scholarly journals Decompression of the internal auditory canal via the retrosigmoid approach in a patient with Camurati-Engelmann disease: illustrative case

2021 ◽  
Vol 1 (4) ◽  
Author(s):  
Salah-Eddine Achahbar ◽  
Thomas Somers ◽  
Tony Van Havenbergh
Keyword(s):  
Facial Nerve ◽  
Skull Base ◽  
Internal Auditory Canal ◽  
Vestibular Function ◽  
Retrosigmoid Approach ◽  
Foraminal Stenosis ◽  
Illustrative Case ◽  
Vestibulocochlear Nerve ◽  
Cranial Neuropathies ◽  
Engelmann Disease

BACKGROUNDCamurati-Engelmann disease (CED) is a rare condition characterized by hyperostosis of the long bones and skull base. Symptoms include contractures and pain in affected extremities but can also include manifestations of cranial hyperostosis such as intracranial hypertension, Chiari malformation, exophthalmia, frontal bossing, and several cranial neuropathies due to cranial foraminal stenosis.OBSERVATIONSThis report describes a 27-year-old patient with suspected CED who developed progressive intermittent facial nerve paresis, hemifacial spasms, and a decrease in hearing. There were no symptoms of increased intracranial pressure or vertigo. Radiological evaluation showed a significant thickening of the skull base with serious bilateral internal auditory canal stenosis. Because of the progressive nature of the aforementioned cranial neuropathies in combination with the correlating severe radiological compression, a surgical decompression of the facial nerve and vestibulocochlear nerve was performed via a retrosigmoid approach with intraoperative monitoring. Postoperative facial nerve function was intact. Hearing and vestibular function were unchanged. There were no more episodes of facial nerve palsy or spasm.LESSONSTo the authors’ knowledge, this is the first report to describe decompression of the internal auditory canal via a retrosigmoid approach for symptomatic facial and cochlear nerve compression in a patient with CED.

scholarly journals Internal auditory canal hypoplasia associated with bilateral vestibulocochlear nerve aplasia and deviant facial nerve course: A case report and MRI findings

2021 ◽  
Vol 15 (5) ◽  
Author(s):  
Licia Pacheco Luna ◽  
Luiz Ricardo Araujo Uchoa ◽  
Luiz Arthur Brasil Gadelha Farias ◽  
Debora Lilian Nascimento Lima ◽  
Pablo Picasso De Araujo Coimbra
Keyword(s):  
Case Report ◽  
Facial Nerve ◽  
Internal Auditory Canal ◽  
Mri Findings ◽  
Vestibulocochlear Nerve

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 Vestibulocochlear Symptoms Caused by Vertebrobasilar Dolichoectasia

2020 ◽  
Vol 13 (2) ◽  
pp. 123-132 ◽  
Author(s):  
Gene Huh ◽  
Yun Jung Bae ◽  
Hyun Jun Woo ◽  
Jung Hyun Park ◽  
Ja-Won Koo ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Cranial Nerve ◽  
Internal Auditory Canal ◽  
Case Series ◽  
Vestibular Function ◽  
Neurovascular Compression ◽  
Vestibulocochlear Nerve ◽  
Radiologic Evaluation ◽  
Vertebrobasilar Dolichoectasia ◽  
Vertebrobasilar Artery

Objectives. Vertebrobasilar dolichoectasia (VBD), an elongation and distension of vertebrobasilar artery, may present with cranial nerve symptoms due to nerve root compression. The objectives of this study are to summarize vestibulocochlear manifestations in subjects with VBD through a case series and to discuss the needs of thorough oto-neurotologic evaluation in VBD subjects before selecting treatment modalities.Methods. Four VBD subjects with vestibulocochlear manifestations were reviewed retrospectively. VBD was confirmed by either brain or internal auditory canal magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). Patient information, medical history, MRI/MRA findings, and audiometry or vestibular function tests were reviewed according to patient’s specific symptom.Results. Of the four subjects, three presented with ipsilesional sensorineural hearing loss (SNHL), three with paroxysmal recurrent vertigo, and two with typewriter tinnitus. The MRI/MRA of the four subjects revealed unilateral VBD with neurovascular compression of cisternal segment or the brainstem causing displacement, angulation, or deformity of the cranial nerve VII or VIII that corresponded to the symptoms.Conclusion. Vestibulocochlear symptoms such as SNHL, recurrent paroxysmal vertigo, or typewriter tinnitus can be precipitated from a neurovascular compression of the vestibulocochlear nerve by VBD. Because proper medical or surgical treatments may stop the disease progression or improve audio-vestibular symptoms in subjects with VBD, a high index of suspicion and meticulous radiologic evaluation are needed when vestibulocochlear symptoms are not otherwise explainable, and if VBD is confirmed to cause audiovestibular manifestation, a thorough oto-neurotologic evaluation should be performed before initial treatment.

