Evolution of techniques for the resection of vestibular schwannomas: from saving life to saving function.

The current state of surgery for vestibular schwannomas (VSs) is the result of a century of step-by-step technical progress by groundbreaking surgeons who transformed the procedure from its hazardous infancy and high mortality rate to its current state of safety and low morbidity rate. Harvey Cushing advocated bilateral suboccipital decompression and developed the method of intracapsular tumor enucleation. Walter Dandy supported the unilateral suboccipital approach and developed the technique of gross-total tumor resection. Microsurgical techniques revolutionized VS surgery to its current status. In this article, the authors review the early history of surgery for VSs with an emphasis on contributions from pioneering surgeons. The authors examined the Cushing Brain Tumor Registry for clues regarding the bona fide intention of Cushing for the resection of these tumors.

Audiologic outcomes with the penetrating electrode auditory brainstem implant.

OBJECTIVE: The penetrating electrode auditory brainstem implant (PABI) is an extension of auditory brainstem implant (ABI) technology originally developed for individuals deafened by neurofibromatosis type 2. Whereas the conventional ABI uses surface electrodes on the cochlear nuclei, the PABI uses 8 or 10 penetrating microelectrodes in conjunction with a separate array of 10 or 12 surface electrodes. The goals of the PABI were to use microstimulation to reduce threshold current levels, increase the range of pitch percepts, and improve electrode selectivity and speech recognition. PATIENTS AND PROTOCOL: In a prospective clinical trial, 10 individuals, all with neurofibromatosis type 2, received a PABI after vestibular schwannoma removal via a translabyrinthine approach. All study participants met strict requirements for informed consent as part of a Food and Drug Administration clinical trial. Approximately 8 weeks after implantation, PABI devices were activated and tested at our tertiary clinical and research facility. Mean follow-up time was 33.8 months. STUDY DESIGN: Using a single-subject design, we measured thresholds and dynamic ranges, electrode-specific pitch percepts, and speech perception performance at regular intervals. RESULTS: Penetrating electrodes produced auditory thresholds at substantially lower charge levels than surface electrodes, a wide range of electrode-specific pitch sensations, and minimal cross-electrode interference and could be used in speech maps either alone or in combination with surface electrodes. However, less than 25% of penetrating electrodes resulted in auditory sensations, whereas more than 60% of surface electrodes were effective. Even after more than 3 years of experience, patients using penetrating electrodes did not achieve improved speech recognition compared with those using surface electrode ABIs. In patients with usable penetrating electrodes, City University of New York Sentence Test scores with sound and visual information were 61.6% in the PABI group and 64.7% in a surface ABI cohort (p = not significant). CONCLUSION: The PABI met the goals of lower threshold, increased pitch range, and high selectivity, but these properties did not result in improved speech recognition.

Auditory brainstem implants.

The development of cochlear implantation has allowed the majority of patients deafened after the development of language to regain significant auditory benefit. In a subset of patients, however, loss of hearing results from destruction of the cochlear nerves, rendering cochlear implantation ineffective. The most common cause of bilateral destruction of the cochlear nerves is neurofibromatosis type 2 (NF2). The hallmark of this genetic disorder is the development of bilateral acoustic neuromas, the growth or removal of which causes deafness in most patients. Patients with NF2 may benefit from direct stimulation of the cochlear nucleus. We describe the development, use, and results of the auditory brainstem implant (ABI), which is typically implanted via craniotomy at the time of tumor removal. Most patients with the implant have good appreciation of environmental sounds, but obtain more modest benefit with regard to speech perception. The majority of patients make use of the implant to facilitate lip reading; some can, to varying degrees, comprehend speech directly. We discuss future directions in central implants for hearing, including the penetrating ABI, the use of ABI in nontumor patients, and the auditory midbrain implant.

Surgical salvage after failed irradiation for vestibular schwannoma.

OBJECTIVES/HYPOTHESIS: Compare vestibular schwannoma (VS) surgical outcome between patients with prior irradiation and those not previously treated. STUDY DESIGN: Retrospective review with matched control group. METHODS: Review of tumor adherence to the facial nerve, facial nerve grade, and complications in 38 patients with radiotherapy as a primary procedure before VS surgical removal and a matched random sample of 38 patients with primary surgery. The majority of the irradiated group had gamma knife radiation therapy. Mean time from irradiation to surgical salvage was 3.3 years (SD = 3.2), with a minimum of 5.2 months and a maximum of 15.8 years. Most (89.5%) patients in each group underwent a translabyrinthine approach. Mean tumor size at surgery was 2.6 cm in each group. RESULTS: The irradiated group had more moderate to severe adherence of tumor than the controls (89% vs. 63%, P < or = .01). They also had a lower rate of good facial function (House-Brackmann grade I/II) (37% vs. 70%) and a higher rate of poor function (grades V or VI) (50% vs. 18%) at follow-up (P < or = .019). Results were similar when including only those with good preoperative function (50% vs. 72% and 32% vs. 15%) but did not achieve statistical significance. Surgical time and complications did not differ. CONCLUSION: Patients who have undergone irradiation for VS and require surgical salvage may have a more difficult surgery and poorer outcomes than those not previously irradiated. When making their initial choice of treatment, patients should be counseled that surgery might be more difficult after failed stereotactic irradiation.

