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https://www.readbyqxmd.com/read/28549773/cochlear-implantation-in-patients-with-otosclerosis-of-the-otic-capsule
#1
Jay Burmeister, Susan Rathgeb, Jacques Herzog
OBJECTIVE: To evaluate outcomes of cochlear implantation of patients with otosclerosis of the otic capsule. STUDY DESIGN: A retrospective case series of 6 patients (7 ears). PATIENTS: 6 patients (7 ears), 5 patients with severe to profound sensorineural hearing loss; 1 patient with mild to profound sensorineural hearing loss, with radiologic evidence of otosclerosis. All patients were adult males, with or without history of stapes surgery...
May 19, 2017: American Journal of Otolaryngology
https://www.readbyqxmd.com/read/28549377/biocompatibility-of-liposome-nanocarriers-in-the-rat-inner-ear-after-intratympanic-administration
#2
Jing Zou, Hao Feng, Rohit Sood, Paavo K J Kinnunen, Ilmari Pyykko
Liposome nanocarriers (LPNs) are potentially the future of inner ear therapy due to their high drug loading capacity and efficient uptake in the inner ear after a minimally invasive intratympanic administration. However, information on the biocompatibility of LPNs in the inner ear is lacking. The aim of the present study is to document the biocompatibility of LPNs in the inner ear after intratympanic delivery. LPNs with or without gadolinium-tetra-azacyclo-dodecane-tetra-acetic acid (Gd-DOTA) were delivered to the rats through transtympanic injection...
December 2017: Nanoscale Research Letters
https://www.readbyqxmd.com/read/28547010/language-development-and-impairment-in-children-with-mild-to-moderate-sensorineural-hearing-loss
#3
Lorna F Halliday, Outi Tuomainen, Stuart Rosen
Purpose: The goal of this study was to examine language development and factors related to language impairments in children with mild to moderate sensorineural hearing loss (MMHL). Method: Ninety children, aged 8-16 years (46 children with MMHL; 44 aged-matched controls), were administered a battery of standardized language assessments, including measures of phonological processing, receptive and expressive vocabulary and grammar, word and nonword reading, and parental report of communication skills...
May 24, 2017: Journal of Speech, Language, and Hearing Research: JSLHR
https://www.readbyqxmd.com/read/28546963/auditory-processing-abilities-of-parkinson-s-disease-patients
#4
Robert L Folmer, Jay J Vachhani, Sarah M Theodoroff, Rachel Ellinger, Amy Riggins
Since Parkinson's Disease (PD) primarily affects older people, a majority of PD patients have age-related hearing loss (HL) that will worsen over time. The goal of this study was to assess peripheral and central auditory functions in a population of PD patients and compare the results with a group of age-matched control subjects. Study participants included 35 adults with PD (mean age = 66.9 ± 11.2 years) and a group of 35 healthy control subjects (mean age = 65.4 ± 12.3 years). Assessments included questionnaires, neuropsychological tests, audiometric testing, and a battery of central auditory processing tests...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28546251/mr-imaging-in-sudden-sensorineural-hearing-loss-time-to-talk
#5
G Conte, F Di Berardino, C Sina, D Zanetti, E Scola, C Gavagna, L Gaini, G Palumbo, P Capaccio, F Triulzi
Sudden sensorineural hearing loss is defined as acute hearing loss of the sensorineural type of at least 30 dB over 3 contiguous frequencies that occurs within a 72-hour period. Although many different causative factors have been proposed, sudden sensorineural hearing loss is still considered "idiopathic" in 71%-85% of cases, and treatments are empiric, not based on etiology. MR imaging implemented with a 3D FLAIR sequence has provided new insights into the etiology of sudden sensorineural hearing loss. Herein, we review the current management trends for patients with sudden sensorineural hearing loss, from the initial clinical diagnosis to therapeutic strategies and diagnostic work-up...
