OBJECTIVE: Pulsatile tinnitus is characterized by hearing the heart beat or respiration in one or both ears. In 15% of patients with pulsatile tinnitus, no cause can be found. Other investigators have suggested that a vascular loop entering the internal auditory meatus can be another cause of arterial, pulse synchronous tinnitus. If so, we should constantly hear the arterial pulsations of the carotid arteries passing through the petrous bone. METHODS: Using magnetic resonance imaging, 17 patients with unilateral pulsatile tinnitus and 46 with non-pulsatile tinnitus were analyzed for the presence of a vascular loop entering into the internal acoustic meatus. Four temporal bones were sectioned to find structural differences between the internal acoustic meatus and the pericarotid area. Four patients with intrameatal vascular loops and ipsilateral pulsatile tinnitus underwent surgery by Teflon interpositioning between the loop and the cochlea. RESULTS: In unilateral pulsatile tinnitus, a statistically highly significant amount of intrameatal vascular loops was noted in comparison to non-pulsatile tinnitus. A well-developed pericarotid venous plexus was found histologically. Three of the four patients who underwent surgery were initially tinnitus free, but pulsations recurred after 3 months in one patient. CONCLUSION: Vascular loops in the internal auditory canal may generate pulsatile tinnitus. It may be treated by placing Teflon between the cochlea and the intrameatal vascular loop. One then does not hear the pulsation of the carotids due to a dampening effect of a pericarotid venous plexus.

Objectives/Hypothesis: Compare vestibular schwannoma (VS) surgical outcome between patients with prior irradiation and those not previously treated.

Acoustic tumors (vestibular schwannomas) are the most common tumor of the cerebellopontine angle (CPA). Microsurgical resection is the traditional method of treatment, with complete tumor removal as the goal. The translabyrinthine approach is used in patients with poor hearing or no hearing and in any tumors larger than 2.5 cm. The middle fossa approach is used for tumors up to 2 cm in size with serviceable hearing. Conservative therapy or “observation” with serial imaging is often advocated for elderly patients with smaller tumors and without significant symptoms. A more recent treatment modality is stereotactic radiotherapy, with the goal of tumor control, defined as tumor regression or stable tumor size. Different forms of radiotherapy include radiosurgery, which uses a single-fraction dose of radiation, and fractionated radiotherapy, which divides doses to minimize damage to normal tissues. Regardless of method, treatment results for small acoustic tumors are generally good, and we typically recommend early intervention. Complete tumor removal has been demonstrated in 99% of patients undergoing microsurgical resection for small tumors, with tumor recurrence rates over all tumor sizes very low (<0.3%). Most reports show good facial nerve function after treatment in >90% of patients with small tumors. Hearing preservation at serviceable levels in patients with preoperative hearing is reported at about 60% in patients with small tumors undergoing middle fossa microsurgical removal. Cranial nerve dysfunction is often delayed following radiotherapy, so tumor control rates and nerve function results with the current generation of lower dose radiotherapy procedures awaits further study.

OBJECTIVE:To assess the effects of the side of implantation (first-side vs second-side vestibular schwannoma); the presence of nonauditory sensations; the general health, expectations, and motivation of the patients; and a support group on the use of a multichannel auditory brainstem implant (ABI) in 12- to 18-year-old patients with neurofibromatosis 2.DESIGN:Since 1992, 21 individuals (age range, 12-18 years) who were deafened by neurofibromatosis 2 have undergone implantation with a multichannel ABI at the House Ear Institute, Los Angeles, Calif. The patients were categorized regarding side of implantation, presence of remaining hearing (in first-side implant recipients), incidence of nonauditory sensations, and ABI use or nonuse. They were also rated on factors of general health, personal motivation, expectations, and family support.RESULTS:Nineteen (95%) of 20 teenagers tested received hearing sensations from their ABIs. Eleven teenagers used their ABIs regularly, but 8 did not. Of the nonusers, 2 had good remaining hearing on the side with the second vestibular schwannoma, 2 had persistent nonauditory sensations, and 4 became program dropouts. None of the dropouts had remaining hearing, significant nonauditory sensations, or poor health; however, they generally rated poorly in terms of personal motivation, expectations, and family support. One patient with good family support returned with excellent ABI results after 4 years' absence.CONCLUSIONS:The multichannel ABI is an effective means of providing hearing sensations to young patients deafened by neurofibromatosis 2. Preoperative counseling regarding the importance of such factors as expectations, personal motivation, and family support is invaluable and can promote successful adaptation to the device. With patience and support, even young nonusers (including program dropouts) can become successful device users.

