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J. Otorhinolaryngol. Hear. Balance Med., Volume 1, Issue 1 (June 2018)

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Cover Story (view full-size image) This image was supplied by our Editor-in-Chief, Prof. Weijia Kong and one of our Editorial Board [...] Read more.
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Open AccessReview Role of Autophagy in Auditory System Development and Survival
J. Otorhinolaryngol. Hear. Balance Med. 2018, 1(1), 7; https://doi.org/10.3390/ohbm1010007
Received: 4 February 2018 / Revised: 6 April 2018 / Accepted: 14 April 2018 / Published: 16 April 2018
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Abstract
Autophagy is a natural catabolic process of the cell that dismantles the useless or dysfunctional components. Autophagy allows the systematic and the lysosomal-mediated deterioration of cellular organelles. During the embryonic development, autophagy plays a critical role by remodeling the tissue and organs of
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Autophagy is a natural catabolic process of the cell that dismantles the useless or dysfunctional components. Autophagy allows the systematic and the lysosomal-mediated deterioration of cellular organelles. During the embryonic development, autophagy plays a critical role by remodeling the tissue and organs of the body, and the deletion of some of the autophagy related genes results in the defective embryonic development. Inner ear is the most sophisticated organ of the body responsible for the sound perception. In mammalian inner ear, autophagy protects the hair cells (HCs) from drug and noise induced damage. In this review, we particularly discuss how autophagy implicates during the auditory system development in mammals and presents its role in age-related hearing loss. Moreover, we discuss the protecting effects of autophagy after noise and drug induced auditory trauma. Full article
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Open AccessArticle Silent Vestibulopathy in Asymmetric Hearing Loss Can Be a Sign of a Cerebellopontine Angle Tumor
J. Otorhinolaryngol. Hear. Balance Med. 2018, 1(1), 6; https://doi.org/10.3390/ohbm1010006
Received: 15 December 2017 / Revised: 6 February 2018 / Accepted: 15 February 2018 / Published: 16 February 2018
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Abstract
The presence of an ipsilateral cerebellopontine angle (CPA) tumor should be ruled out in patients with asymmetric sensorineural hearing loss (ASNHL). Although many patients with CPA tumors have ipsilateral vestibular hypofunction, some of them do not experience dizziness even with ipsilateral vestibular hypofunction.
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The presence of an ipsilateral cerebellopontine angle (CPA) tumor should be ruled out in patients with asymmetric sensorineural hearing loss (ASNHL). Although many patients with CPA tumors have ipsilateral vestibular hypofunction, some of them do not experience dizziness even with ipsilateral vestibular hypofunction. We analyzed the incidence of CPA tumors among patients with ASNHL without subjective dizziness based on the presence of vestibulopathy. We hypothesized that a patient with silent unilateral vestibular hypofunction (UVH) is more likely to be diagnosed with a CPA tumor. Among 157 subjects who underwent MRI for ASNHL, those who did not have “subjective dizziness” were selected. All subjects underwent hearing and vestibular function tests. UVH was diagnosed if canal paresis ≥ 25%, positive head-shake nystagmus, or gain of head-impulse test < 0.8 were detected. The diameters of the CPA tumors were measured along the petrosal ridge on the axial plane of MRI. Among the enrolled subjects, 44 (28.02%) were diagnosed with a CPA tumor. The 37 patients (84.1%) with a CPA tumor had silent UVH, while only 33 of the 113 patients (29.2%) without a CPA tumor had silent UVH (chi-square test, odds ratio = 12.8, p < 0.001). Silent UVH in patients with ASNHL may be a sign of a CPA tumor. Full article
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Open AccessArticle A Study on the Relationship between the Intelligibility and Quality of Algorithmically-Modified Speech for Normal Hearing Listeners
J. Otorhinolaryngol. Hear. Balance Med. 2018, 1(1), 5; https://doi.org/10.3390/ohbm1010005
Received: 17 November 2017 / Revised: 4 December 2017 / Accepted: 7 December 2017 / Published: 8 December 2017
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Abstract
This study investigates the relationship between the intelligibility and quality of modified speech in noise and in quiet. Speech signals were processed by seven algorithms designed to increase speech intelligibility in noise without altering speech intensity. In three noise maskers, including both stationary
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This study investigates the relationship between the intelligibility and quality of modified speech in noise and in quiet. Speech signals were processed by seven algorithms designed to increase speech intelligibility in noise without altering speech intensity. In three noise maskers, including both stationary and fluctuating noise at two signal-to-noise ratios (SNR), listeners identified keywords from unmodified or modified sentences. The intelligibility performance of each type of speech was measured as the listeners’ word recognition rate in each condition, while the quality was rated as a mean opinion score. In quiet, only the perceptual quality of each type of speech was assessed. The results suggest that when listening in noise, modification performance on improving intelligibility is more important than its potential negative impact on speech quality. However, when listening in quiet or at SNRs in which intelligibility is no longer an issue to listeners, the impact to speech quality due to modification becomes a concern. Full article
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Open AccessArticle Head and Neck Paraganglioma: Medical Assessment, Management, and Literature Update
J. Otorhinolaryngol. Hear. Balance Med. 2018, 1(1), 4; https://doi.org/10.3390/ohbm1010004
Received: 8 October 2017 / Revised: 26 November 2017 / Accepted: 3 December 2017 / Published: 8 December 2017
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Abstract
