Frequency of Inner Ear Anomalies Among Cochlear Implant Candidates: A Case Study in the Northwest of Iran

@#Introduction: Globally hearing impairment occurs in about 1 to 2 per 1000 live births. The etiology of severe sensorineural hearing loos (SNHL) is complex and multifactorial. Congenital inner ear abnormality is a major cause of hearing loss in children and cochlear implantation (CI) is a proper treatment in these children. Preoperative CT scan of the temporal bones are used to evaluate inner ear malformations in CI candidates. The frequency of internal ear malformations reported on temporal scan could varied from 20% to 30%. The aim of this study was to evaluate the frequency of inner ear malformations in CT scan of cochlear implant candidates . Methods: In a retrospective cross-sectional descriptive study, 201 infants (105 boys and 96 girls ) with the mean age of 20.5 ± 14.01 month with severe SNHL who are CI candidates were examined by temporal bone imaging with multi-slice CT from March 2014 to March 2015 in CI center of Tabriz University. Results : CT revealed abnormalities of the inner ear in 26 (13%) of infants that most of them with 10 (38.5%) was enlarged vestibular aqueduct (EVA). Also, 31 infants (15.4%) had abnormalities in outer, middle ear or in mastoid aeration. Conclusion: Temporal bone imaging with CT is an essential method and could be suggested as a proper first step for evaluating inner ear malformations in CI candidates.

CT and MRI diagnostic value of the congenital inner ear malformation incomplete partition type Ⅱdeformity combined with spontaneous CSF otorrhea / 实用放射学杂志

Objective To discuss the diagnostic value of high resolution computed tomography (HRCT)and MRI in the congenital inner ear malformation incomplete partition type Ⅱ deformity(Mondini deformity)combined with spontaneous cerebrospinal fluid (CSF)otorrhea.Methods The imaging data including the multiplanar reformation(MPR)images of HRCT,curved planar reforma-tion(CPR)images and MR hydrography images in three patients complicated with recurrent meningitis were retrospectively ana-lyzed.The anatomic changes of the inner ear and middle ear on the affected side were observed,the clinical and imaging features of this disease were summarized.Results One patient had bilateral incidence of the incompletely separated inner ear,combined with semicircular canal dysplasia and cochlear pipe dilation.Two patients had unilateral onset,one of them had concurrent facial nerve ca-nal dysplasia.Humble stapes floor and the defect of the bottom of the internal auditory canal were seen in all the three patients.Con-clusion HRCT with post-processing technology,and MRI have great significance in the diagnosis of the congenital inner ear malfor-mation incomplete partition type Ⅱ deformity (Mondini deformity)combined with spontaneous CSF otorrhea.

Three Cases of Bilateral Semicircular Canal Aplasia with Normal Cochlear Development in the Same Family Member / 대한이비인후과학회지

Semicircular canal aplasia is rare congenital inner ear anomaly. During embryogenesis, congenital malformation of vestibular labyrinth usually associates with cochlear anomalies. Two cases of semicircular canal aplasia with normal or near-normal cochlear development was reported in the English literature. We present three patients with computed tomographic findings of bilateral total semicircular canal aplasia with normal cochlear development in the same family member. Two patients had significant conductive hearing loss due to congenital stapedial anomalies and the other was congenital deafmute. Temporal bone CT scan revealed total absence of the semicircular canals bilaterally and both cochleas were normal in three cases.