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Hearing loss is the most frequent sensory disorder affecting newborns and children. About 1 newborn in every 500 suffer from congenital hearing loss, with approximately half of these having a genetic cause. In the last few decades, the study of genetic hearing loss and related mouse models has unveiled molecular, cellular, and physiological mechanisms of the disease. In addition, effective and safe viral vectors for gene delivery to the inner ear have been generated. A growing number of approaches, including gene replacement, gene silencing, and gene editing, have proved effective in mouse models. This article briefly introduces basic strategies of gene therapy, viral vectors used and surgical methods for gene delivery, and reviews the current works on mouse modes of genetic hearing loss.

Received March 5, 2013 Revised April 9, 2013 Accepted April 10, 2013 Address for correspondence Yong Ho Park, MD Department of Otolaryngology-Head and Neck Surgery, Chungnam National University School of Medicine, 282 Munhwa-ro, Jung-gu, Daejeon 301-721, Korea Tel +82-42-280-7697 Fax +82-42-253-4059 E-mail parkyh@cnu.ac.kr Background and ObjectivesZZThere are several evidences of reduced cochlea blood flow after noise exposure in the cochlea. However, the pathophysiology of blood flow change is still obscure, and endothelins, proteins that constrict blood vessels and play a key role in vascular homeostasis using its receptors may have importance in this respect. In this study, we investigated the expression changes of endothelin-1 (ET-1), endothelin receptor A (ETAR) and B (ETBR) according to auditory threshold change after noise exposure. Materials and MethodZZMice were exposed to different noise to generate transient (group 2) and permanent threshold shift (group 3), respectively. Auditory threshold shifts were evaluated with auditory brainstem response and expression changes of ET-1, ETAR and ETBR after noise exposure were evaluated by immunohistochemistry and real time RT-PCR. ResultsZZAfter noise exposure, the increased ET-1, ETAR and ETBR immunoreactivities were observe in stria vascularis, spiral ligament and spiral ganglion neuron. ET-1 mRNA expressions increased after noise exposure in both group 2 and group 3 compared to those of the control group. At 2 weeks after noise exposure, however, the ET-1 mRNA expressions in group 3 increased compared to that of the control but decreased compared to that of group 2. On the other hand, ETAR mRNA expression increased at 2 weeks after noise exposure in both groups, just after noise exposure in group 2 and at 2 weeks after noise exposure in group 3. ConclusionZZThese results suggest that expression changes of ET-1, ETAR and ETBR might be associated with hearing threshold shift and recovery after noise exposure in the cochlea. Korean J Otorhinolaryngol-Head Neck Surg 2013;56:278-85

Received November 1, 2010 Revised January 6, 2011 Accepted January 6, 2011 Address for correspondence Jin Woong Choi, MD Department of Otolaryngology-Head and Neck Surgery, Research Institute for Medical Sciences, Chungnam National UniversitySchool of Medicine, 282 Munhwa-ro, Jung-gu, Daejeon 301-721, Korea Tel +82-42-280-7698 Fax +82-42-253-4059 E-mail conc-will@hanmail.net Seborrheic keratosis (SK) is a common benign lesion that develops from the proliferation of epidermal cells. Since SK usually occurs on the face, extremities, and upper trunk of the elderly, the finding of this lesion on the auricle is rare. Given the rarity of SK lesions of the auricle and the lack of familiarity with its presentation, otolaryngologists have difficulty diagnosing SK. Authors experienced a case of solitary SK that originated from the auricle in a 65-year-old man. We present this case with a review of the literature. Korean J Otorhinolaryngol-Head Neck Surg 2011;54:231-4 Key WordsZZSeborrheic keratosis ᆞAuricle.

Background and ObjectivesZZBacterial biofilm is thought to contribute to the progression and persistence of chronic rhinosinusitis. There are many studies which demonstrate these structures on chronic rhinosinusitis (CRS), but the majority of the studies just focus on the documentation of their presence and the method for identification. There are also a few researches that show the impact of biofilm on postoperative surgical outcomes. In this study, we demonstrated bacterial biofilm in CRS patients and determined whether they affect clinical courses and outcomes after surgery. Subjects and MethodZZThe mucosa of ethmoid bulla was obtained during endoscopic sinus surgery from 21 CRS patients. Fluorescent microscope and scanning electron microscopy (SEM) were used for the determination of biofilm. The outcomes of the surgery were evaluated by endoscope every two weeks for 6 months. Preoperative symptom scores, CT scores, and the post operative results were compared between patients with biofilm and patients without biofilm. ResultsZZBacterial biofilm were seen in 13 (62%) of the 21 CRS patients. A statistical association existed between the presence of biofilm and the worsened preoperative symptom scores, but the preoperative CT scores were not related to the presence of biofilm. Patients with biofilm had long-lasting postoperative mucosal inflammation and a prolonged healing time, and these differences have statistical significance. ConclusionZZThis study showed that the presence of bacterial biofilm were significantly correlated with worsening preoperative symptom scores and the prolonged postoperative recovery time. Therefore, bacterial biofilm may play an important role in the recalcitrant and resistant nature of CRS. Korean J Otorhinolaryngol-Head Neck Surg 2010;53:349-53 Key WordsZZChronic rhinosinusitis·Biofilm.

Background and Objectives Staphylococcal exotoxins (SEs) have been implicated in the pathogenesis of chronic rhinosinusitis with nasal polyp (CRSwNP). In the current study, we determined the prevalence of specific immunoglobulin E (IgE) antibodies to SEs in serum and polyp tissues of patients with CRSwNP and tried to find out whether there is an association between the presence of SEs-IgE antibody and eosinophilic inflammation. Subjects and Method Blood, nasal polyp and mucosa samples were obtained from 43 patients undergoing endoscopic sinus surgery for CRSwNP and 11 controls undergoing septoplasty without CRS. Specimens were analyzed for the presence of specific IgE antibody to four SEs [staphylococcal enterotoxin A (SEA), staphylococcal enterotoxin B (SEB), staphylococcal enterotoxin C (SEC), toxic shock syndrome toxin 1 (TSST-1)] using ImmunoCAP assay. Eosinophil cationic protein (ECP) in serum and nasal polyp tissue were also analyzed using ImmunoCAP. Eosinophil counts were estimated in polyp tissue. Results SEs-specific IgE antibodies were detected in 13 (30.2%) patients of the CRSwNP group. In contrast, only one (9.1%) control patient had IgE to SEs. Serum ECP level was increased significantly in the CRSwNP group compared with controls. However, there were no significant differences in Lund-MacKay score, the ECP level in the serum and polyp tissue, and eosinophil count in the polyp tissue between the SEs-IgE antibody positive [SEs-IgE Ab (+)] group and the SEs-IgE antibody negative [SEs-IgE Ab (-)] group. Conclusion SEs may play a certain role in the pathogenesis of CRSwNP. However, there is no close correlation between the presence of SEs-IgE antibody and eosinophilic inflammation.