Facial canal bifurcation with inner ear anomalies in a case of Klippel–Feil syndrome: A case report

Author(s):  
S.J.T. Tehrani ◽  
M.Z. Faizah ◽  
T.Y. Kew ◽  
A. Abdullah
1996 ◽  
Vol 89 (2) ◽  
pp. 165-171
Author(s):  
Yuka NAKAGAWA ◽  
Akiko MORI ◽  
Tomoko HOSOKAWA ◽  
Shindow LIN ◽  
Akira Tanaka ◽  
...  

1979 ◽  
Vol 88 (1) ◽  
pp. 72-78 ◽  
Author(s):  
Tetsuya Egami ◽  
Isamu Sando ◽  
Eugene N. Myers

A temporal bone histopathological study was conducted to detect temporal bone anomalies in 20 ears (10 cases) of individuals with congenital heart anomalies. We restricted our study to patients more than one year of age, and to heart anomalies of unknown etiology. The temporal bones were obtained from refrigerated cadavers, fixed in formalin, embedded in celloidin, cut in a horizontal plane, stained with H & E, and mounted on glass slides for light microscopic study. Anomalies observed in the middle ear were: remnants of mesenchymal tissue (8 ears), wide angle of the facial genu (6 ears), persistence of the stapedial artery (5 ears), large defect of the facial canal (4 ears), high jugular bulb (4 ears), and bulky incus (2 ears). Inner ear anomalies consisted of a shortened cochlea (5 ears), anomaly of the horizontal canal (3 ears), anomaly of the posterior canal (2 ears), obliteration of the cochlear aqueduct (2 ears), and patent utriculoendolymphatic valve (1 ear). Most of the anomalies observed appeared to be due to arrested development, and resembled features which may be found at various stages of fetal life. Structural anomalies were more commonly found in the mesoderm than in the ectoderm, and middle ear anomalies were more frequently encountered than anomalies of the inner ear. No definite relationship between these anomalies observed in the temporal bone and hearing problems which had been recorded clinically for these patients could be detected. However, the large defect (more than one third of the circumference) of the facial canal, the high jugular bulb, and the stapedial artery persistence should be recognized as problems since they may be encountered during middle ear surgery.


1987 ◽  
Vol 80 (8) ◽  
pp. 1217-1223 ◽  
Author(s):  
Toshimitsu Kobayashi ◽  
Minoru Takeyama ◽  
Koji Hozawa ◽  
Toshihiko Kikuchi ◽  
Jun Kusakari ◽  
...  

2021 ◽  
pp. 014556132199683
Author(s):  
Wenqi Liang ◽  
Line Wang ◽  
Xinyu Song ◽  
Fenqi Gao ◽  
Pan Liu ◽  
...  

The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyndromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in MYH9 and MYH14, encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient’s hearing was rehabilitated with bilateral cochlear implantation.


1974 ◽  
Vol 84 (7) ◽  
pp. 1188-1201 ◽  
Author(s):  
Makoto Igarashi ◽  
Donald B. Singer ◽  
Bobby R. Alford ◽  
Ted A. Cook
Keyword(s):  

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Alaa Nasser Hussain Zaher ◽  
Tougan Taha Abd El Aziz ◽  
Ahmed Samy Abdelrahman

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).


2012 ◽  
Vol 71 (4) ◽  
pp. 270-275
Author(s):  
Takeshi Masuda ◽  
Kimitaka Kaga

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