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https://www.readbyqxmd.com/read/28822408/-the-application-of-artificial-neural-network-on-the-assessment-of-lexical-tone-production-of-pediatric-cochlear-implant-users
#1
Y T Mao, Z M Chen, L Xu
Objective: The present study was carried out to explore the tone production ability of the Mandarin-speaking children with cochlear implants (CI) by using an artificial neural network model and to examine the potential contributing factors underlining their tone production performance. The results of this study might provide useful guidelines for post-operative rehabilitation processes of pediatric CI users. Methods: Two hundred and seventy-eight prelingually deafened children who received unilateral CI participated in this study...
August 7, 2017: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/28822407/-clinical-observation-on-the-effect-of-glucocorticoid-insensitivity-on-sudden-sensorineural-hearing-loss-patients
#2
J Hou, H Qi, Y H Dai, X M Wei, L Lu, J G Wang, W D She
Objective: To investigate the correlation between the proliferation inhibition effect of glucocorticoid (GC) on peripheral blood mononuclear cell (PBMC) and the pure tone average (PTA) improvement in SSNHL patients. Methods: Sixty inpatients with SSNHL were included from July 2013 to October 2015 in Nanjing Drum Tower Hospital, Medical School of Nanjing University. Peripheral venous blood was collected before receiving treatment, then the PBMC was isolated for GC proliferation inhibition. PBMCs of each patient were cultivated into 4 groups: Group A: PBMCs+ Medium; Group B: PBMCs+ Medium+ lipopolysaccharide (LPS, 1 μmol/L); Group C: PBMCs+ Medium+ LPS+ Dexamethasone; Group D: Medium...
August 7, 2017: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/28821934/whole-exome-sequencing-to-identify-the-cause-of-congenital-sensorineural-hearing-loss-in-carriers-of-a-heterozygous-gjb2-mutation
#3
Thomas Parzefall, Alexandra Frohne, Martin Koenighofer, Andreas Kirchnawy, Berthold Streubel, Christian Schoefer, Klemens Frei, Trevor Lucas
Bi-allelic variations in the gap junction protein beta-2 (GJB2) gene cause up to 50% of cases of newborn hearing loss. Heterozygous pathogenic GJB2 variations are also fivefold overrepresented in idiopathic patient groups compared to the normal-hearing population. Whether hearing loss in this group is due to unidentified additional variations within GJB2 or variations in other deafness genes is unknown in most cases. Whole-exome sequencing offers an effective approach in the search for causative variations in patients with Mendelian diseases...
August 18, 2017: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/28820180/osteogenesis-imperfecta
#4
REVIEW
Joan C Marini, Antonella Forlino, Hans Peter Bächinger, Nick J Bishop, Peter H Byers, Anne De Paepe, Francois Fassier, Nadja Fratzl-Zelman, Kenneth M Kozloff, Deborah Krakow, Kathleen Montpetit, Oliver Semler
Skeletal deformity and bone fragility are the hallmarks of the brittle bone dysplasia osteogenesis imperfecta. The diagnosis of osteogenesis imperfecta usually depends on family history and clinical presentation characterized by a fracture (or fractures) during the prenatal period, at birth or in early childhood; genetic tests can confirm diagnosis. Osteogenesis imperfecta is caused by dominant autosomal mutations in the type I collagen coding genes (COL1A1 and COL1A2) in about 85% of individuals, affecting collagen quantity or structure...
August 18, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28819893/-cochlear-implantation-in-a-girl-with-7q-microdeletion-syndrome
#5
A Roemer, T Lenarz, A Lesinski-Schiedat
One of the rare genetic diseases with sensory hearing loss is the microdeletion 7q syndrome. First described in the 1990s, only 7 cases of patients with this disease are described in the literature. Although this mutation is not well known, otological treatment is necessary if the DFNA5 gene is affected. A mutation in this gene leads to progressive hearing loss. Affected children therefore need regular evaluation of their hearing to ensure adequate treatment with hearing aids at early stages. We now present a case of an affected child with sensory hearing loss, mental retardation and anogenital malformations...