Neurotologic skull base surgery in pediatric patients.

OBJECTIVE: Innovations in diagnosis, surgical techniques, and perioperative care have dramatically improved outcomes in lateral skull base procedures in recent years. There is a belief, however, that children with skull base tumors have yet to benefit from these technological and procedural advances. The purpose of this study is to provide a clinical review of neurotologic skull base surgery in the pediatric population. STUDY DESIGN: Retrospective case review. SETTING: Private practice tertiary referral center. PATIENTS: Eighty-nine pediatric patients undergoing 115 neurotologic procedures for lateral skull base tumors from July 1992 to September 2003. MAIN OUTCOME MEASURES: Initial clinical presentation, tumor type, pre- and postoperative hearing and facial nerve status, treatment course, complications, and functional outcomes. RESULTS: The majority of tumors in this series were vestibular schwannomas, and 65 patients were diagnosed with neurofibromatosis Type 2. Surgical approaches included 70 middle fossa, 40 translabyrinthine, 2 transcochlear, 2 infratemporal fossa, and 1 retrosigmoid craniotomy. Complete tumor removal was accomplished in the majority of cases (97%), with good preservation of facial nerve function (House-Brackmann Grade I or II) in 80% of patients. In patients undergoing middle fossa surgery for hearing preservation, measurable hearing was preserved in 61.4% of cases. The incidence of complications was low. CONCLUSION: With advances in diagnostic procedures and use of current neurotologic techniques, pediatric patients may undergo successful treatment of lateral skull base tumors, with good functional outcomes and minimal morbidity.

The evolution of surgical approaches for posterior fossa meningiomas.

OBJECTIVE: To evaluate surgical outcomes for the treatment of posterior fossa meningiomas at the House Ear Clinic from 1987 to 2001. We review our current treatment algorithm and present our postoperative outcomes with attention to facial and auditory neural preservation. STUDY DESIGN: Retrospective review. SETTING: Tertiary care center. PATIENTS: Medical records of 71 patients who underwent posterior fossa meningioma surgery at the House Ear Clinic were reviewed. INTERVENTION: All patients had surgical removal of their meningioma via translabyrinthine, transcochlear, retrosigmoid, extended middle fossa, or combined petrosal approaches by House Ear Clinic neurotologists and neurosurgeons. MAIN OUTCOME MEASURES: Preoperative and postoperative auditory and facial nerve function data were collected. Patient and tumor characteristics including presenting symptoms, completion of tumor resection, and complications secondary to surgery were also recorded. RESULTS: The most common presenting symptoms in this series were otologic, with hearing loss (61%), tinnitus (58%), and imbalance (58%) as the three most common. Gross total resection was achieved in 67 (94%) patients. Hearing-preservation surgery was attempted in 37 (52%) patients (68% via extended middle fossa or combined approach). Twenty-one patients with preoperative Class A hearing had follow-up audiometric data and 18 (86%) had serviceable hearing preserved. Excluding transcochlear craniotomies, 85% of patients had normal facial nerve function postoperatively. Cerebrospinal fluid leak (6%) was the most common complication. CONCLUSIONS: Advances in microsurgical techniques have greatly changed our management of patients with posterior fossa meningiomas. These changes have reduced postoperative morbidity. Specifically, use of the anterior and posterior petrosal approaches has facilitated facial and auditory neural preservation while not compromising the extent of tumor excision.

The multichannel auditory brainstem implant: how many electrodes make sense?

OBJECT: Development of multichannel auditory brainstem implant (ABI) systems has been based in part on the assumption that audiological outcome can be optimized by increasing the number of available electrodes. In this paper the authors critically analyze this assumption on the basis of a retrospective clinical study performed using the Nucleus 22 ABI surface electrode array. METHODS: The perceptual performances of 61 patients with neurofibromatosis Type 2 were tested approximately 6 weeks after an eight-electrode ABI had been implanted. Of eight implanted electrodes 5.57 +/- 2.57 (mean +/- standard deviation [SD] provided auditory sensations when stimulated. Electrodes were deactivated when stimulation resulted in significant nonauditory side effects or no auditory sensation at all, and also when they failed to provide distinctive pitch sensations. The mean (+/- SD) scores for patients with ABIs were the following: sound-only consonant recognition, 20.4 +/- 14.3 (range 0-65%); vowel recognition, 28.8 +/- 18% (range 0-67%); Monosyllable Trochee Spondee (MTS) word recognition 41.1 +/- 25.3% (range 0-100%); and sentence recognition, 5.3 +/- 11.4% (range 0-64%). Performance in patients in whom between one and three electrodes provided auditory sensation was significantly poorer than that in patients with between four and eight functional electrodes in the vowel, MTS word, and City University of New York (CUNY) sentence recognition tests. The correlation between performance and electrode number did not reach the 0.05 level of significance with respect to the sound effect, consonant, and MTS stress-pattern recognition tests, probably because a satisfactory performance in these tests can be obtained only with temporal cues, that is, without any information about the frequency of the sounds. In the MTS word and the CUNY sentence recognition tests, performance was optimal in the patients with eight functional electrodes. Although all top performers had more than three functional auditory electrodes, no further improvement (asymptotic performance) was seen in those with five or more active electrodes in the consonant, vowel, and sound effect recognition tests. CONCLUSIONS: A minimum of three spectral channels, programmed in the appropriate individual tonotopic order seem to be required for satisfactory speech recognition in most patients with ABI. Due to the limited access to the tonotopic frequency gradient of the cochlear nucleus with surface stimulation, patients with ABI do not receive a wide range of spectral cues (frequency information) with multielectrode (> 5) surface arrays.

