Bilateral microtia, canal atresia and aplasia of cochleovestibular nerve

A six-month-old baby with congenital patent ductus arteriosus (PDA), bilateral microtia and canal atresia was referred for hearing assessment. The audiology assessment revealed bilateral profound hearing loss, which is atypical for a case of pure canal atresia. Imaging was performed much earlier than usual and, as suspected, the patient also had bilateral severe inner ear anomaly. It is extremely rare for a person to have both external and inner ear anomaly because of the different embryological origin. The only suitable hearing rehabilitation option for this kind of patients is brainstem implant. However, the parents had opted for sign language as a form of communication. KEY WORDS: Microtia; canal atresia; cochleovestibular nerve aplasia; inner ear anomaly

Recurrent Meningitis in a Patient with Inner Ear Anomaly after Cochlear Implantation / 대한이비인후과학회지

Cochlear implantation (CI) has been regarded as a safe and effective treatment for patients with severe to profound sensorineural hearing loss. The increase of experience in CI thus has had the effect of leading more children with inner ear anomaly to be considered as cochlear implant candidates. For patients with inner ear anomaly, auditory rehabilitation has been reported to be improved after CI; however, there are several factors such as cerebrospinal fluid leakage, facial nerve injury and abnormal positioning of electrode during surgery and uncertainty of auditory rehabilitation and possibility of the recurrent meningitis after CI that should be considered. Several studies proved that the incidence of otogenic meningitis is higher in patients with inner ear anomaly than in patients with normal inner ear anatomy. We experienced a case of recurrent meningitis due to an unusual cause after cochlear implantation in a patient with inner ear anomaly.

Cochlear Implant in Patients with Incomplete Partition Type III / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Among inner ear anomaly, incomplete partition type III is a quite rare finding which has pathognomonic computerized tomographic finding with bilateral, dilatation of lateral end of internal auditory canal, and deficient or absent bone between internal auditory canal and the basal turn of cochlea. Patients with incomplete partition type III have various range of hearing impairment and in case of severe hearing loss which cannot get benefit from hearing aids, cochlear implantation is indicated. In cochlear implantation for incomplete partition type III, perilymph gusher and abnormal electrode position is highly cautioned. We analyzed cochlear implantation patients with incomplete partition type III to find common intra-operative finding and complication. SUBJECTS AND METHOD: Temporal bone computerized tomographies of 120 patients who received cochlear implantation between September, 2002 and March, 2008 in our hospital were reviewed : the imaging of 4 patients were consistent with typical incomplete partition type III. We reviewed intra-operative finding and postoperative complications along with hearing outcome. RESULTS: During the operation, perilymph gusher was encountered in all cases, and it was tightly sealed with subcutaneous tissue and fat with fibrin glue. In patient 1, electrodes were abnormally positioned in IAC causing facial nerve stimulation. However, the result of postoperative speech perception was good in the patient, and in the rest of patients, the results were various. CONCLUSION: The surgeon should be aware of perilymph gusher when cochlear implantation is performed on patients with characteristic temporal bone computerized tomographic findings and who are suspicious of incomplete partition type III

A Case of Congenital Inner Ear Anomaly Combined with Cerebrospinal Leak Using Hydroxyapatite Cement for Treatment / 대한이비인후과학회지

Cerebrospinal fluid otorrhea is a rare disease entity, especially when brought on by spontaneous causes. The site of leakage should be identified to treat the disorder effectively. Various localizing modality was suggested in the literatures. Intrathecal gadolinium-enhanced magnetic resonance imaging identifies the small leakage site easily and correctly. Hydroxyapatite cement is a calcium phosphate-based material that forms dense paste mixed with water or sodium phosphate solution. Hydroxyapatite cement is remarkable for its excellent biocompatability in interacting with the native bone to osseointergrate with the surrounding bone. We report on our experience of successfully treating a patient with the cerebrospinal fluid leak from the congenital inner ear anomaly using the hydroxyapatite cement.

Diagnosis of Congenital Otogenic CSF Fistula Combined with Recurrent Meningitis in Children / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Otogenic cerebrospinal fluid (CSF)fistula referrs to the abnormal communication between the CSF and the middle ear space due to defects in the inner ear, and is one of the causes of recurrent meningitis. MATERIALS AND METHODS: We report on five children with congenital otogenic CSF fistula, presented by recurrent meningitis and confirmed by surgical exploration. We also propose diagnostic steps for detecting otogenic fistula in the children based on our experiences and paper review. RESULTS: We used the metrizamide CT in the diagnostic procedure for most cases, although not all. The temporal bone CT was an useful initial diagnostic step for clinically suspicious cases. CONCLUSION: No one test or combination of specific tests were found to accurately predict the presence or absence of CSF fistula. It is thought that the only way to diagnose the CSF fistula is by surgical exploration. If the CSF fistula was suspected, aggressive diagnostic evaluation was needed in order to prevent recurrence.