Ultrastructural characteristics of human neurilemoma cell nuclei.

Nuclei of human neurilemoma cells exhibit deep and extensive invaginations of part of their surface. Such invaginations contain cytoplasmic matter. However, in areas of the nucleoplasm distant from the invaginations, small membrane-bound bodies, some of which contain a "nucleoid," occur either singly or grouped together and enclosed within a large membrane body. These small bodies are not considered virus-like. Degenerated nuclei from cultured tumor tissue contain spherical bodies, 130 to 230 nm in diameter, with spikes on their surface similar to those seen on envelopes of herpes-type viruses. Significance of these bodies in vivo and in vitro tumor tissues is not known.

Hypoglossal-facial nerve anastomosis: a review of forty cases caused by facial nerve injuries in the posterior fossa.

Hypoglossal-facial anastomosis has been our procedure of choice in the repair of the permanently injured facial nerve in the cerebellopontine cistern, when the nerve cannot be primarily repaired. Total failures are few and complications are rare. Most results are good to excellent, if assessment is based upon realistic expectations. These include: 1. normal facial symmetry in repose, 2. good midface voluntary motion, 3. no reflex or emotional facial movement, 4. some synkinesis and donor-injected mass facial movement, and 5. surprisingly little functional loss from hypoglossal paralysis. Our experience indicates better results in younger patients and in those repairs completed shortly after injury. These findings correlate well with the experience gained in peripheral nerve repair in the extremities. There appears to be no absolute time period between injury and repair beyond which this anastomosis is definitely contraindicated. Finally, this procedure does not negate adjunctive plastic surgical procedures. Most of our patients have had tarsorrhaphy or physiologic protection of the eye, but few have had corrective cosmetic surgical procedures until the past few years. We have never used cervical sympathectomy to reduce the size of the palpebral fissure. Better surgical procedures to correct both extracranial and intratemporal facial nerve injuries have significantly reduced the indication for anatomosis procedures. Additionally, over the past two decades, the improved diagnostic and surgical techniques for posterior fossa tumors have considerably reduced the incidence of facial paralysis. As these trends continue, the number of patients requiring nerve anastomosis for facial paralysis will continue to decline and what was once the only surgical procedure to repair the paralyzed face will become a rare operation.

A workable approach to the identification of neonatal hearing impairment.

The American Hearing Research Foundation has developed a regional Infant Screening Program in the state of Illinois, designed to meet the vital need for early identification of hearing impairment on a mass scale. The four major components of the program include: 1. Application of the High Risk Register to the general newborn population, 2. Hearing Screening in the Intensive Care Nursery, 3. Electrophysiologic confirmation with Brain Stem Electric Response testing and 4. Subsequent and immediate remediation and/or habilitation. Brain Stem Electric Response Audiometry (BERA) has been shown to be an accurate and sensitive clinical tool for obtaining auditory thresholds in infants from birth. At the Donald T. Forsythe Otologic Laboratory in Mercy Hospital, Chicago, IL, over 250 infants and young children have been tested in a controlled study with BERA to establish or rule-out the diagnosis of hearing impairment. The use of BERA preceded by screening of a refined neonatal population has proven to be an effective method of early identification. The goals of the program are: 1. To identify hearing impaired children prior to passage of the critical periods, 2. To medically or surgically classify and treat and/or auditorily habilitate, and 3. To ultimately improve the quality of life of the child born with hearing impairment.

The acoustic reflex latency test: clinical application.

Acoustic reflex latency is the time interval between onset of an intense auditory stimulus and onset of middle-ear muscle contraction. The hypothesis was proposed that a retrocochlear lesion involving the afferent (VIIIth nerve) portion of the reflex arc should result in a prolongation of the reflex latency. A clinical procedure, the Acoustic Reflex Latency Test (ARLT), its protocol, methods and equipment were developed in order to test this hypothesis clinically. Absolute latency values and interaural latency differences (ILD) were established on 4 populations which included normals, cochlear lesions due to Ménière's disease, cochlear lesions of variable etiology, and 11 cases of surgically confirmed acoustic tumor. Results reveal a dramatic prolongation of latency in the presence of retrocochlear lesions (all tumors). The test was found to be most effective when 1. both ipsilateral and contralateral measurements were made, 2. stimulus frequencies of 100 Hz and 2000 Hz were employed and 3. both Absolute Latency and ILD were considered. The ARLT, while utilizing an indirect method of measurement, is a valid, cost-effective, and simple clinical procedure, yielding relative latency values and requiring only minor modifications of standard impedance equipment. The ARLT appears to have a higher efficiency rate than BERA, while providing comparable diagnostic information at a considerable saving of time, cost and personnel.

