Translabyrinthine resection of neurofibromatosis type 2 associated vestibular and facial schwannomas, repair of facial nerve, and placement of auditory brainstem implant

2014 ◽  
Vol 36 (v1supplement) ◽  
pp. 1 ◽  
Author(s):  
Marc S. Schwartz ◽  
Gregory P. Lekovic ◽  
Derald E. Brackmann ◽  
Courtney C. J. Voelker
Keyword(s):  
Facial Nerve ◽  
Vocal Cord Paralysis ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Facial Nerve Function ◽  
Ipsilateral Side ◽  
Total Resection ◽  
Auditory Brainstem Implant

We present video of gross-total resection of a large cerebellopontine angle tumor consisting of both vestibular and facial schwannoma components via the translabyrinthine route in a patient with neurofibromatosis type 2. The facial nerve is reconstructed using a greater auricular nerve graft, and an auditory brainstem implant is placed. Prior to surgery the patient had no facial nerve function on the operative side and had lost useful hearing. He also had usable vision only on the ipsilateral side and had contralateral vocal cord paralysis.The video can be found here: http://youtu.be/IOkEND-0vhI.

The Auditory Brainstem Implant in Neurofibromatosis Type 2: Experience from the Manchester Programme

Skull Base ◽  
2008 ◽  
Vol 18 (S 01) ◽  
Author(s):  
Simon Freeman ◽  
Richard Ramsden ◽  
Shakeel Saeed ◽  
Martin O'Driscoll ◽  
Deborah Mawman ◽  
...  
Keyword(s):  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Auditory Brainstem Implant

Sporadic unilateral vestibular schwannoma in the pediatric population

2009 ◽  
Vol 4 (2) ◽  
pp. 125-129 ◽  
Author(s):  
Brian P. Walcott ◽  
Ganesh Sivarajan ◽  
Bronislava Bashinskaya ◽  
Douglas E. Anderson ◽  
John P. Leonetti ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Tumor Size ◽  
Pediatric Population ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Nerve Function ◽  
Facial Nerve Function ◽  
Duration Of Symptoms

Object Vestibular schwannomas (VSs) are rare in the pediatric population. Most often, these lesions manifest as a bilateral disease process in the setting of neurofibromatosis Type 2. Even in the absence of additional clinical diagnostic criteria, the presentation of a unilateral VS in a young patient may be a harbinger of future penetrance for this hereditary tumor syndrome. Methods The authors retrospectively reviewed the charts of a cohort of 7 patients who presented with apparently sporadic, unilateral VSs. These patients had previously undergone surgery via translabyrinthine, retrosigmoid, or combined approaches. Clinical outcomes were reviewed with emphasis on facial nerve function and follow-up for signs and symptoms of a heritable disorder. Results All patients underwent microsurgical resection in a multidisciplinary effort by the senior authors. The average tumor size was 4.57 cm, with an average duration of symptoms prior to definitive diagnosis of 31.2 months. The tumor size at the time of presentation followed a trend different from reports in adults, while the duration of symptoms did not. At a follow-up average of 6.3 years (range 1–12 years), 100% of patients demonstrated good facial function (House-Brackmann Grade I or II). No patient in this cohort demonstrated symptoms, objective signs, or genetic analysis indicating the presence of neurofibromatosis Type 2. Conclusions Diagnosis and management of sporadic, unilateral VSs in children is complicated by clinical presentations and surgical challenges unique from their adult counterparts. Careful consideration should be given to a heritable genetic basis for sporadic unilateral VS in the pediatric population. Results of genetic testing do not preclude the necessity for long-term follow-up and systemic investigation. In patients who present with large tumors, preliminary experience leads the authors to suggest that a combined retrosigmoid-translabyrinthine approach offers the greatest opportunity for preservation of facial nerve function.

scholarly journals Restoration of Hearing with an Auditory Brainstem Implant in a Patient with Neurofibromatosis Type 2

2000 ◽  
Vol 40 (10) ◽  
pp. 524-527 ◽  
Author(s):  
Yojiro SEKI ◽  
Hiromichi UMEZU ◽  
Masaaki USUI ◽  
Kozo KUMAKAWA ◽  
Fumiai KUMAGAI ◽  
...  
Keyword(s):  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Auditory Brainstem Implant

Auditory Brainstem Implant: I. Issues in Surgical Implantation

1993 ◽  
Vol 108 (6) ◽  
pp. 624-633 ◽  
Author(s):  
Derald E. Brackmann ◽  
William E. Hitselberger ◽  
Ralph A. Nelson ◽  
Jean Moore ◽  
Michael D. Waring ◽  
...  
Keyword(s):  
Single Channel ◽  
Cranial Nerves ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Electrode Placement ◽  
Anatomic Landmarks ◽  
Auditory Evoked Response ◽  
Auditory Brainstem Implant

Most patients with neurofibromatosis type 2 (NF2) are totally deaf after removal of their bilateral acoustic neuromas. Twenty-five patients with neurofibromatosis type 2 have been implanted with a brainstem electrode during surgery to remove an acoustic neuroma. The electrode is positioned in the lateral recess of the fourth ventricle, adjacent to the cochlear nuclei. The present electrode consists of three platinum plates mounted on a Dacron mesh backing, a design that has been demonstrated to be biocompatible and positionally stable in an animal model. Correct electrode placement depends on accurate identification of anatomic landmarks from the translabyrinthine surgical approach and also on Intrasurglcal electrophysiologic monitoring. Some tumors and their removal can result in significant distortion of the brainstem and surrounding structures. Even in the absence of Identifiable anatomic landmarks, electrode location can be adjusted during surgical placement to find the location that maximizes the auditory evoked response and minimizes activation of other monitored cranial nerves. Stimulation of the electrodes produces auditory sensations in most patients, with results similar to those of single-channel cochlear Implants. A coordinated multldlscipllnary team is essential for successful application of an auditory brainstem implant.

