Biomedical subjects
Publications and source records attributed to S D Rauch.
There now exists a vast literature on Ménière's disease. In this review, we examine closely the foundations of some of the current concepts regarding various aspects of the disorder, including definitions, clinical features, natural history, pathology, pathogenesis, pathophysiology, diagnosis and treatment. We will highlight areas where the current state of knowledge is incomplete and also suggest some avenues for further research.
Previous studies tried to correlate prognosis and response to oral corticosteroids in patients with idiopathic sudden sensorineural hearing loss to such factors as the age of the patient, presence of vertigo, shape of the audiogram, or severity of the hearing loss. However, temporal bone histopathologic evidence shows that idiopathic sudden sensorineural hearing loss may be caused by cochleitis or cochlear nerve neuritis. Herein we report results of a retrospective study of 96 consecutive patients with idiopathic sudden sensorineural hearing loss who were evaluated with auditory brain stem responses and gadolinium-enhanced magnetic resonance imaging. Results of the auditory brain stem response and magnetic resonance imaging were correlated with hearing outcome. Follow-up was available for 65 patients: 14 with abnormal and 51 with normal auditory brain stem responses. The overall rate of hearing recovery or improvement was 65% in the normal auditory brain stem response group compared with 43% in the abnormal auditory brain stem response group (p = 0.07). Among the 38 patients treated with a tapering course of oral corticosteroids, the recovery or improvement rate was 83% for those with normal auditory brain stem responses and 56% for those with abnormal auditory brain stem responses (p < 0.05). Of the 27 patients who did not receive steroid therapy, the improvement rate was 41% in those with normal auditory brain stem responses and 20% in those with abnormal auditory brain stem responses (p = 0.09). Magnetic resonance imaging with gadolinium was obtained on all 14 patients with abnormal auditory brain stem responses but on none with normal auditory brain stem responses.(ABSTRACT TRUNCATED AT 250 WORDS)
A great deal of the controversy associated with perilymph fistula is due to the lack of a sensitive, specific way of detecting and thus diagnosing one. The existing subjective, clinical observations need to be replaced with an objective, scientific methodology having higher specificity and sensitivity for the detection of perilymph fistula. Three detection methods presently under development are presented here. One uses a miniendoscope to observe the middle ear. This procedure is less traumatic than inspection with tympanotomy. Thus it is less likely to create artifacts that can be mistaken for a fistula leakage. The "yes-no" observation decision is to be replaced with a graded quantitative measure. The second method concentrates on detected leakage from inner to middle ear. beta 2-Transferrin, a unique endogenous substance found in perilymph and cerebrospinal fluid, can be detected with one- or two-dimensional gel electrophoresis and immunoblotting. The third method is a system identification fistula test. It measures a change in a physiologic response that is caused by the presence of the fistula. Classic biophysical models of the vestibular end organs can be used to predict the dynamics of responses to systems identification stimuli.
Menière's syndrome of fluctuating sensorineural hearing loss, episodic vertigo, and tinnitus is the clinical presentation resulting from several different pathologic processes. The present study was designed to probe for immunologic factors in Menière's disease. It has previously been shown that the presence of circulating antibodies against a 68-kD protein detected by Western blot immunoassay of serum from patients with idiopathic, progressive, bilateral sensorineural hearing loss (IPB SNHL) is highly correlated with both activity of disease and corticosteroid responsiveness of the hearing loss. The authors recently have identified the 68-kD protein as heat shock protein 70 (HSP70). This study presents a retrospective review of results of Western blot assays in 30 patients meeting strict AAO-HNS diagnostic criteria for Menière's disease. Overall, 47% of patients had anti-HSP70 antibodies. When broken down by clinical pattern, anti-HSP70 antibodies were present in 58.8% of bilateral Menière's disease, 37.5% of contralateral delayed endolymphatic hydrops, and 33.3% of unilateral Menière's disease with IPB SNHL in the second affected ear. Detection of antibodies to HSP may serve to identify a subset of patients with Menière's disease in whom immunologic factors have a pathogenic role.
