Unusual Tumors Obstructing the External Auditory Canal: Report of Two Cases

Primary tumors arising from the external auditory canal (EAC) are rare. We describe two cases of mass lesions within the EAC causing slowly progressive hearing loss without otorrhea or otalgia. Otoendoscopic examination demonstrated total obstruction of the EAC, and pure tone audiometry revealed conductive hearing loss. Based on the findings of the histopathologic examination, one patient was diagnosed with venous hemangioma that was treated using surgical resection, and the other patient was diagnosed with diffuse large B-cell lymphoma (DLBCL) that was treated using external-beam radiation therapy. Although primary tumors in the EAC are rare, both benign tumors such as venous hemangiomas and malignant lesions such as DLBCL should be considered as possible differential diagnoses of mass lesions in the EAC.

Direction-Changing and Direction-Fixed Positional Nystagmus in Patients With Vestibular Neuritis and Meniere Disease

OBJECTIVES: Direction-changing positional nystagmus (PN) was considered to indicate the presence of benign paroxysmal positional vertigo involving lateral semicircular canal in most cases. We investigated the incidence of PN on the supine head-roll test and compared the characteristics of nystagmus in patients with vestibular neuritis (VN) and Meniere disease (MD). METHODS: A retrospective review of patients, who were diagnosed with unilateral VN or unilateral definite MD between September 2005 and November 2011, was conducted. Sixty-five VN patients and 65 MD patients were enrolled. Eye movements were recorded for 30–60 seconds at the positions of sitting, head roll to the right, and head roll to the left, and maximum slow-phase eye velocity was calculated. PN was classified as direction-fixed (paretic or recovery) and direction-changing (geotropic or apogeotropic). RESULTS: Spontaneous nystagmus was observed in 57 patients (87%, the slow-phase eye velocity of 7°/sec±5°/sec) with acute VN, 39 (60%, 2°/sec±1°/sec) with follow-up VN, and 32 (49%, 2°/sec±2°/sec) with MD. Direction-fixed PN was the most common type. Direction-fixed paretic type was most common in acute VN (80%) and follow-up VN (42%), and direction-fixed recovery type was most common in MD (31%). Paretic type was significantly more common in acute VN (80%) than in follow-up VN (42%) and MD (26%), and the recovery type was significantly more common in MD (31%) than in acute VN (3%) and follow-up VN (14%). Direction-changing PN was more common in MD (22%), followed by follow-up VN (14%) and acute VN (9%). CONCLUSION: Though direction-fixed paretic PN was most common in VN and MD patients, direction-changing PN could be observed in a few patients (9%–20%) with peripheral vestibular disorders regardless of the duration from the onset of dizziness, suggesting the presence of otolith-related dizziness.

Influence of Fatigability on the Timing of Reassessment in the Treatment of Posterior Canal Benign Paroxysmal Positional Vertigo / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The early assessment of treatment is not done for benign paroxysmal positional vertigo (BPPV) since the well-known phenomenon of fatigability after a repeated positional test can mimic successful treatment. The aim of this study is to evaluate the clinical implication of ‘fatigability’ after Epley maneuver and to identify the therapeutic efficacy of Epley maneuver in posterior canal BPPV (PC-BPPV). SUBJECTS AND METHOD: This study was prospectively conducted by two dizziness clinics on 51 consecutive patients diagnosed with PC-BPPV. All patients included in the study received Epley maneuver treatment. The therapeutic results were reassessed immediately after a single trial of Epley maneuver. After 30 minutes, results were reassessed repeatedly to confirm the fatigability of diagnostic procedure immediately after treatment. If the treatment was not successful after 30 minutes, Epley maneuver was repeatedly performed until complete resolution. RESULTS: Immediately after the first maneuver, 45 of 51 (88.2%) patients had neither vertigo nor nystagmus during the positional test. All patients demonstrated complete resolution after receiving one to three Epley maneuvers on the day of diagnosis. ‘Fatigability (false negative result)’ was confirmed for only one case (1 of 6 patients, 16.7%), in which nystagmus was observed after 30 minutes but not identified immediately after the first Epley maneuver. CONCLUSION: The therapeutic efficacy of Epley maneuver is very high in PC-BPPV. Considering the possibility of fatigability when reassessment is performed immediately after therapeutic maneuver, clinicians should avoid assessing the outcome immediately after treatment in patients with PC-BPPV.

