Management of far advanced otosclerosis in the era of cochlear implantation.

OBJECTIVE: To evaluate issues pertaining to cochlear implantation in patients with far advanced cochlear otosclerosis. STUDY DESIGN: Prospective cohort. SETTING: Tertiary care referral center. PATIENTS: Eight adult patients (18 years of age or older) referred for management of profound hearing loss, the cause of which was determined to be otosclerosis. INTERVENTION: Cochlear implantation with multichannel cochlear implant device. MAIN OUTCOME MEASURES: Benefit from cochlear implant as measured by CID sentence scores, incidence and management of facial nerve stimulation, and technical issues pertaining to cochlear implantation in this patient population. RESULTS: All patients demonstrated significant improvement in auditory function as measured by performance on CID sentence scores and ability to engage in telephone conversation. Facial nerve stimulation was present in two of eight patients and was managed with deactivation of the stimulating electrodes. Ossification in the basal turn of the cochlea, detected on preoperative computed tomography, necessitated placement of the electrode into the scala vestibuli in two patients and use of a thinner electrode (Nucleus 24) in a third patient. CONCLUSION: Patients with profound hearing loss secondary to otosclerosis derive excellent benefits from cochlear implantation. Surgical implantation may be complicated by ossification of the cochlea, which can be detected on preoperative computed tomography. Electrode activation may be complicated by facial nerve stimulation, which can be addressed with programming strategies.

Tinnitus suppression in patients with cochlear implants.

OBJECTIVE: To determine the degree of tinnitus suppression provided by currently available multichannel cochlear implants and to determine factors that can influence this process. STUDY DESIGN: Prospective cohort. SETTING: Tertiary-care referral center. PATIENTS: Thirty-eight adult patients (18 years of age or older) with severe-to-profound hearing loss and tinnitus who met criteria for cochlear implantation. INTERVENTION: Cochlear implantation with a multichannel cochlear implant device. MAIN OUTCOME MEASURES: Patients rated the intensity of their tinnitus using a semiquantitative scale before and after cochlear implantation. These data were analyzed to determine the significance of the reduction of tinnitus after implantation. Tinnitus levels after implantation were also analyzed to determine whether the level of speech recognition, patient gender, or the implant type influenced the degree of tinnitus reduction. RESULTS: Statistical analysis revealed a significant reduction in tinnitus intensity in patients using cochlear implants, with 35 of 38 patients (92%) experiencing a reduction in tinnitus intensity. All multichannel implants studied afforded similar degrees of tinnitus suppression. The degree of tinnitus reduction was not correlated with speech recognition, as measured by CID Everyday Sentence scores. Female patients had significantly greater degrees of tinnitus before implantation, but both male and female patients demonstrated similar levels of tinnitus after implantation. No patient experienced greater levels of tinnitus after implantation. CONCLUSION: All currently available multichannel cochlear implant devices provide effective and similar levels of tinnitus suppression when activated. Exacerbation of tinnitus as a result of cochlear implantation does not represent a significant risk. The mechanisms by which cochlear implants exert tinnitus suppression are, as yet, unclear.

Prevalence of unsuspected acoustic neuroma found by magnetic resonance imaging.

OBJECTIVES: Acoustic neuromas (ANs) comprise 6% of intracranial tumors. Population and autopsy studies have widely divergent estimates of AN incidence. With widespread use of MRI, asymptomatic ANs will be identified, which should improve estimates of the prevalence of this tumor. METHODS: The reports of all brain MRI scans during a 5-year period were retrospectively searched for the diagnosis of AN. MRIs obtained because of a suspicion of AN were discarded, leaving only the unsuspected ANs. RESULTS: A total of 24, 246 MRI studies were performed during this time period. Seventeen patients had unsuspected ANs. Eight tumors were smaller than 1 cm, 6 were between 1 and 2 cm, and 3 were 2 cm or larger. For all MRI scans, we found 7.0 unsuspected ANs per 10,000 brain MRI studies (0. 07%). CONCLUSION: The true prevalence of AN is likely greater than the 10 per million per year previously reported. This implies that there may be a larger number of asymptomatic ANs than previously suspected.

Extensive intracranial xanthoma associated with type II hyperlipidemia.

