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Papers by Steven Rauch

Research paper thumbnail of Ménière's disease

European Archives of Oto-rhino-laryngology, 1995

There now exists a vast literature on Ménìere's disease. In this review, we examine closely t... more There now exists a vast literature on Ménìere'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 paper thumbnail of Vestibular evoked myogenic potentials in patients with superior semicircular canal dehiscence

Otology & neurotology: official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology

To compare the audiometric and cervical vestibular evoked muscle potential (cVEMP) outcomes of ea... more To compare the audiometric and cervical vestibular evoked muscle potential (cVEMP) outcomes of ears with superior canal dehiscence (SSCD) to outcomes from normal ears and the unaffected ears of unilateral SSCD patients. Retrospective review of clinical testing outcomes. Tertiary care specialty teaching hospital. Three subject groups were used: 21 ears of healthy subjects, ages 21 to 52 years; 58 affected SSCD ears in patients, ages 15 to 65 years; and 26 unaffected ears of unilateral SSCD patients, ages 15 to 65 years. Results of pure-tone audiometry and cervical vestibular evoked muscle potential (cVEMP) testing. SSCD ears differed from normal ears or unaffected ears of unilateral SSCD patients in 2 ways: 1) they had significantly larger air-bone gaps at 250 and 500 Hz, resulting in poorer air-conduction thresholds at those frequencies, and 2) their cVEMP thresholds at 500 Hz were significantly lower. When the cVEMP threshold was adjusted for the size of the air-bone gap on the aud...

Research paper thumbnail of Vestibular evoked myogenic potentials (VEMP) can detect asymptomatic saccular hydrops

The Laryngoscope, 2006

The objective of this study was to explore the useful of vestibular evoked myogenic potential (VE... more The objective of this study was to explore the useful of vestibular evoked myogenic potential (VEMP) testing for detecting endolymphatic hydrops, especially in the second ear of patients with unilateral Ménière disease (MD). This study was performed at a tertiary care academic medical center. Part I consisted of postmortem temporal bone specimens from the temporal bone collection of the Massachusetts Eye & Ear Infirmary; part II consisted of consecutive consenting adult patients (n = 82) with unilateral MD by American Academy of Otolaryngology-Head and Neck Surgery criteria case histories. Outcome measures consisted of VEMP thresholds in patients and histologic saccular endolymphatic hydrops in postmortem temporal bones. Saccular hydrops was observed in the asymptomatic ear in six of 17 (35%) of temporal bones from donors with unilateral MD. Clinic patients with unilateral MD showed elevated mean VEMP thresholds and altered VEMP tuning in their symptomatic ears and, to a lesser degr...

Research paper thumbnail of Results after revision stapedectomy with malleus grip prosthesis

The Annals of otology, rhinology, and laryngology, 2006

Revision stapedectomy with a malleus grip prosthesis is a technically challenging otologic proced... more Revision stapedectomy with a malleus grip prosthesis is a technically challenging otologic procedure. The prosthesis is usually longer and extends deeper into the vestibule than a conventional stapes prosthesis, creating the potential to affect the vestibular sense organs. The prosthesis also bypasses the ossicular joints, which are thought to play a role in protecting the inner ear from large changes in static pressure within the middle ear. The prosthesis is in close proximity to the tympanic membrane, thus increasing the risk for its extrusion. We reviewed our experience with revision stapedectomy with the Schuknecht Teflon-wire malleus grip prosthesis in 36 ears with a mean follow-up of 23 months. The air-bone gap was closed to within 10 dB in 16 ears (44%) and to within 20 dB in 26 ears (72%). The incidence of postoperative sensorineural hearing loss was 8% (3 ears). There were no dead ears. Extrusion of the prosthesis occurred in 1 case (3%). Nearly 50% of patients reported va...