Audiograms derived from the brain stem response.

Nineteen normal and 22 hearing impaired subjects were used in this study. Thresholds were determined by the following methods: 1. behavioral continuous tone, 2. behavioral tone burst, and 3. the brain stem electric response (BER) with tone bursts. A comparison of the BER thresholds with the behavioral thresholds are made for each subject. The difference between these three measures of threshold suggests that audiograms can be derived from the brain stem response threshold. The derived audiogram compares favorably with conventional audiograms. However, it must be remembered that the brain stem response is not hearing.

Temporal bone histopathology of Paget's disease with sensorineural hearing loss and narrowing of the internal auditory canal.

A temporal bone report is presented of a patient with progressive sensorineural hearing loss and advanced Paget's disease involving the skull. The histopathology reveals Pagetic bone invading the internal auditory canal and compressing the cochlear division of the VIIIth cranial nerve resulting in severe neural degeneration. There are no other histopathologic abnormalities present in the cochlea to explain the hearing loss. A low-frequency conductive hearing loss was also present, but no histopathologic correlate could be identified.

The large vestibular aqueduct syndrome.

The radiographic observation of 50 patients, each having an enlarged (greater than 1.5 mm. diameter) vestibular aqueduct, are analyzed. In addition to the aqueduct other associated inner ear anomalies have been identified in 60% of this population including: enlarged vestibule (14); enlarged vestibule and lateral semicircular canal (7); enlarged vestibule and hypoplastic cochlea (4); and hypoplastic cochlea (4). The large equeduct then presumably represents an arrested phase of inner ear development common to all 50 cases. Only 8 of these cases may fall into the Mondini or Mondini-Alexander classification wherein cochlear abnormalities have been identified. The size of the aqueduct ranged from 1.5 to 8 mm. in the anteroposterior diameter; the clinical incidence is 50 in 3700 consecutive cases referred for inner ear tomography. Bilateral involvement is twice as common as unilateral with a female to male predominance of 3:2. Most cases are associated with congenital hearing losses.

Normal opaque cerebellopontine cisternogram.

An attempt has been made through this study to establish a tumor suspect pool of 100 patients, each one highly suspect because of a single or usually several strong clinical indicators of a retrocochlear lesion. In turn each patient has been proven tumor negative by virtue of a normal opaque cerebellopontine cisternogram. This has given us a unique opportunity to critically reassess the clinical indications of VIIIth nerve tumor from a reverse or negative viewpoint. All data from these patients has been carefully analyzed in relation to indication for opaque cisternography, clinical symptomatology, vestibular function, final etiologic diagnosis, and the associated special auditory tests, tomograph of the internal auditory canal, the associated vestibular aqueduct findings. This paper parallels our earlier study of 121 surgically proven schwannomas providing a total tumor suspect pool of 221 patients with a comparable tumor positive-tumor negative distribution.

Acinic cell tumors of salivary gland origin.

The acinic cell tumor of salivary gland origin, once thought to be benign, is now known to be an incidiously slow growing malignant neoplasm with lethal potential. While the degree of malignant behavior of individual acinic cell tumors is notably variable, all must be treated with aggression. Traditional and current methods of treatment are reviewed; and, in conjunction with the tumors herein reported, guidelines for managment of this uncommon malignancy are suggested. Four cases have been reviewed in detail and critically analyzed. The pathology, including features of both light and electron microscopy, in included--particularly in relation to the oncocytoid areas identified in from 10% to 40% of the parenchymal cells of our tumors. Since an accurate histopathologic diagnosis is the first step in the establishment of a proper treatment plan, pitfalls in histologic diagnosis have been stressed.

Sudden hearing loss in the contralateral ear in postoperative acoustic tumor: three case reports.

