Bilateral Cochlear Implantation in Facioscapulohumeral Muscular Dystrophy Presenting Sensorineural Hearing Loss / 대한이비인후과학회지

Facioscapulohumeral muscular dystrophy (FSHD) presents a muscular weakness in the face, shoulder girdle, and legs. In addition, bilateral, progressive, high-frequency sensorineural hearing loss (SNHL) can be expressed. A 3-year-old boy visited with bilateral facial paralysis and bilateral hearing loss. Audiological evaluations revealed bilateral, progressive, sloping SNHL and bilateral hearing aids was used for more than 3 years. Cochlear implantation was carried out on left side when he was 6 years old and on right side when he was 7 years old. Seven months after cochlear implantation on left side, his shoulder muscle weakness was found and the genetic analysis showed decreased D4Z4 repeat size in 4qA allele confirming a diagnosis of FSHD. After auditory rehabilitation using electroacoustic stimulation, his hearing and speech perception were much improved. This case suggests that cochlear implantation can be beneficial in patients with SNHL associated with FSHD.

Long-term Outcome of Cochlear Implant in Patients with Chronic Otitis Media: One-stage Surgery Is Equivalent to Two-stage Surgery

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.

A Case of Post-Traumatic Meniere's Disease

There are only a few reports of post-traumatic Meniere's disease and there is few literature that contains detailed data associated with the disease. We report a case of post-traumatic Meniere's disease. He suffered from tinnitus, fluctuating sensorineural hearing loss, and recurrent vertigo. Symptomatic medical treatment was not helpful and neither was soft tissue plugging around the oval and round windows during exploratory tympanotomy. Three months after soft tissue plugging, endolymphatic sac decompression surgery was performed. The patient's symptoms improved markedly thereafter. The clinical significance of post-traumatic Meniere's disease is described and we present a brief review of the literature.

A Case of Acute Mastoiditis Complicated with Epidural Abscess / 대한이비인후과학회지

Epidural abscess is the most common intracranial complication of acute mastoiditis and may lead to a life-threatening condition if an appropriate treatment is not performed. The treatment of choice is a surgical eradication of lesion, drainage of the abscess, and intravenous antibiotic therapy. We report a case of acute mastoiditis followed by an epidural abscess in the posterior cranial fossa in a 32-month male patient.

Hearing Rehabilitation with Bone Anchored Hearing Aid: Experience in 14 Patients / 대한이비인후과학회지

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.

The Analysis of Prognostic Factor and Treatment Outcome of Malignancies of the External Auditory Canal / 대한이비인후과학회지

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.

CO2 Laser Stapedotomy / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: CO2 laser is known to have optimal tissue characteristics for stapes surgery though it has suboptimal optical characteristics. Surgical experience of stapes surgery using CO2 laser has not been previously reported in Korea. In this study, authors assessed the functional outcomes of stapes surgery using CO2 laser and evaluated its merits and drawbacks. SUBJECTS AND METHOD: We retrospectively reviewed the medical records of 25 patients (28 ears) who underwent CO2 laser stapedotomy between November 2003 and December 2007. The patient's mean age was 42 years (range, 15-70), with the patients consisting of 11 males and 14 females. Follow-up duration ranged from 6 to 49 months and the mean observation time was 18.2+/-13.2 months. Hearing improvements at the final examination were evaluated by airbone gap closure. RESULTS: The pre-operative mean bone conduction threshold, air conduction thresholds and mean air-bone gap were 21.0+/-8.8 (mean+/-SD) dB, 51.3+/-7.2 dB and 30.1+/-7.8 dB, respectively. After stapedotomy, the mean bone conduction and air conduction thresholds were 20.5+/- 9.7 dB, 30.6+/-16.1 dB, respectively, and the mean air-bone gap was 10.1+/-7.1 dB at the last audiologic follow-up. The 'best results' were 18 cases (64.3%) and 'good results' were 8 cases (28.6%). The overall "succeccful" hearing improvement was achieved in 26 ears (92.9%). One case was classified as 'failure' and two cases developed post-operative benign paroxysmal positional vertigo. CONCLUSION: 92.9% of patients achieved satisfactory hearing results using CO2 laser stapedotomy. Although the manipulation of CO2 laser is difficult and limitation in gaining the anterior crus exposure maybe encountered due to suboptimal optical properties, these difficulties were not influential in gaining expertise of the instrument.