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Chronic recurrent parotitis (CRP) is recurrent parotid inflammation with non-obstructive sialectasis. Therapies which produce acinar atrophy or remove the acini are effective in treating CRP. Parotidectomy, tympanic neurectomy, duct ligation, and radiation therapy have either a low success rate or a high risk of morbidity. Intraductal antibiotic instillation has been proposed as a possible method of treatment. We hypothesized that the cytotoxic effects of tetracycline could produce acinar atrophy. A double-blind experiment of intraductal tetracycline instillation was performed in ten rabbits. Acinar atrophy and acute inflammation were found in 40% of the tetracycline treated glands; controls had a complete absence of these histologic changes. These results support the use of tetracycline instillation to produce acinar atrophy and therefore, intraductal tetracycline may be an effective, low-risk therapy for CRP. The clinical features of CRP will be reviewed and therapeutic implications discussed.
The National Temporal Bone, Hearing, and Balance Pathology Resource Registry has been established with funding provided by the National Institute on Deafness and Other Communication Disorders of the National Institutes of Health. The registry is meant to serve as a national resource for researchers and the public to stimulate and facilitate human otopathologic research. It will maintain a computerized database of currently active and inactive temporal bone and auditory brain-stem collections throughout the United States. In addition, it will encourage human temporal bone research by disseminating pertinent information, developing and fostering temporal bone professional educational activities, implementing a national temporal bone acquisition network, and encouraging investigative collaborations in the study of the human temporal bone and brain structures. It will also identify otopathologic collections at risk of being discarded or lost and will develop mechanisms and strategies to conserve them.
The suboccipital-retrosigmoid approach to the internal auditory canal and cerebellopontine angle is being used with increasing frequency for neurotologic surgery, including vestibular nerve section and resection of acoustic neuroma. It offers wide exposure of the cerebellopontine angle and the cranial nerve VII-VIII complex as it courses from the brain stem to the temporal bone. Exposure of the internal auditory canal can be achieved by removing its posterior bony wall. Safe utilization of this approach requires familiarity with the variable position of structures within the petrous bone, including the lateral venous sinus and jugular bulb. We report here a case in which bleeding resulted from injury to a high jugular bulb during surgical exposure of the internal auditory canal via the suboccipital route and discuss the regional anatomy of the jugular bulb based on study of 378 consecutive temporal bone specimens from the collection of the Massachusetts Eye and Ear Infirmary. High jugular bulb was defined as encroachment of the dome of the bulb within 2 mm of the floor of the internal auditory canal. Forty-six percent of scoreable specimens met this criterion. However, when donors less than 6 years of age were excluded, a high jugular bulb was identified in 63% of specimens. Relevance to neurotologic surgery of the posterior fossa is presented.
Transgenic mice carrying the mos proto-oncogene linked to a retroviral transcriptional control sequence display behavioral abnormalities including circling, hyperactivity, head tilt, and bobbing. Axonal degeneration, neuronal chromatolysis, spongiform encephalopathy, gliosis, and inflammatory infiltrates are reportedly found in the central nervous systems of all mutants with the behavioral traits. Hearing was tested by means of broadband free-field rarefaction clicks with auditory brain stem response recorded between vertex and mouth electrodes. No detectable auditory response was elicited in transgenic animals, in contrast to five distinct positive peaks observed in littermate control animals. Light microscopic survey of temporal bone histopathology in mutants revealed extensive degeneration of the organ of Corti with loss of hair cells in all cochlear turns and loss of supporting cells and atrophy of spiral ganglion cells. The spiral limbus was deformed, with replacement of the usual convexity of the superior surface by a flattened trough configuration. Hair cells of the vestibular end organs appeared normal. Pathologic alteration in levels of mos transgene RNA appears to have a direct effect on the structural integrity of the inner ear.
