inner ear malformation
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Author(s):  
Bong Jik Kim ◽  
Hyoungwon Jeon ◽  
Sang-Yeon Lee ◽  
Nayoung Yi ◽  
Jin Hee Han ◽  
...  

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Anandhan Dhanasingh ◽  
Daniel Erpenbeck ◽  
Masoud Zoka Assadi ◽  
Úna Doyle ◽  
Peter Roland ◽  
...  

AbstractIdentification of the inner ear malformation types from radiographs is a complex process. We hypothesize that each inner ear anatomical type has a uniqueness in its appearance in radiographs. The outer contour of the inner ear was captured from the mid-modiolar section, perpendicular to the oblique-coronal plane, from which the A-value was determined from CT scans with different inner ear anatomical types. The mean A-value of normal anatomy (NA) and enlarged vestibular aqueduct syndrome (EVAS) anatomical types was greater than for Incomplete Partition (IP) type I, II, III and cochlear hypoplasia. The outer contour of the cochlear portion within the mid-modiolar section of NA and EVAS resembles the side view of Aladdin’s lamp; IP type I resembles the side-view of the Sphinx pyramid and type II a Pomeranian dog’s face. The steep spiraling cochlear turns of IP type III resemble an Auger screw tip. Drawing a line parallel to the posterior margin of internal auditory canal (IAC) in axial-view, bisecting the cavity into cochlear and vestibular portions, identifies common-cavity; whereas a cavity that falls under the straight-line leaving no cochlear portion identifies cochlear aplasia. An atlas of the outer contour of seventy-eight inner ears was created for the identification of the inner malformation types precisely.


2021 ◽  
Vol 16 (8) ◽  
pp. 1931-1933
Author(s):  
Tran Phan Ninh ◽  
Truong Quang Dinh ◽  
Thieu-Thi Tra My ◽  
Bui-Thi Phuong Thao ◽  
Bui Khac Hieu ◽  
...  

Author(s):  
Anagha R. Joshi ◽  
Anisha O. Talreja ◽  
Mrinalini M. Rahatgaonkar ◽  
Harshal A. Badgujar

AbstractExternal ear malformations are commonly associated with middle ear malformations due to their closely related embryogenesis. Inner ear anomalies are less consistently seen due to their independent development. Cross-sectional imaging has come to play a critical role in the workup of congenital deafness in the pediatric age group. To optimize speech and language development, early diagnosis and treatment are very crucial. Identification of these anomalies is additionally important when operative intervention is being planned because they may complicate or perhaps preclude the surgery.


Author(s):  
Ashish Kumar Gupta ◽  
Mohnish Grover ◽  
Sunil Samdani ◽  
N. Sushmitha

Genes ◽  
2021 ◽  
Vol 12 (5) ◽  
pp. 613
Author(s):  
Ahmet M. Tekin ◽  
Marco Matulic ◽  
Wim Wuyts ◽  
Masoud Zoka Assadi ◽  
Griet Mertens ◽  
...  

Incomplete partition type III (IP-III) is a relatively rare inner ear malformation that has been associated with a POU3F4 gene mutation. The IP-III anomaly is mainly characterized by incomplete separation of the modiolus of the cochlea from the internal auditory canal. We describe a 71-year-old woman with profound sensorineural hearing loss diagnosed with an IP-III of the cochlea that underwent cochlear implantation. Via targeted sequencing with a non-syndromic gene panel, we identified a heterozygous c.934G > C p. (Ala31Pro) pathogenic variant in the POU3F4 gene that has not been reported previously. IP-III of the cochlea is challenging for cochlear implant surgery for two main reasons: liquor cerebrospinalis gusher and electrode misplacement. Surgically, it may be better to opt for a shorter array because it is less likely for misplacement with the electrode in a false route. Secondly, the surgeon has to consider the insertion angles of cochlear access very strictly to avoid misplacement along the inner ear canal. Genetic results in well describes genotype-phenotype correlations are a strong clinical tool and as in this case guided surgical planning and robotic execution.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Tawfiq Khurayzi ◽  
Fida Almuhawas ◽  
Abdulrahman Alsanosi ◽  
Yassin Abdelsamad ◽  
Úna Doyle ◽  
...  

AbstractThe A-value used in cochlear duct length (CDL) estimation does not take malformed cochleae into consideration. The objective was to determine the A-value reported in the literature, to assess the accuracy of the A-value measurement and to evaluate a novel cochlear measurement in distinguishing malformed cochlea. High resolution Computer Tomography images in the oblique coronal plane/cochlear view of 74 human temporal bones were analyzed. The A-value and novel C-value measurement were evaluated as predictors of inner ear malformation type. The proximity of the facial nerve to the basal turn was evaluated subjectively. 26 publications report on the A-value; but they do not distinguish normal vs. malformed cochleae. The A-values of the normal cochleae compared to the cochleae with cochlear hypoplasia, incomplete partition (IP) type I, -type II, and -type III were significantly different. The A-value does not predict the C-value. The C-values of the normal cochleae compared to the cochleae with IP type I and IP type III were significantly different. The proximity of the facial nerve to the basal turn did not relate to the type of malformation. The A-value is different in normal vs. malformed cochleae. The novel C-value could be used to predict malformed anatomy, although it does not distinguish all malformation types.


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