Name:
Translabyrinthine resection of NF2 associated vestibular schwannoma with cochlear implant insertion
Description:
Translabyrinthine resection of NF2 associated vestibular schwannoma with cochlear implant insertion
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Duration:
T00H10M27S
Embed URL:
https://stream.cadmore.media/player/b37e7173-00c6-4a92-ba5a-98ab7e4482cc
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https://cadmoreoriginalmedia.blob.core.windows.net/b37e7173-00c6-4a92-ba5a-98ab7e4482cc/21-122.mp4?sv=2019-02-02&sr=c&sig=2%2F4Jt%2BtNGKMrYpbSrBuF8L%2BAztL6seMUxe0nVONh1VY%3D&st=2025-05-09T14%3A53%3A12Z&se=2025-05-09T16%3A58%3A12Z&sp=r
Upload Date:
2021-10-27T00:00:00.0000000
Transcript:
Language: EN.
Segment:0 .
[MUSIC PLAYING]
SPEAKER: In this video, we present the case of a translabyrinthine resection of a neurofibromatosis type 2-associated vestibular schwannoma with simultaneous cochlear implant insertion. The patient is a 24-year-old medical student monitor of the the superregional NF2 service based in Manchester. She was diagnosed with bilateral vestibular schwannomata managed initially with radiological observation. However, slowly progressive growth was demonstrated on recent surveillance imaging, more so of the left side of the tumor.
SPEAKER: Intracranial imaging from 2020 demonstrated an increase in the maximal linear diameter of the tumor of around 2 mm when compared to imaging from 1 year previously. There was also progressive hearing loss in the left ear. Postcontrast T1 with an MRI demonstrated the presence of bilateral vestibular schwannomata, larger on the left side as previously described. There was evidence of tumor extension laterally to the fundus of the internal auditory canal and into the vestibule.
SPEAKER: There were no intracranial meningiomas, or ependymomas. Successive audiometry demonstrated a progressive deterioration in pure tone average and speech discrimination score in the left ear. Patient's most recent pure tone audiogram is displayed on the slide, demonstrating severe to profound sensorineural hearing loss in the left ear. The patient had previously trialed a hearing aid in this ear and not found it to be helpful due to amplification of background noise and the inability to focus on individual conversations due to distortion, a problem commonly encountered with the use of hearing aids in NF2.
SPEAKER: Hearing in the right ear was normal. Given the degree of preoperative hearing loss, the patient was not a candidate for hearing preservation surgery. Although stereotactic radiosurgery was considered, we elected against this approach due to the poorer tumor control in NF2 associated with vestibular schwannomata as compared to sporadic tumors, the small but nonetheless present risk of malignant transformation of the tumor in a patient with a tumor predisposition syndrome, and the suggestion in the literature of poorer hearing outcomes associated with cochlear implant insertion in the setting of an irradiated vestibular schwannoma.
SPEAKER: The decision to proceed with a translabyrinthine approach and resection of the tumor with simultaneous cochlear implant insertion was taken for a number of reasons. Firstly, the progressive growth of the tumor and loss of hearing in the MC lateral ear justified an active approach rather than continued conservative management. Moreover, the tumor was of a size whereby preservation of facial nerve function was highly likely, which was an absolute priority for the patient.
SPEAKER: Due to the lateral extension of the tumor into the vestibule, the translabyrinthine approach was preferred, as we felt this provided the best chance of achieving a gross total resection and minimizing the requirement for further interventions in future. Given the presence of a contralateral VS, effective hearing rehabilitation in the affected ear would be even more valuable in the event of deterioration in hearing on the contralateral side.
SPEAKER: Finally, the patient had recently graduated from medical school, and the hiatus between graduation and commencing work as a doctor offered an opportunity to perform the surgery at a time when it was likely to be minimally disruptive for the patient, both personally and professionally. Cochlear nerve preserving surgery with concomitant cochlear implant insertion is an approach supported by our own institutional experience, as well as by recent systematic reviews demonstrating positive results with cochlear implantation in the setting of NF2, particularly when compared to auditory brainstem implantation.
SPEAKER: Following the induction of general anesthesia, the patient was positioned supine with the head turned to the right in a gel head ring. A curved retroauricular incision was planned. Electrodes for facial nerve EMG, facial MEPs, and electronically evoked auditory brainstem response were placed. Given that we wished to monitor facial motor evoked potentials and continuous facial EMG, inhaled anesthetic agents and muscle relaxants were avoided by our anesthesiology team.