August 17, 2017: HNO
https://www.readbyqxmd.com/read/28819426/central-role-of-cemip-in-tumorigenesis-and-its-potential-as-therapeutic-target
#6
REVIEW
Li Li, Lin-Hai Yan, Shwetha Manoj, Ying Li, Lu Lu
CEMIP (KIAA1199) was identified as migratory indicator protein which had been crudely studied in the last decade. Firstly its mutation site was reported to cause hearing loss due to the folding change of protein structure, meanwhile the over-expression of CEMIP referred to dreadful invasion and uncontrolled proliferation of tumor with distant metastasis, dedifferentiation, and limited survival opportunity of patients. Especially, over-expressed CEMIP also protected malignant tumor from strict microenvironment in hypoxia, low glucose and cracked barrier, leading to enhanced adaptability of tumor by stimulating the Wnt, EGFR, FGFR pathway...
2017: Journal of Cancer
https://www.readbyqxmd.com/read/28818524/loss-of-sestrin-2-potentiates-the-early-onset-of-age-related-sensory-cell-degeneration-in-the-cochlea
#7
Celia Zhang, Wei Sun, Ji Li, Binbin Xiong, Mitchell D Frye, Dalian Ding, Richard Salvi, Mi-Jung Kim, Shinichi Someya, Bo Hua Hu
Sestrin 2 (SESN2) is a stress-inducible protein that protects tissues from oxidative stress and delays the aging process. However, its role in maintaining the functional and structural integrity of the cochlea is largely unknown. Here, we report the expression of SESN2 protein in the sensory epithelium, particularly in hair cells. Using C57BL/6J mice, a mouse model of age-related cochlear degeneration, we observed a significant age-related reduction in SESN2 expression in cochlear tissues that was associated with early onset hearing loss and accelerated age-related sensory cell degeneration that progressed from the base towards the apex of the cochlea...
August 14, 2017: Neuroscience
https://www.readbyqxmd.com/read/28818455/adipose-derived-stromal-cells-enhance-auditory-neuron-survival-in-an-animal-model-of-sensory-hearing-loss
#8
Philipp Schendzielorz, Maike Vollmer, Kristen Rak, Armin Wiegner, Nashwa Nada, Katrin Radeloff, Rudolf Hagen, Andreas Radeloff
BACKGROUND: A cochlear implant (CI) is an electronic prosthesis that can partially restore speech perception capabilities. Optimum information transfer from the cochlea to the central auditory system requires a proper functioning auditory nerve (AN) that is electrically stimulated by the device. In deafness, the lack of neurotrophic support, normally provided by the sensory cells of the inner ear, however, leads to gradual degeneration of auditory neurons with undesirable consequences for CI performance...
August 14, 2017: Cytotherapy
https://www.readbyqxmd.com/read/28818308/a-honey-bee-can-threat-ear-sudden-sensorineural-hearing-loss
#9
Ufuk Düzenli, Nazım Bozan, Abdurrahman Ayral, Abdülaziz Yalınkılıç, Ahmet Faruk Kıroğlu
Sudden sensorineural hearing loss is an otologic emergency. Many etiological factors can lead to this pathology. Honey bee (Apis mellifera) sting may lead to local and systemic reactions due to sensitization of the patient. In this paper we described a sudden sensorineural hearing loss occurred after honey bee sting.
August 2, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28817812/the-mir-34a-bcl-2-pathway-contributes-to-auditory-cortex-neuron-apoptosis-in-age-related-hearing-loss
#10
Qiuhong Huang, Yongkang Ou, Hao Xiong, Haidi Yang, Zhigang Zhang, Suijun Chen, Yongyi Ye, Yiqing Zheng
HYPOTHESIS: The miR-34a/Bcl-2 signaling pathway may play a role in the mechanisms related to age-related hearing loss (AHL) in the auditory cortex. BACKGROUND: The auditory cortex plays a key role in the recognition and processing of complex sound. It is difficult to explain why patients with AHL have poor speech recognition, so increasing numbers of studies have focused on its central change. Although micro (mi)RNAs in the central nervous system have recently been increasingly reported to be associated with age-related diseases, the molecular mechanisms of AHL in the auditory cortex are not fully understood...
