Congenital Internal Auditory Canal Stenosis With Facial Palsy

Author(s):  
Tsun-Min Lu ◽  
Nai-Wei Hsueh ◽  
Lin Hung-Ching
2019 ◽  
Vol 40 (2) ◽  
pp. e167-e169
Author(s):  
Tsun-Min Lu ◽  
Nai-Wei Hsueh ◽  
Lin Hung-Ching

2020 ◽  
Vol 44 (3) ◽  
pp. 256-259
Author(s):  
Se-Heum Park ◽  
Woo-Jin Kim ◽  
Yun-Jung Lim ◽  
Cheol-Won On ◽  
Ji-Ho Park ◽  
...  

Internal auditory canal (IAC) stenosis with hypoplasia of the facial and vestibulocochlear nerves is a rare cause of congenital facial palsy. In this case report, a 3-month-old female infant was referred for a neurological developmental assessment for developmental delay and congenital facial palsy. Upon evaluation of developmental delay, hearing loss was detected. Following a magnetic resonance imaging scan of the brain and a computed tomography scan of the temporal bone, IAC stenosis with hypoplasia of facial and vestibular nerves was diagnosed. This is a rare case of IAC stenosis in an infant with initial presentations of left facial palsy and developmental delay associated with hearing loss in the left ear. We strongly suggest that IAC stenosis be considered a cause of congenital facial palsy in infants, especially in patients with developmental delay. In infants with congenital facial palsy, a thorough physical examination and neurological developmental assessment should be performed.


2008 ◽  
Vol 39 (2) ◽  
pp. 116-119 ◽  
Author(s):  
Kao-Min Lin ◽  
Chao-Ching Huang ◽  
Joseph Hang Leung

2009 ◽  
Vol 25 (5) ◽  
pp. 628-632
Author(s):  
Inn-Chi Lee ◽  
Chien-Han Tsao ◽  
Pen-Hua Su ◽  
Jui-Ming Hu ◽  
Yan-Yan Ng ◽  
...  

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.


2017 ◽  
Vol 131 (8) ◽  
pp. 676-683 ◽  
Author(s):  
E Tahir ◽  
M D Bajin ◽  
G Atay ◽  
B Ö Mocan ◽  
L Sennaroğlu

AbstractObjectives:The bony cochlear nerve canal is the space between the fundus of the internal auditory canal and the base of the cochlear modiolus that carries cochlear nerve fibres. This study aimed to determine the distribution of bony labyrinth anomalies and cochlear nerve anomalies in patients with bony cochlear nerve canal and internal auditory canal atresia and stenosis, and then to compare the diameter of the bony cochlear nerve canal and internal auditory canal with cochlear nerve status.Methods:The study included 38 sensorineural hearing loss patients (59 ears) in whom the bony cochlear nerve canal diameter at the mid-modiolus was 1.5 mm or less. Atretic and stenotic bony cochlear nerve canals were examined separately, and internal auditory canals with a mid-point diameter of less than 2 mm were considered stenotic. Temporal bone computed tomography and magnetic resonance imaging scans were reviewed to determine cochlear nerve status.Results:Cochlear hypoplasia was noted in 44 out of 59 ears (75 per cent) with a bony cochlear nerve canal diameter at the mid-modiolus of 1.5 mm or less. Approximately 33 per cent of ears with bony cochlear nerve canal stenosis also had a stenotic internal auditory canal and 84 per cent had a hypoplastic or aplastic cochlear nerve. All patients with bony cochlear nerve canal atresia had cochlear nerve deficiency. The cochlear nerve was hypoplastic or aplastic when the diameter of the bony cochlear nerve canal was less than 1.5 mm and the diameter of the internal auditory canal was less than 2 mm.Conclusion:The cochlear nerve may be aplastic or hypoplastic even if temporal bone computed tomography findings indicate a normal cochlea. If possible, patients scheduled to receive a cochlear implant should undergo both computed tomography and magnetic resonance imaging of the temporal bone. The bony cochlear nerve canal and internal auditory canal are complementary structures, and both should be assessed to determine cochlear nerve status.


2007 ◽  
Vol 127 (11) ◽  
pp. 1226-1230 ◽  
Author(s):  
Mitsuya Suzuki ◽  
Yuki Saito ◽  
Munetaka Ushio ◽  
Tatsuya Yamasoba ◽  
Izumi Hatta ◽  
...  

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P152-P152 ◽  
Author(s):  
Marc Cohen ◽  
Claudia Kirsch ◽  
Abie Mendelsohn ◽  
Akira Ishiyama

Objectives 1) To retrospectively review the pathophysiology and radiologic features of delayed facial palsy after stapedectomy. 2) To discuss the management strategy of this unusual problem. Methods 450 stapedectomies performed at our institution were retrospectively reviewed from 1997 to 2007. A total of 2 patients in this series developed a delayed facial paralysis postoperatively. The clinical presentation, radiographic characteristics on magnetic resonance imaging (MRI), and the management of these patients are presented. Results A total of 2 patients out of 450 stapedectomies within a 10-year period developed the rare complication of delayed facial paralysis (postoperative days 12 and 41, respectively). Both patients developed rapidly progressive complete facial nerve paralysis preceded by periauricular pain and dysguesia. No sign of infection was present in the operated ear. MRI with gadolinium of the internal auditory canal demonstrated gadolinium enhancement of the labyrinthine portion of the facial nerve as seen in Bell's palsy. Both patients were treated with oral corticosteroids and antiviral medications. Subsequent imaging revealed decreased enhancement of the facial nerve with complete resolution of facial paralysis. Conclusions In the rare complication of delayed facial paralysis after stapedectomy, MR imaging is a useful diagnostic tool to dictate the treatment strategy. Delayed facial paralysis following stapedectomy can be treated medically when the MRI scan demonstrates enhancement of the facial nerve in the labyrinthine segment.


2012 ◽  
Vol 18 (2) ◽  
pp. 179-182
Author(s):  
Sathiya Murali ◽  
Arpana Shekhar ◽  
S Shyam Sudhakar ◽  
Kiran Natarajan ◽  
Mohan Kameswaran

Internal auditory canal (IAC) stenosis is a rare cause of sensorineural hearing loss. Patient may present with symptoms of progressive facial nerve palsy, hearing loss, tinnitus and giddiness. High resolution temporal bone CT-scan and magnetic resonance imaging (MRI) are the important tools for diagnosis. No specific management has been devised. Here is presentation of a case of unilateral (left) IAC stenosis with profound hearing loss and progressive House Brackmann Grade III-IV facial weakness. The diameter of the IAC was less than 2 mm on high resolution temporal bone computed tomography (HRCT) scan. It was managed by facial nerve decompression by translabyrinthine approach in an attempt to prevent further deterioration of facial palsy. DOI: http://dx.doi.org/10.3329/bjo.v18i2.12014 Bangladesh J Otorhinolaryngol 2012; 18(2): 179-182


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