Careful presurgical otologic assessment of the contralateral ear in acoustic tumor patients is essential, not only to establish baseline data but more importantly to resolve the intense patient anxiety that arises almost immediately when the diagnosis is presented. Any subsequent reduction in hearing acuity in the contralateral ear after the tumor ear has been deafened, poses serious psychological, socio-economic and medical problems. Three detailed case reports of sudden hearing loss in the contralateral ear of postoperative acoustic tumor patients are presented. The lesion sites include: the middle ear, cochlear, and retrocochlear. In each case, hearing returned to its previous or near-previous levels. Allergic factors were implicated in two cases.

The contralateral ear in acoustic tumors and hearing conservation.

Hearing conservation in acoustic tumor surgery is an admirable goal and a logical extension of micro neurotologic surgery, but ideal candidates for conservation are few, and attempts to preserve hearing in those few fail in about half of all cases. Conserved hearing most often falls far beyond a range that will provide binaural function and is, therefore, of questionable use. The problem of hearing conservation in acoustic tumor surgery is rationally approached based upon the implications of the total presurgical overview, particularly with reference to age, general health, tumor size, and especially hearing in the tumor ear, the contralateral ear, and interaural relationships. Technical considerations, the morbidity and mortality, and, finally, probability factors are reviewed from the literature, The patient's consideration regarding potentials for hearing conservation, his need for conservation, and alternative options are discussed. Rehabilitative options for unilateral hearing impairment are discussed. A review of the amplification requirements of 45 acoustic tumor patients - 5 or more years postoperative - indicated that three-quarters had normal hearing in the contralateral ear, 24% of whom required CROS amplification; 67% of the remaining quarter had mild to moderate hearing losses and were effectively wearing BiCROS aids. None were rehabilitated with monaural aids; 20% of the entire group studied had demonstrated some degree of additional hearing loss in the non-tumor ear since surgery.

Electromagnetic semi-implantable hearing device: phase I. Clinical trials.

Conventional hearing aids have improved significantly in recent years; however, amplification of sound within the external auditory canal creates a number of intrinsic problems, including acoustic feedback and the need for a tight ear mold to increase usable gain. Nonacoustic alternatives which could obviate these encumbrances have not become practical due to inefficient coupling (piezoelectric techniques) or unfeasible power requirements (electromagnetic techniques). Recent technical advances, however, prompted a major clinical investigation of a new electromagnetic, semi-implantable hearing device. This study presents the details of clinical phase I, in which an electromagnetic driver was coupled with a target magnet temporarily affixed onto the lateral surface of the malleus of six hearing aid users with sensorineural losses. The results indicate that the electromagnetic hearing device provides sufficient gain and output characteristics to benefit individuals with sensorineural hearing loss. Significant improvements compared to conventional hearing aids were noted in pure-tone testing and, to a lesser degree, in speech discrimination. Subjective responses were quite favorable, indicating that the electromagnetic hearing device 1. produces no acoustic feedback; 2. works well in noisy environments; and 3. provides a more quiet, natural sound than patients' conventional hearing aids. These favorable results led to phase II of the project, in which patients with surgically amendable mixed hearing losses were implanted with the target magnet incorporated within a hydroxyapatite ossicular prosthesis. The results of this second-stage investigation were also encouraging and will be reported separately.

Relative frequency of inferior vestibular schwannoma.

Until quite recently, acoustic tumors were not diagnosed until they had reached relatively large size. Vestibular function tests were positive in a large percentage of these cases. Since vestibular function tests are limited to lesions of the superior branch of the vestibular nerve, it was thought that 90% of vestibular schwannomas arose from the superior vestibular nerve. Recently, as the diagnostic efficiency of auditory tests has improved, many small tumors are being detected. As the size of the tumors decreases, the efficiency of vestibular function testing has also decreased. A review of histologic and surgical literature demonstrates that the earlier estimate is inaccurate, and that only 50% of vestibular schwannomas originate on the superior branch of the vestibular nerve. This warrants a critical review of the value of vestibular function tests in the evaluation of suspected vestibular schwannomas.

Acoustic reflex testing in otoneurology.