August 18, 2017: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/28816700/recovery-of-tinnitus-and-sensorineural-hearing-loss-due-to-lysis-of-arachnoid-adhesions-in-the-posterior-cranial-fossa-is-there-a-novel-etiology-in-neurotological-disorders
#11
Raşit Cevizci, Alper Dilci, Ahmet Mahmut Tekin, Yıldırım Bayazıt
We reported the recovery of sensorineural hearing loss and tinnitus in a 22-year-old man after complete removal of intracranial portion of jugular foramen schwannoma via the retrosigmoid approach. The aim of this case report was to present the excision of a large jugular foramen schwannoma via the retrosigmoid approach and to describe the improvement of sensorineural hearing loss related to arachnoid inflammations due to chronic arachnoiditis after suboccipital craniectomy. The recovery of sensorineural hearing loss and tinnitus after release of arachnoid adhesions may indicate the clinical significance of these adhesions or arachnoiditis, which should also be considered and investigated in the etiology of other neurotological diseases...
August 2017: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/28816698/intralabyrinthine-vestibular-schwannoma-responsive-to-intratympanic-gentamicin-treatment
#12
Edoardo Covelli, Luigi Volpini, Chiara Filippi, Silvia Tarantini, Vania Marrone, Simonetta Monini, Maurizio Barbara
Intralabyrinthine schwannoma (ILS) is a rare benign tumor that affects the ends of cochlear and vestibular nerves. In a majority of the cases, it occurs with unilateral progressive sensorineural hearing loss. Less frequent symptoms include tinnitus, imbalance, vertigo, or fullness. The advent of magnetic resonance imaging allows early diagnosis and enables an appropriate therapeutic protocol. This report describes a case of intravestibular schwannoma, with fluctuating hearing loss and intractable vertigo, treated with intratympanic gentamicin...
August 2017: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/28816695/audiological-and-radiological-characteristics-in-incomplete-partition-malformations
#13
Merve Özbal Batuk, Betül Çiçek Çınar, Burçe Özgen, Gonca Sennaroğlu, Levent Sennaroğlu
OBJECTIVE: To compare the audiological and radiological findings of patients with incomplete partition malformations (IPs) and analyze the relationship between the audiological and radiological findings. MATERIALS AND METHODS: The study included 84 patients (168 ears) with IPs as follows: 26 patients with Type I;IP-I (41 ears), 54 patients with Type II;IP-II (108 ears), and 4 patients with Type III;IP-III (8 ears). Remaining 11 ears were diagnosed with other inner ear malformations...
August 2017: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/28816694/evaluation-of-hearing-handicap-in-adults-with-auditory-neuropathy-spectrum-disorder
#14
Prashanth Prabhu
OBJECTIVE: The present study attempted to evaluate hearing handicap in adults with auditory neuropathy spectrum disorder (ANSD). The study also attempted to determine if gender, pure tone average, speech identification scores (SIS), and reported duration of hearing loss could predict the hearing handicap in adults with ANSD. MATERIALS AND METHODS: Hearing Handicap Inventory for Adults and Hearing Handicap Questionnaire were administered to 50 adults with ANSD. RESULTS: Using both the scales, there was a significant hearing handicap in both the social and emotional domains in adults with ANSD...
August 2017: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/28816692/questionnaires-in-patients-with-unilateral-sudden-sensorineural-hearing-loss
#15
Gyu Ho Hwang, Jae Woo Joo, In Sik Song, Yoon Chan Rah, June Choi
OBJECTIVE: In this study, we evaluated the prognostic value of the Dizziness Handicap Inventory (DHI) and the Tinnitus Handicap Inventory (THI) in patients with unilateral sudden sensorineural hearing loss (SSNHL). MATERIALS AND METHODS: In total, 101 patients with unilateral SSNHL (44 women, 57 men), who were admitted and treated at our institution between December 2012 and June 2014, were included in the study. All patients completed the questionnaires for DHI and THI during their admission and were treated with bed rest and oral methylprednisolone (1 mg/kg, which was eventually tapered)...