Xanthomas are associated with a spectrum of medical conditions, most commonly disorders of lipid storage and lipid metabolism. They occur primarily in the subcutaneous tissues, especially along the Achilles tendon and the extensor tendons of the hands. Intracranial xanthomas are extremely rare. We present a case of an extensive xanthoma of the temporal bone in a patient with hyperlipidemia.

Sound- and pressure-induced vertigo associated with dehiscence of the roof of the superior semicircular canal.

In many types of peripheral vertigo, imaging is not part of the initial evaluation. We present a patient with sound- and pressure-induced vertigo associated with bony dehiscence of the roof of the superior semicircular canal. The diagnosis of this new entity can only be made by high-resolution coronal CT imaging of the temporal bones. In patients with this symptom complex, CT should be performed early in the diagnostic workup.

Treatment of malignant neoplasms of the lateral cranial base with the combined frontotemporal-anterolateral approach: five-year follow-up.

Technical advances in accessing the lateral cranial base have permitted disease in this area previously deemed inoperable to be resected. The procedures required to effect an oncologically adequate resection are often long and accompanied by the potential for serious, even life-threatening, complications. Although it has been demonstrated that such disease can be extirpated, the question of whether such heroic surgery improves long-term survival remains unanswered. We retrospectively reviewed the records of 25 patients who underwent a combination of frontotemporal craniotomy with other, more conventional, anterolateral procedures (eg, infratemporal fossa approach, maxillectomy, orbitectomy, mandibulopharyngectomy) to resect stage IV malignant disease of the lateral to midcranial base between 1983 and 1990. Perioperative deaths occurred in 2 patients, 1 patient died of unrelated causes free of disease, and 2 patients were lost to follow-up, leaving 20 patients with a minimum 5-year evaluation. Five (25%) of the 20 patients we monitored were free of disease. Of those patients in whom recurrent disease developed, local control was achieved in about 50%; however in 80% of those with recurrence, metastatic disease developed. Surgical treatment of selected stage IV malignant disease of the lateral to midcranial base appears to have provided long-term disease-free survival to 25% of patients in this series who would otherwise have had little hope of survival.

Effect of healed tympanic membrane perforations on umbo velocity in the rat.

Heterodyne laser interferometry was used to measure tympanic membrane (TM) velocity at the umbo during acoustic stimulation in 2 groups of rats. The control group had a normal TM, while in the other group the TM was perforated and then allowed to heal for at least 28 days. Umbo velocity functions for constant sound pressure level stimuli were obtained for test tones between 0.3 and 40.0 kHz in each animal. The results revealed that velocity was the same in the control and healed TMs below 3.0 kHz. Above 5.0 kHz, the velocity response in the healed ear was between 3 and 12 dB smaller than in the control ears. Histologic evaluation of the healed perforation revealed a thick fibrous reaction between the epidermal and lamina propria layers. The results indicated that the added mass of the scar tissue changed the middle- and high-frequency TM responses.

Lipomas of the internal auditory canal and cerebellopontine angle.

OBJECTIVE: To evaluate lipomas of the internal auditory canal (IAC) and cerebellopontine angle (CPA). STUDY DESIGN: Retrospective review. METHODS: Review of a multi-institutional series of 17 lipomas of the IAC/CPA, combined with a Medline review of the 67 cases reported in the world literature. RESULTS: This series of 17 IAC/CPA lipomas is the largest reported series to date, bringing the total number of documented cases to 84. There appears to be a nearly 2:1 male to female predominance. Sixty percent were left-sided lesions, and three were bilateral. Hearing loss, dizziness, and tinnitus were the most common presenting symptoms. Surgical resection was performed in 52 (62%) of these lesions; however, total tumor removal was accomplished in only 17 (33%), which is most likely because of the fact that these tumors tend to have a poorly defined matrix and a dense adherence to neurovascular structures. Sixty-eight percent of patients experienced a new deficit postoperatively, 11% were unchanged, and only 19% improved with no new deficit. Only one documented case of tumor growth was identified; however, the reported follow-up was short (average, less than 3 years). CONCLUSION: With the magnetic resonance imaging techniques now available, lipomas can be reliably differentiated from other masses within the CPA and IAC, so histopathologic diagnosis is rarely necessary. Because of the potential for significant morbidity with resection of these lesions, we believe that conservative follow-up is the best treatment option for patients with these rare lesions. Surgery is indicated only when significant progressive or disabling symptoms are present.