OBJECTIVE: Meningiomas arising primarily within the internal auditory canal (IAC) are notably rare. By far the most common tumors that are encountered in this region are neuromas. We report a series of eight patients with meningiomas of the IAC, analyzing the clinical presentations, surgical management strategies, and clinical outcomes. METHODS: The charts of the patients, including histories and audiograms, imaging studies, surgical records, discharge letters, histological records, and follow-up records, were reviewed. RESULTS: One thousand eight hundred meningiomas were operated on between 1978 and 2002 at the Neurosurgical Department of Nordstadt Hospital. Among them, there were 421 cerebellopontine angle meningiomas; 7 of these (1.7% of cerebellopontine angle meningiomas) were limited to the IAC. One additional patient underwent surgery at the Neurosurgical Department of the International Neuroscience Institute, where a total of 21 cerebellopontine angle meningiomas were treated surgically from 2001 to 2003. As a comparison, the incidence of intrameatal vestibular schwannomas during the same period, 1978 to 2002, was 168 of 2400 (7%). There were five women and three men, and the mean age was 49.3 years (range, 27–59 yr). Most patients had signs and symptoms of vestibulocochlear nerve disturbance at presentation. One patient had sought treatment previously for total hearing loss before surgery. No patient had a facial paresis at presentation. The neuroradiological workup revealed a homogeneously contrast-enhancing tumor on magnetic resonance imaging in all patients with hypointense or isointense signal intensity on T1- and T2-weighted images. Some intrameatal meningiomas showed broad attachment, and some showed a dural tail at the porus. In all patients, the tumor was removed through the lateral suboccipital retrosigmoid approach with drilling of the posterior wall of the IAC. Total removal was achieved in all cases. Severe infiltration of the facial and vestibulocochlear nerve was encountered in two patients. There was no operative mortality. Hearing was preserved in five of seven patients; one patient was deaf before surgery. Postoperative facial weakness was encountered temporarily in one patient. CONCLUSION: Although intrameatal meningiomas are quite rare, they must be considered in the differential diagnosis of intrameatal mass lesions. The clinical symptoms are very similar to those of vestibular schwannomas. A radiological differentiation from vestibular schwannomas is not always possible. Surgical removal of intrameatal meningiomas should aim at wide excision, including involved dura and bone, to prevent recurrences. The variation in the anatomy of the faciocochlear nerve bundle in relation to the tumor has to be kept in mind, and preservation of these structures should be the goal in every case.

A retrospective clinical analysis was performed to evaluate the effectiveness of the preauricular infratemporal fossa (ITF) surgical approach using modifications based on tumor pathology and extension, without compromising outcomes. Patients were surgically treated for tumors involving the ITF via a preauricular surgical approach during 1990 to 2000. Their clinical charts were reviewed to determine the association among pathological variables, details of the surgical procedure, and outcomes. Tumors in 65 patients were categorized as "malignant" and "benign." The malignant group included 44 patients (mean age, 49.5 years). Squamous cell carcinoma was the most common pathology followed by sarcomas. To achieve complete tumor resection, the ITF approach and dissection were combined with other procedures in 74% of these patients. No surgical complications were encountered in 74.4%, and a clinical cure was obtained in 55% of patients (follow-up, 2 years). The benign group included 21 patients (mean age, 36.7 years). Juvenile angiofibromas and meningiomas constituted most of the tumors in this group. An ITF approach alone was sufficient to achieve complete tumor excision in 66.7% of these patients. A clinical cure was achieved in 85% of patients (follow-up, 2 years), and 76.2% had no surgical complications. Chi-square tests revealed significant correlations between tumor extensions and surgical treatment variables. These were more evident in the malignant group, indicating the use of wider surgical exposures and more aggressive, extirpative surgery. The preauricular surgical approach to the ITF can be used to achieve a complete resection of a variety of tumors arising from or extending into the ITF. This approach can be tailored to the nature of the tumor and its extensions.