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 Combined use of diffusion tensor tractography and multifused contrast-enhanced FIESTA for predicting facial and cochlear nerve positions in relation to vestibular schwannoma

2015 ◽  
Vol 123 (6) ◽  
pp. 1480-1488 ◽  
Author(s):  
Masanori Yoshino ◽  
Taichi Kin ◽  
Akihiro Ito ◽  
Toki Saito ◽  
Daichi Nakagawa ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Vestibular Schwannoma ◽  
Diffusion Tensor ◽  
Internal Auditory Canal ◽  
Cochlear Nerve ◽  
Diffusion Tensor Tractography ◽  
Vestibulocochlear Nerve ◽  
Contrast Enhanced ◽  
Combined Use ◽  
Single Modality

OBJECT The authors assessed whether the combined use of diffusion tensor tractography (DTT) and contrast-enhanced (CE) fast imaging employing steady-state acquisition (FIESTA) could improve the accuracy of predicting the courses of the facial and cochlear nerves before surgery. METHODS The population was composed of 22 patients with vestibular schwannoma in whom both the facial and cochlear nerves could be identified during surgery. According to DTT, depicted fibers running from the internal auditory canal to the brainstem were judged to represent the facial or vestibulocochlear nerve. With regard to imaging, the authors investigated multifused CE-FIESTA scans, in which all 3D vessel models were shown simultaneously, from various angles. The low-intensity areas running along the tumor from brainstem to the internal auditory canal were judged to represent the facial or vestibulocochlear nerve. RESULTS For all 22 patients, the rate of fibers depicted by DTT coinciding with the facial nerve was 13.6% (3/22), and that of fibers depicted by DTT coinciding with the cochlear nerve was 63.6% (14/22). The rate of candidates for nerves predicted by multifused CE-FIESTA coinciding with the facial nerve was 59.1% (13/22), and that of candidates for nerves predicted by multifused CE-FIESTA coinciding with the cochlear nerve was 4.5% (1/22). The rate of candidates for nerves predicted by combined DTT and multifused CE-FIESTA coinciding with the facial nerve was 63.6% (14/22), and that of candidates for nerves predicted by combined DTT and multifused CE-FIESTA coinciding with the cochlear nerve was 63.6% (14/22). The rate of candidates predicted by DTT coinciding with both facial and cochlear nerves was 0.0% (0/22), that of candidates predicted by multifused CE-FIESTA coinciding with both facial and cochlear nerves was 4.5% (1/22), and that of candidates predicted by combined DTT and multifused CE-FIESTA coinciding with both the facial and cochlear nerves was 45.5% (10/22). CONCLUSIONS By using a combination of DTT and multifused CE-FIESTA, the authors were able to increase the number of vestibular schwannoma patients for whom predicted results corresponded with the courses of both the facial and cochlear nerves, a result that has been considered difficult to achieve by use of a single modality only. Although the 3D image including these prediction results helped with comprehension of the 3D operative anatomy, the reliability of prediction remains to be established.

