scholarly journals Genetics of Inner Ear Malformations: A Review

2021 ◽  
Vol 11 (4) ◽  
pp. 524-536
Author(s):  
Davide Brotto ◽  
Flavia Sorrentino ◽  
Roberta Cenedese ◽  
Irene Avato ◽  
Roberto Bovo ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
18Th Century ◽  
Sensorineural Hearing ◽  
Genetic Mutations ◽  
Inner Ear Malformations ◽  
Ear Malformations ◽  
Inner Ear Anomaly ◽  
First Descriptions

Inner ear malformations are present in 20% of patients with sensorineural hearing loss. Although the first descriptions date to the 18th century, in recent years the knowledge about these conditions has experienced terrific improvement. Currently, most of these conditions have a rehabilitative option. Much less is known about the etiology of these anomalies. In particular, the evolution of genetics has provided new data about the possible relationship between inner ear malformations and genetic anomalies. In addition, in syndromic condition, the well-known presence of sensorineural hearing loss can now be attributed to the presence of an inner ear anomaly. In some cases, the presence of these abnormalities should be considered as a characteristic feature of the syndrome. The present paper aims to summarize the available knowledge about the possible relationships between inner ear malformations and genetic mutations.

scholarly journals Prevalence & features of inner ear malformations among children with congenital sensorineural hearing loss: A Public Cochlear Implant Centre Experience

2020 ◽  
Vol 36 (7) ◽  
Author(s):  
Jawwad Ahmed ◽  
Ghulam Saqulain ◽  
Muhammad Iqbal javed Khan ◽  
Mobeen Kausar
Keyword(s):  
Hearing Loss ◽  
Cochlear Implant ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Demographic Data ◽  
Sensorineural Hearing ◽  
Profound Hearing Loss ◽  
Creative Commons ◽  
Inner Ear Malformations ◽  
Ear Malformations

Objective: To determine the prevalence and features of inner ear anomalies in children with congenital profound hearing loss who presented at our cochlear implant center based on imaging studies. Methods: This retrospective study reviewed charts of children with congenital SNHL, who presented to Department of Otolaryngology & Auditory Implant Centre, Capital Hospital Islamabad over a period of 2 years from 1st May 2017 to 30th April 2019. These included 481 cases of both genders aged between 1 to 12 years. After gathering demographic data, audiological data, computed tomography findings of the temporal bone were analyzed. Data was analyzed using SPSS 22. Results: The Inner Ear Malformations were identified in 48(10%) children including 28 (58.33%) males and 20 (41.67%) female. Most 20(41.67%) presented at >3-5 years of age followed by 19(39.58%) at 2-3 years. However, no significant association of gender (p=0.57, p=0.076) and age of presentation (p=0.344, p=0.697) for right and left ears was noted with inner ear malformations. The most common anomaly noted were CLA, CH-III and CH-II in decreasing order of frequency in both ears. Conclusion: The prevalence of IEM’s was found to be 48(10%). Commonest anomalies noted were CLA, CH-III and CH-II. No significant association of gender and age of presentation was noted with type of anomaly in both ears. doi: https://doi.org/10.12669/pjms.36.7.3134 How to cite this:Ahmed J, Saqulain G, Khan MIJ, Kausar M. Prevalence & features of inner ear malformations among children with congenital sensorineural hearing loss: A Public Cochlear Implant Centre Experience . Pak J Med Sci. 2020;36(7):---------.  doi: https://doi.org/10.12669/pjms.36.7.3134 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Inner ear malformations in patients with sensorineural hearing loss: detection with gradient-echo (3DFT-CISS) MRI

1996 ◽  
Vol 38 (3) ◽  
pp. 278-286 ◽  
Author(s):  
J. W. Casselman ◽  
R. Kuhweide ◽  
W. Ampe ◽  
G. D'Hont ◽  
E. F. Offeciers ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Hearing ◽  
Gradient Echo ◽  
Inner Ear Malformations ◽  
Ear Malformations