Three parameters of the acoustic reflex have been used in otoneurologic diagnosis: threshold (ART), decay (ARD), and latency (ARL). Each test is defined, put in historical perspective, and discussed in relation to diagnostic implications. We recommend that these three tests - ART, ARD, and ARL - be used as a group and describe how they are implemented in the audiologic diagnosis of acoustic tumors and other lesions affecting the auditory system in the cerebellopontine angle and brain stem.

Hearing conservation in acoustic tumor surgery: pro's and con's.

This article presents an overview of the points to be made both for and against the consideration of hearing conservation in acoustic tumor surgery based on current available data in the literature. Age, general health, tumor size, and hearing - in the tumor ear and in the contralateral ear as well as the interaural relationship - are the presurgical factors discussed. Either the middle fossa or posterior fossa approach may be utilized for hearing conservation. The technical factors relating to surgical anatomy, specific risks, and the probability that hearing may be preserved by these approaches have been abstracted from a review of the literature. Finally, the patient's considerations and his need for the conservation of his hearing are presented, including realistic alternatives to surgical conservation.

Identification of allergic factors in middle ear effusions.

Allergic factors are important, but not the only etiologic force in middle ear effusion. Nor do bacterial and viral infections, nasopharyngeal mass lesions, neuromuscular or regional anatomic abnormalities rank as sole etiologic factors. Some children have multiple components to their problem that requires specific identification before a workable approach to management can be tailored for each of them. Identification and management of allergic factors requires special skills and a greater commitment of time. Yet the marked increase in total yield when allergic techniques are added to the armamentarium of the otolaryngologist make these factors both worthwhile and satisfying.

Allergic factors in management of middle ear effusions.

There is no single specific therapy for the treatment of middle ear effusions and, therefore, a holistic approach to management is necessary. Current management involves multiple therapeutic modalities of which we believe tube and allergy are most efficient.

Monitoring of the glycerol test with sinusoidal harmonic acceleration in Menière's disease patients.

Changes in auditory pure tone threshold and speech discrimination and the phase and gain in the sinusoidal harmonic acceleration test were measured in subjects with endolymphatic hydrops (Meniere's disease) following the administration of glycerol. Although all subjects showed a marked improvement in auditory function, a comparable change in vestibular function, as measured by the sinusoidal harmonic acceleration test, was not observed.

Acoustic reflex latency test in the evaluation of nontumor patients with abnormal brainstem latencies.

The acoustic reflex latency test (ARLT), an indirect measure of neural conduction time utilizing the electroimpedance method, was used to evaluate 43 highly suspect, nontumor patients who demonstrated abnormal latency results on brainstem electric response audiometry (BERA), false-positive for tumor. Normative values and criteria for tumor-positive ARLT results were established on a control group using a second generation test unit, comprised of dual impedance bridges and a digital signal averager, designed especially to perform the ARLT. When cut-off criteria for both absolute reflex latency and interaural latency difference (ILD) values were applied to this BERA false positive population, the ARLT correctly identified 93% of patients as having end-organ lesions. Differences in ARLT and BERA test results are attributed to differences in 1) method of measurement, 2) characteristics of the eliciting signal, and 3) generation of the response. These factors are discussed as sources of variability. Both ARLT and BERA are recommended as tandem procedures when screening for acoustic or angle tumors.

Effects of conductive hearing loss on auditory brainstem response.

The purpose of this paper is not to propose that auditory brainstem response (ABR) be utilized for the assessment of conductive losses, but to define the effects of conductive hearing loss on the ABR when such a complication occurs. Conductive losses attenuate cochlear stimulation. Since wave V latency is inversely related to stimulus intensity, the magnitude of the conductive loss should be a predictor of the wave V latency delay. In this study, ABR wave V latencies from patients with known conductive losses due to canal occlusion, middle ear effusion, ossicular fixation and chain interruption were compared with latency values calculated from the magnitude of the loss. In those patients with occlusion of the external auditory canal and middle ear effusion, the shift of the wave V latency-intensity function correlated well with the air-bone gap. This correlation was poor for patients with ossicular chain disorders. In mixed hearing losses, the increased wave V latency due to the conductive component may totally mask an increase in latency caused by a retrocochlear component.