The Light Cupula: An Emerging New Concept for Positional Vertigo

Benign paroxysmal positional vertigo (BPPV) is the most common type of positional vertigo. A canalolithiasis-type of BPPV involving the lateral semicircular canal (LSCC) shows a characteristic direction-changing positional nystagmus (DCPN) which beats towards the lower ear (geotropic) on turning the head to either side in a supine position. Because geotropic DCPN in LSCC canalolithiasis is transient with a latency of a few seconds, the diagnosis can be challenging if geotropic DCPN is persistent without latency. The concept of “light cupula” has been introduced to explain persistent geotropic DCPN, although the mechanism behind it requires further elucidation. In this review, we describe the characteristics of the nystagmic pattern in light cupula and discuss the current evidence for possible mechanisms explaining the phenomenon.

Positional Vertigo Showing Direction-Changing Positional Nystagmus after Chronic Otitis Media Surgery: Is It Benign Paroxysmal Positional Vertigo? / 대한평형의학회지

This case report describes a patient who developed positional vertigo after surgery for chronic otitis media on the right side. Canal wall up mastoidectomy was performed, and the stapes was moderately mobilized during removal of the inflammatory granulation tissues that were attached to it. Immediately after the surgery, positional vertigo developed. The patient showed weakly left-beating spontaneous nystagmus in a seated position. Examination of positional nystagmus revealed geotropic direction-changing positional nystagmus with a prolonged duration and weak intensity in a supine head-roll test, which may be caused by a change in inner ear fluids due to a disruption of inner ear membrane around the oval window or penetration of toxic materials into the labyrinth during surgery.

Effects of an Online Imagery-Based Treatment Program in Patients with Workplace-Related Posttraumatic Stress Disorder: A Pilot Study

OBJECTIVE: We developed easily accessible imagery-based treatment program for patients with post-traumatic stress disorder (PTSD) related to workplace accidents and investigated the effects of the program on various PTSD related symptoms. METHODS: The program was based on an online platform and consisted of eight 15-min sessions that included script-guided imagery and supportive music. Thirty-five patients with workplace-related PTSD participated in this program 4 days per week for 4 weeks. Its effects were examined using self-report questionnaires before and after the take-home online treatment sessions. RESULTS: After completing the 4-week treatment program, patients showed significant improvements in depressed mood (t=3.642, p=0.001) based on the Patient Health Questionnaire-9 (PHQ-9), anxiety (t=3.198, p=0.003) based on the Generalized Anxiety Disorder seven-item (GAD-7) scale, and PTSD symptoms (t=5.363, p < 0.001) based on the Posttraumatic Stress Disorder Check List (PCL). In particular, patients with adverse childhood experiences exhibited a greater degree of relief related to anxiety and PTSD symptoms than those without adverse childhood experiences. CONCLUSION: The present results demonstrated that the relatively short online imagery-based treatment program developed for this study had beneficial effects for patients with workplace-related PTSD.

Clinical Pharmacokinetics of Caffeine in Korean Preterm Infants with Apnea of Prematurity

PURPOSE: Caffeine shows wide interindividual pharmacokinetic (PK) variation, and therapeutic drug monitoring (TDM) may be needed. The PK profile of caffeine in Korean preterm neonates was investigated, and factors influencing the clearance of caffeine were analyzed. METHODS: Fifty-nine preterm neonates receiving caffeine for apnea of prematurity were enrolled in the study (gestational age, 29.5±2.2 weeks and birth weight [BW], 1,318±358 g). Caffeine (20 mg/kg) was intravenously administered to each neonate as a loading dose, followed by a maintenance dose of 5-10 mg/kg/d. A total of 190 serum concentrations were measured for population PK analysis and modeling using nonlinear mixed-effects model (NONMEM®) software. RESULTS: The mean serum concentration of caffeine was 15.4±4.5 mg/L (range 7.8-33.0 mg/L). High serum concentrations (>20 mg/L) were noted in 36 samples (29%). At the first measurement of serum caffeine, the mean postmenstrual age was 33.9±2.3 weeks, mean BW was 1,802±471 g, mean duration of treatment was 7.4±9.4 days, and mean sampling time after the last dose was 21.8±2.1 hours. In the population PK analysis, the clearance was 0.033 L/h and volume of distribution was 0.371 L. Typical clearance was calculated as 0.0293×(BW/70)1.33. Among the subjects receiving 5 mg/kg/d caffeine, the most significant risk factor associated with high serum concentrations (>20 mg/L) was low BW (P=0.024). CONCLUSION: BW was the only covariate that influenced caffeine clearance in preterm neonates. Preterm neonates with low BW should be carefully monitored for apnea and adverse reactions in addition to undergoing TDM.

