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

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

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: 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: Co-administration of kanamycin (KM) with the loop diuretic ethacrynic acid (EA) has been known to produce a rapid and profound hearing loss in adult animals. The objective of this study was to see if monitoring the hearing status during intravenous infusion of EA could minimalize individual variability and to evaluate the correlation between the dose of EA and the body weight (wt). MATERIALS AND METHOD: Twenty cats with the mean age of 24 weeks+/-3.7 (range, 20.6-28.3 weeks) and the mean weight of 3.27 kg+/-0.75 (range 2.4-4.75 kg) received a subcutaneous injection of KM (300 mg/kg) followed by an intravenous infusion of EA (1 mg/min). Click evoked auditory brainstem responses (ABRs) were recorded to monitor the hearing during the infusion. When the ABR thresholds rose to levels in excess of 90 dB SPL, the infusion of EA was stopped. The histopathologies for sections of apex, middle, base of cochlea were examined after 6 months. RESULTS: There was a significant positive correlation (p<.001, r2=.583) between the EA dose and body weight. Cochlear histopathology showed an absence of organ of Corti and decrease of spiral ganglion cells in the majority of cochleas, especially in the basal turn. The extent of loss of spiral ganglion cells was dependent on their distance from the round window. CONCLUSION: Monitoring the animal's hearing status during the procedure ensured that the dose of EA was optimized for individual animals. Thus, the positive correlation between the EA dose and body weight should be considered should in designing the animal models of controlled high frequency hearing loss.
Adult ; Aminoglycosides ; Animals ; Body Weight ; Cats ; Cochlea ; Deafness ; Ethacrynic Acid ; Evoked Potentials, Auditory, Brain Stem ; Hearing ; Hearing Loss ; Humans ; Infusions, Intravenous ; Injections, Subcutaneous ; Kanamycin ; Models, Animal ; Organ of Corti ; Organothiophosphorus Compounds ; Spiral Ganglion

BACKGROUND AND OBJECTIVES: Bone anchored hearing aid (BAHA) is an alternative method applicable to patients with chronic draining ear, congenital aural atresia and single sided deafness, who cannot benefit from conventional air conduction hearing aids. The objective of this study was to evaluate the experience of 14 patients who underwent BAHA surgery. SUBJECTS AND METHOD: We retrospectively reviewed the medical records of 14 patients who underwent BAHA surgery. Preoperative pure tone air and bone conduction thresholds and air-bone gap, postoperative BAHA-aided thresholds were measured. Hearing improvement as a result of implantation and complications related to implant were evaluated. RESULTS: The most common indication for BAHA was congenital aural atresia (8 patients) and the rest consisted of chronic otitis media (3 patients) and unilateral sensorineural hearing loss (3 patients). The average threshold improvement with BAHA was 40 dB and 34 dB in patients with congenital aural atresia and chronic otitis media, respectively. Patients with unilateral hearing loss had a postoperative aided threshold of 25 dB. Complications were limited to the periabutment skin problem in two patients. One patient received revision surgery replacing the diseased skin with split-thickness skin graft from a thigh and the other patient received surgery to remove the abutment and the wound was closed with rotation flap, leaving the fixture underneath the skin. CONCLUSION: BAHA could be one of the safe and reliable treatment options available for auditory rehabilitation. Systematic evaluation for candidate selection might be needed to increase hearing gain and decrease co-morbidity.
Bone Conduction ; Deafness ; Ear ; Hearing ; Hearing Aids ; Hearing Loss, Conductive ; Hearing Loss, Sensorineural ; Hearing Loss, Unilateral ; Humans ; Medical Records ; Otitis Media ; Postoperative Complications ; Retrospective Studies ; Skin ; Suture Anchors ; Thigh ; Transplants