Clinical Features of Children with Congenital Malformations of Inner Ear in Sensorineural Hearing Loss

Objective:To study the clinical features of inner ear malformations with sensorineural hearing loss(SNHL)in children.Methods: Retrospective chart review,860 patients with SNHL,of which 125 children(225 ears) with inner ear malformations diagnosed with temporal bone computed tomography(CT) scans who referred to the Ear,Nose and Throat(ENT) Department of Children Medical Center from February 2005 to January 2010,109 cases due to hearing loss and other 3 cases due to external ear malformations,13 cases due to face abnormality,were retrospectively studied.Results: A total of 109(87.2%) cases were found hearing loss,52 cases(41.6%)mothers were noted to have caught a cold in first trimester of pregnancy.Hearing loss was typically bilateral in 100 ears(80%),and profound sensorineural hearing loss in 166 ears(73.8%).Of the total,3 ears were accompanied by outer and middle ear malformations,13 ears accompanied by middle ear malformations.Among the 124 patients,2(4 ears)were found to be Michel deformity,1(1 ears) was found to be Cochlear aplasia,10(14 ears)were found to be common cavity deformity,2(2 ears)incomplete partition,2(2 ear) cochleovestibular hypoplaisa,19(29 ears)incomplete partition II(Mondini deformity),91(170 ears) accompanied by large vestibular aqueduct,30(46 ears)semicircular canal deformity,internal auditory canal deformity 35(49ears).Conclusions: The patients with large vestibular aqueduct are more in patients with inner ear malformations than in patients with other inner ear malformations.Hearing loss is typically bilateralor total deafness.CT examinations are of great importance for the diagnosis and treatment of children with inner ear malformations.