scholarly journals Unilateral internal auditory canal stenosis with progressive facial weakness

2012 ◽  
Vol 18 (2) ◽  
pp. 179-182
Author(s):  
Sathiya Murali ◽  
Arpana Shekhar ◽  
S Shyam Sudhakar ◽  
Kiran Natarajan ◽  
Mohan Kameswaran
Keyword(s):  
Hearing Loss ◽  
High Resolution ◽  
Facial Nerve ◽  
Temporal Bone ◽  
Internal Auditory Canal ◽  
Facial Weakness ◽  
Profound Hearing Loss ◽  
Canal Stenosis ◽  
Temporal Bone Ct ◽  
Magnetic Resonance Imaging Mri

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

Congenital internal auditory canal stenosis

2003 ◽  
Vol 117 (10) ◽  
pp. 784-787 ◽  
Author(s):  
Seung Kuk Baek ◽  
Sung Won Chae ◽  
Hak Hyun Jung
Keyword(s):  
Hearing Loss ◽  
High Resolution ◽  
Facial Nerve ◽  
Ct Scan ◽  
Sensorineural Hearing Loss ◽  
Temporal Bone ◽  
Nerve Palsy ◽  
Facial Nerve Palsy ◽  
Internal Auditory Canal ◽  
Canal Stenosis

Congenital internal auditory canal stenosis is a rare cause of sensorineural hearing loss in children. A retrospective analysis including clinical manifestation and radiological findings was made for seven patients who were diagnosed with congenital internal auditory canal stenosis from 1996 to 2002. Chief presenting symptoms were hearing loss, facial nerve palsy, dizziness, and tinnitus. Hearing loss including deafness was found in five cases, vestibular function loss in four cases, and profound functional loss of facial nerve in two cases. In all cases, the diameter of the internal auditory canal was less than 2 mm on high-resolution temporal bone computed tomography (CT) scan. Two cases revealed bilateral internal auditory canal stenosis, and others were unilaterally involved cases. Congenital internal auditory canal stenosis can be an important cause of sensorineural hearing loss, facial nerve palsy, and vestibular dysfunction. High resolution temporal bone CT scan and magnetic resonance (MR) imaging were important tools for diagnosis.

scholarly journals Chances of Improvement in Cases of Vestibular Schwannoma Presenting with Facial Nerve Weakness: Presentation of Two Cases and Literature Review

2018 ◽  
Vol 80 (01) ◽  
pp. 040-045
Author(s):  
Ahmed Rizk ◽  
Marcus Mehlitz ◽  
Martin Bettag
Keyword(s):  
Hearing Loss ◽  
Facial Nerve ◽  
Male Patient ◽  
Vestibular Schwannoma ◽  
Internal Auditory Canal ◽  
Retrosigmoid Approach ◽  
Neurophysiological Monitoring ◽  
Facial Weakness ◽  
First Case ◽  
The Brain

Background and Study Aim Facial nerve (FN) weakness as a presenting feature in vestibular schwannoma (VS) is extremely rare. We are presenting two different cases of VS with significant facial weakness and reviewed the literature for similar cases. Methods and Results We are presenting two cases of VS with significant facial weakness. The first case was a 63-year-old male patient presented with 3 weeks' history of severe left-sided facial weakness (House–Brackmann [HB] grade V) and hearing loss. Magnetic resonance imaging (MRI) of the brain showed a tumor in the left internal auditory canal. Gross total removal with anatomical and physiological FN preservation was performed through a retrosigmoid approach under neurophysiological monitoring. FN function improved postoperatively to HB grade II after 16 months. The other case was 83-year-old male patient presented with sudden left-sided hearing loss and severe facial weakness (HB grade V). MRI of the brain 2.5 years before showed a left-sided (Class-T3A) cystic VS. The tumor was asymptomatic; wait-and-scan strategy was advised by the treating neurologist. Recent MRI of the brain showed approximately three times enlargement of the tumor with brain stem compression, extensive cystic changes, and suspected intratumoral hemorrhage. Surgery was performed; the tumor was subtotally removed through a retrosigmoid approach with intraoperative FN monitoring. The FN was anatomically preserved; however, physiological preservation was not possible. Severe facial weakness with incomplete lid closure persisted postoperatively. Conclusion Surgical treatment could be offered to cases of suspected VS presenting with facial weakness, as these cases may still have a chance for improvement especially in laterally located tumors.

