochlear Implantation	Surgical insertion of an electronic hearing device (COCHLEAR IMPLANTS) with electrodes to the COCHLEAR NERVE in the inner ear to create sound sensation in patients with residual nerve fibers.	Cochlear Prosthesis Implantation,Cochlear Implantations,Cochlear Prosthesis Implantations,Implantation, Cochlear,Implantation, Cochlear Prosthesis,Implantations, Cochlear,Implantations, Cochlear Prosthesis,Prosthesis Implantation, Cochlear,Prosthesis Implantations, Cochlear