Surgical outcomes of early congenital cholesteatoma: minimally invasive transcanal approach.

OBJECTIVES/HYPOTHESIS: To introduce a simple and alternative surgical technique, minimally invasive transcanal myringotomy (MITM), for early stage congenital cholesteatoma in children and to evaluate the feasibility and results of MITM for management of early stage congenital cholesteatoma with respect to its effectiveness and safety. STUDY DESIGN: Retrospective review. METHODS: Between August 2008 and September 2012, a total of 36 patients with congenital cholesteatoma met the inclusion criteria and were analyzed. Patient medical records, including demographic characteristics, intraoperative findings, and follow-up records, were reviewed. RESULTS: Subjects consisted of 23 males (64%) and 13 females (36%), and the age at operation ranged from 12 months to 6 years (mean age = 3 years and 6 months). The number of congenital cholesteatoma was as follows: 26 patients at stage I and 10 patients at stage II. The follow-up duration was between 12 and 56 months, with an average of 30 months. There were no postoperative complications such as tympanic membrane perforation, dizziness, or secondary middle ear infection. Among 36 patients who had undergone the MITM approach for the treatment of congenital cholesteatoma, five (13.8%) showed recurrence and underwent a second-look operation. CONCLUSIONS: On the basis of our data, the MITM approach is a useful surgical technique for early stage congenital cholesteatoma in children. It has many advantages, in that there is no external wound and it is a simple surgical technique that involves easy postoperative care, a short operation time and hospitalization period, avoidance of serious complications, and easy repeatability for recurrence.

Voxel-wise analysis of diffusion tensor imaging for clinical outcome of cochlear implantation: retrospective study.

OBJECTIVES: To evaluate retrospectively, the possible difference in diffusion tensor imaging (DTI) metric of fractional anisotropy (FA) between good and poor surgical outcome cochlear implantation (CI) patients using investigator-independent voxel-wise analysis. METHODS: Eighteen patients (11 males, 7 females; mean age, 5.9 years) with profound sensorineural hearing loss underwent DTI scans using a 3.0 Tesla magnetic resonance scanner. Among the 18 patients, 10 patients with categories of auditory performance (CAP) score over 6 were classified into the good outcome group and 8 patients with CAP score below 6 were classified into the poor outcome group. The diffusion tensor scalar measure was calculated from the eigenvalues of the tensor on a voxel-by-voxel basis from each subject and two-sample t-test evaluation between good and poor outcome subjects were performed for each voxel of FA values, across the entire brain, with a voxel-wise intensity threshold of P<0.0005 (uncorrected) and a contiguous cluster size of 64 voxels. Individual values of FA were measured by using the region-of-interest based analysis for correlation analysis with CAP scores, open sentence and open word scores. RESULTS: Two-sample t-test evaluation using SPM voxel-wise analysis found significantly higher FA values at the several brain areas including Broca's area, genu of the corpus callosum, and auditory tract in good outcome subjects compared to poor outcome subjects. Correlation analyses between FA and CAP scores, open sentence and open word scores revealed strong correlations at medial geniculate nucleus, Broca's area, genu of the corpus callosum and auditory tract. CONCLUSION: Investigator-independent voxel-based analysis of DTI image demonstrated that good outcome subjects showed better neural integrity at brain areas associated with language and auditory functions, suggesting that the conservation of microstructural integrity of these brain areas is important. Preoperative functional imaging may be helpful for CI.

Construction of a DNA chip for screening of genetic hearing loss.

OBJECTIVES: Hearing loss is the most common sensory disorder in humans and genetic causes are estimated to cause more than 50% of all incidents of congenital hearing loss. To develop an efficient method for a genetic diagnosis of hearing loss, we have developed and validated a genetic hearing loss DNA chip that allows the simultaneous analysis of 7 different mutations in the GJB2, SLC26A4, and the mtDNA 12S rRNA genes in Koreans. METHODS: A genotyping microarray, based on the allele-specific primer extension (ASPE) method, was used and preliminary validation was examined from the five patients and five controls that were already known their genotypes by DNA sequencing analysis. RESULTS: The cutoff Genotyping index (GI) of genotyping for each mutation was set up and validated to discriminate among the genotypes. The result of the DNA chip assay was identical to those of previous results. CONCLUSION: We successfully designed the genetic hearing loss DNA chip for the first time in Korea and it would be useful for a clinical genetic diagnosis of hearing loss. Further consideration will be needed in order to examine the accuracy of this DNA chip with much larger patient sample numbers.

Endoscopic repairment of septal perforation with using a unilateral nasal mucosal flap.

OBJECTIVES: Nasal septal perforation is an anatomic defect of the cartilaginous and bone tissues of the nasal septum. Many approaches and techniques to repair nasal septal perforations have been reported on. The purpose of this paper is to report on our surgical technique and the results of the treatment for nasal septal perforations. METHODS: From May 2001 to March 2008, 14 patients (12 males and 2 females; mean age: 41.3 yr) were enrolled. The mean perforation size was 15 mm, and all the perforations were located at the cartilaginous portion. Our surgical technique is based on an endoscope-assisted endonasal approach, with dissection of unilateral advanced mucosal flaps with using a temporalis fascia graft. The follow-up periods ranged from 3 to 23 months (mean follow-up period: 8 months). RESULTS: Using our surgical technique on 14 patients, 12 cases (85.7%) of septal perforation were closed without complication. The remaining two patients (14.3%) had incomplete closures (about 2-3 mm) without any significant symptoms related to the remaining perforation. CONCLUSION: Our technique is a viable procedure with a high success rate for achieving closure of nasal septal perforations. It has the advantages of shortening the operative time, no external incision and avoiding any other perforation during the operation. Therefore, we consider it to be a good alternative for repairing nasal septal perforations.