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.

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.

Context.—Lipochoristomas (lipomatous choristomas) are rare tumors of the acoustic nerve (cranial nerve VIII/vestibulocochlear nerve) within the internal acoustic canal and sometimes the cerebellopontine angle, and are histogenetically believed to be congenital malformations. Their clinically indolent behavior has recently prompted a more conservative management protocol in a quest for maximal nerve/hearing preservation. This approach contrasts sharply with that for the common internal acoustic canal/cerebellopontine angle tumors, the neuroepithelial neoplasms (acoustic schwannomas and meningiomas), which behave more aggressively and have more prominent clinical manifestations. Owing to their rarity, the clinicopathologic features of cranial nerve VIII lipochoristomas have been obtained mainly through case reports. Objective.—We present the clinicopathologic features of 11 cases of lipochoristomas of cranial nerve VIII. Design.—The 11 cases were documented between 1992 and 2003. We performed complete clinical reviews with histologic, histochemical, and immunohistochemical analyses of formalin-fixed, paraffin-embedded tumor samples. Results.—The patients were 8 men and 3 women with hearing loss of the right ear (5 patients) or the left ear (6 patients). No patient had bilateral tumors. All lipochoristomas histologically possessed mature adipose tissue admixed with varied amounts of mature fibrous tissue, tortuous thick-walled vessels, smooth muscle bundles, and skeletal muscle fibers, the latter verified with immunohistochemistry. Conclusions.—The histomorphologic and immunophenotypic evidence showed that these tumors are better characterized as choristomas than as simple “lipomas,” as they have been labeled in the past. Their overall nonaggressive clinical nature in addition to the characteristic radiologic and histomorphologic findings are important clinicopathologic features for the pathologist to recognize and differentiate, especially during frozen section evaluations, in order to direct the neurosurgeon to a more appropriate conservative therapeutic intervention.

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.

Background: Paragangliomas are rare and highly heritable tumours of neuroectodermal origin that often develop in the head and neck region. Germline mutations in the mitochondrial complex II genes, SDHB, SDHC, and SDHD, cause hereditary paraganglioma (PGL). Methods: We assessed the frequency of SDHB, SDHC, and SDHD gene mutations by PCR amplification and sequencing in a set of head and neck paraganglioma patients who were previously managed in two otolaryngology clinics in the USA. Results: Fifty-five subjects were grouped into 10 families and 37 non-familial cases. Five of the non-familial cases had multiple tumours. Germline SDHD mutations were identified in five of 10 (50%) familial and two of 37 (∼5%) non-familial cases. R38X, P81L, H102L, Q109X, and L128fsX134 mutations were identified in the familial cases and P81L was identified in the non-familial cases. Both non-familial cases had multiple tumours. P81L and R38X mutations have previously been reported in other PGL families and P81L was suggested as a founder mutation. Allelic analyses of different chromosomes carrying these mutations did not show common disease haplotypes, strongly suggesting that R38X and P81L are potentially recurrent mutations. Germline SDHB mutations were identified in two of 10 (20%) familial and one of 33 (∼3%) non-familial cases. P131R and M71fsX80 were identified in the familial cases and Q59X was identified in the one non-familial case. The non-familial case had a solitary tumour. No mutations could be identified in the SDHC gene in the remaining four families and 20 sporadic cases. Conclusions: Mutations in SDHD are the leading cause of head and neck paragangliomas in this clinic patient series. SDHD and SDHB mutations account for 70% of familial cases and ∼8% of non-familial cases. These results also suggest that the commonness of the SDHD P81L mutation in North America is the result of both a founder effect and recurrent mutations.

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.

The case of a 55-year-old woman with a middle ear mass is presented. The preoperative diagnostic workup, including an audiogram and imaging studies, and the histopathologic findings of the tumor are reviewed. The tumor, a schwannoma, arose from Jacobson's nerve in the middle ear. The surgical anatomy of Jacobson's nerve and the surgical approach to this tumor and to other tumors of the middle ear space are discussed. Tumors of the tympanic cavity are rare, with the exception of cholesteatoma; otherwise, the most common among them are paraganglioma and facial nerve neuroma. This report represents the first documented case of a schannoma arising from Jacobson's nerve in the tympanic cavity.

