Program DescriptionEvery time the ear surgeon operates, a significant number of events occur and are anticipated. Many of these are routine and predictable. However, sometimes the unexpected occurs, which can be during the preoperative assessment, during surgery, or after surgery. Treatment outcomes are dependent on the surgeon’s handling of unexpected findings or occurrences as well as the decisions made regarding what is best for the patient. This miniseminar presents cases of 4 common otologic diagnoses where the surgeon was faced with unanticipated situations that challenged his diagnostic and surgical skills; where every decision made affected the final outcome. The disease entities presented are Meniere’s disease, otosclerosis, chronic otitis media with cholesteatoma, and chronic otitis media without cholesteatoma. In each case presented, the panelist will discuss his decisions regarding whether or not to image the patient, whether or not to recommend surgery, what was found intraoperatively, and what was encountered that required difficult but necessary intraoperative decisions. The panelists are all expert otologic surgeons with many years of experience in handling complicated otologic problems. The emphasis of the miniseminar is not to present unusual cases, but instead is to present common problems where unexpected findings significantly challenged the surgeon and complicated the case. In Meniere’s disease, various approaches in evaluation and treatment are used by otolaryngologists. It is anticipated that after this miniseminar the attendee will have a better understanding of how to approach Meniere’s disease systematically and be able to anticipate potential problems. Otosclerosis may seem straight forward, but a myriad of anatomical variations exist that may confuse the surgeon. In addition, other entities may mimic otosclerosis. This miniseminar should help the attendee more accurately prepare for otosclerosis surgery and its potential variations. Chronic otitis media with and without cholesteatoma is a continuum of disease that can challenge all ear surgeons. By presenting ways to evaluate these diseases with awareness of possibly encountering unanticipated findings, this miniseminar should help the attendee feel more comfortable handling chronic otitis media and its many presentations. The emphasis of this miniseminar is for both general otolaryngologists and otologists to become aware of and be able to diagnose and treat the unpredictable challenges that are so often seen in common otologic conditions and how to make the correct intraoperative decisions.Educational Objectives1) Understand that common otologic diseases may present unanticipated challenges during diagnosis and treatment. 2) Learn how to anticipate diagnostic dilemmas and be able to make appropriate treatment recommendations. 3) Learn how to recognize unusual surgical presentations and how to make the right intraoperative decision.

With the rapid expansion in the introduction of computer integrated manufacturing (CIM), the allotment of cell level functions and the placing of cell level systems become important. This paper describes the trend and the future image of the functions of cell level systems, that is, the cell computer/controller as system components of flexible manufacturing cells (FMC).

Objectives: To determine if the addition of transtympanic steroids to oral steroids improves hearing recovery in idiopathic sudden sensorineural hearing loss (ISSNHL).

Computed tomography (CT) of a polypoid mass in the external auditory canal demonstrated a heterogeneous, enhancing mass located in the right temporal bone, with soft-tissue extension into the external auditory canal and anterior middle ear. Magnetic resonance (MR) imaging demonstrated an irregular mass with heterogeneous signal on T2-weighted images, isointense signal on T1weighted images, and contrast enhancement. The anterior wall of the external canal, the medial aspect of the right temporomandibular joint, and the masticator space were involved. The lesion invaded the lateral aspect of the carotid canal, but it did not encase the carotid artery. There was erosion of the anterior floor of the middle cranial fossa and invasion of the anterior temporal lobe. Mesenchymal chondrosarcoma is a rare subtype of chondrosarcoma. Ninety-nine percent of conventional Address correspondence and reprint requests to Barry E. Hirsch, M.D., Department of Otolaryngology, University of Pittsburgh, 200 Lothrop Street, Suite 500, Pittsburgh, PA 15213, U.S.A. Email: hirschbe@msx.upmc.edu