Research paper thumbnail of Temporal bone studies of the human peripheral vestibular system. Normative vestibular hair cell data

The Annals of otology, rhinology & laryngology. Supplement, 2000

Quantitative studies of the vestibular system with serially sectioned human temporal bones have b... more Quantitative studies of the vestibular system with serially sectioned human temporal bones have been limited because of difficulty in distinguishing hair cells from supporting cells and type I from type II hair cells. In addition, there is only a limited amount of normative data available regarding vestibular hair cell counts in humans. In this study, archival temporal bone sections were examined by Nomarski (differential interference contrast) microscopy, which permitted visualization of the cuticular plate and stereociliary bundle so as to allow unambiguous identification of hair cells. The density of type I, type II, and total numbers of vestibular hair cells in each of the 5 sense organs was determined in a set of 67 normal temporal bones that ranged from birth through 100 years of age. The mean total densities at birth were 76 to 79 cells per 0.01 mm2 in the cristae, 68 cells per 0.01 mm2 in the utricle, and 61 cells per 0.01 mm2 in the saccule. The ratio of type I to type II h...

Research paper thumbnail of Evaluating Inhibition of Motoneuron Firing From Electromyogram Data to Assess Vestibular Output Using Vestibular Evoked Myogenic Potentials

Ear and Hearing, 2015

Vestibular evoked myogenic potentials (VEMPs) are due to vestibular responses producing brief inh... more Vestibular evoked myogenic potentials (VEMPs) are due to vestibular responses producing brief inhibitions of muscle contractions that are detectable in electromyographic (EMG) responses. VEMP amplitudes are traditionally measured by the peak to peak amplitude of the averaged EMG response (VEMPpp) or by a normalized VEMPpp (nVEMPpp). However, a brief EMG inhibition does not satisfy the statistical assumptions for the average to be the optimal processing strategy. Here, it is postulated that the inhibition depth of motoneuron firing is the desired metric for showing the influence of the vestibular system on the muscle system. The authors present a metric called "VEMPid" that estimates this inhibition depth from the EMG data obtained in a usual VEMP data acquisition. The goal of this article was to compare how well VEMPid, VEMPpp, and nVEMPpp track inhibition depth. To find a robust method to compare VEMPid, VEMPpp, and nVEMPpp, realistic physiological models for the inhibition of VEMP EMG signals were made using VEMP data from four measurement sessions on each of the five normal subjects. Each of the resulting 20 EMG-production models was adjusted to match the EMG autocorrelation of an individual subject and session. Simulated VEMP traces produced by these models were used to compare how well VEMPid, VEMPpp, and nVEMPpp tracked model inhibition depth. Applied to simulated and real VEMP data, VEMPid showed good test-retest consistency and greater sensitivity at low stimulus levels than VEMPpp or nVEMPpp. For large-amplitude responses, nVEMPpp and VEMPid were equivalent in their consistency across subjects and sessions, but for low-amplitude responses, VEMPid was superior. Unnormalized VEMPpp was always worse than nVEMPpp or VEMPid. VEMPid provides a more reliable measurement of vestibular function at low sound levels than the traditional nVEMPpp, without requiring a change in how VEMP tests are performed. The calculation method for VEMPid should be applicable whenever an ongoing muscle contraction is briefly inhibited by an external stimulus.

Research paper thumbnail of Meniere's syndrome and endolymphatic hydrops. Double-blind temporal bone study

The Annals of otology, rhinology, and laryngology, 1989

A systematic double-blind assessment of case histories and histopathologic findings in temporal b... more 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.

Research paper thumbnail of Vestibular Evoked Myogenic Potential (VEMP) in Patients With M??ni??re???s Disease With Drop Attacks