Long Term Outcomes of Early Cochlear Implantation in Korea

OBJECTIVES: The objective of this study was to compare the long-term auditory performance and language skill depending on the age of cochlear implantation in the Korean population. We especially tried to separate the effect of maturation/development from that of the age at implantation. METHODS: Eighty-six pre-lingual children with profound hearing loss who underwent a cochlear implantation before the age of six and had been followed for more than 3 yr were included in this study prospectively. Categories of Auditory Performance (CAP) and Korean Picture Vocabulary Test (K-PVT) were serially followed up. In order to separate the age at implantation effect, K-PVT results were readjusted to the child's chronological age in the normal hearing population. RESULTS: When the CAP and K-PVT scores were directly compared without chronological readjustment, we failed to show a significant difference for improvements according to the age at implantation. Early cochlear implantation was associated with better language development, only when the K-PVT scores were readjusted to percentile scores of their chronological age. CONCLUSION: Early cochlear implantation was associated with better language development even within the critical period. This advantage may be recognized only when the effect of the age at implantation is separated from the effect of maturation/development.

Role of Radiotherapy for Squamous Cell Carcinoma of the External Auditory Canal and Middle Ear / 대한방사선종양학회지

PURPOSE: To investigate the role of radiotherapy for squamous cell carcinomas of the external auditory canal and middle ear. MATERIALS AND METHODS: A series of 35 patients who were treated at a single institution from 1981 through 2007 were retrospectively analyzed. Thirteen patients were treated by radiotherapy alone; four by surgery only and 18 by a combination of surgery and radiotherapy. The total radiation dose ranged from 39~70 Gy (median, 66 Gy) in 13~35 fractions for radiotherapy alone and 44~70 Gy (median, 61.2 Gy) in 22~37 fractions for the combined therapy. Clinical end-points were the cause of specific survival (CSS) and local relapse-free survival (LRFS). The median follow-up time was 2.8 years (range, 0.2~14.6 years). RESULTS: The 3-year CSS and LRFS rate was 80% and 63%, respectively. Based on a univariate analysis, performance status and residual disease after treatment had a significant impact on CSS; performance status and histologic grade for LRFS. Patients treated by radiotherapy alone had more residual disease following the course of treatment compared to patients treated with the combined therapy; 69% vs. 28%, respectively. CONCLUSION: Our results suggest that radiation alone was not an inferior treatment modality for CSS compared to the combined therapy for squamous cell carcinoma of the external auditory canal and middle ear. However, local failure after radiotherapy is the main issue that will require further improvement to gain optimal local control.

Outcome Analysis of Cochlear Implantation in Elderly Patients / 대한이비인후과학회지

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.

Analysis of Risk Factors for the Occurrence of Residual Cholesteatoma after Congenital Cholesteatoma Surgery / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: A higher residual rates after surgery have been reported in patients with congenital cholesteatoma than those with acquired cholesteatoma. The aim of this study was to document the risk factors of residual cholesteatoma after surgery for congenital cholesteatoma. SUBJECTS AND METHOD: From 1989 to 2006, 90 patients with congenital cholesteatoma treated at the Department of Otorhinolaryngology, Seoul National University Hospital were investigated retrospectively. Data were analyzed according to the location, type, stage, ossicular involvement, and initial surgery types for residual cholesteatoma. RESULTS: Residual cholesteatoma was detected in 18.9% (17/90) of total patients. With respect to residual cholesteatoma, significant risk factors were found with the open type, mastoid involvement and stapes supra-structure erosion. On the other hand, location, initial surgery types did not show statistical significance. CONCLUSION: The Staged 2nd look operation should be performed for patients with congenital cholesteatoma, especially in either case of the open type, advanced stage or in the presence of supra-structure erosion.