The Study of Combined Voice Therapy with Intralaryngeal Injection

BACKGROUND AND OBJECTIVES: Though voice therapy is proven to be effective in academia, treatment results and satisfaction level are inconsistent in clinics. The inconsistency may rise from diverse vocal cords misuse patterns in patient population as they suffer from different diseases or have bad phonation habits. In order to overcome the limitation, we uniformized patients' vocal cords with injection laryngoplasty prior to voice therapy. The efficacy of voice therapy and consistency in treatment results after injection laryngoplasty were assessed. MATERIALS AND METHODS: Patients diagnosed with vocal nodules were either treated with injection laryngoplasty followed by voice therapy (combined treatment group) or voice therapy only (voice therapy group). Each group consisted of 15 patients. Acoustic measures (jitter, shimmer, NHR), aerodynamic measures (MPT, Psub, MAFR), and subjective auditory conscious measures (K-VHI, K-VRQOL) between two groups were analyzed. RESULTS: After treatments, both groups improved in terms of acoustic, aerodynamic, and subjective auditory conscious measures compared to pre-treatment. Combined treatment group had statistically significantly greater improvement in shimmer, P in K-VHI, and PF in K-VRQOL compared to voice therapy group. CONCLUSION: Injection laryngoplasty treatment prior to voice therapy synergizes in treating patients compared to voice therapy only.

Correlation between Quantitative Electroencephalogram Findings and Neurocognitive Functions in Patients with Obsessive-Compulsive Disorder and Schizophrenia

OBJECTIVES: Obsessive-compulsive disorder (OCD) and schizophrenia have many common clinical and neurocognitive features. However, not all of them share the same underlying mechanism. The aim of this study was to discover evidences that indicate a pathophysiological mechanism specific to OCD by comparing correlations of quantitative electroencephalography (QEEG) patterns and neurocognitive function in patients with OCD and schizophrenia. METHODS: Resting-state QEEG data of total 265 patients were acquired retrospectively and parameters such as absolute power, relative power and peak frequency were analyzed from the data. Stroop test and Trail Making Test results as well as demographic features were reviewed for this study. The correlation of neurocognitive functions and brain electrical activities in each group were assessed and compared by correlation analysis. RESULTS: Compared with the OCD group, the schizophrenia group performed poorly in neurocognitive tests. Mean values of QEEG parameters in patients with OCD and schizophrenia did not show significant differences. Both absolute and relative power of alpha rhythm in central and frontal regions showed significant positive correlation with Stroop test results in OCD patients. CONCLUSIONS: Findings in this study shows distinctive correlations between frontal executive dysfunction and frontal alpha rhythm in the OCD patients, both of which might be a candidate for endophenotype underlying obsessive rumination.

Diagnosis of Severe Protein C Deficiency Confirmed by Presence of Rare PROC Gene Mutation

