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S D Rauch

Publications and source records attributed to S D Rauch.

At least 19 recordsLinked to original sources

Recognition of a dominant epitope in bovine heat-shock protein 70 in inner ear disease.

OBJECTIVE: To investigate the specificity of antibodies to heat-shock protein 70 (HSP70) in patients with idiopathic, progressive, bilateral sensorineural hearing loss (IPBSNHL) and Meniere disease. STUDY DESIGN: Test immunoreactivity of patients' sera using recombinant human (rh) and bovine (rb) HSP70, as well as segments representing different regions of bovine HSP70 as antigen. METHODS: Sera were tested by Western blotting. RESULTS: Of 52 patients with IPB-SNHL, 40 sera reacted only with rbHSP70; 12 reacted with both rbHSP70 and rhHSP70. Sera from 13 patients with IPBSNHL and from 8 with Meniere disease were tested on the panel of bovine HSP70 segments. Eleven and 7 samples, respectively, reacted with amino acid segment 427-461 from the carboxy (C)-terminal region of the molecule. CONCLUSION: In IPBSNHL and Meniere disease, antibodies are directed primarily against an epitope(s) within the C-terminal region of HSP70 where diversity in sequence among different species, including possible pathogens, is greatest. These findings may provide clues to the pathogenesis or specific serodiagnosis (or both) of diseases of the inner ear.

Animals

Intratympanic gentamicin for treatment of intractable Meniere's disease: a preliminary report.

Topical administration of aminoglycoside antibiotics in the middle ear can achieve "chemical labyrinthectomy" in patients with intractable Meniere's disease. Herein we report our results of intratympanic gentamicin therapy in 21 patients using two different dosing protocols, twice weekly and twice daily (b.i.d.). Both hearing and vertigo outcome were evaluated. Complete control of episodic vertigo was achieved initially in 20 of 21 patients (95.2%). However, 6 of 20 responders (30%) developed relapsing symptoms within 12 months. Retreatment was successful in 75% of these patients. Overall, hearing was preserved or improved in 62% of cases, worse in 24%, and not yet tested in 14%. When the cumulative dose of gentamicin was < or = 4 injections in the first week, only 1 of 14 (7.1%) lost hearing. Intratympanic gentamicin offers better risk/benefit outcome than other invasive therapies for intractable Meniere's disease.

Administration, Topical

Efficacy of tympanomastoid surgery for control of infection in active chronic otitis media.

The efficacy of surgery in controlling infection in 272 tympanomastoidectomy procedures for chronic otitis media (COM) was assessed by means of a four-point rating scale that incorporated both symptoms and signs, such as the presence or absence of otorrhea and granulation tissue. Of the 272 procedures, 170 were performed for COM with cholesteatoma and 102 were for active COM with granulation tissue but no cholesteatoma. Forty-seven percent were primary procedures, and 53% were revisions. Minimum follow-up was 12 months for all cases, with a mean of 30 months. Adequate control of infection occurred in 248 (91%) of the 272 cases. Of the 24 cases (9%) that developed persistent infection, 10 were controlled with a combination of oral and topical antibiotics and/or delayed skin grafting in the office. Thus overall satisfactory control of infection was achieved in 258 of 272 cases (95%). The outcome was influenced by the diagnostic category of COM: COM with cholesteatoma did significantly better than COM with granulation tissue (P = 0.02). The outcome was not influenced by the following variables: primary versus revision surgery, canal wall-up versus canal wall-down surgery, and extent of disease. The results suggest that active COM with granulation tissue may be more difficult to control than COM with cholesteatoma.

Administration, Oral

Revision stapedectomy: intraoperative findings, results, and review of the literature.

Seventy-four revision stapedectomies performed consecutively over 10 years (1986 to 1995) were reviewed retrospectively. The most common intraoperative findings were incus erosion, prosthesis displacement, and oval window closure. Incus erosion was more frequently associated with multiple revisions. The postoperative results were reported using the conventional method (postoperative air minus preoperative bone) as well as the guidelines recently published by the American Academy of Otolaryngology--Head and Neck Surgery (postoperative air minus postoperative bone), with success rates of postoperative air-bone gap closure to within 10 dB after revision surgery of 51.6% and 45.6%, respectively. Patients with persistent conductive hearing loss (large residual air-bone gaps) after primary stapedectomy had poorer postrevision hearing results. Sensorineural hearing loss (defined as a drop in bone pure-tone average of more than 10 dB) occurred in four cases (5.4%). The number of revision surgeries, variations in operative techniques using laser or drill, and the ossicle to which the prosthesis was attached did not statistically affect the postoperative air-bone gaps. These results were compared with previously published data.

Adult

Nucleus 22 cochlear implantation results in postmeningitic deafness.

Cochlear implant surgery was performed on 13 patients with postmeningitic deafness (seven adults, six children). Two adults and two children (30.8%) had severe labyrinthitis ossificans requiring radical "drill-out." Five of 13 (38.5%) had some bone growth requiring partial drill-out, and four of 13 (30.8%) had normal insertion with no drill-out. Hearing results for patients with no bone growth were similar to nonmeningitic patients; three of four (75%) had open-set speech recognition. Performance of patients with total drill-out was poor; "auditory only" performance was limited to detection and pattern perception of speech, and no patients had open-set speech recognition. Results for patients with partial drill-out were similar to results in patients with no bone growth. Labyrinthitis ossificans not only presents surgical challenges to cochlear implantation but may also adversely affect hearing outcome.

Adolescent

Serum antibodies to heat shock protein 70 in sensorineural hearing loss.

OBJECTIVE: To identify the 68-kd target of antibody in serum samples from patients with idiopathic, progressive, bilateral sensorineural hearing loss. DESIGN: To purify target protein from renal extracts using gel filtration, ion-exchange chromatography, and polyacrylamide gel electrophoresis and to transfer to nitrocellulose membranes. The purified protein was digested with trypsin, and peptide fragments were separated by high-pressure liquid chromatography. RESULTS: One fraction obtained by high-performance liquid chromatography contained a peptide of 2776 molecular weight. The sequence of a stretch of 22 amino acids within this peptide was identical to that of amino acids 424 through 445 of heat shock protein 70 (HSP70). On Western blotting, monoclonal antibody directed against HSP70 (but capable of recognizing both constitutive HSP70 [HSC70] and stress-inducible HSP70) reacted with the purified 68-kd protein. We compared the reactivity of serum samples from six patients with idiopathic, progressive, bilateral sensorineural hearing loss, as well as monoclonal antibody to HSC70, and monoclonal antibody to HSP70 with renal extract. The pattern obtained suggested that patient antibodies are preferentially directed at HSP70. CONCLUSION: The target of antibody in serum samples from patients with idiopathic, progressive, bilateral sensorineural hearing loss is HSP70.

Amino Acid Sequence

Ménière's disease.

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.

Animals

Significance of auditory brain stem response and gadolinium-enhanced magnetic resonance imaging for idiopathic sudden sensorineural hearing loss.

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)

Administration, Oral

Perilymph fistula pathophysiology.

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.

Biomarkers

Serum antibodies against heat shock protein 70 in Menière's disease.

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.

Adult

Intraductal tetracycline therapy for the treatment of chronic recurrent parotitis.

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.

Adult

Re: RBRVS.

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Communism

The National Temporal Bone, Hearing, and Balance Pathology Resource Registry.

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.

Brain

High jugular bulb: implications for posterior fossa neurotologic and cranial base surgery.

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.

Adult

Malformation and degeneration in the inner ear of mos transgenic mice.

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.

Animals