Hearing Changes after Stapes Surgery in Otosclerosis / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Hearing loss is the main symptom of otosclerosis. Stapes surgery could restore the hearing ability, but the amount of restoration was changed as time passed. The aims of this study were to analyze the outcome of primary stapes surgery in otosclerosis and to compare hearing changes in stapedectomy and stapedotomy group. SUBJECTS AND METHOD: This study involved 221 ears, which were operated on for otosclerosis in Seoul National University Hospital from 1983 to 2005. We analyzed the hearing results of stapes surgery regarding air conduction, bone conduction, air-bone gap using the operation methods in preoperative, postoperative 1, 3, 6, 12, 24 months and after 36-months period. RESULTS: Stapedectomy was performed in 75 ears and stapedotomy in 146 ears. There were no differences of mean age, preoperative hearing between stapedectomy and stapedotomy group. In the postoperative period, hearing thresholds were significantly decreased compared with preoperative period in each group. The stapedectomy group showed more improvement of air conduction and reduction of AB gap than the stapedotomy group. Results of bone conduction showed no difference after the postoperative 12 months period between the two groups. Hearing improvement went on upto postoperative 12 months. Development of sensorineural hearing loss was found in two patients (2.7%) in the stapedectomy group and in one patient (0.68%) in the stapedotomy group. CONCLUSION: Stapedectomy showed better hearing results, but had more risks of sensorineural hearing loss in this study.

Long Term Treatment Results of Endolymphatic Sac Decompression in Meniere's Disease / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The treatment result of endolymphatic sac decompression (ELSD) is controversial especially after a long term follow-up period. The aims of this study are to review the long term treatment outcome of ELSD and to analyzethe factors associated with the long term prognosis. SUBJECTS AND METHOD: A retrospective review of medical records was performed. Sixteen patients (18 ears) who had been diagnosed with definite Meniere's disease, had undergone ELSD and been followed up for more than 2 years were included in this study. The average follow up period was 88.6 months, with the range spanning from 25.7 to 243.4 months. All results were described according to the 1995 AAO-HNS criteria. RESULTS: After a long term follow-up, vertigo was successfully controlled in 66.6% and hearing was preserved or improved in 58.8%. Tinnitus and aural fullness were relieved in 27.3% and 60.0% respectively. The preoperative hearing threshold (p=0.03) and caloric test (p=0.05) showed a close relationship with the long term vertigo control after ELSD. CONCLUSION: Although vertigo and hearing may deteriorate again after 2 years, ELSD generally seems to be able to control vertigo even after a long term follow up. Preoperative hearing threshold and caloric test may be able to serve as prognostic factors.

Clinical Outcomes of Cochlear Reimplantation Due to Device Failure

OBJECTIVES: The aim of this study was to evaluate the clinical features of cochlear reimplantation due to device failure. METHODS: The medical records of 30 patients who had undergone a revision cochlear implantation were retrospectively reviewed. Causes of revision operations, number of electrode channels inserted, and postoperative speech performances were analyzed. RESULTS: Device failure (N=12, 38.7%) and hematoma (N=3, 9.6%) were the two most common reasons for revision surgery. In patients with device failure, the number of electrode channels reinserted was equal to, or more than the number of channels inserted during initial implantation. Speech performance scores remained the same, or improved after reimplantation in patients with device failure. CONCLUSION: Device failure was the most common cause of revision operation in patients with cochlear implanttion. Contrary to expectation, new electrodes were fully inserted without difficulty in all reimplantation cases. Intracochlear damage due to reimplantation appeared to be clinically insignificant.