scholarly journals Inner ear salivary gland choristoma extending to the middle ear with congenital profound hearing loss and facial palsy: a case report

2021 ◽  
Vol 50 (1) ◽  
Author(s):  
Yoshiharu Yamanobe ◽  
Naoki Oishi ◽  
Takanori Nishiyama ◽  
Makoto Hosoya ◽  
Kaoru Ogawa
Keyword(s):  
Hearing Loss ◽  
Salivary Gland ◽  
Facial Nerve ◽  
Inner Ear ◽  
Middle Ear ◽  
Internal Auditory Canal ◽  
Residual Tumor ◽  
Partial Resection ◽  
Profound Hearing Loss ◽  
Rare Benign Tumor

Abstract Background Salivary gland choristoma (SGCh) is a rare benign tumor reported in several unusual sites, such as the gastrointestinal tract, the optic nerve, and the internal auditory canal, but never reported in the inner ear. Case presentation An 8-year-old girl with a history of left profound congenital hearing loss presented to us with ipsilateral progressive severe facial nerve palsy (House-Brackmann Grade VI). The left tympanic membrane was swollen with a pulsatile tumor. Radiological investigations revealed a multilocular tumor in the inner ear extending into the middle ear and internal auditory canal (IAC). We performed a partial resection of the tumor by transmastoid approach to preserve the anatomical structure of the facial nerve. The tumor was pathologically diagnosed as SGCh. Two years after surgery, her facial function recovered to House-Brackmann Grade II and the residual tumor did not show regrowth on MRI. Conclusions Although the natural course of this rare tumor is unknown, a partial resection is an acceptable treatment procedure when functional recovery of the facial nerve is anticipated. Graphical abstract

Narrow internal auditory canal syndrome: parasaggital reconstruction

2000 ◽  
Vol 114 (5) ◽  
pp. 392-394 ◽  
Author(s):  
Yang-Sun Cho ◽  
Dong Gyu Na ◽  
Jae Yun Jung ◽  
Sung Hwa Hong
Keyword(s):  
Magnetic Resonance ◽  
High Resolution ◽  
Facial Nerve ◽  
Temporal Bone ◽  
Magnetic Resonance Image ◽  
Internal Auditory Canal ◽  
The Other ◽  
Vestibulocochlear Nerve ◽  
Computed Tomographic ◽  
Canal Syndrome

Narrow internal auditory canal (IAC) syndrome is a malformation of the temporal bone, that is defined as an IAC diameter of only 1–2 mm on high-resolution computed tomographic scans (HRCT). This syndrome is known to be caused by the absence (aplasia or hypoplasia) of the vestibulocochlear nerve. We present a case of unilateral narrow IAC syndrome which was diagnosed by HRCT. The aplasia of the vestibulocochlear nerve was confirmed using parasigittal reconstruction magnetic resonance image (MRI). The IAC was composed of two separate canals, one of which contained a facial nerve and the other was empty with aplasia of the vestibulocochlear nerve.

scholarly journals Neurenteric cyst of the temporal bone: A case report

2021 ◽  
Vol 9 ◽  
pp. 2050313X2110679
Author(s):  
Timothy Fan ◽  
Ezer Benaim ◽  
Jorge Lee Diaz ◽  
Charles MacDonald
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Facial Nerve ◽  
Temporal Bone ◽  
Complete Resolution ◽  
Goblet Cells ◽  
Neurenteric Cyst ◽  
Rare Entity ◽  
Facial Weakness ◽  
History Of

Neurenteric cysts are rare, developmental malformations mainly found in the spinal canal. The authors report on a 29-year-old woman who presented with congenital left-sided hearing loss and a 9 days history of left ear pain and facial weakness (House–Brackmann IV). Radiological examination revealed a complex 7 mm cystic structure involving the petrous and mastoid portions of the left temporal bone. Intraoperatively, a red-brown multicystic mass was discovered and excised completely. Histologic findings showed simple columnar epithelium with goblet cells and bilayers of submucosal musculature, resembling normal intestinal linings. Post resection, the patient regained nearly full facial nerve capabilities (House–Brackmann II) with complete resolution of pain, but no changes to her hearing. The objectives of this case report are to describe the clinical, radiologic, intraoperative, and pathologic characteristics of an extradural temporal neurenteric cyst with sensorineural deficits to contribute to the growing knowledge regarding this rare entity.

