Nystagmus-based approach to vertebrobasilar stroke presenting as vertigo without initial neurologic signs.

BACKGROUND: We aimed to investigate the clinical courses and common nystagmus of isolated vertigo patients with vertebrobasilar stroke. METHODS: The patients who presented with isolated acute spontaneous vertigo with spontaneous nystagmus (acute vestibular syndrome) at the Emergency Department were retrospectively analyzed. They were referred to the Otolaryngology Department due to the absence of neurologic signs or even of imaging abnormalities after the initial examination at the Emergency Department. Various clinical features, including presenting symptoms, delayed neurologic signs, the site of infarction, and videonystagmographic (VNG) findings were analyzed. RESULTS: Of the 468 cases of acute vestibular syndrome, 23 (4.9%) cases of radiologically proven vertebrobasilar stroke were identified. Of the 23 patients, 17 (74%) showed aggravation of vertigo or delayed neurologic signs during the admission. In the analysis of VNG, 11 (48%) cases of direction-changing gaze-evoked nystagmus, 7 (30%) cases of fixation failure in the caloric test, 6 (27%) cases of periodic alternating nystagmus, and 4 (17%) cases of atypical head-shaking nystagmus were presented. Stroke occurred in the cerebellum (n=18, 78%), medulla (n=4, 17%), and pons (n=1, 4%). CONCLUSION: In the early stage of vertebrobasilar stroke, an accurate diagnosis was difficult in the Emergency Department even though a radiologic study was performed, but various VNG abnormalities and delayed neurologic signs could help to diagnose whether the origin is central or not.

A Novel Frameshift Mutation of the USH2A Gene in a Korean Patient with Usher Syndrome Type II.

Usher syndrome type II (USH2) is the most common form of Usher syndrome, characterized by moderate to severe hearing impairment and progressive visual loss due to retinitis pigmentosa. It has been shown that mutations in the USH2A gene are responsible for USH2. The authors herein describe a 34-year-old Korean woman with the typical clinical manifestation of USH2; she had bilateral hearing disturbance and progressive visual deterioration, without vestibular dysfunction. Molecular genetic study of the USH2A gene revealed a novel frameshift mutation (c.2310delA; Glu771LysfsX17). She was heterozygous for this mutation, and no other mutation was found in USH2A, suggesting the possibility of an intronic or large genomic rearrangement mutation. To the best of our knowledge, this is the first report of a genetically confirmed case of USH2 in Korea. More investigations are needed to delineate genotype-phenotype correlations and ethnicity-specific genetic background of Usher syndrome.

Allergic diseases in children with otitis media with effusion.

OBJECTIVES: Recent studies have shown that allergic diseases may be associated with the pathogenesis of recurrent otitis media with effusion (OME). We aimed to assess the relationship between OME and allergic diseases and other types of disease in children with OME. We also evaluated the between group differences in the characteristics of middle ear effusion. MATERIALS AND METHODS: We evaluated 370 patients diagnosed with OME between January 2007 and December 2012 and, as a control group, 100 children with no medical history of OME but who had undergone blood tests and MAST-CLA (multiple allergosorbent test - chemiluminescent assay) were selected. RESULT: Among the allergic diseases, the incidence of allergic rhinitis alone was significantly higher in children with OME (33.8%) than without OME (16.0%) (p<0.05). The rate of adenoid, but not tonsil, hypertrophy was significantly greater in patients with than without OME also (p<0.05). When we evaluated the characteristics of middle ear effusion (MEE) in patients with OME, we found that 186 had serous, 129 had mucous and 55 had purulent MEE. Of these patients, 75 (40.3%), 36 (27.9%) and 14 (25.5%), respectively, had allergic rhinitis and the rates of allergic rhinitis and asthma were significantly higher in the serous group than in the mucous group (p<0.05). CONCLUSION: Allergic rhinitis was significantly more frequent among pediatric patients with than without OME, although the rates of other allergic diseases did not differ in these two groups. The likelihoods of allergic rhinitis and asthma were higher in patients with serous than with mucous MEE.

Repair of spontaneous cerebrospinal fluid otorrhea from defect of middle cranial fossa.

Spontaneous cerebrospinal fluid (CSF) otorrhea is defined as CSF otorrhea where there are no identifiable causes including previous trauma, surgery, infection, neoplasm or congenital anomaly. The condition is rare. The origin of CSF leak is commonly a defect in the tegmen of the middle cranial fossa. The pathophysiology of spontaneous CSF otorrhea is unclear. Two theories of the etiology of bony defects of the temporal bone are the congenital bony defect theory and arachnoid granulation theory. The authors experienced a case of a 49-year-old female patient admitted with the complaint of persistent right ear fullness. Computed tomography revealed a large defect of the middle fossa and suspicious CSF otorrhea through the defect of tegmen tympani. Repair was successful with multiple bone chips using the transmastoid approach. The postoperative course was good and there has been no recurrence of the CSF leakage.

