Cochlear Implant Outcomes and Genetic Mutations in Children with Ear and Brain Anomalies

Table 1

Suspected main aetiology of hearing loss among 143 children who underwent cochlear implantation and presented ear or brain anomalies.

Aetiology	Number of patients	%
Unknown	27	18.9
Cytomegalovirus infection	26	18.2
GJB2 mutations	24	16.8
Acquired conditions (prematurity, perinatal suffering/icterus)	16	11.2
Enlarged vestibular aqueduct (EVA)	14 (4 Pendred syndromes)	9.8
Cochleovestibular malformations	14	9.8
Meningitis/encephalitis	12/1	9.1
CHARGE association	3	2.1
Hydrocephalus	2	1.4
Waardenburg syndrome (with dilated vestibule and bilateral cochlear dysplasia)	1	0.7
Möbius syndrome (with microtia and facial nerve aplasia)	1	0.7
Williams syndrome (with EVA and semicircular canal dysplasia)	1	0.7
Other conditions (neurosurgery)	1	0.7
Total	143	100