The Laryngoscope, 2006

In this retrospective study, we tested the hypothesis that vestibular evoked myogenic potential (... more In this retrospective study, we tested the hypothesis that vestibular evoked myogenic potential (VEMP) thresholds are more often elevated or absent in patients with Ménière's disease experiencing Tumarkin drop attacks than in other patients with Ménière's disease. Subjects included normal subjects (n = 14) and patients with unilateral Ménière's disease by AAO-HNS (1995) diagnostic criteria with (n = 12) and without (n = 82) Tumarkin drop attacks at a large specialty hospital otology service. VEMP threshold testing was conducted using 250, 500, and 1,000 Hz tone burst stimuli. VEMP responses were present in at all frequencies in both ears of all normal subjects. In unaffected ears of patients with unilateral Ménière's disease, VEMPs were undetectable in 13% of measurements attempted. This number rose to 18% in affected ears of patients with unilateral Ménière's disease and to 41% in Meniere ears with Tumarkin drop attacks. Frequency tuning of the VEMP response in normal subjects showed lowest thresholds at 500 Hz. In Meniere ears, the tuning was altered such that the 500-Hz thresholds were higher than the 1,000-Hz thresholds. There was a gradient of threshold elevation and altered tuning that corresponded to the gradient of worsening disease. Our findings support the hypothesis that Tumarkin drop attacks arise from advanced disease involving the saccule and that VEMP may be a clinically valuable metric of disease severity or progression in patients with Ménière's disease.

Research paper thumbnail of Chronic Otitis Media

Evidence-Based Otolaryngology, 2008

ABSTRACT A computerized PubMed search of MEDLINE 1966-December 2005 was performed. The medical su... more ABSTRACT A computerized PubMed search of MEDLINE 1966-December 2005 was performed. The medical subject heading “otitis media, suppurative” was exploded and the resulting articles were cross-referenced with those mapping to the subject headings “anti-bacterial agents,” “macrolides,” “fluoroquinolones,” “clindamycin,” or “lactams,” yielding 227 publications. These articles were then reviewed to identify those that met the following inclusion criteria: 1) patient population without cholesteatoma with discharging ears through a chronic perforation, 2) intervention with topical antibiotic drops versus placebo or antiseptic drops, 3) outcome measured in terms of cessation of otorrhea. Because of the richness of the literature, randomized controlled trials (RCTs) were specifically extracted as the best evidence available. Articles in which random allocation could not be confirmed or in which other interventions were allowed were excluded. The bibliographies of the articles that met these inclusion criteria and relevant reviews were manually checked to ensure no further relevant articles could be indentified.

Research paper thumbnail of Efficacy of Tympanomastoid Surgery for Control of Infection in Active Chronic Otitis Media

The Laryngoscope, 1997

The efficacy of surgery in controlling infection in 272 tympanomastoidectomy procedures for chron... more 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. Fortyseven 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 andor 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.

Research paper thumbnail of Nucleus 22 Cochlear Implantation Results in Postmeningitic Deafness

The Laryngoscope, 1997

Cochlear implant surgery was performed on 13 patients with postmeningitic deafness (seven adults,... more 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.

Research paper thumbnail of Revision Stapedectomy: Intraoperative Findings, Results, and Review of the Literature

The Laryngoscope, 1997

Seventy-four revision stapedectomies performed consecutively over 10 years (1986 to 1995) were re... more 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.

Research paper thumbnail of Neuropathological changes in transgenic mice carrying copies of a transcriptionally activated Mos protooncogene

Proceedings of the National Academy of Sciences, 1990

Independent transgenic mouse lines carrying the mouse Mos protooncogene linked to a retroviral tr... more Independent transgenic mouse lines carrying the mouse Mos protooncogene linked to a retroviral transcriptional control sequence display behavioral abnormalities including circling, head tilting, and head bobbing. This dominant phenotype shows various degrees of penetrance in different transgenic founder animals and lines. Neuronal and axonal degeneration, gliosis, and inflammatory infiltrates are found in all transgenic mouse lines in which behavioral traits are present. Recordings of auditory-evoked potentials in mice of one of these lines demonstrate that transgenic mice are deaf; in these mice spiral ganglia degenerate and most of the cochlear hair cells are absent. By using an S1 nuclease protection assay, we have detected RNA expression of the transgene in all tissues examined and, in particular, at high levels in brain. In situ hybridization experiments show that Mos expression can be detected in specific areas of the central nervous system. Lesions are present in areas with demonstrable overexpression ofMos.