Rate of isolation and trends of antimicrobial resistance of multidrug resistant pseudomonas aeruginosa from otorrhea in chronic suppurative otitis media.

OBJECTIVES: To assess the rate of isolation of Pseudomonas aeruginosa (PA) and multidrug-resistant PA (MDR-PA) from patients with chronic suppurative otitis media (CSOM) otorrhea and the annual trend of antibiotic-resistance. METHODS: Otorrhea samples were collected aseptically from 1,598 CSOM patients. The rate of bacterial isolation and the results of antibiotic susceptibility testing were evaluated retrospectively. RESULTS: The PA isolation rate from CSOM otorrhea was 24.4%. Of the 398 isolated strains tested for their susceptibilities to 10 antibiotics, 395 strains showed definitive results. Of these, 183 (46.3%) were susceptible to whole antibiotics and 212 (53.7%) was resistant to more than 1 antibiotics, with the frequency of antibiotics-resistance increasing significantly over time. Although strains susceptible to all antibiotics decreased over time, the rate of isolation of MDR-PA did not change significantly. Resistance to aminoglycosides and quinolones was higher than to other antibiotics and significantly increased over time, whereas resistance to other antibiotics showed no trend. CONCLUSION: MDR-PA, assessed using five individual antibiotics and six antibiotic-classes, showed no tendency to increase or decrease over time. This may have been due to increased concern about antibiotic-resistant bacterial strains, leading to improved infection control within hospitals and healthcare centers.

Clinical analysis of auricular benign masses.

BACKGROUND AND OBJECTIVES: Auricular masses are growths on the outer ear that have not been well characterized clinically. We assessed the clinical nature and treatment of auricular benign masses in patients at our institution. SUBJECTS AND METHODS: We retrospectively identified 63 patients with auricular benign masses who underwent excision and biopsy from May 1970 to April 2011. We determined the site, cause, size, pathology and postoperative results of these auricular masses. RESULTS: Auricular benign masses occurred most commonly on the lobule (44.4%), followed by the tragus (20.6%), crus of helix (11.1%), triangular fossa (6.3%), crus of antihelix-antitragus (3.1%) and scapha (1.5%). Pathologically, the most common type of auricular mass was epidermal cysts (25.3%), followed by hypertrophic scar (12.6%), fibrous tissue-accessory ear (9.5%), chronic inflammation-nevus (7.9%), keloid (6.3%), hemangioma (4.7%), and skin tag-seborrheic keratosis (3.1%). CONCLUSIONS: The most common site of auricular benign masses is the lobule and most common pathology is an epidermal cyst.

Comparison of the clinical relevance of traditional and new classification systems of temporal bone fractures.

Temporal bone fractures are traditionally classified as transverse, longitudinal or mixed. Since these categories have shown little association with clinical symptoms, new classifications have been introduced, including those related to the involvement of the petrous bone and otic capsule. We have formulated a new classification based on the involvement of four parts of the temporal bone (squama, tympanic, mastoid, and petrous) and assessed which of these classification systems is the most rational using a retrospective chart review in hospital settings (KyungHee Medical Center, Seoul, Korea and Samsung Changwon Hospital, Changwon, Korea). The association between each classification and clinical symptoms was examined by analyzing temporal bone computed tomography scans of 129 patients diagnosed as temporal bone fractures over the past 7 years. Using the traditional classification, there was a significant correlation between transverse fractures and the incidence of sensorineural hearing loss. Patients with petrous bone fractures had significantly higher incidence rates of sensorineural hearing loss, vertigo, and eardrum perforation than patients without petrous bone involvement. Involvement of the otic capsule was significantly associated with sensorineural hearing loss and the severity of hearing loss. The associations of the traditional classification and the classification according to the involvement of the otic capsule, four parts of temporal bone with clinical symptoms were not high. Petrous bone fractures were significantly associated with sensorineural hearing loss, vertigo, and eardrum perforation, suggesting that this classification may be optimally associated with clinical symptoms including hearing and the results of otological examination.

Clinical efficacy of initial intratympanic steroid treatment on sudden sensorineural hearing loss with diabetes.

