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Large vestibular aqueduct

Kemal Koray Bal, Onur Ismi, Helen Bucioglu, Yusuf Vayısoğlu, Kemal Gorur
Sudden hearing loss in the only hearing ear cases are rarely published in the English literature; most of the cases are idiopathic. It is an otologic emergency needing urgent treatment. Delayed diagnosis can interfere with patient's social life with interrupting the verbal communication. In this case report we presented a 33-year-old female patient having sudden sensorineural hearing loss in the only hearing ear diagnosed as bilateral large vestibular aqueduct syndrome.
2016: Case Reports in Otolaryngology
Ahmad M Aldhafeeri, Abdulrahman A Alsanosi
OBJECTIVE: To review the difficulties that can occur during cochlear implant surgery in patients with inner ear abnormalities and the management thereof. METHODS: A retrospective chart review of 316 patients who received cochlear implants was conducted. The data collected included the types of inner ear anomalies, intraoperative findings, and the clinical management strategies. A review of the literature was also performed. RESULTS: A total of 24 patients with inner ear malformations who underwent 28 total cochlear implant procedures were identified...
January 2017: International Journal of Pediatric Otorhinolaryngology
Yunlong Li, Baosheng Zhu, Jie Su, Yifei Yin, Fangqing Yu
Large vestibular aqueduct syndrome (LVAS) is a type of hearing loss characterized by an autosomal recessive inheritance. LVAS has been shown to be associated with mutations in SLC26A4 gene. In the present study, we report the clinical, genetic and molecular characterization of a Chinese family with LVAS. By using the targeted sequence capture and next-generation sequencing, we identified heterozygous mutations of SLC26A4 p.I188T (c.563T > C), p.L582LfsX4 (c.1746 delG) and p.E704K (c.2110G > A) in the affected individual of this family, of which SLC26A4 p...
December 2016: International Journal of Pediatric Otorhinolaryngology
Ahmad M Aldhafeeri, Abdulrahman A Alsanosi
OBJECTIVES: To determine the prevalence of inner ear anomalies and the frequency of different anomaly types among cochlear implant recipients. METHODS: This study included a retrospective chart review of all patients who received cochlear implants between January 2009 and January 2013 in King Abdulaziz University Hospital cochlear implant program in Riyadh, Saudi Arabia. All subjects underwent thin-cut CT of the temporal bone and MRI. The collected data included age, gender, and CT and MRI findings regarding temporal bone anomalies...
October 2016: Saudi Medical Journal
Charlotta Kämpfe Nordström, Göran Laurell, Helge Rask-Andersen
HYPOTHESIS: The human vestibular aqueduct (VA) shows great anatomical variations, and imaging can be difficult, so we need more data on the normal anatomy of the VA for better radiologic evaluation of large vestibular aqueduct syndrome (LVAS). BACKGROUND: The normal anatomy of the human VA was analyzed in micro-dissected human temporal bones. METHODS: The study is based on two sets of human temporal bones. One set of 32 human temporal bones was selected from a collection of 50 micro-dissected specimens...
September 14, 2016: Otology & Neurotology
Elke Loos, Nicolas Verhaert, Annelore Willaert, Koenraad Devriendt, Ann Swillen, Robert Hermans, Katya Op de Beeck, Greet Hens
The 22q11 deletion syndrome (22q11DS), the most frequent microdeletion syndrome in humans, presents with a large variety of abnormalities. A common abnormality is hearing impairment. The exact pathophysiological explanation of the observed hearing loss remains largely unknown. The aim of this study was to analyze the middle and inner ear malformations as seen on computer tomographic imaging in patients with 22q11DS. We retrospectively reviewed the charts of 11 22q11DS patients who had undergone a CT of the temporal bone in the past...
