Sean Flanagan

Cystic vestibular schwannoma: classification, management, and facial nerve outcomes.

Objective: Review of postoperative morbidity and facial nerve outcomes of cystic vestibular schwannoma (CVS) patients compared with solid vestibular schwannoma (SVS) patients and a proposal for a new CVS classification system. Study Design: Retrospective review. Setting: Tertiary care facility. Patients: Ninety-six patients with surgically treated CVS (1998-2008). Outcomes were assessed in a subpopulation of 57 patients with greater than or equal to 1-year follow-up compared with 57 SVS patients. Intervention: Fifty-six CVS patients underwent the enlarged translabyrinthine approach with transapical extension (Type I), and 1 patient underwent a transcochlear/transzygomatic approach. Main Outcome Measure: Preoperative and postoperative (at least 1 yr) House-Brackmann facial nerve (HBFN) grade evaluation. Results: Favorable HBFN grades (I-III) were observed in 46 (81%) CVS patients, and unfavorable HBFN grades (IV-VI) were seen in 11 (19%) CVS patients. Comparison of tumor size and 1-year HBFN grades showed significant, moderate to strong, Pearson correlation (0.38). Comparison of long-term facial nerve outcomes with a sample of 57 matched SVS patients showed no significant difference (p = 0.74). When the tumor was adherent to the facial nerve and a dissection plane could not be developed between the cyst wall and the nerve, only subtotal resection could offer the CVS patients a normal facial nerve outcome. Conclusion: In most CVS cases, complete resection should be foreseen. Central and thick-walled tumors can be removed in almost all cases. However, when peripheral thin-walled, adherent, cystic tumors are confronted and the cysts are medially or anteriorly located, we recommend subtotal resection, leaving portions of the cyst walls on neurovascular structures and on the facial nerve. This surgical strategy allows us to improve facial nerve outcomes and to reduce complications.

Intracranial facial nerve grafting after removal of vestibular schwannoma

OBJECTIVE The objectives of this study were to evaluate outcomes from facial nerve (FN) cable grafting in patients who experienced FN transection during vestibular schwannoma removal and to compare the FN outcomes of patients who underwent FN grafting by using fibrin glue with those of patients who underwent FN grafting by using microsuture. MATERIAL AND METHODS We retrospectively evaluated a series of 33 patients in whom FN grafting was achieved either by using microsuture (8 cases) or fibrin glue (25 cases). Immediate repair of the FN was performed in all cases at the time of initial resection. The patients FN function was assessed preoperatively, in the immediate postoperative period, and at 3, 6, 9, and 12 months or more postoperatively using the House-Brackmann grading system. All patients had at least 1-year follow-up. RESULTS At 12 months, a House-Brackmann grade III was achieved in 75% of those who underwent cable nerve graft interposition by using microsuture and in 76% of those who underwent cable nerve graft interposition by using fibrin glue. Analysis of final FN function outcomes demonstrated no statistically significant difference in FN outcomes between the 2 groups (P = .891, Mann-Whitney U test; P = .1, Fisher exact test). CONCLUSIONS The functional results after FN cable grafting by using fibrin glue exclusively were equivalent to those obtained with microsuture. However, the technique of FN repair by means of fibrin glue is technically simple, less time-consuming, and imparts less trauma on the nerve than does the traditional suture method.

Outcomes in the use of intra-tympanic gentamicin in the treatment of Ménière's disease.

The pathogenesis of Ménières disease is associated with a disorder of ionic homeostasis, with the pathologic correlate being endolymphatic hydrops. Despite uncertainty as to its particular mode of action, it is accepted wisdom that intra-tympanic gentamicin has a definite therapeutic role in the control of symptoms in patients who fail to respond to medical therapy. This study reports an evaluation of the efficacy of intra-tympanic gentamicin in the treatment of Ménières disease and also presents a simple, reliable, safe method of administering gentamicin for this purpose. A retrospective review of 56 patients undergoing intra-tympanic gentamicin treatment for Ménières disease was conducted. Response to treatment was analysed using a patient survey and examination of pure-tone averages. An overall significant improvement in vertigo symptoms of 81.3 per cent was found. There was a 21.4 per cent rate of significant hearing loss, defined as greater than 10 dB, with an average loss in this group of 18.5 dB. A single dose of gentamicin applied directly to the round window resulted in a high rate of control of vertigo, with acceptably low rates of hearing loss.

CNAP To Predict Functional Cochlear Nerve Preservation in NF-2: Cochlear Implant or Auditory Brainstem Implant.