OBJECTIVE: The purpose of this study is to compare the efficacy of intratympanic steroid injection (ITSI) with that of systemic steroids as an initial treatment of sudden sensorineural hearing loss (SNHL) with diabetes. STUDY DESIGN: Prospective, nonrandomized multicenter clinical trial. SETTING: Multicenter study in Busan and Masan, South Korea. SUBJECTS AND METHODS: A total of 114 sudden SNHL patients who were diagnosed with diabetes were divided into peroral (PO) group (n = 48), intravenous (IV) group (n = 32), and intratympanic (IT) group (n = 34). In the PO group, prednisolone was used orally for 10 days, per schedule. In the IV group, prednisolone was administered intravenously for seven days, followed by oral administration of tapered doses for another several days. In the IT group, dexamethasone was injected into the tympanic cavity four times within a two-week period. Hearing outcome was assessed before and after the treatment. RESULTS: All groups showed significant improvement with criteria of 15 dB (P < 0.05). However, there was no significant difference in hearing gain and recovery rate among groups (P > 0.05). Systemic steroid treatment was stopped for two patients in the IV group and for one in the PO group due to uncontrolled hyperglycemia. However, in the IT group, there were no patients who failed to control their blood sugar level. CONCLUSION: ITSI is as effective as systemic steroid treatment for sudden SNHL patients with diabetes and it can avoid undesirable side effects. Therefore, we consider ITSI to be a more reasonable alternative as an initial treatment for sudden SNHL patients with diabetes.

Combination treatment of Cetuximab and photodynamic therapy in SNU-1041 squamous cancer cell line.

Cetuximab (Erbitux) has been highlighted for its anti-proliferative effects in solid tumors and it is currently used as an adjuvant modality with other anti-cancer treatments. Photodynamic therapy (PDT) is used widely in many specialties of medicine. This study evaluated the efficacy of a combination treatment of two modalities (Cetuximab, PDT) both in vivo and in vitro. The SNU-1041 cell line was used for both the in vitro and in vivo studies. The in vivo and in vitro experiments were each classified into four groups, control group, Cetuximab applied group, PDT applied group and combined modality group. A migration study was performed to determine the anti-migration effect of Cetuximab, and a MTT assay was performed to compare the anti-proliferative effect of the modalities in vitro. For the in vivo study, the cells were implanted into 5-week-old nude mice. The measured volume of the tumor for each group was compared as a function of time. In the migration study, the control group showed a longer migration length than the Cetuximab applied group. In the MTT assay, the combination modality group showed less survival than the uni-modality groups. The measured tumor size after treatment showed that the combination treatment was more effective than the single modalities. PDT and Cetuximab are treatment modalities that target different molecular pathways. A combination of these two treatment modalities was found to more effective than an individual treatment. However, further studies will be needed to determine the optimal dose of the photosensitizer and Cetuximab.

A novel splice site mutation in the EYA1 gene in a Korean family with branchio-oto (BO) syndrome.

Branchio-oto-renal (BOR) and branchio-oto (BO) syndromes are autosomal dominant hereditary disorders characterized by the presence of hearing loss and branchial fistulae and cysts, with (BOR syndrome) or without (BO syndrome) renal malformations of varying degrees of severity. Mutations in the human homologous of the Drosophila eyes absent (EYA1) gene are frequently the cause of BOR/BO syndrome. Here we describe a Korean family with BO syndrome; the proband had preauricular pit, cup-shaped auricles, branchial fistula, and hearing loss, without renal involvement. Molecular genetic study revealed a novel mutation occurring in the consensus acceptor splice site of intron 8 (c.868-2A > G) in the EYA1 gene. To the best of our knowledge, this is the first report of a splice site mutation in a family with BO syndrome without renal involvement, further extending the phenotypic-genotypic heterogeneity of BOR/BO syndrome.

Proapoptotic effects of NF-kappaB on cisplatin-induced cell death in auditory cell line.

CONCLUSIONS: The findings suggest that nuclear factor (NF)-kappaB is activated by cisplatin and plays a proapoptotic role during cell death in the auditory cell line HEI-OC1. OBJECTIVES: Cisplatin is a very effective antineoplastic drug but in high doses it shows ototoxicity by inducing apoptosis of hair cells in the cochlea. NF-kappaB is a transcription factor regulating apoptosis in many organs and tissues. This study examined the role of NF-kappaB in the apoptotic pathway induced by cisplatin in the auditory cell line, HEI-OC1. MATERIALS AND METHODS: Apoptotic cell death was identified by measuring caspase 3 activity and immunostaining with anti-caspase 3 antibody after cisplatin treatment (50 microM) for 24 h. To investigate the role of NF-kappaB in apoptotic cell death, HEI-OC1 cells treated with 50 microM of cisplatin were immunostained with anti-NF-kappaB (p65) antibody. Two different NF-kappaB inhibitors, Bay 11-7085 and SN-50, were co-incubated with cisplatin (50 microM) for 24 h and caspase 3 activity was assayed. RESULTS: Immunostaining with anti-caspase 3 antibody and caspase 3 assay showed that cisplatin induced apoptosis in HEI-OC1 cells. After cisplatin treatment, NF-kappaB (p65) was activated to translocate from the cytoplasm into the nucleus. Co-treatment with NF-kappaB inhibitors reduced the cisplatin-induced apoptosis of HEI-OC1 cells.