Protein C (PROC) deficiency is caused by mutations in the PROC gene on chromosome 2q14.3. Patients with PROC deficiency typically present distinguished purpura, intracerebral and intravascular coagulopathy, and ophthalmologic complications. Here, we report a rare severe form of PROC deficiency resulting from a compound heterozygosity in PROC. The patient was a 5-day-old female neonate born at 39 weeks of gestation with a birth weight of 2,960 g. She was transferred to our hospital with running a fever at 38.5℃ and with dark red patches on her feet. At admission, a complete blood count showed no specific findings, but levels of PROC and protein S were abnormally low (1% and 68%, respectively). Magnetic resonance imaging revealed intracerebral hemorrhaging and parenchymal damage with dysplasia of the brain. Ophthalmologic examination revealed vitreous hemorrhaging with retinal detachment. Genetic testing revealed a missense mutation (Arg211Trp) and a frameshift mutation (Gly239Serfs*8) in PROC, inherited from the father and mother, respectively. The patient recovered from purpura after undergoing ventriculoperitoneal shunting and treatment with fresh frozen plasma, warfarin sodium, and PROC concentrate. This is the first report of severe neonatal PROC deficiency with purpura fulminans, vitreous hemorrhage, and intracerebral hemorrhage confirmed via PROC genetic testing, which identified a rare compound heterozygosity of PROC.

Bilateral Internal Auditory Canal Metastasis of Non-small Cell Lung Cancer / Journal of the Korean Cancer Association, 대한암학회지

We report on a patient with brain metastasis involving bilateral internal auditory canal from non-small cell lung cancer (NSCLC). A 49-year-old woman who had been diagnosed with NSCLC (T2aN1M0) complained of persistent vertigo and bilateral tinnitus for three months. The patient had refused all treatments, including surgery and chemotherapy; however, she sought alternative medicine. The patient's hearing loss showed rapid progression bilaterally, and rotatory vertigo with peripheral-type nystagmus developed. Magnetic resonance imaging of the brain showed irregular nodular enhancement within both internal auditory canals with leptomeningeal enhancement and multiple intracranial metastasis. The patient was treated with epidermal growth factor receptor-tyrosine kinase inhibitor, and the tumor showed partial response. This was a rare case of multiple brain metastases involving bilateral internal auditory canal from known NSCLC presenting with vertigo and hearing loss.

Persistent Positional Vertigo in a Patient with Sudden Sensorineural Hearing Loss: A Case Report

Because inner ear organs are interconnected through the endolymph and surrounding endolymphatic membrane, the patients with sudden sensorineural hearing loss (SSNHL) often complain of vertigo. In this study, we report a patient with SSNHL accompanied by persistent positional vertigo, and serial findings of head-roll tests are described. At acute stage, head-roll test showed persistent geotropic direction-changing positional nystagmus (DCPN), which led to a diagnosis of SSNHL and ipsilateral light cupula. Although vertigo symptom gradually improved, positional vertigo lasted for more than 3 weeks. At this chronic stage, persistent apogeotropic DCPN was observed in a head roll test, which led to a diagnosis of the heavy cupula. Although the mechanism for the conversion of nystagmus direction from geotropic to apogeotropic persistent DCPN is unclear, the change of specific gravity of the endolymph might be one of the plausible hypothetical explanations.

The Clinical Characteristics of Colonic Pseudo-obstruction and the Factors Associated with Medical Treatment Response: A Study Based on a Multicenter Database in Korea

Colonic pseudo-obstruction (CPO) is defined as marked colonic distension in the absence of mechanical obstruction. We aimed to investigate the clinical characteristics of CPO and the factors associated with the response to medical treatment by using a multicenter database in Korea. CPO was diagnosed as colonic dilatation without mechanical obstruction by using radiologic and/or endoscopic examinations. Acute CPO occurring in the postoperative period in surgical patients or as a response to an acute illness was excluded. CPO cases were identified in 15 tertiary referral hospitals between 2000 and 2011. The patients' data were retrospectively reviewed and analyzed. In total, 104 patients (53 men; mean age at diagnosis, 47 yr) were identified. Seventy-seven of 104 patients (74%) showed a transition zone on abdominal computed tomography. Sixty of 104 patients (58%) showed poor responses to medical treatment and underwent surgery at the mean follow-up of 7.4 months (0.5-61 months). Younger age at the time of diagnosis, abdominal distension as a chief complaint, and greater cecal diameter were independently associated with the poor responses to medical treatment. These may be risk factors for a poor response to medical treatment.