Long-term hearing preservation after middle fossa removal of vestibular schwannoma.

OBJECTIVE: We sought to determine long-term hearing preservation in vestibular schwannoma patients after undergoing middle fossa resection. STUDY DESIGN, SETTING, AND OUTCOME MEASURES: We conducted a retrospective chart review of patients undergoing middle fossa resection from 1990 to 1995 at a tertiary care center. Pure-tone thresholds, before resection and at least 5 years after resection, and speech discrimination scores are reported. RESULTS: Seventy percent of patients with immediate postoperative hearing maintained serviceable hearing at more than 5 years after surgery. Pure-tone average in the operative ear changed at the same rate as hearing in the unoperated ear during this follow-up period. CONCLUSIONS: More than two thirds of patients who underwent middle fossa resection of a vestibular schwannoma with some hearing postoperatively maintain that hearing at greater than 5 years of follow-up. Surgery alone does not have a negative impact on long-term hearing preservation.

Use of a multichannel auditory brainstem implant for neurofibromatosis type 2.

Neurofibromatosis type 2 (NF 2) typically results in deafness due to disruption of the cochlear nerves, making peripheral devices such as cochlear implants ineffective. Auditory brainstem implants (ABIs), for direct electrical stimulation of the cochlear nucleus, have been used to provide auditory stimulation in this group of patients. Currently, 141 patients have been implanted in our institution, most recently using an advanced multichannel device. We report results of a recent series of 86 patients who received ABIs. Of this group, 60 had successful implantation, recovered from surgery, responded successfully to stimulation and underwent a full course of device programming and audiologic testing. This group had significant improvement in scores on several audiologic tests compared to baseline. When used to augment lip reading, improvement was also seen. The degree of improvement varied considerably among patients. ABI is a useful device for deaf patients with NF 2. As measured by audiologic testing, many patients receive substantial benefit with regard to sound and speech comprehension.

Multichannel auditory brainstem implant: update on performance in 61 patients.

OBJECT: Neurofibromatosis Type 2 (NF2) has typically resulted in deafness after surgical removal of bilateral vestibular schwannomas (VSs). Cochlear implants are generally ineffective for this kind of deafness because of the loss of continuity in the auditory nerve after tumor removal. The first auditory brainstem implant (ABI) in such a patient was performed in 1979 at the House Ear Institute, and this individual continues to benefit from electrical stimulation of the cochlear nucleus complex. In 1992, an advanced multichannel ABI was developed and a series of patients with NF2 received this implant to study the safety and efficacy of the device. METHODS: At the time of first- or second-side VS removal, patients received an eight-electrode array applied to the surface of the cochlear nucleus within the confines of the lateral recess of the fourth ventricle. The device was activated approximately 6 weeks after implantation. and patients were tested every 3 months for the 1st year after the initial stimulation, and annually thereafter. The protocol included a comprehensive battery of psychophysical and speech perception tests. CONCLUSIONS: The multichannel ABI proved to be effective and safe in providing useful auditory sensations in most patients with NF2. The ABI improved patients' ability to communicate compared with the lipreading-only condition, it allowed the detection and recognition of many environmental sounds, and in some cases it provided significant ability to understand speech by using just the sound from the ABI (with no lipreading cues). Its performance in most patients has continued to improve for up to 8 years after implantation.

Growth of postoperative remnants of unilateral vestibular nerve schwannoma: role of the vestibular ganglion.

Histopathological examination of seven temporal bones from patients who underwent a removal of vestibular nerve schwannomas by the translabyrithine or middle fossa approaches has demonstrated small tumor remnants that failed to grow as long as 25 years after surgery. In spite of the high incidence of residual tumors, the clinical recurrence rate of tumors operated at our institution by the translabyrinthine or middle fossa approaches is low (0.3%). Immunohistochemical labeling of dividing cells demonstrated that segments of tumor adjacent to the vestibular nerve and ganglion contained more dividing cells than were present in areas of the tumor at a distance from them.