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.
Child ; Cochlear Implantation ; Cochlear Implants ; Ear, Inner ; Electrodes ; Facial Nerve Injuries ; Hearing Loss, Sensorineural ; Humans ; Incidence ; Meningitis ; Uncertainty

OBJECTIVES: Although it is well recognized that a small meatus after canal wall down (CWD) tympanomastoidectomy can cause a lifetime problem, unsatisfactory results are frequently encountered. We herein introduce a novel technique, perichondrial posterior fixation (PPF), to maintain a wide external auditory canal (EAC), to minimize postoperative wound infection due to the smaller dead space and to improve the posterior auricular cosmetic outcome. METHODS: A total of 73 patients who underwent CWD tympanomastoidectomy were included. Interventions are CWD tympanomastoidectomy with the PPF technique. Review of the medical records and evaluation of the postoperative size of the meatus and the extent of the cavum conchal cartilage buried within the mastoid cavity by taking digital photographs. RESULTS: Thirty males and 43 females were included and the mean age was 44.1 yr (range, 6 to 66 yr). The mean follow-up duration was 26 months (range, 12 to 56 months). All ears maintained a clean and large external meatus. The posterior auricular cavum conchal cartilage was successfully prevented from being buried into the mastoid cavity in all ears. The extent of cartilage buried into the mastoid cavity was much reduced compared to the conventional technique. CONCLUSION: The PPF technique, which is a novel meatoplasty technique in CWD tympanomastoidectomy, seems to be effective in maintaining a large external meatus and improving the cosmetic outcome with minimal risk of complications.
Cartilage ; Cosmetics ; Ear ; Ear Canal ; Female ; Follow-Up Studies ; Humans ; Male ; Mastoid ; Medical Records ; Surgical Wound Infection

Brain hernia into the middle ear cavity is quite a rare entity which is often caused by chronic otitis media with cholesteatoma and surgical complications. Other unusual factors such as extension of the intracranial meningioma, primary temporal bone meningioma and aberrant arachnoid granulation can also cause hernia of brain tissue. Brain hernia can be diagnosed through temporal bone computed tomography (TBCT) and brain magnetic resonance image (MRI), which show the location of bony defect and similar signal intensity between the herniated tissue and brain. We present a 50-year old female patient, who suffered from otorrhea 8 years ago; otorrhear disappeared 4 years ago and rhinorrhea 1 years ago. She had not have any history of chronic otitis media and otologic surgery. The herniated cerebellum was diagnosed through the TBCT and brain MRI. She was successfully cured after cerebellar reduction operation without any postoperative complications.
Arachnoid ; Brain ; Cerebellum ; Cerebrospinal Fluid Otorrhea ; Cholesteatoma ; Ear, Middle ; Encephalocele ; Female ; Hernia ; Humans ; Magnetic Resonance Spectroscopy ; Meningioma ; Otitis Media ; Postoperative Complications ; Temporal Bone

BACKGROUND AND OBJECTIVES: Transient evoked otoacoustic emission (TEOAE) has an increasingly signficant role in pediatric otological practice such as screening hearing in newborn babies. To assess the potential applicability of the TEOAE measurements on the sequential monitoring of pediatric patients with middle ear effusion (MEE), we investigated the effects of MEE on the TEOAE responses. MATERIALS AND METHODS: TEOAE responses were recorded from 33 patients (56 ears) before and after ventilation tube insertion. RESULT: In the 37 cases with MEE, we found that the average band reproducibility below 2 kHz was recovered significantly after the tube placement, although the band reproducibility at 5 kHz was diminished. In the 19 cases without MEE, no significant changes in echo amplitude and band reproducibility were noted in the postoperative TEOAE measurements compared to the preoperative measurements. CONCLUSION: The results of this study suggest that an improvement in echo response and band reproducibility less than 2 kHz in serial TEOAE measurements may indicate the resolution of MEE in children with OME.
Child ; Hearing ; Humans ; Infant, Newborn ; Mass Screening ; Otitis Media with Effusion* ; Otitis Media* ; Otitis* ; Ventilation