Suprajugular extension of the retrosigmoid approach: microsurgical anatomy

2014 ◽  
Vol 121 (2) ◽  
pp. 397-407 ◽  
Author(s):  
Ken Matsushima ◽  
Michihiro Kohno ◽  
Noritaka Komune ◽  
Koichi Miki ◽  
Toshio Matsushima ◽  
...  
Keyword(s):  
Skull Base ◽  
Cerebellopontine Angle ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Glossopharyngeal Nerve ◽  
Retrosigmoid Approach ◽  
Illustrative Case ◽  
Microsurgical Anatomy ◽  
Total Removal ◽  
Skull Base Approach

Object Jugular foramen tumors often extend intra- and extracranially. The gross-total removal of tumors located both intracranially and intraforaminally is technically challenging and often requires a combined skull base approach. This study presents a suprajugular extension of the retrosigmoid approach directed through the osseous roof of the jugular foramen that allows the removal of tumors located in the cerebellopontine angle with extension into the upper part of the foramen, with demonstration of an illustrative case. Methods The cerebellopontine angles and jugular foramina were examined in dry skulls and cadaveric heads to clarify the microsurgical anatomy around the jugular foramen and to define the steps of the suprajugular exposure. Results The area drilled in the suprajugular approach is inferior to the acoustic meatus, medial to the endolymphatic depression and surrounding the superior half of the glossopharyngeal dural fold. Opening this area exposed the upper part of the jugular foramen and extended the exposure along the glossopharyngeal nerve below the roof of the jugular foramen. In the illustrative case, a schwannoma originating from the glossopharyngeal nerve in the cerebellopontine angle and extending below the roof of the jugular foramen and above the jugular bulb was totally removed without any postoperative complications. Conclusions The suprajugular extension of the retrosigmoid approach will permit removal of tumors located predominantly in the cerebellopontine angle but also extending into the upper part of the jugular foramen without any additional skull base approaches.

Middle Skull Base Approach With Posterolateral Mobilization of the Geniculate Ganglion to Access the Clival Regions

2011 ◽  
Vol 69 (suppl_1) ◽  
pp. ons88-ons94 ◽  
Author(s):  
Hiroki Morisako ◽  
Takeo Goto ◽  
Takashi Nagata ◽  
Isao Chokyu ◽  
Tsutomu Ichinose ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Skull Base ◽  
Surgical Techniques ◽  
Internal Auditory Canal ◽  
Geniculate Ganglion ◽  
Nerve Function ◽  
Facial Nerve Function ◽  
Base Approach ◽  
Patient Morbidity ◽  
Skull Base Approach

Abstract BACKGROUND: Clival lesions remain one of the most challenging intracranial tumors to treat surgically. Many skull base approaches have been described to improve resection and to decrease patient morbidity. OBJECTIVE: To describe a middle skull base approach with posterolateral mobilization of the geniculate ganglion of the facial nerve to access the clival regions. METHODS: Three patients with petroclival chordoma and 1 patient with petroclival meningioma underwent surgical resection of lesions with our new procedure. Surgical techniques consisted of temporal craniotomy and exposure of the facial nerve from the tympanic segment to the labyrinthine segment, keeping the fundus of the internal auditory canal intact. The geniculate ganglion was mobilized posterolaterally, followed by drilling of the cochlea and exposure of the Dorello canal. RESULTS: Four lesions were successfully removed with complete preservation of facial nerve function. CONCLUSION: This approach is a modification of the Goel procedure in which the facial nerve from the tympanic segment to the cisternal segment was totally mobilized. Our procedure carries less risk to the facial nerve function than the Goel procedure and provides sufficiently wide exposure of clival lesions.

Duplicate internal auditory canals with facial and vestibulocochlear nerve dysfunction

2011 ◽  
Vol 126 (1) ◽  
pp. 66-71 ◽  
Author(s):  
T Y Kew ◽  
A Abdullah
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Facial Nerve ◽  
Clinical Presentation ◽  
Facial Nerve Palsy ◽  
Internal Auditory Canal ◽  
Resonance Imaging ◽  
Radiological Findings ◽  
Vestibulocochlear Nerve ◽  
Nerve Dysfunction

