scholarly journals New Imaging Findings of Incomplete Partition Type III Inner Ear Malformation and Literature Review

2020 ◽  
Vol 41 (6) ◽  
pp. 1076-1080
Author(s):  
R. Hong ◽  
Q. Du ◽  
Y. Pan
Keyword(s):  
Literature Review ◽  
Inner Ear ◽  
Imaging Findings ◽  
Type Iii ◽  
Inner Ear Malformation ◽  
Incomplete Partition

Duplicated internal auditory canal with inner ear malformation: Case report and literature review

2018 ◽  
Vol 45 (2) ◽  
pp. 351-357 ◽  
Author(s):  
Yoshitaka Takanashi ◽  
Tetsuaki Kawase ◽  
Yasuko Tatewaki ◽  
Jun Suzuki ◽  
Izumi Yahata ◽  
...  
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Inner Ear ◽  
Internal Auditory Canal ◽  
Inner Ear Malformation

scholarly journals A New Pathogenic Variant in POU3F4 Causing Deafness Due to an Incomplete Partition of the Cochlea Paved the Way for Innovative Surgery

Genes ◽  
2021 ◽  
Vol 12 (5) ◽  
pp. 613
Author(s):  
Ahmet M. Tekin ◽  
Marco Matulic ◽  
Wim Wuyts ◽  
Masoud Zoka Assadi ◽  
Griet Mertens ◽  
...  
Keyword(s):  
Inner Ear ◽  
Surgical Planning ◽  
Internal Auditory Canal ◽  
Pathogenic Variant ◽  
Liquor Cerebrospinalis ◽  
Clinical Tool ◽  
Inner Ear Malformation ◽  
Cochlear Implant Surgery ◽  
Incomplete Partition ◽  
Genetic Results

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.

scholarly journals A novel cochlear measurement that predicts inner-ear malformation

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Tawfiq Khurayzi ◽  
Fida Almuhawas ◽  
Abdulrahman Alsanosi ◽  
Yassin Abdelsamad ◽  
Úna Doyle ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Inner Ear ◽  
Type I ◽  
Basal Turn ◽  
Type Iii ◽  
A Value ◽  
Inner Ear Malformation ◽  
C Value ◽  
Temporal Bones ◽  
Value Measurement

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.

Cochlear Implant in Patients with Incomplete Partition Type III

2009 ◽  
Vol 52 (6) ◽  
pp. 492 ◽  
Author(s):  
Jae Hyuk Choi ◽  
Kyu Yup Lee ◽  
Eun Jung Lim ◽  
Sang Heun Lee
Keyword(s):  
Cochlear Implant ◽  
Type Iii ◽  
Incomplete Partition

scholarly journals Hereditary angioedema type III (estrogen-dependent) report of three cases and literature review*

2013 ◽  
Vol 88 (4) ◽  
pp. 578-584 ◽  
Author(s):  
Amanda Rodrigues Miranda ◽  
Ana Paula Fusel de Ue ◽  
Dominique Vilarinho Sabbag ◽  
Wellington de Jesus Furlani ◽  
Patricia Karla de Souza ◽  
...  
Keyword(s):  
Literature Review ◽  
Clinical Features ◽  
Oral Contraceptives ◽  
Hereditary Angioedema ◽  
Genetic Alterations ◽  
C1 Inhibitor ◽  
Type Iii ◽  
Triggering Factors ◽  
Encoding Gene ◽  
And Control

In this article, three cases of hereditary angioedema (HAE) type III (estrogen-dependent or with normal C1 inhibitor) are reported. The HAE was initially described in women of the same family in association with high-leveled estrogenic conditions such as the use of oral contraceptives and pregnancy. There is no change in the C1 inhibitor as happens in other types of hereditary angioedema, and mutations are observed in the encoding gene of the XII factor of coagulation in several patients. The current diagnosis is mainly clinical and treatment consists in the suspension of the triggering factors and control of acute symptoms. A brief review of physiopathology, clinical features, genetic alterations and treatment are also presented.

Role of Computed Tomography and Magnetic Resonance Imaging in detecting the Prevalence of inner ear anomalies among cochlear implant candidates

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Alaa Nasser Hussain Zaher ◽  
Tougan Taha Abd El Aziz ◽  
Ahmed Samy Abdelrahman
Keyword(s):  
Magnetic Resonance Imaging ◽  
Computed Tomography ◽  
Hearing Loss ◽  
Cochlear Implant ◽  
Inner Ear ◽  
Resonance Imaging ◽  
High Resolution Computed Tomography ◽  
Vestibular Aqueduct ◽  
Incomplete Partition ◽  
Ear Anomalies

Abstract Background Hearing loss management using cochlear implants in patients with inner ear anomalies has long been discussed in the otology community. Magnetic resonances imaging (B,/IRI) and Computed tomography (CT) play important roles in the preoperative assessment of inner ear abnormalities such as cochlear nerve deficiency and variant anatomy as these abnormalities may not only affect the decision of the implantation procedure or the patient's prognosis regarding auditory improvement, but also the risk of complications. Objective To examine the prevalence of inner ear anomalies among cochlear implant recipients in patients with congenital sensorineural hearing loss among the pediatric age group in the Demerdash hospital, Ain Shams university using High resolution computed tomography (HRCT) and MRI imaging. Methods A retrospective descriptive study over the course of 9 months that included all patients that are candidates for cochlear implant referred to the Radiology department, Ain Shams University Hospitals for a preoperative imaging in the form of CT and VIRI scans. Results CT and MRI scans of 33 patients who had congenital hearing loss and were candidates for cochlear implantation with total 66 ears were reviewed. Inner ear anomalies were identified in 8 patients representing a prevalence (24.2%) with 14 ear diseased. Anomalies were seen bilaterally in 6 patients and unilaterally in 2 patients. Among the 14 diseased ear, 9 ears (64.3%) were seen with incomplete partition Il, 7 ears (50%) were seen with enlarged vestibular aqueduct, 4 ears (28.6%) were seen with cochlear hypoplasia, 3 ears (21.4%) were seen with semicircular canal aplasia, 2 ears (14.3%) were seen with incomplete partition type I, 2 ears (14.3%) were seen with cochlear nerve aplasia, 2 ears with cochlear aplasia (14.3%), I ear (7.1%) was seen with common cavity ear (7.1%) with complete labyrinthine aplasia. Conclusion Prevalence of inner ear anomalies among cochlear implant candidates was 24.2%. This result is consistent with results worldwide and the most common anomalies were Incomplete partition Il and large vestibular aqueduct. Abbreviations Computed tomography (CT), Magnetic resonance imaging (MRI), High resolution computed tomography (HRCT), Internal auditory canal (IAC), Cerebellopontine angle (CPA).

scholarly journals Cochlear implantation in cases with incomplete partition type III (X-linked anomaly)

2008 ◽  
Vol 265 (11) ◽  
pp. 1439-1439
Author(s):  
Armagan Incesulu ◽  
Baki Adapinar ◽  
Cem Kecik
Keyword(s):  
Cochlear Implantation ◽  
Type Iii ◽  
Incomplete Partition
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