Background: Phospholipase C-γ (PLC-γ) plays a crucial role in immune responses and is related to the pathogenesis of various inflammatory disorders. In this study, we investigated the role of PLC-γ and the therapeutic effect of the PLC-specific inhibitor U73122 using orbital fibroblasts from patients with Graves’ orbitopathy (GO). Methods: The expression of phospholipase C gamma 1 (PLCG1) and phospholipase C gamma 2 (PLCG2) was evaluated using polymerase chain reaction in GO and normal orbital tissues/fibroblasts. The primary cultures of orbital fibroblasts were treated with non-toxic concentrations of U73122 with or without interleukin (IL)-1β to determine its therapeutic efficacy. The proinflammatory cytokine levels and activation of downstream signaling molecules were determined using Western blotting. Results: PLCG1 and PLCG2 mRNA expression was significantly higher in GO orbital tissues than in controls (P<0.05). PLCG1 and PLCG2 mRNA expression was significantly increased (P<0.05) in IL-1β, tumor necrosis factor-α, and a cluster of differentiation 40 ligand-stimulated GO fibroblasts. U73122 significantly inhibited the IL-1β-induced expression of proinflammatory molecules, including IL-6, IL-8, monocyte chemoattractant protein-1, cyclooxygenase-2, and intercellular adhesion molecule-1 (ICAM-1), and phosphorylated protein kinase B (p-Akt) and p38 (p-p38) kinase in GO fibroblasts, whereas it inhibited IL-6, IL-8, and ICAM-1, and p-Akt and c-Jun N-terminal kinase (p-JNK) in normal fibroblasts (P<0.05). Conclusion PLC-γ-inhibiting U73122 suppressed the production of proinflammatory cytokines and the phosphorylation of Akt and p38 kinase in GO fibroblasts. This study indicates the implications of PLC-γ in GO pathogenesis and its potential as a therapeutic target for GO.

OBJECTIVES: P1 is a robust positivity at a latency of 50-150 msec in the auditory evoked potential of young children. It has been reported that over the first 2-3 years of life, there is a rapid decrease of the latency and the mean P1 latency in adults with normal hearing is approximately 60 msec. This study was designed to evaluate the change of the P1 latency in Koreans with normal hearing according to age and to compare this with the P1 latency of young patients with profound sensorineural hearing loss before and/or after cochlear implantation. METHODS: Among the patients who visited the Department of Otorhinolaryngology at Seoul National University Hospital from June 2007 to September 2009, the P1 response was recorded in 53 patients in the normal hearing group, in 13 patients in the pre-cochlear implantation (CI) group and in 10 patients in the post-CI group. A synthesized consonant-vowel syllable /ba/ was used to elicit the evoked responses. The evoked responses were collected using the center of the frontal head. For each subject, an individual grand average waveform was computed by averaging the ten recordings. The P1 latency was visually identified as a robust positivity in the waveform. RESULTS: For the normal hearing group, the P1 latency showed the pattern of shortening as the age increased (coefficient, -0.758; P<0.001). For the pre-CI group, 10 cases showed delayed latencies and 3 cases did not show the P1 wave. For the post-CI group, the P1 latencies showed a less delayed tendency than those of the pre-CI group, but this was not statistically different. CONCLUSION: This report provides the standard value of the P1 latency at each age in Koreans for the first time and the findings support that the maturation of the central auditory pathways could be measured objectively using the P1 latency.
Adult ; Auditory Pathways ; Child ; Cochlear Implantation ; Evoked Potentials, Auditory ; Head ; Hearing ; Hearing Loss, Sensorineural ; Humans ; Otolaryngology

The result of a series of thirteen translabyrinthine removals of acoustic neurinomas are reported. From 1990 March to 1993 May, thirteen patients with acoustic neuromas underwent translabyrinthine removal at Seoul National University Hospital with cooperation of Department of Neurosurgery and Otolaryngology. In four patients with the medium-sized(11-20 mm) tumors, complete removal with preservation of the facial nerve was possible. In nine patients with the large(21-40 mm) tumors, complete removal of the tumor was possible in seven. Preservation of the facial nerve was possible in all but one, whose facial nerve was incidentally transected at the distal intracanalicular portion and was anastomosed end-to-endly. In one, postoperative leakage of cerebrospinal fluid required reoperation. The results of translabyrinthine approaches were compared with those of suboccipital approaches performed during the same period. In medium-sized or large acoustic tumors, translabyrinthine approach was comparable with suboccipital approach in the rate of total tumors removal, preservation of the facial nerves, and complication.
Acoustics* ; Cerebrospinal Fluid ; Facial Nerve ; Humans ; Neuroma, Acoustic* ; Neurosurgery ; Otolaryngology ; Reoperation ; Seoul