The ideal management of bilateral vestibular schwannomas (VSs) involves complete tumor resection with preservation of hearing in at least one ear. While auditory brainstem implants (ABIs) have represented a significant advance in the management of neurofibromatosis type 2 (NF-2) patients, hearing rehabilitation is far from ideal. More recently, cochlear implantation has been used in selected cases of NF-2, following tumor removal, where the patient is left with bilateral profound hearing loss but with anatomical continuity of the cochlear nerve. In selected cases, cochlear implant (CI) has given superior results to ABI. The Gruppo Otologico experience in managing NF-2 patients consists of 29 patients treated between December 1996 and December 2007 out of a total of 1723 VSs. Thirty-nine tumors have been removed, with 10 patients having had bilateral tumor removed. Ten ABIs have been implanted and 5 CIs placed. We present a case that illustrates some of the difficulties encountered in the management of NF-2 VS and provides a basis for discussion of a technique to assist intraoperative decision-making to achieve optimal hearing rehabilitation. In the setting of a negative fast auditory brainstem response (ABR), but preserved cochlear nerve action potential (CNAP), the option of a CI can be considered. However, where the fast ABR and the morphology and amplitude of the CNAP are significantly degraded at the brainstem, the placement of an ABI should be strongly considered at the time of tumor removal. This technique must be further evaluated, but it represents an adjunct in this difficult area for the neurotologist. To this end we propose that a multicenter trial be undertaken to further elucidate the role of intraoperative monitoring to determine the functional preservation of the cochlear nerve.

Quality of Life in Vestibular Schwannoma Patients Managed by Surgical or Conservative Approaches.

Objective The aim of our study was to assess quality of life (QOL) among patients who underwent microsurgical excision of vestibular schwannoma (VS) compared with those managed conservatively. Study Design Retrospective study. Setting Tertiary care center. Patients There was a total sample population of 376 patients diagnosed with a unilateral VS. Intervention A total of 223 patients with unilateral VS returned the mailed questionnaires. These were then divided into two groups—78 that had undergone microsurgical excision and 145 that were managed conservatively. Subgroups within these primary groups were created for analysis. Main Outcome Measure The primary outcome measure was the Medical Outcomes Study 36 Items Short Form (SF-36). The Dizziness Handicap Inventory test, Hearing Handicap Inventory test, and Tinnitus Handicap Inventory were also used. Results The surgically managed group had a worse QOL when compared with the conservatively managed group using SF-36, significantly so in the domains of physical role limitation and social functioning. Trends were seen toward a better QOL in some domains in the subgroups of male patients and patients younger than 65 years. Worse QOL scores in the Tinnitus Handicap Inventory were seen in the subgroups with larger tumor size. Finally, on correlation analysis between all handicap inventories and SF-36, handicap due to disequilibrium had the strongest correlation with worsening of QOL. In SF-36, the vitality domain showed the greatest correlation with otologic handicap overall, whereas the role emotional domain showed the least. Conclusion This study found that worse QOL scores for surgically managed versus conservatively managed VS patients are most significant in the areas of physical role limitation and social functioning. In some areas, patients who are male and younger report better QOL. Handicap due to disequilibrium seems to have the greatest negative impact on QOL. These factors should be considered when counseling patients regarding approach to VS, in the context of an experienced management program.

Microsurgery of skull base paragangliomas

Representing the pinnacle of skull base surgery, paraganglioma management requires the advanced surgical skills and expertise that are presented by Dr. Mario Sanna and his team in this comprehensive reference. It incorporates extensive surgical and radiological data compiled over 20 years at the preeminent Gruppo Otologica Clinic in Piacenza, Italy, and features the exquisite intraoperative photographs and schematic diagrams that are a hallmark of the highly regarded Sanna texts. All head and neck and skull base surgeons will find their knowledge, technical proficiency, and ability to manage these challenging tumors vastly increased by this informative resource. Special Features: * Step-by-step descriptions of the full range of head and neck paraganglioma procedures, including the infratemporal fossa approach type A, the extreme lateral approach, and the transcervical approach, plus guidance on managing complex tympanojugular paragangliomas* More than 1,800 clearly labeled, high-quality intraoperative photographs correlated to full-color schematic drawings for an in-depth understanding of techniques* Detailed discussion of managing the internal carotid artery during procedures, including the neuroradiological use of stents* Pathology-oriented structure that allows the reader to identify a lesion at a particular stage and then follow the critical thinking, assessment, pre-operative management, and intra-operative course taken by the surgeon * Comprehensive sections on pathology, physiology, anatomy, epidemiology, and new genetic breakthroughs that lay the groundwork for the surgical chapters* Tips, hints, and pitfalls at the end of each chapter that offer valuable insights for managing different clinical scenarios Demonstrating the complex decision-making process in many actual cases and offering practical advice for handling complications, this book puts the reader into the operating room with the surgeon. It is indispensable for all head and neck and skull base surgeons who manage paragangliomas and related pathologies, as well as for otolaryngologists, neurosurgeons, maxillofacial surgeons, radiologists, and interventional radiologists involved in assessing these intricate conditions.