Clinical Outcomes of Cochlear Reimplantation Due to Device Failure

OBJECTIVES: The aim of this study was to evaluate the clinical features of cochlear reimplantation due to device failure. METHODS: The medical records of 30 patients who had undergone a revision cochlear implantation were retrospectively reviewed. Causes of revision operations, number of electrode channels inserted, and postoperative speech performances were analyzed. RESULTS: Device failure (N=12, 38.7%) and hematoma (N=3, 9.6%) were the two most common reasons for revision surgery. In patients with device failure, the number of electrode channels reinserted was equal to, or more than the number of channels inserted during initial implantation. Speech performance scores remained the same, or improved after reimplantation in patients with device failure. CONCLUSION: Device failure was the most common cause of revision operation in patients with cochlear implanttion. Contrary to expectation, new electrodes were fully inserted without difficulty in all reimplantation cases. Intracochlear damage due to reimplantation appeared to be clinically insignificant.

Treatment Result of Mastoidectomy in Pediatric Chronic Suppurative Otitis Media / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Chronic suppurative otitis media (CSOM) is a common infectious disease in childhood of poor hygiene group. In addition, in industrialized countries, with rising popularity of tympanostomy tubes for management of otitis media with effusion, CSOM is a potential problem in any child who had inserted ventilating tubes previously. Hearing loss following CSOM can negatively affect learning, speech, and cognitive function, so effective and timely management of CSOM is mandatory. The surgical procedure may be necessary in some children with CSOM, but when it is performed at a too early age, there might be more chances of recurrence than adults. SUBJECTS AND METHOD: Used in the study were 51 ears from patients who received tympanomastoidectomy due to CSOM in tertiary referral center from 1995 through 2004. The mean age was 9 years and 6 months. Cholesteatoma was found in 30 cases (58.8%). Age at operation, hearing outcomes, and the surgical results were retrospectively reviewed. RESULTS: Canal wall down mastoidectomy was performed in 23 cases and canal wall up procedure in 28 cases. Revision surgery was performed in 9 cases (17.6%) and mean duration between first and revision surgery was 27 months. In the cases of cholesteatoma, the rate of revision surgery was different between under 6 years old and over 7 years old group (p=.045). The age at 1st operation and postoperative hearing outcome had no correlation. CONCLUSION: Since the1st operation is performed earlier, the rate of revision may increase, especially in cases of cholesteatoma.

Clinical Features and Solutions of Facial Nerve Stimulation after Cochlear Implantation in Deaf Children / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Facial nerve stimulation (FNS) as a complication of cochlear implantation can produce significant discomfort, limit effective use of cochlear implant, and require extensive reprogramming in some patients. The purpose of this study is to review the clinical features of children with FNS after cochlear implantation and to discuss its possible solutions. SUBJECTS AND METHOD: Thirteen children who had FNS after cochlear implantation were included. Their medical records were reviewed retrospectively regarding the presence of inner ear anomaly (IEA), the programming techniques for cochlear implant, timing and progression of FNS, and the management of it. RESULTS: Ten out of 13 children (76.9%) with FNS had IEA. In those 10 patients with IEA, FNS appeared within 6 months from the operation and showed a tendency of being relevant to all electrodes. Authors used four methods to eliminate FNS. They included (a) turning off the specific electrodes when FNS seems related to some specific electrodes, (b) changing the coding strategy or the programming mode, which proved not to be effective, (c) reducing the C-level, which resulted in severe narrowing of dynamic range as well as a relative control of FNS, and (d) surgical exploration in specific cases. CONCLUSION: FNS after CI is at greater risk for IEA. FNS in those cases can interfere with the progression of speech development. This should be sufficiently informed of the parents of CI candidates with IEA preoperatively. Surgical exploration can be reserved for elimination of FNS in specific cases.