SPEAKER: Following initial skin incision, a myocutaneous flap was raised anteriorly and secured with fishhooks to expose the mastoid. With the mastoid exposed, a cortical mastoidectory was performed in standard fashion with skeletonization of the sigmoid sinus, exposure of middle fossa dura, and delineation of the Fallopian canal.
SPEAKER: Exposed dura was cauterized prior to posterior mobilization of the sigmoid sinus and bony labyrinthectomy. Following entry into the vestibule, tumor tissue was identified and removed.
SPEAKER: Gutters were drilled at both the inferior and the superior aspect of the internal auditory canal, with the aim of exposing it through 270 degrees. Following this, the bone overlying the internal auditory canal was elevated and gently removed. After removal of the bone surrounding the internal auditory canal, the incus was removed and the epitympanotomy window widened to allow access to the round window for the placement of the golf club eABR electrode.
SPEAKER: It was not possible to record eABR waveforms at this juncture, due to interference from stimulation of the facial nerve. The dura overlying the tumor was opened with scissors, and the dural edges tacked out of the operative field with stay sutures. Arachnoid webs inferior to the tumor were divided to release CSF.
SPEAKER: A 45-degree endoscope was introduced, providing clear views of the facial and vestibulocochlear nerves as they exited the brainstem, as well as an intervening loop of AICA on the schwannoma arising more laterally. Endoscopic views were also obtained of the dorsal cochlear nucleus.
SPEAKER: However, attempts at eABR recording using a ball electrode were once again impeded by interference from facial nerve stimulation. Tumor dissection was initiated with the establishment of a plane between the tumor and the cochlear nerve, using microdissecting instruments. The CP angle component of the tumor was partially debulked before moving laterally to the internal auditory canal, where further drilling of the fundus of the internal auditory canal exposed the most lateral aspect of the tumor.
SPEAKER: The tumor was meticulously dissected from lateral to medial, with avulsion of the superior vestibular nerve to facilitate exposure of the underlying facial nerve. Final stages of the resection involved the removal of tumor components adherent to the cochlear nerve and resection of the CP angle component. Due to the lack of a clear plane between the lateral aspect of the facial nerve and the tumor, the final stages of tumor resection were facilitated by sharp dissection.
SPEAKER: A tiny volume of tumor residuum was left on the facial nerve, as we felt further attempts at establishing a plane between this very small aggregation of tumor cells and the nerve would compromise postoperative facial function. Final overview confirms near total resection with anatomical preservation of the facial and cochlear nerves. Also visible is the preserved loop of AICA, which was interposed between the tumor and the cochlear and facial nerves, as well as lying in between the cochlear and facial nerves.
SPEAKER: The facial nerve was stimulating briskly at 0.05 mA at the conclusion of tumor resection. Following tumor resection, the cochlear implant was inserted. Given that facial nerve monitoring was no longer required, short-acting muscle relaxants were administered by our anesthesiologists to allow for accurate recording of eABRs. The Eustachian tube and middle ear were obliterated with muscle, and abdominal fat was packed into the petrosectomy defect and secured with a dural sealant.
SPEAKER: The scalp was closed in layers with absorbable sutures to the skin. eABR recordings were successfully recorded while stimulating using the implant, as demonstrated on the recordings obtained by the audiologist. The patient emerged from anesthesia with no neurological deficits and grade 1 facial function. She recovered well and was discharged on postoperative day 4, following suturing of a minor CSF leak on the floor.
SPEAKER: She experienced no vestibular symptoms postoperatively, which we attribute at least partially to our administration of intratympanic gentamicin preoperatively, which has been instrumental in reducing our length of stay following VS surgery. The patient will continue to be reviewed under the auspices of the NF2 service in Manchester, with a plan for continued radiological surveillance of the contralateral vestibular schwannoma.
SPEAKER: The patient returned for programming of her cochlear implant 6 weeks postoperatively and was able to hear on stimulation of all 22 cochlear implant electrodes with no known auditory side effects. The patient will return for ongoing cochlear implant programming and audiometric follow-up. It is clear that the decision-making with respect to the management of NF2- associated vestibular schwannomata is invariably complex.
SPEAKER: However, this case serves to illustrate that in judiciously selected patients, resection of their vestibular schwannoma with cochlear nerve preservation, and cochlear implant insertion, allows for a definitive treatment of the tumor alongside hearing rehabilitation while offering a high chance of facial nerve preservation in tumors of the appropriate size.