November 2016: American Journal of Medical Genetics. Part A
Ayako Kanno, Hideki Mutai, Kazunori Namba, Noriko Morita, Atsuko Nakano, Noboru Ogahara, Tomoko Sugiuchi, Kaoru Ogawa, Tatsuo Matsunaga
OBJECTIVES/HYPOTHESIS: To determine the frequency of the incomplete partition type III anomaly and the genetic and clinical features associated with POU3F4 mutations in children with hearing loss. STUDY DESIGN: Retrospective case series from 2000 to 2014 at the National Hospital Organization Tokyo Medical Center and collaborating hospitals. METHODS: A total of 1,004 patients (from 938 families) who had hearing loss by 10 years of age and had undergone computed tomography scanning of their temporal bones were enrolled in this genetic, clinical, and radiological study...
August 31, 2016: Laryngoscope
Youyou Guo, Yongmei Li, Chun Zeng, Jingjie Wang, Yi Liu, Ping Yin, Dan Liao
OBJECTIVE: To explore. HRCT and MRI three-dimensional fast imaging employing steady state ac-quisition(3D-FIESTA) imaging features and clinical characteristics of bilateral large vestibular aqueduct syndrome(LVAS). METHOD: The imaging and clinical features of 14 cases of bilateral LVAS identified over a 5-year periodwere retrospectively analyzed. All patients underwent HRCT and MRI 3D-FIESTA scanning of head and neck;MRI three dimensional reconstructions of internal acoustical meatus were also completed at the same time...
March 2016: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
S Vashist, S Singh
OBJECTIVES: The purpose of this prospective study was to measure surgical outcomes in cases of cerebrospinal fluid gusher in cochlear implant surgery. MATERIALS AND METHODS: Demographic, radiological, neurophysiological and surgical results were evaluated in nine cases of patients with cerebrospinal fluid gusher during cochlear implant surgery, out of 164 unilateral cochlear implant procedures. Review of literature and our management technique is discussed. RESULTS: Nine cases had cerebrospinal fluid leak during surgery...
June 2016: European Annals of Otorhinolaryngology, Head and Neck Diseases
Kristianna Mey, Michael Bille, Per Cayé-Thomasen
OBJECTIVE: To explore specific clinical issues, surgical results, and complications of 80 cochlear implantations (CI) in 55 patients with Pendred syndrome (PS) or non-syndromic enlarged vestibular aqueduct (NSEVA). BACKGROUND: Previous studies have focused either on unselected case series or on populations with mixed cochlear malformations. PS/NSEVA accounts for up to 10% of congenital SNHL, rendering this a large group of cochlear implant candidates. The abnormal inner ear anatomy of these patients may be associated with a lower surgical success rate and a higher rate of complications...
October 2016: Acta Oto-laryngologica
Ayako Nishio, Taku Ito, Hui Cheng, Tracy S Fitzgerald, Philine Wangemann, Andrew J Griffith
SLC26A4 mutations cause fluctuating and progressive hearing loss associated with enlargement of the vestibular aqueduct (EVA). SLC26A4 encodes a transmembrane anion exchanger called pendrin expressed in nonsensory epithelial cells of the lateral wall of cochlea, vestibular organs and endolymphatic sac. We previously described a transgenic mouse model of EVA with doxycycline (dox)-inducible expression of Slc26a4 in which administration of dox from conception to embryonic day 17.5 (DE17.5) resulted in hearing fluctuation between 1 and 3months of age...
August 4, 2016: Neuroscience
Bao Zhou, Shaolian Lin, Youhui Lin, Zheming Fang, Shengnan Ye, Rong Zhang
OBJECTIVE: To investigate imaging and audiology features of temporal bone and analyze the classification and prevalence of inner ear abnormalities in children with sensorineural hearing loss. METHOD: Children who were diagnosed with sensorineural hearing loss were examined by high resolution CT and the inner ear fluid of MRI. And each chart was retrospectively reviewed to determine the imaging and audiology features. RESULT: There were 125 patients(232 ears) found with inner ear malformation in 590 children with SNHL...
November 2015: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
Mohamed M El-Badry, Nasr M Osman, Haytham Mamdouh Mohamed, Fatma M Rafaat
OBJECTIVE: The main objective of the current work is to increase the sensitivity of the radiological diagnosis of the large vestibular aqueduct syndrome (LVAS). The specific aims were to compare between the two famous criteria to diagnose large vestibular aqueduct (LVA), (i.e., Valvassori and Cincinnati), to correlate between vestibular aqueduct (VA) measurements in the axial view and those in 45° oblique reformate in children with LVAS, and to define radiological criteria to diagnose LVA in the 45° oblique reformate...