Developmental Analysis of Calretinin Using Quantitative Analysis in Rat Cochlea / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Calretinin is a neuron specific, high affinity cytosolic calcium-binding protein of the EF-hand family. In the mammalian inner ear, it is expressed in the organ of Corti and most of the spiral ganglion neurons. Authors observed the change of expression and amount of calretinin according to the maturation in the rat cochleae. MATERIALS AND METHOD: Sprague-Dawley rat cochleae collected from each stage (postnatal day (P) P5, P17, P35) were analyzed using 2D gel electrophoresis, proteomic analysis, Western blot analysis, and fluorescence immunocytochemistry. RESULTS: In P17 and P35, calretinin was identified at an isoelectric point (pI) of 4.9 and a molecular weight of 29 kDa in the analysis of the rat cochlea proteins using proteomic analysis. P17 and P35 revealed remarkable existence of calretinin in 2D gel electrophoresis and the Western blot, whereas P5 demonstrated no existence in 2D gel electrophoresis and weak expression in the Western blot. In fluorescence immunocytochemistry, P17 and P35 showed intense calretinin immunoreactivity in the inner hair cells and most ganglion neurons, but P5 displayed no immunoreactivity in inner hair cells and very weak expression in spiral ganglion cells. CONCLUSION: Compared with the early neonatal stage, an amount of calretinin remarkably increases during the critical period of the cochlear maturation and is maintained until the young adult stage. These results suggest that calretinin may have a specific role as a calcium-binding protein since the cochlear maturation in the rat inner ear.

Immunologic Screening Test and Type II Collagen Autoimmunity in Meniere's Disease

BACKGROUND AND OBJECTIVES:Altered immunity against one's own tissue, especially inner ear, is in part responsible for the development of Meniere's disease (MD). Since immunologic screening test results are usually nonspecific, even if tested for those patients suspicious of autoimmune inner ear disease, they do not seem to provide valuable information. The aim of this study is to evaluate the validity of immunologic screening laboratory tests for MD patients with type II collagen (CII) autoimmunity and to discuss the diagnostic role in this localized immunologic disease. MATERIALS AND METHOD:Thirty-six patients of MD in which immunologic screening laboratory test result was available were included in this study and their clinical features were described according to the criteria of AAO-HNS (1995). They were divided into two groups, CII(+) (N=8) or CII(-) (N=28) according to the presence of anti-CII antibody determined by ELISA method as described earlier. Rheumatoid factor (RF), Fluorescent antinuclear antibody (FANA), immunoglobulin G, M and A, and complement 3 and 4 were included for immunologic screening test. Individual test and clinical features were compared between groups. RESULTS:Any single test did not show significant correlation between groups. But RF, total IgG and the proportion of patients more than at least one marker are higher in CII(+) group with borderline significance. CONCLUSION:Higher positive rate of immunologic screening test may support the immunologic involvement in CII(+) group. However the role of this screening test seems to be limited in a localized disease like MD compared systemic immunologic disorder, such as rheumatoid arthritis.

A Preliminary Study of Predictive Factors for Language and Cognitive Development after Cochlear Implant in Congenital Deaf Children / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The cognitive function of the cochlear implant (CI) candidates and psychological factors of their family may have influence on the rehabilitation after CI. However, only a few studies have been reported. The aim of the study was to examine the cognitive and language abilities of deaf children before CI. We also assessed educational opportunities that deaf children have been given, emotional problems of their parents, and investigated the relationship of these variables to the cognitive function of the deaf children. SUBJECTS AND METHOD: Sixteen deaf children from 5 to 7 years old and 100 parents having a deaf child participated in the study. The psychological test battery was administered to the deaf children to assess intelligence, attention and motor coordination. Questionnaires were given to the parents to get the medical and educational history of their child, the familial characteristics, parental attitude and emotional problems. RESULTS: Congenital deaf children showed high social adaptation abilities on Social Maturity Scale and borderline level intelligence in non-linguistic intelligence test. However, they showed low scores on visual attention task and significantly decreased visual-motor coordination on complex tasks. Seventeen parents (18.5%) met the criteria of depressive disorder by Beck Depressive Inventory. The parental stress and depressive mood were negatively correlated with the language and cognitive development of their child. CONCLUSION: The result of this study suggests that preoperative cognitive development of deaf children and emotional problem of their parents may affect on the development of language and cognitive abilities during the rehabilitation period after cochlear implantation.