This study analyzed features of total and segmental spiral ganglion cell populations in children with normal ears and those with various pathological conditions. Sixty-three human temporal bone specimens, obtained from 43 children 4 days to 9 years of age, were studied histopathologically. These specimens were divided into 5 diagnostic groups: group 1, normal ears (13 ears); group 2, congenital infectious diseases (13 ears); group 3, chromosomal aberrations (11 ears); group 4, multiple craniofacial anomalies with hereditary or genetic causes (21 ears); and group 5, perinatal and postnatal asphyxia (5 ears). Eighteen of the 63 ears had documented profound deafness. In either normal ears (group 1) or those with various pathological conditions (groups 2 through 5), the total number of ganglion cells did not change as a function of age during the first 10 years. The total number of ganglion cells was significantly larger in group 1 (33,702) than in each of groups 2, 3, 4, and 5 (p < .01), and the number was significantly larger in group 2 than in each of groups 4 and 5 (p < .01 and p < .05, respectively). The ratio of basal to apical ganglion cell populations remained constant in both normal and pathological ears. Each ratio of the number of basal and apical ganglion cells in groups 2, 3, 4, and 5 to the mean number in group 1 (basal and apical survival ratios) was at least approximately 40%. There was no statistical difference between these Two ratios in groups 2, 3, 4, and 5. The mean (±SD) total number of ganglion cells in ears with documented profound deafness was 15,417 ± 5,944, which is approximately 40% of those present in normal ears. Our results suggest that normally, cochlear neurons are completely present at birth and minimally regress during the first decade of life. In addition, although intergroup differences among various pathological groups were present, the majority of pathological ears had more than 10,000 spiral ganglion cells present. Cochlear implantation has gradually been recognized as an effective and reliable tool for rehabilitation of children who have profound deafness, even congenitally or prelingually deafened children. On the basis of the results obtained in this study, we discuss the implications for cochlear implantation in children.

OBJECTIVE:This study describes the histopathologic findings of a patient with severe bilateral sensorineural hearing loss after perinatal and postnatal hypoxia and asphyxia.STUDY DESIGN:Histopathologic examination on the temporal bones.SETTING:The study was performed at the Elizabeth McCullough Knowles Otopathology Laboratory, Division of Otopathology, Department of Otolaryngology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.RESULTS:Histopathologic examination on the left temporal bone revealed severe atrophy of the organ of Corti throughout the entire cochlea, decrease in the number of the spiral ganglion cells especially in the basal turn, and mild atrophy of saccular macula. In the right temporal bone, similar abnormalities were observed in the inner ear, but the changes were milder than those in the left temporal bone. No other distinct pathologic finding was observed in either ear.CONCLUSION:These findings suggest that the presence of severe hypoxic ischemia causes cochleosaccular atrophy. To our knowledge, this is the first histopathologic case report describing the long-term effect of perinatal and postnatal hypoxia and asphyxia that produced cochleosaccular abnormalities in the human inner ear.

Six temporal bone-eustachian tube (ET) specimens with cholesterol granuloma (CG), obtained from 6 children 6 months to 15 years of age, were studied histopathologically to obtain further information about the pathogenesis of CG. We observed CG in the mastoid air cells in 5 ears, the mastoid antrum in 1 ear, the aditus ad antrum in 2 ears, and the epitympanum in 1 ear. All 6 cases exhibited a large amount of remaining mesenchyme that was in continuity with the hematopoietic bone marrow in the locations in which CG was present. All cases demonstrated otitis media with effusion and inflammation of the ET. Apparent morphological abnormalities of the ET and its associated structures, including hypoplastic ET cartilage and an abnormal tensor veli palatini muscle, were noted in 3 of the 6 cases. Furthermore, the posterior cartilaginous portion of the ET that includes its narrowest portion was completely filled with effusion in 2 of the 3 cases with the ET anomaly. The findings obtained were compared with data from age-matched control cases. Our results suggest that the source of erythrocytes in the remaining mesenchyme is the hematopoietic bone marrow. The pathogenesis of CG in children is likely promoted by ET dysfunction resulting in failure of ventilation of the middle ear.