Rehabilitation of Age-Related Hearing Loss Patients / 대한이비인후과학회지

Age-related hearing loss or presbycusis is the progressive hearing loss that affects both peripheral and central structures of the auditory system. It is characterized by increased hearing thresholds along with decrease in auditory perception. Presbycusis is permanent and irreversible, causing serious consequences both for the people with hearing impairment and for those around them. Because hearing loss in elderly is closely related with the quality of life, rehabilitation intervention through the use of hearing aids and hearing assistance technology and implementation of communication program are very important. There is a clear understanding of the importance of availability of the range of rehabilitation interventions in accordance with active involvement from the hearing impaired elderly who should be taught of self-management skills. Rehabilitation intervention should focus on optimizing availability and adherence to the rehabilitation program to truly benefit age-related hearing loss patients.

Current Trends in the Treatment of Subjective Tinnitus at University Hospitals in Korea / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The effective management of subjective tinnitus should start with an accurate diagnosis based on an appropriate classification. Since there is no gold standard for managing subjective tinnitus, clinicians can select from various treatment options after considering the multifactorial etiology of tinnitus. This study surveyed otologists at university hospitals in Korea to identify the treatments used for subjective tinnitus and to obtain basic information on evidence-based medicine for treating tinnitus. SUBJECTS AND METHOD: A five-major-item questionnaire on current tinnitus treatments was sent by email to otologists at 37 university hospitals in Korea; 30 (81.1%) replied. RESULTS: The mean incidence of tinnitus in otology outpatient clinics was 22.7% (range 10-40%). Common treatments were oral pharmacological therapy, regular counseling with tinnitus retraining or cognitive behavioral therapy and hearing aids. Tinnitus retraining therapy and hearing aids were considered the most effective when the visual analog scale scores were 7.0 and 6.6, respectively, and considered safe when the scores were 9.9 and 9.3. Ginkgo biloba and benzodiazepines were the most frequently prescribed drugs, although their reported effectiveness was questionable. Intra-tympanic steroid injection was not considered effective (3.8) or safe (6.3). Somatosensory-based treatments such as treating neck muscle or temporomandibular joint disorders were also used to relieve a subgroup of somatic tinnitus. CONCLUSION: Our results showed trends similar to those in other countries, yet we have not reached the level of evidence-based clinical practice due to the lack of reliable and effective treatment options. Further research on tinnitus-treatments is needed, particularly about randomized controlled studies with blinding.

Standardization for a Korean Adaptation of the International Outcome Inventory for Hearing Aids: Study of Validity and Reliability / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The evaluation of subjective outcomes of hearing aid fitting is of paramount importance along with audiometric improvement. The aim of this study was to develop a Korean adaptation of the International Outcome Inventory for Hearing Aids (IOI-HA) and to determine its reliability and validity. SUBJECTS AND METHOD: The translation of IOI-HA into the Korean version (K-IOI-HA) was completed through a process of forward translation, reconciliation, reverse translation and cognitive debriefing. K-IOI-HA was administered to 101 patients using hearing aids and visiting any the 10 referral hospitals. Reliability was evaluated by Cronbach's alpha coefficient and by test-retest analysis. Validity was assessed by confirmatory factor analysis and criterion validity based on the results of pure tone audiometry. RESULTS: K-IOI-HA showed a good internal consistency (Cronbach's alpha=0.83) and a high test-retest reliability (r=0.943, p<0.01). Validity checked by confirmatory factor analysis also showed good construct validity. CONCLUSION: The Korean version of IOI-HA is a reliable and valid tool for evaluating subjective outcomes of hearing aids.

A Case of Herpes Zoster Oticus without Facial Nerve Palsy Associated with Vertigo and Hearing Loss / 대한평형의학회지

Herpes zoster oticus (HZO) is characterized by facial nerve palsy, otalgia and auricular vesicles on the affected side and accepted to be caused by the reactivation of varicella zoster virus (VZV) in the geniculate ganglion. Vestibulocochlear deficits are known to be frequently accompanied by HZO. Unusual clinical manifestations such as only vertigo without facial nerve palsy or hearing loss has been reported. We report a case of 27-year-old man presented with vertigo, sensorineural hearing loss and vesicular eruptions on the left auricle without facial nerve palsy. Serologic test revealed that the patient was positive for immunoglobulin G (IgG) and IgM antibodies against VZV.