scholarly journals High resolution temporal bone computerized tomography in paediatric sensorineural hearing loss prior to cochlear implantation

2020 ◽  
Vol 6 (2) ◽  
pp. 282
Author(s):  
Suhail Rafiq ◽  
Fahad Shafi ◽  
Ajaz Mohiuddin ◽  
Sajjad A. Dar
Keyword(s):  
Hearing Loss ◽  
High Resolution ◽  
Cochlear Implant ◽  
Sensorineural Hearing Loss ◽  
Temporal Bone ◽  
Computerized Tomography ◽  
Cochlear Implantation ◽  
Internal Auditory Canal ◽  
Vestibular Aqueduct ◽  
Hrct Temporal Bone

<p class="abstract"><strong>Background:</strong> Congenital sensorineural hearing loss (SNHL) is one of the most common birth defects with incidence of approximately 1:1000 live births. Imaging plays an important role in the work up of cochlear implant candidates not only to identify inner ear congenital and acquired abnormalities or cochlear nerve anomalies but also to detect temporal bone abnormalities or variations that may alter surgical approach. Preoperative evaluation of cochleovestibular anatomy is an important component of the cochlear implant evaluation. The objective of the study was high resolution computerized tomography (HRCT) assessment of congenital ear anomalies before cochlear implantation.</p><p class="abstract"><strong>Methods:</strong> This prospective study was conducted in the Department of Radio diagnosis and Imaging, GMC, Srinagar. 24 children in the age group of 1 to 12 years with unidentified causes of bilateral SNHL were subjected to HRCT over a period of 7 months from January to July 2019.  </p><p class="abstract"><strong>Results:</strong> Eighteen patients had normal radiological scans and 6 had congenital anomalies. We had one each case of common cavity, bilateral labrynthine aplasia, incomplete partition type 1, Mondini’s deformity with dilated vestibular duct, Internal auditory canal stenosis and bilateral large vestibular aqueduct. Out of 18 patients without congenital anomaly, two had Korner’s septum and giant jugular bulb which were important for operating surgeon.</p><p><strong>Conclusions:</strong> HRCT temporal bone is superior at identifying the bony labyrinth, including enlarged vestibular aqueduct and caliber of internal auditory canal. HRCT temporal bone should be supplemented by magnetic resonance imaging especially for cochlear nerve assessment. It is the initial imaging modality of choice for assessment of congenital SNHL. </p>

scholarly journals Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes

2021 ◽  
pp. 014556132199683
Author(s):  
Wenqi Liang ◽  
Line Wang ◽  
Xinyu Song ◽  
Fenqi Gao ◽  
Pan Liu ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Next Generation Sequencing ◽  
Inner Ear ◽  
Internal Auditory Canal ◽  
Cochlear Nerve ◽  
Transverse Diameter ◽  
Congenital Hearing Loss ◽  
Canal Stenosis ◽  
Ear Anomalies ◽  
Generation Sequencing

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.

scholarly journals Congenital unilateral facial palsy revealing a facial nerve agenesis: a case report and review of the literature

2019 ◽  
Vol 5 (1) ◽  
pp. 20180029
Author(s):  
Yaotse Elikplim Nordjoe ◽  
Ouidad Azdad ◽  
Mohamed Lahkim ◽  
Laila Jroundi ◽  
Fatima Zahrae Laamrani
Keyword(s):  
Case Report ◽  
Facial Nerve ◽  
Temporal Bone ◽  
Facial Paralysis ◽  
Facial Palsy ◽  
Rare Condition ◽  
Review Of The Literature ◽  
Moebius Syndrome ◽  
Temporal Bone Ct

Facial nerve aplasia is an extremely rare condition that is usually syndromic, namely, in Moebius syndrome. The occurrence of isolated agenesis of facial nerve is even rarer, with only few cases reported in the literature. We report a case of congenital facial paralysis due to facial nerve aplasia diagnosed on MRI, while no noticeable abnormality was detected on the temporal bone CT.

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