This study compared long-term speech performance after cochlear implantation (CI) between surgical strategies in patients with chronic otitis media (COM). Thirty patients with available open-set sentence scores measured more than 2 yr postoperatively were included: 17 who received one-stage surgeries (One-stage group), and the other 13 underwent two-stage surgeries (Two-stage group). Preoperative inflammatory status, intraoperative procedures, postoperative outcomes were compared. Among 17 patients in One-stage group, 12 underwent CI accompanied with the eradication of inflammation; CI without eradicating inflammation was performed on 3 patients; 2 underwent CIs via the transcanal approach. Thirteen patients in Two-stage group received the complete eradication of inflammation as first-stage surgery, and CI was performed as second-stage surgery after a mean interval of 8.2 months. Additional control of inflammation was performed in 2 patients at second-stage surgery for cavity problem and cholesteatoma, respectively. There were 2 cases of electrode exposure as postoperative complication in the two-stage group; new electrode arrays were inserted and covered by local flaps. The open-set sentence scores of Two-stage group were not significantly higher than those of One-stage group at 1, 2, 3, and 5 yr postoperatively. Postoperative long-term speech performance is equivalent when either of two surgical strategies is used to treat appropriately selected candidates.
Adult ; Aged ; Cholesteatoma, Middle Ear/epidemiology ; Chronic Disease/therapy ; Cochlear Implantation/*adverse effects ; Cochlear Implants/*adverse effects ; Female ; Hearing Loss, Sensorineural/*surgery ; Humans ; Inflammation/epidemiology ; Male ; Middle Aged ; Otitis Media/*surgery ; Retrospective Studies ; *Speech Articulation Tests ; Treatment Outcome ; Young Adult

BACKGROUND AND OBJECTIVES: The purpose of this study was to analyze the survival rate treatment modalities and certain prognostic factors in patients with external auditory canal (EAC) cancer using the University of Pittsburgh TNM Staging System. SUBJECTS AND METHOD: Medical records and radiological findings of 33 patients treated for EAC cancer between January 1995 and January 2009 were reviewed retrospectively. The median follow-up period was 65 months (range, 12-182 months). RESULTS: The most common histological type was squamous cell carcinoma (19 patients; 57.6%), followed by adenoid cystic carcinoma (9 patients; 27.3%), basal cell carcinoma (1 patient), adenocarcinoma (1 patient), rhabdomyosarcoma (1 patient), undifferentiated carcinoma (1 patient) and malignant small round cell tumor (1 patient). Seven of 33 patients died of EAC cancer and the overall survival rate was 70.6%. There was a significant difference in the survival rate with respect to TNM stages (p=.031). The 10 yr-survival rate of patients with stage I disease was 100.0%, whereas those of stages II, III and IV were 50.0, 80.0 and 58.3%, respectively. Five of 7 patients with recurrence had a history of initial positive resection margin. CONCLUSION: Early detection and proper surgical treatment with sufficient resection margin are essential for disease-specific survival and prevention of recurrence in patients with EAC cancer.
Adenocarcinoma ; Aminocaproic Acids ; Carcinoma ; Carcinoma, Adenoid Cystic ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Ear Canal ; Follow-Up Studies ; Humans ; Medical Records ; Neoplasm Staging ; Prognosis ; Recurrence ; Retrospective Studies ; Rhabdomyosarcoma ; Survival Rate ; Treatment Outcome

BACKGROUND AND OBJECTIVES: Surgical indications and effect of auditory rehabilitation have not been established in elderly deaf patients who received cochlear implant (CI). The aim of this study is to analyze the outcomes of CI with respect to auditory performance and surgical complication in elderly deaf patients. SUBJECTS AND METHOD: Nineteen postlingually deafened patients who were 60 years and older at the time of implantation were included in the study from April 1992 to October 2006. They were compared to a group of 53 younger patients (<60 years) at the time of implantation. Surgical complications were identified and speech performance was evaluated preoperatively and postoperatively at 1 month, 3 months, 6 months, 1 year and 2 years. RESULTS: In the etiology, progressive hearing loss was most common in both groups. Perioperative complications such as temporary dizziness, wound problem, tinnitus, and temporary facial weakness showed no significant differences between elderly and control groups despite higher incidence of underlying disease in elderly patients. Preoperative speech performance (audiologic K-CID) was significantly better in control group than in elderly group, but there were no significant differences in postoperative period. CONCLUSION: The outcomes of CI in the elderly patients have been comparable with those of younger adults. Advanced age alone could not preclude anyone from being candidates of CI.
Adult ; Aged ; Cochlear Implantation ; Cochlear Implants ; Deafness ; Dizziness ; Hearing Loss ; Humans ; Incidence ; Postoperative Period ; Tinnitus