Imaging And Audiology Analysis Of The Congenital Inner Ear Malformations

Objective:To investigate imaging and audiology features of temporal bone and analyze the classification and prevalence of inner ear abnormalities in children with sensorineural hearing loss(SNHL).Methods : Selected 590 children diagnosed sensorineural hearing loss with audiology detection in the otolaryngology head and neck surgery of first affiliated hospital of fujian medical university.Age between 3 months to 8years and 9months.The average age is 3.5 years.All of them were examined by high resolution CT and the inner ear fluid of MRI.And then retrospective chart review their imaging and audiology features.Results: There were 127 patients(232 ears) found with inner ear malformation in590 children with SNHL, 105 of them had bilateral hearing loss and 22 had unilateral hearing loss. According to Sennaroglu’S classification, Michel deformity were found in 2 patients(2 ears)(0.9%).14(26 ears)(11.2%) were found to be cochlear dysplasia;8(15 ears)(6.5%) IP-I malformation; 18(ears)(14.6%) Mondini deformity; 51(95ears)(40.1%) vestibular malformation; 73(127ears)(54.7%)semicircular canal malformation;10(18ears)(7.8%) internal auditory canal malformation;42(79ears) large vestibular aqueduct. Between the patients with internal auditory canal narrowing,there were 3(6ears) vestibulocochlear nerve absent or tiny. About 21.71% were inner ear malformation in severe and profound hearing loss ears, and under moderate level there were 13.41% deformity rate.The difference was statistically significant(0.01<P<0.025).Conclusion: Congenital inner ear malformations is one of the leading causes of SNHL. CT and MRI examinations are the first choice to the diagnosis and classification of inner ear malformations,and they have important clinical significance to guide the cochlear implant operation. Inner ear malformations are almost bilateral and hearing loss of them are profoud.Of them cochleovestibular malformations and large vestibular aqueduct are more than others. Between the patients with cochleovestibular malformations Mondini deformity are more thanothers. Vestibular and semicircular canal malformation are often accompanied by other parts malformation of the inner ear. And internal auditory canal narrowing are often accompanied by vestibulocochlear nerve dysplasia.Among the children with SNHL,deformity rate in the severe and profound hearing loss ears is higher than the one under moderate level.People with normal hearing can also exist inner ear malformations.