May 25, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28544920/acquisition-of-who-question-comprehension-in-german-children-with-hearing-loss
#6
Eva Wimmer, Monika Rothweiler, Martina Penke
For children with sensorineural hearing loss the ability to understand wh-questions might be particularly challenging because they often have only restricted access to spoken language input during optimal periods of language acquisition. In previous research it has been suggested that this restricted input during critical stages in language acquisition might lead to syntactic deficits that persist into adolescence. In this study we want to pursue this issue by investigating the comprehension of wh-questions in German children with bilateral sensorineural hearing loss...
May 13, 2017: Journal of Communication Disorders
https://www.readbyqxmd.com/read/28544110/waardenburg-syndrome-a-rare-cause-of-inherited-neuropathy-due-to-sox10-mutation
#7
Petya Bogdanova-Mihaylova, Michael D Alexander, Raymond Pj Murphy, Sinéad M Murphy
Waardenburg syndrome (WS) is a rare disorder comprising sensorineural deafness and pigmentation abnormalities. Four distinct subtypes are defined based on the presence or absence of additional symptoms. Mutations in six genes have been described in WS. SOX10 mutations are usually associated with a more severe phenotype of WS with peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy and Hirschsprung disease. Here we report a 32 year-old man with a novel heterozygous missense variant in SOX10 gene, who presented with congenital deafness, Hirschsprung disease, iris heterochromia, foot deformity and intermediate conduction velocity length-dependent sensorimotor neuropathy...
May 22, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28542515/a-novel-frameshift-mutation-of-smpx-causes-a-rare-form-of-x-linked-nonsyndromic-hearing-loss-in-a-chinese-family
#8
Zhijie Niu, Yong Feng, Lingyun Mei, Jie Sun, Xueping Wang, Juncheng Wang, Zhengmao Hu, Yunpeng Dong, Hongsheng Chen, Chufeng He, Yalan Liu, Xinzhang Cai, Xuezhong Liu, Lu Jiang
X-linked hearing impairment is the rarest form of genetic hearing loss (HL) and represents only a minor fraction of all cases. The aim of this study was to investigate the cause of X-linked inherited sensorineural HL in a four-generation Chinese family. A novel duplication variant (c.217dupA, p.Ile73Asnfs*5) in SMPX was identified by whole-exome sequencing. The frameshift mutation predicted to result in the premature truncation of the SMPX protein was co-segregated with the HL phenotype and was absent in 295 normal controls...
2017: PloS One
https://www.readbyqxmd.com/read/28541783/cyclosporine-causes-no-hearing-defect-in-paediatric-patients-with-nephrotic-syndrome
#9
Belde Kasap-Demir, Derya Özmen, Günay Kırkım, Ersoy Doğan, Alper Soylu, Bülent Şerbetçioğlu, Salih Kavukçu
OBJECTIVE: We aimed to evaluate the ototoxicity of cyclosporine A (CsA) in children with nephrotic syndrome (NS). DESIGN: Data of paediatric patients with NS followed in paediatric nephrology department were evaluated retrospectively, and hearing functions were evaluated by pure tone audiometry (PTA) and transient evoked otoacoustic emissions (TEOAEs). Age, gender, type of NS, duration and cumulative doses of immunosuppressives were noted. STUDY SAMPLE: The patients who had received CsA (n: 16) and immunosuppressives other than CsA (n: 13) for at least 6 months formed two patient groups and healthy cases formed a control group (n: 20)...
May 25, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/28535812/current-insights-in-noise-induced-hearing-loss-a-literature-review-of-the-underlying-mechanism-pathophysiology-asymmetry-and-management-options
#10
REVIEW
Trung N Le, Louise V Straatman, Jane Lea, Brian Westerberg
BACKGROUND: Noise-induced hearing loss is one of the most common forms of sensorineural hearing loss, is a major health problem, is largely preventable and is probably more widespread than revealed by conventional pure tone threshold testing. Noise-induced damage to the cochlea is traditionally considered to be associated with symmetrical mild to moderate hearing loss with associated tinnitus; however, there is a significant number of patients with asymmetrical thresholds and, depending on the exposure, severe to profound hearing loss as well...