Otolaryngological manifestations of Creutzfeldt-Jakob disease.

Creutzfeldt-Jakob disease (CJD) is one of the uniformly fatal spongiform encephalopathies that is characterized clinically by an unrelenting progression of myoclonus, dementia, and ataxia. Since many of these patients will develop cerebellar abnormalities, some may present to the otolaryngologist with dizziness. Hearing loss, however, to our knowledge, has not been reported. We describe a patient with CJD who presented with hearing loss and vague symptoms of imbalance, and whose disease progressed rapidly and fatally despite an extensive initial workup that was otherwise unrevealing. A review and discussion of the otolaryngological manifestations of CJD is presented. The otolaryngologist should be aware that CJD can present with otolaryngological manifestations, and with proper diagnosis extensive workups may be avoided.

Facial nerve stimulation from cochlear implants.

OBJECTIVE: To evaluate the incidence of facial nerve stimulation from cochlear implants and to better define the segment of nerve being stimulated and the causes of stimulation. STUDY DESIGN: Retrospective patient case review and a temporal bone dissection study. SETTING: A tertiary care setting. PATIENTS: All patients given a cochlear implant at the Hospital of the University of Pennsylvania. This encompassed only adult patients. INTERVENTION: All patients had surgical insertion of either a 3M single channel, Nucleus 22-channel, or CLARION multichannel cochlear implant. MAIN OUTCOME MEASURES: Demonstration of facial nerve stimulation with a cochlear implant and determination of affected electrodes; measurement of electrode location and distances between the labyrinthine segment of the facial nerve and the cochlea in temporal bone dissections: and determination of the relationship between the labyrinthine facial nerve and the cochlea using computed tomography evaluation. RESULTS: The overall incidence of facial nerve stimulation using all three devices was 14% (8 of 58). Otosclerosis and otosyphilis appear to be predisposing conditions to stimulation. The mid-cochlear electrodes, located near the labyrinthine facial nerve, appear to cause stimulation of the VIIth nerve most commonly. Computed tomographic evaluation of the bone between the labyrinthine fallopian canal and the cochlea may provide some indication of potential facial nerve problems. CONCLUSION: Facial nerve stimulation from the use of cochlear implants is more prevalent in patients with otosclerosis and otosyphilis. The labyrinthine segment of the facial nerve is the most likely area being stimulated in most patients. Preoperative computed tomographic evaluation may be beneficial in determining the possibility of this problem.

Brainstem origins of the n18 component of the somatosensory evoked response.

Proposed generator sites for the N18 component of the somatosensory evoked potential (SEP) range in location from the medulla to the thalamus. Additional knowledge regarding the generators of the N18 will be important in interpreting the results of intra-operative monitoring during skull base surgery and providing the surgeon more specific information. The goal of this study was to use both intracranial electrical recording and the effects of acute brainstem ischemia in humans to further define the generators of N18. Monopolar electrodes were used to record SEP (after median nerve stimulation) from the brainstem surface in eight patients undergoing posterior fossa surgical procedures. Recordings were made from various locations, from the cervico-medullary junction to the level of the aqueduct of Sylvius. As the electrode moved rostrally on the brainstem surface, the difference in latencies between the scalp N18 potential and the electrode potential approached zero, suggesting an upper pontine-lower midbrain origin of the N18 potential. These findings were supported by the lack of change in the N18 potentials of ten patients with basilar tip aneurysms who experienced marked changes of their N20/P22 potentials during temporary occlusion of the distal basilar artery.

Pleomorphic adenoma of the parotid.

Pleomorphic adenoma of the parotid is the most common tumor of salivary gland origin, accounting for 60 to 70 percent of all benign salivary gland tumors. This lesion usually presents as a slow-growing painless mass inferior to the pinna of the ear. The diagnosis is based on clinical presentation, magnetic resonance imaging or computed tomography, and fine-needle aspiration biopsy. The treatment is wide excision in which the entire capsule is removed but the facial nerve is spared. Proper diagnosis and treatment are necessary to prevent the complications of tumor recurrence and malignant transformation. Carcinoma expleomorphic adenoma arises in longstanding tumors and has a five-year recurrence rate of 75 percent.