Head and neck paraganglioma (HNPGL) are rare, highly vascular; typically slow growing and mostly benign neoplasms arising from paraganglia cells. HNPGL cause morbidity via mass effect on adjacent structures (particularly the cranial nerves), invasion of the skull base and, rarely, catecholamine secretion with
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Head and neck paraganglioma (HNPGL) are rare, highly vascular; typically slow growing and mostly benign neoplasms arising from paraganglia cells. HNPGL cause morbidity via mass effect on adjacent structures (particularly the cranial nerves), invasion of the skull base and, rarely, catecholamine secretion with associated systemic effects. The last decade has seen significant progress in the understanding of HNPGL genetics, with pertinent implications for diagnostic assessment and management of patients and their relatives. The implicated genes code for three of the five subunits of mitochondrial enzyme succinate dehydrogenase (SDH); recent literature reports that approximately one third of all HNPGL are associated with SDH mutations—a prevalence significantly greater than traditionally thought. There are distinct phenotypical syndromes associated with mutations in each individual SDH subunit (SDHD, SDHB, SDHC, and SDHAF2). This article focuses on the clinical features of HNPGL, the implications of HNPGL genetics, and the current evidence relating to optimal identification, investigation, and management options in HNPGL, which are supported by reference to a personal series of 60 cases. HNPGL require a systematic and thorough assessment to appropriately guide management decisions, and a suggested algorithm is presented in this article. Recent developments are particularly pertinent to surgeons of multiple disciplines, including otolaryngology, neurosurgery, vascular, and general surgery. Full article
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Open AccessReview Endoplasmic Reticulum Stress in Hearing Loss
J. Otorhinolaryngol. Hear. Balance Med. 2018, 1(1), 3; https://doi.org/10.3390/ohbm1010003
Received: 16 October 2017 / Revised: 26 October 2017 / Accepted: 30 October 2017 / Published: 1 November 2017
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Abstract
The endoplasmic reticulum (ER) plays important roles in coordinating protein biosynthesis and secretion in the cell. Accumulation of misfolded and/or unfolded proteins in the ER causes ER stress and the so-called unfolded protein response (UPR). The UPR alleviates ER stress through blocking protein
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The endoplasmic reticulum (ER) plays important roles in coordinating protein biosynthesis and secretion in the cell. Accumulation of misfolded and/or unfolded proteins in the ER causes ER stress and the so-called unfolded protein response (UPR). The UPR alleviates ER stress through blocking protein synthesis and activating expression of chaperone genes, whereas prolonged UPR could induce cell death. Recent research has showed that ER stress and UPR are involved in hearing loss. Accordingly, animal experiments showed that chemical chaperones or ER stress inducers alleviate environment-related hearing loss, whereas ER stress inhibitor has been used to treat certain types of hereditary deafness. Further investigations are needed to fully understand the detailed mechanisms of how ER stress contributes to the loss of auditory function, which will help us to eventually develop ER-stress-related treatment of various types of deafness. Full article
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Open AccessArticle Effects of Retinoid Treatment on Cochlear Development, Connexin Expression and Hearing Thresholds in Mice
J. Otorhinolaryngol. Hear. Balance Med. 2018, 1(1), 2; https://doi.org/10.3390/ohbm1010002
Received: 5 July 2017 / Revised: 10 August 2017 / Accepted: 17 October 2017 / Published: 23 October 2017
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Abstract
Mutations in GJB2, gene coding for connexin 26 (Cx26), and GJB6, gene coding for connexin 30 (Cx30), are the most common genetic defects causing non-syndromic hereditary hearing loss. We previously reported that overexpression of Cx26 completely rescues the hearing in a mouse model
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Mutations in GJB2, gene coding for connexin 26 (Cx26), and GJB6, gene coding for connexin 30 (Cx30), are the most common genetic defects causing non-syndromic hereditary hearing loss. We previously reported that overexpression of Cx26 completely rescues the hearing in a mouse model of human GJB6 null mutations. The results suggest that therapeutic agents up-regulating the expression of Cx26 may potentially be a novel treatment for non-syndromic hereditary deafness caused by Cx30 null mutations. Retinoids are a family of vitamin A derivatives that exert broad and profound effects on cochlear protein expression including connexins. They are readily available and already utilized as therapeutic agents for recurrent otitis media and hearing loss due to noise exposure. In this study, we characterized the expression of Cx26 and Cx30 in the postnatal inner ear by different retinoids including retinyl palmitate (RP), the main source of vitamin A in over-the-counter (OTC) supplements, retinyl acetate (RAc) which is an isomer of RP, and all-trans-retinoic acid (ATRA), the most active retinoid derivative. The results revealed ATRA significantly increased cochlear Cx26 expression and improved hearing in Cx30 knockout (KO) mice by 10 dB suggesting its potential benefits as a therapeutic agent. In contrast, RP selectively reduced cochlear Cx30 expression and did not improve hearing thresholds at the dosages we tested. Full article
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Open AccessEditorial Welcome to Journal of Otorhinolaryngology, Hearing and Balance Medicine
J. Otorhinolaryngol. Hear. Balance Med. 2018, 1(1), 1; https://doi.org/10.3390/ohbm1010001
Received: 24 February 2017 / Revised: 24 February 2017 / Accepted: 24 February 2017 / Published: 28 February 2017
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Abstract
To date, much progress has been achieved in the field of Otorhinolaryngology—Head and Neck surgery, as well as hearing and balance science and medicine.[...] Full article
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