Background: The advent of cochlear implantation has revolutionized the options afforded to the deaf population. With the increase in the prevalence of this procedure have come larger experiences in the associated technical challenges and complications. Results. We present the evaluation and management of a patient with an unusual complication of improper placement of the implant electrode into the carotid canal and its management. We discuss the anatomy of the carotid artery and its proximity to the cochlea to emphasize the potential risk to this large vessel. Conclusions. Damage to the carotid canal and the carotid artery is a potential risk of cochlear implant surgery. When available, we recommend intraoperative electrical testing of the cochlear implant be performed. If there is doubt as to the placement of the electrode, a radiograph should be obtained before the patient is taken out of the operating room to avoid this complication.

Haginomori, Shin-Ichi; Sando, Isamu; Miura, Makoto; Orita, Yorihisa; Hirsch, Barry E. Author Information

Four temporal bone specimens, obtained from three individuals 1--6 years of age with Noonan syndrome (NS), were studied histopathologically. All four specimens were accompanied by similar inner ear abnormalities including the reduced number of spiral ganglion cells, enlarged lateral semicircular canal, and dislocated endolymphatic sac and vestibular aqueduct. The mean population of spiral ganglion cells (15,699 cells) was approximately half of those (32,978 cells) in four age-matched control cases. In addition, they had several middle ear abnormalities including the remaining mesenchyme and dehiscence of the facial canal. To our knowledge, this is the first report to describe the histopathological temporal bone findings in patients with NS. We discuss the implications of the observed abnormalities with regard to clinical issues.

OBJECTIVETo describe a histopathologic analysis of a human temporal bone demonstrating patulous changes of the eustachian tube (ET) and its surrounding structures following radiation therapy.DESIGNRetrospective histopathologic case review and comparison with an age-matched control.SETTINGElizabeth McCullough Knowles Otopathology Laboratory, University of Pittsburgh School of Medicine, Pittsburgh, Pa.RESULTSA widened patulous ET was verified by demonstrating fibrous tissue replacement of the surrounding supporting structures related to the ET. The ET lumen was patulous and wider than the control case. Ostmann fatty tissue, the levator veli palatini muscle, and submucosal glands around the ET cartilage were replaced by dense connective tissue.CONCLUSIONThis is the first histopathologic report, to our knowledge, demonstrating the effects on the ET lumen and supporting structures following acute weight loss, possible tumor infiltration, and radiation changes for carcinoma of the oropharynx.

Tinnitus, a buzzing or ringing in the ear, may be pulsatile or continuous (nonpulsatile). The distinction, with a detailed clinical evaluation, determines the most appropriate imaging study. Pulsatile tinnitus suggests a vascular neoplasm, vascular anomaly, or vascular malformation. Most of the neoplasms are glomus tympanicum and glomus jugulare tumors. Vascular anomalies may cause pulsatile tinnitus, but the mechanism is unknown, and another (treatable) cause should be sought. Most neoplasms and anomalies are best seen on bone algorithm computed tomographic (CT) studies. Dural vascular malformations are often elusive on all cross-sectional imaging studies; conventional angiography may be necessary to make this diagnosis. Flow-sensitive magnetic resonance (MR) images show vascular loops compressing the eighth cranial nerve. Carotid dissections, aneurysms, atherosclerosis, and fibromuscular dysplasia can be identified on both MR imaging or MR angiographic studies and CT or CT angiographic studies. Otosclerosis and Paget disease are CT diagnoses. Benign intracranial hypertension often has no abnormal imaging findings. For patients with nonpulsatile tinnitus, MR imaging is the study of choice to exclude a vestibular schwannoma or other neoplasm of the cerebellopontine angle cistern. Multiple sclerosis and a Chiari I malformation are rare causes of pulsatile tinnitus, also best seen on MR studies. Many patients with tinnitus have no abnormal imaging findings.