compared with advertised noise reduction rate of the best available ear-level protectors. RESULTS: All suppressors offered significantly greater noise reduction than ear level protection, usually more than 50% better. Noise reduction of all ear level protectors is unable to reduce the impulse pressure below 140 dB for certain common firearms, an international standard for prevention of sensorineural hearing loss. CONCLUSION: Modern muzzle level suppression is vastly superior to ear level protection and the only available form of suppression capable of making certain sporting arms safe for hearing. The inadequacy of standard ear protectors with certain common firearms is not recognized by most hearing professionals or their patients and should affect the way hearing professionals counsel patients and the public. Cryopyrin-Associated Periodic Syndromes: ENT Manifestations Neda Ahmadi, MD (presenter); Jeffrey Hung Kim, MD; Carmen Brewer, PhD; Christopher Zalewski, MA; Kelly King, AuD OBJECTIVE: Cryopyrin-associated periodic syndromes (CAPS) represent a spectrum of CIAS1 gene-mediated autoinflammatory diseases characterized by recurrent episodes of inflammation. The three distinct entities, from severe to mild, include neonatal-onset multisystem inflammatory disease (NOMID), Muckle-Wells syndrome (MWS), and familial cold autoinflammatory syndrome (FCAS). We present the largest reported series of patients with CAPS. The objective is to understand the pathogenesis, otolaryngologic and audiologic manifestations of CAPS. METHOD: Comprehensive data regarding genotype, clinical manifestations, fluid attenuation inversion recovery magnetic resonance imaging (FLAIR MRI) of ear, and audiology were extracted retrospectively (2003-2009). RESULTS: Fifty-seven (34-NOMID, 7-NOMID/MWS, 10MWS and 6-FCAS) patients with a diagnosis of CAPS were identified. CIAS1 mutations were present in 7(79%)-NOMID, 6(86%)-NOMID/MWS, 7(70%)-MWS, and 6(100%)-FCAS patients. Comprehensive audiologic data was available on 42(62%)-NOMID, 7(50%)-NOMID/MWS, 11(55%)-MWS and 12(100%)-FCAS ears. Distribution of hearing loss is as follows: normal hearing in 8(19%)-NOMID, 2(33%)-NOMID/ MWS, and 8(73%)-MWS ears; conductive hearing loss in 4(10%)-NOMID ears; and mixed hearing loss in 5(12%)-NOMID, and 1(14%)-NOMID/MWS ears. Sensorineural hearing loss (SNHL) worsening at increasing frequencies was the most common type of hearing loss present in 25(60%)-NOMID, 4(57%)-NOMID/MWS, and 3(27%)-MWS ears. All of the FCAS patients had normal hearing except 2, which had SNHL at 4000-8000 Hertz. On FLAIR MRI sequence, cochlear enhancement was noted in 27(79%)-NOMID, 5(71%)-NOMID/ MWS, 3(30%)-MWS, and 1(17%)-FCAS patients.
P91 CONCLUSION: CIAS1 pathway-mediated CAPS is associated with unregulated autoinflammation in the cochlea and hearing loss. Timely diagnosis can lead to initiation of early treatment with anakinra (recombinant IL-1 receptor antagonist), which has been shown to improve the inflammatory symptoms and hearing loss. Curcumin Attenuates Oxidative Damage of SGNs Wenwen Liu, MD (presenter); Zhaomin Fan, MD; Haibo Wang, MD OBJECTIVE: To investigate the effect of curcumin on peroxynitrite-induced damage in rat spiral ganglion neurons (SGNs) as well as to delineate the possible mechanisms underlying its action. METHOD: The experiments were performed on primary cultured rat SGNs. Cell viability was measured by MTT assay. Cellular glutathione (GSH) content, superoxide dismutase (SOD) activity and malonaldehyde (MDA) levels were assessed by spectrophotometer respectively. The mRNA expressions of Apaf-1, Caspase-9, Caspase-3, Bcl-2, and Bax were examined by RT-PCR while the protein expression of cytochrome c, Caspase-3, Bcl-2 and Bax proteins were determined by western blot. RESULTS: The activity of SOD and level of GSH in SGN were notably reduced after exposure to peroxynitrite (100 M) for 24 h, whereas, the level of MDA was significantly increased. Pretreatment with Curcumin improved the levels of SOD and GSH, decreased the elevation of MDA. The peroxynitrite induced cytochrome c release from mitochondria and subsequent activation of Caspase-3 and cell apoptosis. After pretreatment with curcumin 15 M for 12 h, curcumin abrogated cytochrome c release, blocked activation of caspase 3, and altered the expression of Bcl-2 family. CONCLUSION: Curcumin may attenuate peroxynitrite-induced oxidative damages in SGNs by reducing intracellular production of ROS and protecting mitochondria from oxidative damage. Do Topical Photoactivated Antimicrobials Cause Ototoxicity? Tulio Valdez, MD (presenter); Kourosh Parham, MD, PhD OBJECTIVE: 1) Evaluate acute ototoxicity of potential novel ototopical antimicrobial, photoactivated methyl delta-aminolevulinic acid (M-ALA), after intratympanic administration and light activation in a murine model. 2) Identify any local inflammatory changes after ototopic application of M-ALA. METHOD: Design: Non-randomized controlled trial. Interventions: Ten CBA/J mice received daily intratympanic injections for 7 days of 10 mM M-ALA solution followed by light activation via tympanic illumination with 640 nm wavelength
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