August 2017: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/28816204/deep-band-modulated-phrase-perception-in-quiet-and-noise-in-individuals-with-auditory-neuropathy-spectrum-disorder-and-sensorineural-hearing-loss
#16
Hemanth Narayan Shetty, Vishal Kooknoor
CONTEXT: Deep band modulation (DBM) improves speech perception in individuals with learning disability and older adults, who had temporal impairment in them. However, it is unclear on perception of DBM phrases at quiet and noise conditions in individuals with auditory neuropathy spectrum disorder (ANSD) and sensorineural hearing loss (SNHL), as these individuals suffer from temporal impairment. AIM: The aim is to study the effect of DBM and noise on phrase perception in individuals with normal hearing, SNHL, and ANSD...
July 2017: Noise & Health
https://www.readbyqxmd.com/read/28815995/progressive-macrothrombocytopenia-and-hearing-loss-in-a-large-family-with-diaph1-related-disease
#17
Akira Ganaha, Tadashi Kaname, Ayano Shinjou, Yasutsugu Chinen, Kumiko Yanagi, Teruyuki Higa, Shunsuke Kondo, Mikio Suzuki
In this study, we describe a Japanese family with progressive hearing loss and macrothrombocytopenia. Using next-generation and Sanger sequencing analyses, we identified a heterozygous variant in exon 27 of the DIAPH1 gene (NM_005219), c.3637C>T, p.R1213X. All patients in the family had sensorineural hearing loss and macrothrombocytopenia. None of the patients exhibited a tendency to bleed. No pathogenic variants were found in the MYH9 gene. Hearing loss began with high-frequency loss during early childhood and progressed to severe hearing loss involving all frequencies...
August 16, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28815315/emerging-gene-therapies-for-genetic-hearing-loss
#18
REVIEW
Hena Ahmed, Olga Shubina-Oleinik, Jeffrey R Holt
Gene therapy, or the treatment of human disease using genetic material, for inner ear dysfunction is coming of age. Recent progress in developing gene therapy treatments for genetic hearing loss has demonstrated tantalizing proof-of-principle in animal models. While successful translation of this progress into treatments for humans awaits, there is growing interest from patients, scientists, clinicians, and industry. Nonetheless, it is clear that a number of hurdles remain, and expectations for total restoration of auditory function should remain tempered until these challenges have been overcome...
August 16, 2017: Journal of the Association for Research in Otolaryngology: JARO
https://www.readbyqxmd.com/read/28814992/feasibility-of-a-supplemental-phonological-awareness-intervention-via-telepractice-for-children-with-hearing-loss-a-preliminary-study
#19
Sue Ann S Lee, Brittany Hall, Sherry Sancibrian
The goal of the current study was to examine the feasibility of a telepractice intervention to improve phonological awareness skills in children with hearing loss as compared to a conventional in-person intervention. Twenty children with hearing loss participated in this study. Two groups of ten children each received a supplemental phonological awareness intervention either via telepractice or an in-person service delivery model. Within each of the two groups, five children were enrolled in preschool or kindergarten and five children were enrolled in first or second grade...
2017: International Journal of Telerehabilitation
https://www.readbyqxmd.com/read/28814843/effects-of-hearing-loss-on-speech-recognition-under-distracting-conditions-and-working-memory-in-the-elderly
#20
Wondo Na, Gibbeum Kim, Gungu Kim, Woojae Han, Jinsook Kim
PURPOSE: The current study aimed to evaluate hearing-related changes in terms of speech-in-noise processing, fast-rate speech processing, and working memory; and to identify which of these three factors is significantly affected by age-related hearing loss. METHODS: One hundred subjects aged 65-84 years participated in the study. They were classified into four groups ranging from normal hearing to moderate-to-severe hearing loss. All the participants were tested for speech perception in quiet and noisy conditions and for speech perception with time alteration in quiet conditions...
2017: Clinical Interventions in Aging
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