OBJECTIVE: The best management for patients with small- to medium-sized vestibular schwannomas (VS) is controversial. METHODS: A prospective cohort study of 82 patients with unilateral, unoperated VS less than 3 cm undergoing surgical resection (n = 36) or radiosurgery (n = 46). Patients undergoing resection were younger (48.2 yr versus 53.9 yr, P = 0.03). The groups were similar with regard to hearing loss, associated symptoms, and tumor size. The mean follow-up period was 42 months (range, 12-62 mo). RESULTS: Normal facial movement and preservation of serviceable hearing was more frequent in the radiosurgical group at 3 months (P < 0.001), 1 year (P < 0.001), and at the last follow-up examination (P < 0.01) compared with the surgical resection group. Patients undergoing surgical resection had a significant decline in the following subscales of the Health Status Questionnaire 3 months after surgery: physical functioning (P = 0.006), role-physical (P < 0.001), energy/fatigue (P = 0.02), and overall physical component (P = 0.004). Patients in the surgical resection group continued to have a significant decline in the physical functioning (P = 0.04) and bodily pain (P = 0.04) subscales at 1 year and in bodily pain (P = 0.02) at the last follow-up examination. The radiosurgical group had no decline on any component of the Health Status Questionnaire after the procedure. The radiosurgical group had lower mean Dizziness Handicap Inventory scores (16.5 versus 8.4, P = 0.02) at the last follow-up examination. There was no difference in tumor control (100 versus 96%, P = 0.50). CONCLUSION: Early outcomes were better for VS patients undergoing stereotactic radiosurgery compared with surgical resection (Level 2 evidence). Unless long-term follow-up evaluation shows frequent tumor progression at currently used radiation doses, radiosurgery should be considered the best management strategy for the majority of VS patients.

Patient outcomes after vestibular schwannoma management: a prospective comparison of microsurgical resection and stereotactic radiosurgery.

Object The aim of this study was to evaluate and present the results of current surgical treatment of vestibular schwannomas (VSs) and to report the refinements in the operative technique. Methods The authors performed a retrospective study of 200 consecutive patients who had undergone VS surgery over a 3-year period. Patient records, operative reports, follow-up data, and neuroradiological findings were analyzed. The main outcome measures were magnetic resonance imaging, neurological status, patient complaints, and surgical complications. Complete tumor removal was achieved in 98% of patients. Anatomical preservation of the facial nerve was possible in 98.5% of patients. In patients treated for tumors with extension Classes T1, T2, and T3, the rate of facial nerve preservation was 100%. By the last follow-up examination, excellent or good facial nerve function had been achieved in 81% of the cases. By at least 1 year postsurgery, no patients had total facial palsy. In the patients with preserved hearing,...

Improved preservation of hearing and facial nerve function in vestibular schwannoma surgery via the retrosigmoid approach in a series of 200 patients

Sudden sensorineural hearing loss (SSNHL) is commonly encountered in audiologic and otolaryngologic practice. SSNHL is most commonly defined as sensorineural hearing loss of 30dB or greater over at least three contiguous audiometric frequencies occurring within a 72-hr period. Although the differential for SSNHL is vast, for the majority of patients an etiologic factor is not identified. Treatment for SSNHL of known etiology is directed toward that agent, with poor hearing outcomes characteristic for discoverable etiologies that cause inner ear hair cell loss. Steroid therapy is the current mainstay of treatment of idiopathic SSNHL in the United States. The prognosis for hearing recovery for idiopathic SSNHL is dependent on a number of factors including the severity of hearing loss, age, presence of vertigo, and shape of the audiogram.

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Sudden sensorineural hearing loss: a review of diagnosis, treatment, and prognosis.