Use of preoperative MR to predict dural, perineural, and venous sinus invasion of skull base tumors.

PURPOSE: To assess the accuracy of MR imaging in predicting dural, venous sinus, and perineural invasion by skull base tumors. METHODS: The preoperative MR images of 22 patients who had resection of skull base neoplasms were evaluated for the following characteristics: dural enhancement, pial enhancement, local perineural invasion by adjacent tumor, and venous sinus invasion by tumor. The greatest width of dural enhancement was measured, and the character of dural enhancement was noted. The pathologic and surgical reports were reviewed retrospectively with specific attention to dural, venous, and local perineural invasion. RESULTS: Of the 22 patients studied, dural invasion by tumor was confirmed in eight patients, vascular invasion in six patients, and perineural invasion in four patients. The sensitivity of dural enhancement in predicting invasion was 88%, the specificity 50%, and the accuracy 64%. When enhancement and focal nodularity were present, the sensitivity remained at 88%; however, specificity was 100% and accuracy 95%. If the dural enhancement was more than 5 mm thick, sensitivity, specificity, and accuracy were 75%, 100%, and 91%, respectively. Predicting tumor invasion of the dura by the presence of pial enhancement was 50% sensitive and 100% specific. Venous sinus/jugular vein invasion was predicted with 100% sensitivity, 94% specificity, and 95% accuracy. Local perineural invasion was predicted with 100% sensitivity, 50% specificity, and 59% accuracy. CONCLUSIONS: The presence of pial enhancement, focal dural nodules, or dural thickening of more than 5 mm is highly accurate in predicting the presence of neoplastic dural invasion. Linear enhancement of dura does not imply dural infiltration by tumor. Venous invasion by tumor can be predicted accurately with preoperative MR imaging.

The effect of tympanic membrane perforation size on umbo velocity in the rat.

The tympanic membrane (TM) in adult rats was surgically exposed and laser interferometry was used to measure TM velocity at the umbo for frequencies between 1.0 and 40.0 kHz. Velocity measures were obtained for five conditions: TM intact, and four progressively larger holes cut into the posterior region of the membrane. Photomicrographs of each condition were used to calculate the percentage of pars tensa lost to the perforation. The relation between TM velocity and stimulus sound pressure level (SPL) was also examined for each of the conditions. The results revealed a systematic loss in low-frequency velocity as perforation size increased. These observations were consistent with clinical reports of low-frequency hearing loss in the perforated human TM. The rat appears to be a successful model for studying this form of conductive pathology.

Surgical management of transcranial hypoglossal schwannomas.

Hypoglossal schwannomas are rare cranial base neoplasms that originate in the posterior cranial fossa and extend through the hypoglossal canal in a dumbell fashion. Recent experience with this unusual neoplasm prompted a search for an operative approach to remove the lesion with a minimization of postoperative morbidity from bleeding and iatrogenic cranial nerve deficits. This report describes the use of the transcondylar approach with monitoring of the lower basal cranial nerves to totally remove a transcranial hypoglossal schwannoma with no new-onset cranial nerve palsies. The regional anatomy of the hypoglossal nerve and canal is reviewed in relation to the operative approach.

Carotid artery invasion by head and neck masses: prediction with MR imaging.

PURPOSE: To determine the value of magnetic resonance (MR) imaging in predicting resectability of head and neck neoplasms around the carotid arteries. MATERIALS AND METHODS: Forty-nine patients (28 male patients and 21 female patients aged 17-79 years; mean, 57.3 years) with head and neck masses and clinical evidence of carotid wall invasion underwent MR imaging. T1-weighted, T2-weighted, and gadolinium-enhanced T1-weighted images were analyzed to determine circumferential involvement of 53 arteries by tumor. RESULTS: More than 270 degrees of circumferential involvement was considered suggestive of unresectability of the malignant neoplasm; 270 degrees or less was considered lack of invasion. The sensitivity of MR imaging for determination of unresectable disease was 100% (12 of 12 cases), specificity was 88% (36 of 41), and accuracy was 91% (48 of 53). Accuracy was 100% for squamous cell carcinoma (n = 29). CONCLUSION: Tumor that encompasses more than 270 degrees of the carotid artery probably cannot be removed from the artery. Tumor that involves 270 degrees or less of the artery can be removed.