Decision making for solitary vestibular schwannoma and contralateral Meniere's disease.

The existence of dual inner ear pathology such as unilateral Meniere’s disease (MD) with a contralateral vestibular schwannoma (VS) is very rare, but provides the otologist with a significant management dilemma. In this study, we present 5 cases of unilateral disabling MD with a contralateral VS in the better hearing ear. Conservative management of the VS is mandated unless there are impending complications, with management directed toward controlling the vertigo attributed to MD. If and when the VS requires intervention, or the hearing in that ear deteriorates to unserviceable levels, cochlear implant of the ear affected by MD prior to addressing the VS provides optimal management.

Utility of 3D printed temporal bones in pre-surgical planning for complex BoneBridge cases

With the advent of single-sided hearing loss increasingly being treated with cochlear implantation, bone conduction implants are reserved for cases of conductive and mixed hearing loss with greater complexity. The BoneBridge (BB, MED-EL, Innsbruck, Austria) is an active fully implantable device with no attenuation of sound energy through soft tissue. However, the floating mass transducer (FMT) part of the device is very bulky, which limits insertion in complicated ears. In this study, 3D printed temporal bones of patients were used to study its utility in preoperative planning on complicated cases. Computed tomography (CT) scans of 16 ears were used to 3D print their temporal bones. Three otologists graded the use of routine preoperative planning provided by MED-EL and that of operating on the 3D printed bone of the patient. Data were collated to assess the advantage and disadvantage of the technology. There was a statistically significant benefit in using 3D printed temporal bones to plan surgery for difficult cases of BoneBridge surgery compared to the current standard. Surgeons preferred to have the printed bones in theatre to plan their drill sites and make the transition of the planning to the patient’s operation more precise. 3D printing is an innovative use of technology in the use of preoperative planning for complex ear surgery. Surgical planning can be done on the patient’s own anatomy which may help to decrease operating time, reduce cost, increase surgical precision and thus reduce complications.

Cochlear Implantation in Ménière's Disease With and Without Labyrinthectomy.

OBJECTIVE To investigate outcomes of cochlear implantation (CI) in patients with Ménières disease (MD) with and without surgical labyrinthectomy. STUDY DESIGN Retrospective study. SETTING Multiple tertiary referral centers. SUBJECTS Thirty one ears from 27 patients (17 men, 10 women, aged 42-84) with CI in ipsilateral MD ear. INTERVENTION CI in ears with intact labyrinths (Group 1), CI with simultaneous surgical labyrinthectomy (Group 2), and CI sequential to surgical labyrinthectomy (Group 3). MAIN OUTCOME MEASURE Within-subject improvement on Bamford Kowal Bench test or City University of New York open set sentence tests. RESULTS Majority of ears achieved excellent open-set speech recognition by 12 months post-CI, irrespective of intervention group. Preoperative details including patient age and sex, implant, MD and previous intervention, and audiological test results did not significantly affect outcomes. Patients with MD undergoing CI only may experience vestibular dysfunction which may cause long-term concerns. Incidental finding was noted of eight ears with fluctuating symptoms in ipsilateral ear during 12-month period post-CI, with five of eight ears showing objective fluctuating impedances and mapping. CONCLUSION CI in MD can yield good hearing outcomes in all three groups and this is possible even after a long delay after labyrinthectomy. Bilateral MD patients are complex and prospective quality of life (QoL) measures would be beneficial in being better able to manage the vestibular outcomes as well as the audiological ones.

Jugular Foramen Meningiomas II: An Otologist's Approach, Perspective, and Experience

Meningiomas are common intracranial tumors, usually of benign and slow-growing behavior, with a tendency to invade the dura and infiltrate bone. Involvement of the jugular fossa, however, is rare, with primary involvement exceedingly so. This chapter reviews the current literature on meningiomas of this location and highlights the differences in diagnostic features and management options compared to the far more common pathology arising in this area, namely jugulo-tympanic paragangliomas and lower cranial nerve schwannomas. References to our series of 13 jugular foramen meningiomas is made to further emphasize these points. Significant advances in diagnostics, surgical and anaesthetic techniques have been made since the pioneering times, allowing curative surgical management in most cases. Despite these advances, however, both the pathology and the complex anatomy of the jugular foramen make this condition an extremely difficult one to manage. The variability of tumor extension, its rarity, and its associated morbidity of treatment make a prescriptive management protocol impossible. Jugular foramen meningiomas (JFM) are most often considered in the differential diagnosis of jugulo-tympanic paragangliomas, which make up 90% of jugular fossa tumors. This is followed by schwannomas of the lower cranial nerves, and then JFMs.