Perceptual consequences of disrupted auditory nerve activity were systematically studied in 21 subjects who had been clinically diagnosed with auditory neuropathy (AN), a recently defined disorder characterized by normal outer hair cell function but disrupted auditory nerve function. Neurological and electrophysical evidence suggests that disrupted auditory nerve activity is due to desynchronized or reduced neural activity or both. Psychophysical measures showed that the disrupted neural activity has minimal effects on intensity-related perception, such as loudness discrimination, pitch discrimination at high frequencies, and sound localization using interaural level differences. In contrast, the disrupted neural activity significantly impairs timing related perception, such as pitch discrimination at low frequencies, temporal integration, gap detection, temporal modulation detection, backward and forward masking, signal detection in noise, binaural beats, and sound localization using interaural time differences. These perceptual consequences are the opposite of what is typically observed in cochlear-impaired subjects who have impaired intensity perception but relatively normal temporal processing after taking their impaired intensity perception into account. These differences in perceptual consequences between auditory neuropathy and cochlear damage suggest the use of different neural codes in auditory perception: a suboptimal spike count code for intensity processing, a synchronized spike code for temporal processing, and a duplex code for frequency processing. We also proposed two underlying physiological models based on desynchronized and reduced discharge in the auditory nerve to successfully account for the observed neurological and behavioral data. These methods and measures cannot differentiate between these two AN models, but future studies using electric stimulation of the auditory nerve via a cochlear implant might. These results not only show the unique contribution of neural synchrony to sensory perception but also provide guidance for translational research in terms of better diagnosis and management of human communication disorders.

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Perceptual consequences of disrupted auditory nerve activity

This study examines auditory brainstem responses (ABR) elicited by rising frequency chirps. The time course of frequency change for the chirp theoretically produces simultaneous displacement maxima by compensating for travel-time differences along the cochlear partition. This broadband chirp was derived on the basis of a linear cochlea model [de Boer, “Auditory physics. Physical principles in hearing theory I,” Phys. Rep. 62, 87–174 (1980)]. Responses elicited by the broadband chirp show a larger wave-V amplitude than do click-evoked responses for most stimulation levels tested. This result is in contrast to the general hypothesis that the ABR is an electrophysiological event most effectively evoked by the onset or offset of an acoustic stimulus, and unaffected by further stimulation. The use of this rising frequency chirp enables the inclusion of activity from lower frequency regions, whereas with a click, synchrony is decreased in accordance with decreasing traveling velocity in the apical region. The use of a temporally reversed (falling) chirp leads to a further decrease in synchrony as reflected in ABR responses that are smaller than those from a click. These results are compatible with earlier experimental results from recordings of compound action potentials (CAP) [Shore and Nuttall, “High synchrony compound action potentials evoked by rising frequency-swept tonebursts,” J. Acoust. Soc. Am. 78, 1286–1295 (1985)] reflecting activity at the level of the auditory nerve. Since the ABR components considered here presumably reflect neural response from the brainstem, the effect of an optimized synchronization at the peripheral level can also be observed at the brainstem level. The rising chirp may therefore be of clinical use in assessing the integrity of the entire peripheral organ and not just its basal end.

Auditory brainstem responses with optimized chirp signals compensating basilar-membrane dispersion

Objective: To determine whether prognostic indicators for hearing preservation could be identified in patients with vestibular schwannoma undergoing middle fossa craniotomy resection. Study Design: Prospective case review. Setting: Private practice tertiary referral center. Patients: 333 patients with serviceable hearing and vestibular schwannoma resected by middle fossa craniotomy from 1992 to 1998. Main Outcome Measures: Potential prognostic indicators, including tumor size and nerve of origin, preoperative pure-tone average, speech discrimination, distortion product otoacoustic emission testing, age, auditory brainstem response (ABR), and electronystagmography. Results: Postoperative hearing near preoperative levels was attained in 167 patients (50%), with an American Academy of Otolaryngology-Head and Neck Surgery Class A hearing result in 33% and a Class B result in 26%. Comparison of potential prognostic indicators between groups with hearing preserved and the group with no measurable hearing revealed significant differences in preoperative hearing, ABR, and tumor origin data. Better preoperative hearing, shorter intraaural wave V latency, shorter absolute wave V latency, and superior vestibular nerve origin were associated with higher rates of hearing preservation. Conclusions: Preoperative hearing status, ABR, and intraoperative tumor origin data were shown to be of value as prognostic indicators.