Modern immunologic techniques of immunostaining, immunoblotting, and creation of monoclonal antibodies are gaining wide application in studies of development, function, and pathology of the ear. These techniques require a source of inner ear tissue for production of antigen extract. Human tissue is not readily available, and other mammalian species common in auditory research are small in size. Bovine temporal bones are readily available, and the membranous portions of the inner ear are abundant and easily accessible. Herein we report our technique for acquisition and dissection of bovine temporal bones and preparation and preservation of inner ear antigen.
In view of the growing popularity of office videolaryngoscopy, surprisingly little objective data exist as to its influence on clinical practice. We reviewed 150 consecutive laryngeal recordings in an attempt to determine what effect videolaryngoscopy has had on our practice and the patient care provided.
A retrospective review of all stapedectomies performed at the Massachusetts Eye and Ear Infirmary over a 13 month period was undertaken to compare the results of argon laser stapedectomy to conventional footplate fenestration techniques. Despite the fact that it was new and unfamiliar to the surgical staff, the complication rate was lower with the argon laser than with footplate fenestration by traditional pick or drill techniques. The incidence of footplate fracture or removal preventing use of a small fenestra technique was five times as likely to occur with traditional instruments as with the argon laser (p < 0.025). In revision stapedectomy sensorineural hearing loss was four times as likely to occur with traditional instruments as with the argon laser (p < or = 0.05). The argon laser did not alter the operating time. Though not ideal for every case, the argon laser endo-otoprobe offers the surgeon a significant new tool to improve the outcome in stapes surgery.
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For some patients, the transcutaneous bone-conduction implant offers a viable alternative to conventional amplification. However, this option should be employed only after considering the relative advantages and disadvantages of conventional medical management and, when feasible, the fitting of air-conduction amplification. The cases presented here illustrate some important factors to consider in the selection and fitting of bone-implant candidates.
Prader-Willi syndrome (PWS) is a sporadic disorder in which about half of cases have a 15q12 deletion. Although a small number of cases have other rearrangements involving 15q12, the rest of the cases appear to have normal chromosomes. Clinical similarities among all these patients regardless of the karyotype strongly suggests a common etiology. To investigate the nature of this common etiology, we analyzed sister chromatid exchange (SCE) at the 15q11-13 region in 10 PWS patients with the chromosome deletion, 12 PWS patients with normal chromosomes, and 11 normal control individuals. While SCE at the q11-13 region was absent on the 15q12 deleted chromosome, the percentage of SCE on chromosome 15 at q11 was statistically higher for PWS with normal chromosomes (10.1%) compared to that for normal controls (1.9%) and the normal homologue (2.2%) in deleted patients (chi 2 = 7.7982, df = 2, P less than 0.025). The data suggest relative instability of DNA at the 15q11 region in PWS patients.
A systematic double-blind assessment of case histories and histopathologic findings in temporal bones in the collection at the Massachusetts Eye and Ear Infirmary was performed to test the hypothesis that clinical Meniere's syndrome is associated with endolymphatic hydrops demonstrated histopathologically at death. Thirteen of 13 cases of clinical Meniere's syndrome were found to have endolymphatic hydrops not attributable to other causes. However, some patients with idiopathic endolymphatic hydrops did not exhibit clinical Meniere's syndrome as revealed in their medical records. These results challenge the dogma that endolymphatic hydrops per se generates the symptoms of Meniere's syndrome.
Mucoceles involving any of the paranasal sinuses may present with ophthalmic disturbances. Maxillary antral mucoceles can encroach on the inferior orbit with ocular displacement and compression, proptosis or enophthalmos, lower lid distortion, tethering of extraocular muscles, and ptosis. Periocular pain, erosion of the inferior orbital rim, infraorbital nerve compression, epiphora, and inner canthal swellings are other important presentations. Postoperative mucoceles may involve only a portion of the antrum. Five illustrative cases are presented.