Clinical Characteristics of 7 Patients with Lateral Semicircular Canal Dysplasia / 대한평형의학회지

BACKGROUND AND OBJECTIVES: Dysplasia of lateral semicircular canal (LSCC) is the most common inner ear malformation, because LSCC is the last single structure to be formed during inner ear embryogenesis. There have been several case reports about the LSCC anomaly which showed clinical spectrum ranging from no symptom to severe sensorineural hearing loss. The authors investigated the clinical characteristics of 7 patients with LSCC dysplasia. MATERIALS AND METHODS: The medical records of 7 patients who were diagnosed as LSCC dysplasia from temporal bone computed tomography were retrospectively reviewed. We analysed the findings of history taking, pure tone audiometry, speech audiometry, and caloric tests. RESULTS: The LSCC dysplasia was observed in all of 7 patients in which 5 showed unilaterally confined LSCC dysplasia, 1 showed bilaterally confined LSCC dysplasia, and 1 exhibited bilateral LSCC dysplasia combined with bilateral posterior semicircular canal dysplasia. From 7 patients, hearing loss was chief complaint only in 2 patients. Pure tone audiometry revealed sensorineural hearing loss in one patient, and conductive hearing loss in the other patient. Two patients complained of whirling type vertigo, and 3 complained of nonspecific dizziness. CONCLUSION: The patients with LSCC dysplasia may show variable symptoms such as sensorineural hearing loss, conductive hearing loss, vertigo and nonspecific dizziness.

Cervical Vestibular-Evoked Myogenic Potentials Using Vibration and Sound in Normal Subjects / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The aim of this study was to investigate and compare the incidence and the characteristics of parameters of cervical vestibular-evoked myogenic potentials (cVEMPs) using air-conducted (AC) and bone-conducted (BC) stimulations in normal subjects. SUBJECTS AND METHOD: Twenty-four normal subjects (48 ears) with normal hearing and no previous history of dizziness were included. cVEMP responses were recorded by surface electrodes on sternocleidomastiod muscles in response to AC and BC stimuli sequentially. Variances of parameters, including thresholds, amplitudes and interaural amplitude difference ratios (IADR), were analyzed and compared. RESULTS: cVEMP responses were clearly observed in all 48 ears in both AC and BC cVEMP tests. There was no significant difference in latencies (p1 and n1). However, the thresholds in BC cVEMP testing were significantly lower than those in AC cVEMP testing, when compared to the maximum stimulation level. Interaural amplitude difference ratios showed no significant difference in between the two tests, although amplitudes at maximum stimulation intensity in BC cVEMP testing were significantly larger than those in AC cVEMP testing. CONCLUSION: This study shows that BC cVEMP testing shows lower thresholds compared to AC cVEMP testing in normal subjects, suggesting that the threshold value can be used as a parameter in detecting vestibular dysfunction in the clinic. Further studies in patients with various vestibular disorders are needed.

Standardization for a Korean Version of Hearing Handicap Inventory for the Elderly / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: This study has been performed to determine the reliability and validity of the Korean version of Hearing Handicap Inventory for the Elderly (HHIE), which has been adapted to the Korean population. SUBJECTS AND METHOD: For the Korean version of HHIE (K-HHIE), the processes of independent forward translation, backward translation and reconciliation were standardized using subjects older than 65 years old were recruited from the 10 referral-hospitals. Reliability and validity were evaluated by Cronbach's alpha coefficient, confirmatory factor analysis and test-retest analysis. Hearing impairment was assessed using pure tone audiometry, and the result was used as the gold standard. RESULTS: The recruited K-HHIEs were 169. The reliability of K-HHIE, checked by Cronbach's alpha coefficient, was as high as 0.95. Scale-item correlation coefficient was even higher than 0.97, which shows very high reliability of K-HHIE. Test-retest reliability and the correlation coefficients of social/situational, emotional and total scores of K-HHIE were as high as 0.73, 0.82 and 0.78, respectively. The validity of K-HHIE, checked by confirmatory factor analysis, also showed good construct validity. There was high correlation between hearing level and the scores of K-HHIE, which is another indicative result of its high validity. CONCLUSION: We have developed and validated Korean version of HHIE, which has good reliability and validity. It seems to be suitable enough for clinical use and research studies in patients with hearing impairment.