Prognostic factors for hearing preservation in vestibular schwannoma surgery.

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 intrasurgical 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 multidisciplinary team is essential for successful application of an auditory brainstem implant.

Auditory Brainstem Implant: I. Issues in Surgical Implantation:

The auditory brainstem Implant (ABI) restores some hearing sensations to patients deafened by bilateral acoustic tumors. Electrodes are stable for more than 10 years. In most cases nonaudltory side...

Auditory brainstem implant: II. Postsurgical issues and performance.

HYPOTHESIS The aim of this study was to show that a new auditory brain stem response (ABR) measure, the stacked derived-band ABR amplitude, can detect small acoustic intracanalicular tumors missed by standard ABR measures. BACKGROUND Recent studies clearly have shown that standard ABR latency measures have inadequate sensitivity to detection of small intracanalicular acoustic tumors. Consequently, despite its relatively low cost and wide availability, the standard ABR test has been replaced as a diagnostic screening tool by magnetic resonance imaging (MRI) with gadolinium (GdDTPA) contrast. However, screening with MRIs can be problematic because of their high cost, limited availability, and impact on patient comfort. Thus, an ABR method capable of detecting small tumors with good specificity would be an invaluable cost-effective screening tool for reducing the number of patients without tumor imaged. METHODS Derived-band ABRs were obtained to 63-dB normal hearing level (nHL) clicks using high-pass noise-masking procedures. The new measure is the wave V amplitude of a stacked ABR constructed by temporally aligning wave V of each derived-band ABR and then summing the time-shifted responses. RESULTS In a series of 25 tumor cases, 5 small (< or = 1 cm) intracanalicular tumors, missed by standard ABR latency measures, were detected by this new stacked ABR method. The stacked wave V ABR amplitudes in all five cases were significantly lower than those obtained in a group of normal-hearing individuals without tumors. CONCLUSIONS A new ABR measure, the stacked derived-band ABR amplitude, is sensitive to the presence of small intracanalicular tumors in patients and has excellent specificity for the absence of tumors in normal-hearing individuals. This method, in combination with standard ABR measures, appears promising both as a cost-effective approach to reducing the number of patients without tumors imaged and as a method for acoustic tumor screening when MRI scans: 1) are unavailable; 2) are not appropriate because of patient comfort; and 3) need to be justified because of their cost.

Successful detection of small acoustic tumors using the stacked derived-band auditory brain stem response amplitude

Because our recent clinical experiences suggested a possible association between hearing loss and systemic lupus erythematosus (SLE), we prospectively studied the hearing of 30 patients hospitalized because of exacerbation of SLE Twenty-nine of the 30 patients were receiving immunosuppressive therapy at the time of testing We found an 8% incidence of substantial, previously undetected hearing loss without attributable cause Hearing loss could not be correlated to age, sex, disease activity, organ-system involvement, laboratory test abnormalities, or duration of symptoms of SLE Otolaryngologists treating patients with unexplainable hearing loss--particularly if it is sudden, fluctuating, or rapidly progressive--are alerted to the possibility of underlying systemic autoimmunity We advise physicians who frequently treat patients with SLE to include questions on hearing in the review of systems and to refer the patient with hearing complaints for thorough otologic evaluation

Ralph A. Nelson

Papers

Prognostic factors for hearing preservation in vestibular schwannoma surgery.

Auditory Brainstem Implant: I. Issues in Surgical Implantation:

Auditory brainstem implant: II. Postsurgical issues and performance.

Successful detection of small acoustic tumors using the stacked derived-band auditory brain stem response amplitude

Sensorineural Hearing Loss Associated with Systemic Lupus Erythematosus