OBJECTIVE/HYPOTHESIS:To determine the heritable proportion of paraganglioma (PGL) and identify clinical features associated with heritable PGL. STUDY DESIGN:Patients diagnosed with head and neck PGLs, identified retrospectively through clinical otolaryngology practices and/or participation in previous PGL research studies, were given a medical and family history questionnaire. METHODS:Questionnaire information was used to classify participants as having "heritable" or "non-heritable" cases of PGL. Classification of the participants identified through otolaryngology clinics was used to estimate the heritable proportion of PGL. Statistical analysis was performed to identify significant differences in the clinical characteristics of the heritable versus non-heritable groups. RESULTS:Among the otolaryngology clinic population, 35% were classified as having heritable PGL. Individuals with heritable PGL were younger on average than those with non-heritable PGL. The majority of non-heritable participants were female, but there was an equal gender ratio among the heritable participants. Individuals diagnosed with a carotid body tumor (CBT) were 5.8 times more likely to be classified as heritable than those diagnosed with PGL at other anatomic locations. CONCLUSIONS:Approximately 35% of individuals who present to an otolaryngologist with a head and neck PGL have inherited a predisposition for this growth. Among individuals diagnosed with head and neck PGL, those diagnosed with CBT are 5.8 times more likely to have an inherited predisposition than those diagnosed with PGL at other anatomic locations.

Augmented or turbulent flow within the transverse-sigmoid-jugular venous system is one of the more common causes of pulsatile tinnitus. Characteristically, the pulsation can be temporarily muted or even abolished by relatively gentle compression of the ipsilateral neck. As patients may be quite disturbed by this symptom, clinicians understandably are tempted to bring relief through ligation of the jugular vein in the neck and/or compression of the sigmoid sinus and jugular bulb within the temporal bone. The purpose of this communication is to warn surgeons against undertaking this measure because it risks serious neurologic injury. As a group, we have encountered series of patients in whom venous ligation has triggered florid pseudotumor cerebri accompanied by severe headaches, progressive visual loss, and other disturbing neurologic symptoms. Noisy flow originating from the jugulo-sigmoid system venous system usually indicates either stenosis with downstream turbulence or augmented flow through a dominant system. The latter may occur due to stenosis or atresia of the contralateral system. The flow through the noisy side can be essential to maintaining physiological intracranial hemodynamics. Ill-advised interruption can trigger serious intracranial venous hypertension. Closure at the sigmoid sinus level or jugular bulb is especially perilous because it eliminates the alternative pathway via the petrosal sinuses. While we would have preferred to present a formal case series, these patients were not under our direct care but rather came to our attention through the medical–legal process. In our opinion, there is rarely, if ever, an indication for interruption of a major venous pathway solely for the purpose of alleviating pulsatile tinnitus. Robert K. Jackler, M.D. Derald E. Brackmann, M.D. Aristides Sismanis, M.D.

OBJECTIVE: We evaluated hearing outcomes in patients with sudden hearing loss and vestibular schwannoma who underwent a hearing preservation operation for tumor resection in an effort to determine whether a history of sudden sensorineural hearing loss has an impact on subsequent hearing preservation surgery. METHODS: Retrospective chart review of 45 patients operated between 1990 and 1998. Patients were divided into “Recovery” (n = 22) and “No Recovery” (n = 23) groups based on preoperative hearing recovery. Hearing preservation was assessed using the AAO-HNS hearing classification system. RESULTS: Measurable hearing was preserved in 73% of patients, with 47% having good postoperative hearing (AAO-HNS Classes A-B). There was no significant difference in hearing outcome from patients presenting with progressive hearing loss (45% Classes A-B). There was also no difference in postoperative hearing between the “Recovery” and “No Recovery” groups. CONCLUSIONS: Patients with sudden hearing loss and vestibular schwannoma have the same chance of hearing preservation after tumor removal as those with progressive loss. Preoperative recovery of hearing is not predictive of hearing preservation. Available data support the nerve compression theory as the mechanism of sudden hearing loss in patients with vestibular schwannoma.