May 23, 2017: Journal of Otolaryngology—Head & Neck Surgery
https://www.readbyqxmd.com/read/28534734/the-impact-of-single-sided-deafness-upon-music-appreciation
#11
Sarah Meehan, Elizabeth A Hough, Gemma Crundwell, Rachel Knappett, Mark Smith, David M Baguley
BACKGROUND: Many of the world's population have hearing loss in one ear; current statistics indicate that up to 10% of the population may be affected. Although the detrimental impact of bilateral hearing loss, hearing aids, and cochlear implants upon music appreciation is well recognized, studies on the influence of single-sided deafness (SSD) are sparse. PURPOSE: We sought to investigate whether a single-sided hearing loss can cause problems with music appreciation, despite normal hearing in the other ear...
May 2017: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/28534732/evaluation-of-adaptive-noise-management-technologies-for-school-age-children-with-hearing-loss
#12
Jace Wolfe, Mila Duke, Erin Schafer, Christine Jones, Lori Rakita
BACKGROUND: Children with hearing loss experience significant difficulty understanding speech in noisy and reverberant situations. Adaptive noise management technologies, such as fully adaptive directional microphones and digital noise reduction, have the potential to improve communication in noise for children with hearing aids. However, there are no published studies evaluating the potential benefits children receive from the use of adaptive noise management technologies in simulated real-world environments as well as in daily situations...
May 2017: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/28534728/hearing-loss-and-age-induced-changes-in-the-central-auditory-system-measured-by-the-p3-response-to-small-changes-in-frequency
#13
Kathy R Vander Werff, Kerrie L Nesbitt
BACKGROUND: Recent behavioral studies have suggested that individuals with sloping audiograms exhibit localized improvements in frequency discrimination in the frequency region near the drop in hearing. Auditory-evoked potentials may provide evidence of such cortical plasticity and reorganization of frequency maps. PURPOSE: The objective of this study was to evaluate electrophysiological evidence of cortical plasticity related to cortical frequency representation and discrimination abilities in older individuals with high-frequency sensorineural hearing loss (SNHL)...
May 2017: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/28532971/clinical-and-audiological-characteristics-of-1000hz-audiometric-notch-patients
#14
Hyun-Min Lee, Seok-Hyun Kim, Jae-Hwan Jung, Se-Joon Oh, Soo-Keun Kong, Il-Woo Lee
PURPOSE: Among the hearing loss patients, we can confirm that the hearing loss of the specific frequency decreases, such as the 2000Hz notch in otosclerosis and the 4000Hz notch (c5-dip) in noise-induced hearing loss. The 1000Hz notch (c3-dip), however, is rarely studied. We fortuitously encountered a group of patients with a 1kHz hearing loss and report it with a review of the literature. METHODS: Otological history, audiogram, diagnosis, occupation, and history of noise exposure were reviewed from charts and telephone interview, and compared between c3-dip and c5-dip patients (n=98)...
April 1, 2017: American Journal of Otolaryngology
https://www.readbyqxmd.com/read/28532627/regenerative-medicine-in-hearing-recovery
#15
REVIEW
Edi Simoni, Giulia Orsini, Milvia Chicca, Simone Bettini, Valeria Franceschini, Alessandro Martini, Laura Astolfi
Hearing loss, or deafness, affects 360 million people worldwide of which about 32 million are children. Deafness is irreversible when it involves sensory hair cell death because the regenerative ability of these cells is lost in mammals after embryo development. The therapeutic strategies for deafness include hearing aids and/or implantable devices. However, not all patients are eligible or truly benefit from these medical devices. Regenerative medicine based on stem cell application could play a role in both improvement of extant medical devices and in vivo recovery of auditory function by regeneration of inner ear cells and neurons...