Congenital inner ear malformations without sensorineural hearing loss

2008 ◽  
Vol 35 (1) ◽  
pp. 121-126 ◽  
Author(s):  
Kumiko Yukawa ◽  
Satoshi Horiguchi ◽  
Mamoru Suzuki
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Hearing ◽  
Inner Ear Malformations ◽  
Ear Malformations

Congenital inner ear malformations without sensorineural hearing loss in children

2009 ◽  
Vol 73 (10) ◽  
pp. 1484-1487 ◽  
Author(s):  
Michio Ozeki ◽  
Zenichiro Kato ◽  
Hideo Sasai ◽  
Kazuo Kubota ◽  
Michinori Funato ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Hearing ◽  
Inner Ear Malformations ◽  
Ear Malformations

Inner ear malformations in patients with sensorineural hearing loss: detection with gradient-echo (3DFT-CISS) MRI

1996 ◽  
Vol 38 (3) ◽  
pp. 278-286 ◽  
Author(s):  
J. W. Casselman ◽  
R. Kuhweide ◽  
W. Ampe ◽  
G. D'Hont ◽  
E. F. Offeciers ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Hearing ◽  
Gradient Echo ◽  
Inner Ear Malformations ◽  
Ear Malformations

scholarly journals A Case of Perilymphatic Fistula with Inner Ear Anomaly Diagnosed Preoperatively by the Cochlin-Tomoprotein Detection Test

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Kana Lee ◽  
Naoki Ochi ◽  
Kohei Yamahara ◽  
Kunihiko Makino ◽  
Tetsuo Ikezono
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Clinical Course ◽  
Sudden Hearing Loss ◽  
Sensorineural Hearing ◽  
Fistula Repair ◽  
Perilymphatic Fistula ◽  
Ear Anomalies ◽  
Inner Ear Anomaly

We present a case of perilymphatic fistula (PLF) with inner ear anomalies having sudden, progressive sensorineural hearing loss and describe the fistula repair surgeries. We focus on the diagnosis methods of PLF and clinical course of PLF with inner ear anomaly. The cochlin-tomoprotein (CTP) detection test is very useful for the surgeons to encourage the earlier operation to sudden hearing loss cases. It is also helpful to define the diagnosis of PLF after operation. We could not get the good result as to hearing from the fistula repair surgery mainly because surgery was held 1 month after the onset. The results of the case, as well as recommendations of other reports, suggest that patients with sudden sensorineural hearing loss and PLF may need repair surgery within at most 2 weeks from the onset. We describe how to diagnose PLF more accurately using CTP detection combined with intraoperative findings.

Sudden Sensorineural Hearing Loss Associated with Inner Ear Anomaly

2005 ◽  
Vol 26 (2) ◽  
pp. 241-246 ◽  
Author(s):  
Makoto Sugiura ◽  
Tsutomu Nakashima ◽  
Shinji Naganawa ◽  
Yasutaka Otake ◽  
Toru Mukaida ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Inner Ear Anomaly

scholarly journals Radiological diagnosis of the inner ear malformations in children with sensorineural hearing loss

BJR|Open ◽  
2019 ◽  
Vol 1 (1) ◽  
pp. 20180050
Author(s):  
Bernadine Quirk ◽  
Adam Youssef ◽  
Mario Ganau ◽  
Felice D'Arco
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Significant Proportion ◽  
Sensorineural Hearing ◽  
Vestibulocochlear Nerve ◽  
Membranous Labyrinth ◽  
Pictorial Review ◽  
Integral Role ◽  
Ear Malformations

Malformations in either the inner ear, vestibulocochlear nerve (VIIIth) or auditory cortex of the brain can lead to congenital sensorineural hearing loss (SNHL). In most cases the underlying disorders involve the membranous labyrinth at a microscopic level and therefore radiological examinations are entirely normal. In a significant proportion however (up to 20%), there are abnormalities visualized in the inner ear and/or the VIIIth nerve; the type of abnormality is relevant for the surgical planning of a cochlear implant. Imaging and the accurate radiological identification of the affected inner ear structures therefore plays an integral role in the clinical evaluation of sensorineural hearing loss. In this pictorial review, we describe the main malformations of the inner ear in view of recent classifications and briefly explore the surgical implications.

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