AbstractObjective:We report an extremely rare case of duplication of the internal auditory canal associated with dysfunction of both the facial and vestibulocochlear nerves. We also review the literature regarding the integrity of the facial and vestibulocochlear nerves in such cases.Case report:A 34-year-old man presented with unilateral, right-sided, sensorineural hearing loss and facial nerve palsy since childhood. Facial nerve function was observed to be House–Brackmann grade III. Computed tomography and magnetic resonance imaging demonstrated ipsilateral duplicate, vacant internal auditory canals. Based on the clinical presentation, we interpreted these radiological findings as aplasia of the vestibulocochlear nerve and severe hypoplasia of the facial nerve.Conclusion:To our best knowledge, this is the first report of vestibulocochlear nerve aplasia and severe facial nerve hypoplasia in a case of ipsilateral duplication of the internal auditory canal. High resolution gradient echo magnetic resonance imaging sequences are advocated for assessment of neural integrity in patients with an abnormal internal auditory canal and facial and/or vestibulocochlear nerve dysfunction.

scholarly journals Facial Nerve Schwannoma: The Rare/Great Mimicker of Vestibular Schwannoma/Neuroma

2021 ◽  
Author(s):  
Deepthi Pathapati ◽  
Kiran Barla ◽  
Monal Dayal ◽  
Rajitha Gati ◽  
Praveen Kumar Lakota
Keyword(s):  
Facial Nerve ◽  
Schwann Cells ◽  
Benign Tumors ◽  
Facial Weakness ◽  
Vestibulocochlear Nerve ◽  
Seventh Nerve ◽  
Clinical Presentations ◽  
Topographical Anatomy ◽  
Facial Nerve Schwannoma ◽  
Early Surgical Intervention

AbstractSchwannomas are benign tumors arising from Schwann cells which are a protective casing of nerves, composing myelin sheath and can develop in any nerve where Schwann cells are present. Most common are vestibulocochlear nerve schwannomas. Facial nerve schwannomas (FNSs) are uncommon tumors involving seventh nerve of which geniculate ganglion involvement is most common. Clinical presentations and the imaging appearances of FNSs are influenced by the topographical anatomy of the facial nerve and vary according to the segments involved. We report a case of 73-year-old man presenting with right side facial weakness of lower motor neuron type involvement. Computed tomography and magnetic resonance imaging are clinching the diagnosis. An early diagnosis is important in containing the disease facilitating early surgical intervention.

Multi-Disciplinary Skull Base Conference and its Effects on Patient Management

2021 ◽  
pp. 000348942110212
Author(s):  
Nathan Kemper ◽  
Scott B. Shapiro ◽  
Allie Mains ◽  
Noga Lipschitz ◽  
Joseph Breen ◽  
...  
Keyword(s):  
Skull Base ◽  
Medical Center ◽  
Academic Medical Center ◽  
Significant Proportion ◽  
Internal Auditory Canal ◽  
Patient Characteristics ◽  
Management Plan ◽  
Case Review ◽  
Retrospective Case ◽  
Lateral Skull Base

Objective: Examine the effects of a multi-disciplinary skull base conference (MDSBC) on the management of patients seen for skull base pathology in a neurotology clinic. Methods: Retrospective case review of patients who were seen in a neurotology clinic at a tertiary academic medical center for pathology of the lateral skull base and were discussed at an MDSBC between July 2019 and February 2020. Patient characteristics, nature of the skull base pathology, and pre- and post-MDSBC plan of care was categorized. Results: A total of 82 patients with pathology of the lateral skull base were discussed at a MDSBC during an 8-month study period. About 54 (65.9%) had a mass in the internal auditory canal and/or cerebellopontine angle while 28 (34.1%) had other pathology of the lateral skull base. Forty-nine (59.8%) were new patients and 33 (40.2%) were established. The management plan changed in 11 (13.4%, 7.4-22.6 95% CI) patients as a result of the skull base conference discussion. The planned management changed from some form of treatment to observation in 4 patients, and changed from observation to some form of treatment in 4 patients. For 3 patients who underwent surgery, the planned approach was altered. Conclusions: For a significant proportion of patients with pathology of the lateral skull base, the management plan changed as a result of discussion at an MDSBC. Although participants of a MDSBC would agree of its importance, it is unclear how an MDSBC affects patient outcomes.

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