The multichannel auditory brainstem implant (ABI) has been used successfully to treat deafness in individuals with neurofibromatosis type II. The device has been implanted in nearly 150 recipients worldwide, and clinical trials with the device are approaching completion. The implantation and fitting of the multichannel ABI differ significantly from cochlear implantation, and the processes are illustrated in a series of case studies. Performance data also are included from recipients with up to 7 years experience.

Objective: To review characteristics of and outcome in patients undergoing microvascular decompression of the vestibulocochlear nerve. Patients studied had a diagnosis of disabling positional vertigo caused by a vascular loop compressing the VIIIth cranial nerve.Study Design: Retrospective chart review and telephone interview.Setting: Private practice tertiary neurotologic referral center.Patients: Twenty patients with disabling positional vertigo underwent 25 retrosigmoid craniotomies for microvascular decompression between November 1990 and June 1999. The 4 men and 16 women ranged in age from 30 to 71 years (mean age, 46 yr).Main Outcome Measures: Charts were reviewed and patients were contacted by telephone and asked to rate severity of symptoms (tinnitus and dizziness) on a 4-point scale (none = 1, mild = 2, moderate = 3, and severe = 4) before and after surgery. They were also asked to rate their overall disability from their symptoms on the six-point scale established by the American Academy of Otolaryngology-Head and Neck Surgery. Preoperative and postoperative four-frequency (500 Hz, 1 kHz, 2 kHz, and 4 kHz) pure-tone average and speech discrimination scores were calculated and compared. Complications of surgery are also reported.Results: Postoperative tinnitus score and dizziness score showed significant improvement from preoperative scores (p less than or equal to 0.047 and p less than or equal to 0.001, respectively). with 80% of patients improved in dizziness rating: 85% improved in their overall disability rating, and the difference from preoperative to postoperative was significant (p less than or equal to 0.001). The mean postoperative pure-tone averages ( 15.4 dB) and speech discrimination scores (99%) did not differ from preoperative scores ( 11.9 dB and 98%). One patient lost all vestibular function in the operated ear (hearing remained intact) as the only complication of surgery. When asked, 83% of patients responded that they would have the surgery again.Conclusions: Diagnosing disabling positional vertigo secondary to vascular compression of the VIIIth cranial nerve remains the clinical challenged a clear history Plus air-contrast computed tomographic or magnetic resonance imaging make the diagnosis. Microvascular decompression of the vestibulocochlear nerve is a safe and effective operation for these carefully selected patients.

OBJECTIVETo define the indications for surgery in lesions of the internal auditory canal (IAC) and cerebellopontine angle (CPA) in an only hearing ear.STUDY DESIGNRetrospective case series.SETTINGTertiary referral center.PATIENTSSeven patients with lesions of the IAC and CPA who were deaf on the side opposite the lesion. Five patients had vestibular schwannoma (VS), and one each had meningioma and progressive osseous stenosis of the IAC, respectively. The opposite ear was deaf from three different causes: VS (neurofibromatosis type 2 [NF2]), sudden sensorineural hearing loss, idiopathic IAC stenosis.INTERVENTION(S)Middle fossa removal of VS in five, retrosigmoid resection of meningioma in one, and middle fossa IAC osseous decompression in one.MAIN OUTCOME MEASUREHearing as measured on pure-tone and speech audiometry.RESULTSPreoperative hearing was class A in four patients, class B in two, and class C in one. Postoperative hearing was class A in three patients, class B in one, class C in two, and class D in one.CONCLUSIONSAlthough the vast majority of neurotologic lesions in an only hearing ear are best managed nonoperatively, in highly selected cases surgical intervention is warranted. Surgical intervention should be considered when one or more of the following circumstances is present: (1) predicted natural history of the disease is relatively rapid loss of the remaining hearing, (2) substantial brainstem compression has evolved (e.g., large acoustic neuroma), and/or (3) operative intervention may result in improvement of hearing or carries relatively low risk of hearing loss (e.g., CPA meningioma).