Use of a Multichannel Auditory Brainstem Implant for Neurofibromatosis Type 2

2003 ◽  
Vol 81 (1-4) ◽  
pp. 110-114 ◽  
Author(s):  
Marc S. Schwartz ◽  
Steven R. Otto ◽  
Derald E. Brackmann ◽  
William E. Hitselberger ◽  
Robert V. Shannon
Keyword(s):  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Auditory Brainstem Implant

scholarly journals Cochlear Implantation in Patients with Neurofibromatosis Type 2 and Patients with Vestibular Schwannoma in the Only Hearing Ear

2012 ◽  
Vol 2012 ◽  
pp. 1-12 ◽  
Author(s):  
Erika Celis-Aguilar ◽  
Luis Lassaletta ◽  
Javier Gavilán
Keyword(s):  
Cochlear Implants ◽  
Vestibular Schwannoma ◽  
Cochlear Implantation ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Environmental Sound ◽  
Auditory Brainstem Implant ◽  
Surgical Option

Cochlear implants are a new surgical option in the hearing rehabilitation of patients with neurofibromatosis type 2 (NF2) and patients with vestibular schwannoma (VS) in the only hearing ear. Auditory brainstem implant (ABI) has been the standard surgical treatment for these patients. We performed a literature review of patients with NF2 and patients with VS in the only hearing ear. Cochlear implantation (CI) provided some auditory benefit in all patients. Preservation of cochlear nerve integrity is crucial after VS resection. Results ranged from environmental sound awareness to excellent benefit with telephone use. Promontory stimulation is recommended although not crucial. MRI can be performed safely in cochlear implanted patients.

Early Proactive Management of Vestibular Schwannomas in Neurofibromatosis Type 2

Neurosurgery ◽  
2001 ◽  
Vol 49 (2) ◽  
pp. 274-283 ◽  
Author(s):  
Derald E. Brackmann ◽  
Jose N. Fayad ◽  
William H. Slattery ◽  
Rick A. Friedman ◽  
John Diaz Day ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Neurofibromatosis Type ◽  
Hearing Preservation ◽  
Neck Surgery ◽  
Neurofibromatosis Type 2 ◽  
Nerve Function ◽  
Speech Discrimination ◽  
Facial Nerve Function ◽  
Proactive Management

Abstract OBJECTIVE The treatment of patients with neurofibromatosis Type 2 has always been challenging for neurosurgeons and neurotologists. Guidelines for appropriate management of this devastating disease are controversial. METHODS A retrospective study of 28 patients with neurofibromatosis Type 2 who underwent 40 middle fossa craniotomies for excision of their acoustic tumors is reported. Eleven patients underwent bilateral procedures. The study focused on hearing preservation and facial nerve results for this group of patients. The 16 male patients and 12 female patients ranged in age (at the time of surgery) from 10 to 70 years, with a mean age of 22.6 years. The mean tumor size was 1.1 cm (range, 0.5–3.2 cm), and the majority of tumors were less than 1.5 cm. RESULTS Measurable hearing was preserved in 28 ears (70%), with 42.5% being within 15 dB pure-tone average and 15% speech discrimination score of preoperative levels. In 55% of cases there was no change in the hearing class, as defined by the American Academy of Otolaryngology-Head and Neck Surgery. Of the 11 patients who underwent bilateral operations, 9 (82%) retained some hearing bilaterally. After 1-year follow-up periods (mean, 12.8 mo), 87.5% of patients exhibited normal facial nerve function (House-Brackmann Grade I). CONCLUSION Early surgical intervention to treat acoustic tumors among patients with neurofibromatosis Type 2 is a feasible treatment strategy, with high rates of hearing and facial nerve function preservation.

The restoration of hearing in neurofibromatosis type 2

1995 ◽  
Vol 109 (5) ◽  
pp. 385-389 ◽  
Author(s):  
R. Laszig ◽  
W. P. Sollmann ◽  
N. Marangos
Keyword(s):  
Electrical Stimulation ◽  
Auditory Nerve ◽  
Signal Transmission ◽  
Neurofibromatosis Type ◽  
Auditory Pathway ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Auditory Brainstem Implant ◽  
Stimulation Of

AbstractCentral electrical stimulation of the auditory pathway can allow hearing in patients sufferingfrom deafness localized in the auditory nerve. Developments in a multi-channel auditory brainstem implant based on the Nucleus Mini 22 Cochlear implant with transcutaneous signal transmission is discussed. The devices have been implanted in nine European patients suffering from Neurofibromatosis Type 2. Preliminary speech perception results and patient satisfaction are encouraging, and the data presented include some limited open speech recognition.

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