May 19, 2017: Cytotherapy
https://www.readbyqxmd.com/read/28530253/sensitivity-and-specificity-of-vestibular-bedside-examination-in-detecting-viii-cranial-nerve-schwannoma-with-sensorineural-sudden-unilateral-hearing-loss-as-presenting-symptom
#16
L Califano, F Salafia, M G Melillo, S Mazzone
The objectives of this study were to identify signs of vestibular nerve suffering through a bedside vestibular examination protocol in case of sudden sensorineural unilateral hearing loss without spontaneous signs of vestibular impairment and to propose a bed-side vestibular examination based protocol for the focused execution of gadolinium-enhanced magnetic resonance imaging (MRI) only if a vestibular schwannoma is suspected. 96 patients, 52 men, 44 women, mean age 57.73 +/- 12.85 years, suffering from sudden sensorineural unilateral hearing loss, which presented neither vertigo nor spontaneous nystagmus, were enrolled...
May 22, 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/28524840/a-novel-de-novo-gata-binding-protein-3-mutation-in-a-patient-with-hypoparathyroidism-sensorineural-deafness-and-renal-dysplasia-syndrome
#17
Xue-Ying Chu, Yue-Peng Li, Min Nie, Ou Wang, Yan Jiang, Mei Li, Wei-Bo Xia, Xiao-Ping Xing
No abstract text is available yet for this article.
June 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28521807/case-report-a-novel-frameshift-mutation-in-the-mitochondrial-cytochrome-c-oxidase-ii-gene-causing-mitochondrial-disorder
#18
Laura Kytövuori, Mikko Kärppä, Hannu Tuominen, Johanna Uusimaa, Markku Saari, Reetta Hinttala, Kari Majamaa
BACKGROUND: Mitochondrial cytochrome c oxidase 2, MT-CO2, encodes one of the three subunits, which form the catalytic core of cytochrome c oxidase (COX), complex IV. Mutations in MT-CO2 are rare and the associated phenotypes are variable including nonsyndromic and syndromic forms of mitochondrial diseases. CASE PRESENTATION: We describe a 30-year-old man with cognitive decline, epilepsy, psychosis, exercise intolerance, sensorineural hearing impairment, retinitis pigmentosa, cataract and lactic acidosis...
May 18, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28516965/benefits-of-active-middle-ear-implants-over-hearing-aids-in-patients-with-sloping-high-tone-hearing-loss-comparison-with-hearing-aids
#19
J M Lee, J H Jeon, I S Moon, J Y Choi
In this retrospective chart review we compared the subjective and objective benefits of active middle ear implants (AMEIs) with conventional hearing aids (HAs) in patients with sloping high tone hearing loss. Thirty-four patients with sensorineural hearing loss were treated with AMEIs. Of these, six had sloping high tone hearing loss and had worn an HA for more than 6 months. Objective assessments, a pure-tone audiogram, as well as a word recognition test, and the Korean version of the Hearing in Noise Test (K-HINT), and a subjective assessment, the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire, were performed...
June 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/28516283/developmental-window-of-sensorineural-deafness-in-biotinidase-deficient-mice
#20
Kathleen June Maheras, Kirit Pindolia, Barry Wolf, Alexander Gow
Biotinidase deficiency is an autosomal recessively inherited disorder that results in the inability to recycle the vitamin, biotin. If untreated, the disorder can result in a range of neurological and cutaneous symptoms, including sensorineural deficits and deafness. To understand early mechanistic abnormalities that may precede more generalized and nonspecific effects of metabolic deficits such as weight loss and acidosis, we have analyzed auditory brainstem responses (ABRs) in biotinidase-deficient knockout (Btd (-/-) ) mice in the periweaning period with or without dietary biotin supplementation...
May 17, 2017: Journal of Inherited Metabolic Disease
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