Cochlear Implantation in a Mondini Malformation of the Inner Ear and the Management of Perilymphatic Gusher

2015 ◽  
pp. 66-71
Author(s):  
Markus C. Dahm ◽  
Benno P. Weber ◽  
Thomas Lenarz
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation

scholarly journals Hearing loss in inner ear and systemic autoimmune disease: A systematic review of post‐cochlear implantation outcomes

2021 ◽  
Author(s):  
Jonathan Lee ◽  
Kirsty Biggs ◽  
Jameel Muzaffar ◽  
Manohar Bance ◽  
Peter Monksfield
Keyword(s):  
Systematic Review ◽  
Hearing Loss ◽  
Autoimmune Disease ◽  
Inner Ear ◽  
Cochlear Implantation ◽  
Systemic Autoimmune Disease

Cochlear Implantation Outcomes in Patients With Autoimmune and Immune-Mediated Inner Ear Disease

2010 ◽  
Vol 31 (8) ◽  
pp. 1337-1342 ◽  
Author(s):  
Saba Aftab ◽  
Maroun T. Semaan ◽  
Gail S. Murray ◽  
Cliff A. Megerian
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Immune Mediated ◽  
Inner Ear Disease

Cochlear implantation in patients afflicted with inner ear malformations – the Pécs experience

2018 ◽  
Vol 16 (4) ◽  
pp. 232-237
Author(s):  
Peter Bako ◽  
Adrienn Nemeth ◽  
Krisztian Molnar ◽  
Tamas Toth ◽  
Kinga Harmat ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Inner Ear Malformations ◽  
Ear Malformations

B111 Cochlear implantation in congenital middle and inner ear malformations in children

2011 ◽  
Vol 75 ◽  
pp. 30
Author(s):  
H. Skarzynski ◽  
M. Porowski ◽  
M. Mrówka ◽  
P. Mlotkowska-Klimek ◽  
A. Lorens ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Inner Ear Malformations ◽  
Ear Malformations

Role of Electrically Evoked Auditory Brainstem Response in Cochlear Implantation of Children With Inner Ear Malformations

2008 ◽  
Vol 29 (5) ◽  
pp. 626-634 ◽  
Author(s):  
Ana H. Kim ◽  
Paul R. Kileny ◽  
H. Alexander Arts ◽  
Hussam K. El-Kashlan ◽  
Steven A. Telian ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Auditory Brainstem Response ◽  
Auditory Brainstem ◽  
Inner Ear Malformations ◽  
Ear Malformations ◽  
Brainstem Response

Vestibular evoked myogenic potentials of children with inner ear malformations before and after cochlear implantation

2008 ◽  
pp. 1-8
Author(s):  
Yulian Jin ◽  
Yukiko Shinjo ◽  
Yusuke Akamatsu ◽  
Tatsuya Yamasoba ◽  
Kimitaka Kaga
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Vestibular Evoked Myogenic Potentials ◽  
Inner Ear Malformations ◽  
Before And After ◽  
Ear Malformations

Measurement of the Human Cochlear Spiral Curvature In Vivo

2013 ◽  
Vol 284-287 ◽  
pp. 1552-1558
Author(s):  
Jen Fang Yu ◽  
Kun Che Lee
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Inner Ear ◽  
Cochlear Implantation ◽  
Resonance Imaging ◽  
Basal Turn ◽  
Magnetic Resonance Imaging Mri ◽  
Human Inner Ear

This research aims to characterize the geometry of the human cochlear spiral in vivo by measuring curvature and length. Magnetic resonance imaging (MRI) was used to visualise the human inner ear in vivo. The inner ear was imaged in 12 ears in 7 subjects recruited. Visualisation of the cochlear spiral was enhanced by T2 weighting and further processing of the raw images. The spirals were divided into 3 segments: the basal turn segment, the middle turn segment and the apex turn segment. The length and curvature of each segment were measured. The measured lengths of cochlear spiral are consistent with data in the literature derived from anatomical dissections. Overall, the apex turn segment of the cochlear had the greatest degree of curvature. A detailed description of the cochlear spiral is provided, using measurements of curvature and length. This data will provide a valuable reference in the development of cochlear implantation procedures.

scholarly journals Detailed Clinical Features of Deafness Caused by a Claudin-14 Variant

2019 ◽  
Vol 20 (18) ◽  
pp. 4579 ◽  
Author(s):  
Kitano ◽  
Kitajiri ◽  
Nishio ◽  
Usami
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Cochlear Implantation ◽  
Vestibular Function ◽  
Sound Field ◽  
Speech Discrimination ◽  
Epithelial Barrier Function ◽  
Pathogenic Variants ◽  
Novel Variant ◽  
Claudin 14

Tight junctions are cellular junctions that play a major role in the epithelial barrier function. In the inner ear, claudins, occludin, tricellulin, and angulins form the bicellular or tricellular binding of membrane proteins. In these, one type of claudin gene, CLDN14, was reported to be responsible for human hereditary hearing loss, DFNB29. Until now, nine pathogenic variants have been reported, and most phenotypic features remain unclear. In the present study, genetic screening for 68 previously reported deafness causative genes was carried out to identify CLDN14 variants in a large series of Japanese hearing loss patients, and to clarify the prevalence and clinical characteristics of DFNB29 in the Japanese population. One patient had a homozygous novel variant (c.241C>T: p.Arg81Cys) (0.04%: 1/2549). The patient showed progressive bilateral hearing loss, with post-lingual onset. Pure-tone audiograms indicated a high-frequency hearing loss type, and the deterioration gradually spread to other frequencies. The patient showed normal vestibular function. Cochlear implantation improved the patient’s sound field threshold levels, but not speech discrimination scores. This report indicated that claudin-14 is essential for maintaining the inner ear environment and suggested the possible phenotypic expansion of DFNB29. This is the first report of a patient with a tight junction variant receiving a cochlear implantation.

Down's syndrome with abnormal inner ear: Is it suitable for cochlear implantation?

2013 ◽  
Vol 17 (2) ◽  
pp. 25-28 ◽  
Author(s):  
H. Eyzawiah ◽  
A. Suraya ◽  
A. Asma
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Down's Syndrome ◽  
Down’S Syndrome
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