February 2016: International Journal of Pediatric Otorhinolaryngology
Aleena Shafi Jallu, Majid Jehangir, Waqar Ul Hamid, Rafiq Ahmad Pampori
Computerized tomography (CT) and magnetic resonance (MR) are complementary in the imaging of the labyrinth, the internal auditory canal and the brain in children with sensorineural hearing loss who are being evaluated for cochlear implantation. An accurate anatomical description of the inner ear is essential in the preoperative work up. Computerized tomography visualizes the bony structures, whereas MR can discern soft-tissue components including intra labyrinthine fluid, cerebrospinal fluid (CSF), nerves, and vessels within the IAC...
December 2015: Indian Journal of Otolaryngology and Head and Neck Surgery
Michihiko Sone, Tadao Yoshida, Kyoko Morimoto, Masaaki Teranishi, Tsutomu Nakashima, Shinji Naganawa
OBJECTIVES/HYPOTHESIS: Pathologic third window lesions, such as superior semicircular canal dehiscence syndrome (SCDS) or large vestibular aqueduct syndrome (LVAS), cause several auditory and vestibular symptoms, which might affect perilymphatic pressure and induce endolymphatic hydrops (EH). In this study, the existence of EH in subjects with SCDS or LVAS was investigated using contrast-enhanced magnetic resonance imaging (MRI). STUDY DESIGN: Case series at university hospital...
June 2016: Laryngoscope
Rabindra Pradhananga, Kiran Natarajan, AmarNath Devarasetty, Mohan Kameswaran
Introduction Large vestibular aqueduct syndrome (LVAS) is characterized by the enlargement of the vestibular aqueduct associated with sensorineural hearing loss. It is the most common radiographically detectable inner ear anomaly in congenital hearing loss. LVAS may occur as an isolated anomaly or in association with other inner ear malformations. Objective To report three cases of isolated LVAS with a focus on preoperative assessment, surgical issues, and short-term postoperative follow-up with preliminary auditory habilitation outcomes...
October 2015: International Archives of Otorhinolaryngology
Nicholas L Deep, Joseph M Hoxworth, David M Barrs
No abstract text is available yet for this article.
February 2016: Laryngoscope
T Ito, A Nishio, P Wangemann, A J Griffith
Hearing loss of patients with enlargement of the vestibular aqueduct (EVA) can fluctuate or progress, with overall downward progression. The most common detectable cause of EVA is mutations of SLC26A4. We previously described a transgenic Slc26a4-insufficient mouse model of EVA in which Slc26a4 expression is controlled by doxycycline administration. Mice that received doxycycline from conception until embryonic day 17.5 (DE17.5; doxycycline discontinued at embryonic day 17.5) had fluctuating hearing loss between 1 and 6 months of age with an overall downward progression after 6 months of age...
December 3, 2015: Neuroscience
Galdino E Valvassori, Jack D Clemis
No abstract text is available yet for this article.
September 2015: Laryngoscope
So Young Kim, Ah Reum Kim, Nayoung K D Kim, Min Young Kim, Eun-Hee Jeon, Bong Jik Kim, Young Eun Han, Mun Young Chang, Woong-Yang Park, Byung Yoon Choi
BACKGROUND: Despite the prevalence of CDH23 mutations in East Asians, its large size hinders investigation. The pathologic mutation p.P240L in CDH23 is common in East Asians. However, whether this mutation represents a common founder or a mutational hot spot is unclear. The prevalence of CDH23 mutations with prelingual severe-to-profound sporadic or autosomal recessive sensorineural hearing loss (arSNHL) is unknown in Koreans. METHODS: From September 2010 to October 2014, children with severe-to-profound sporadic or arSNHL without phenotypic markers, and their families, were tested for mutations in connexins GJB2, GJB6 and GJB3...
August 13, 2015: Journal of Translational Medicine
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