Research paper thumbnail of Normalization Reduces Intersubject Variability in Cervical Vestibular Evoked Myogenic Potentials

Otology & Neurotology, 2014

Objective: Cervical vestibular evoked myogenic potentials are used to assess saccular and inferio... more Objective: Cervical vestibular evoked myogenic potentials are used to assess saccular and inferior vestibular nerve function. Normalization of the VEMP waveform has been proposed to reduce the variability in vestibular evoked myogenic potentials by correcting for muscle activation. In this study, we test the hypothesis that normalization of the raw cervical VEMP waveform causes a significant decrease in the intersubject variability. Study Design: Prospective cohort study. Setting: Large specialty hospital, department of otolaryngology. Subjects: Twenty healthy subjects were used in this study. Intervention: All subjects underwent cervical vestibular evoked myogenic potential testing using short tone bursts at 250, 500, 750, and 1,000 Hz. Both intersubject and intrasubject variability was assessed. Main Outcome Measures: Variability between raw and normalized peak-to-peak amplitudes was compared using the coefficient of variation. Intrasubject variability was assessed

Research paper thumbnail of Vestibular Evoked Myogenic Potentials Show Altered Tuning in Patients with M??ni??re???s Disease

Otology & Neurotology, 2004

Research paper thumbnail of Vestibular Evoked Myogenic Potentials versus Vestibular Test Battery in Patients with Ménière’s Disease

Otology & Neurotology, 2004

The present study was undertaken to assess the sensitivity of vestibular evoked myogenic potentia... more The present study was undertaken to assess the sensitivity of vestibular evoked myogenic potentials testing to side-of-disease in Ménière's disease patients and to test the hypothesis that information supplied by vestibular evoked myogenic potentials is complementary to that provided by a conventional vestibular test battery. Study Design: Prospective cohort study. Setting: Large specialty hospital, department of otolaryngology. Subjects: Twenty consenting adults (9 men and 11 women) with unilateral Ménière's disease by American Academy of Otolaryngology-Head and Neck Surgery diagnostic criteria. Interventions: All subjects underwent bilateral vestibular evoked myogenic potentials testing using ipsilateral broadband click and short-toneburst stimuli at 250, 500, and 1,000 Hz. All subjects also underwent electronystagmography and sinusoidal vertical axis rotation testing. Main Outcome Measures: Accuracy of side-of-disease assignment by vestibular evoked myogenic potentials, caloric asymmetry, and multivariate analysis. Results: Side-of-disease assignment was most accurate using caloric asymmetry with a 5% interaural difference criterion, achieving 85% correct assignment. The next best method was

Research paper thumbnail of NATIONAL DEAFNESS AND OTHER COMMUNICATION DISORDERS ADVISORY COUNCIL

____________________________________ 1For the record, it is noted that members absent themselves ... more ____________________________________ 1For the record, it is noted that members absent themselves from the meeting when the Council is discussing applications (a) from their respective institutions or (b) in which a real or apparent conflict of interest might occur. This procedure applies only to individual discussion of an application and not to "en bloc" actions.

Research paper thumbnail of Measurements of stapes velocity in live human ears

Hearing Research, 2009

Sound-induced stapes velocity (Vs) was measured intraoperatively in 14 patients undergoing cochle... more Sound-induced stapes velocity (Vs) was measured intraoperatively in 14 patients undergoing cochlear implantation. All 14 patients had no history of middle-ear pathology, and their ossicular chains appeared normal on intraoperative inspection and palpation. The magnitude of the mean Vs (normalized by simultaneously-measured ear-canal sound pressure) was stiffness-dominated at frequencies below 1 kHz, increased up to ~4 kHz, and then decreased at higher frequencies. The phase of the mean velocity was +0.2 periods at 0.3 kHz, and gradually became a phase lag at higher frequencies. The mean Vs measured in this study was similar to that of seven ears reported in the only other published study of live human measurements . We also made measurements of Vs in fresh cadaveric temporal bones using a technique identical to that used in live ears, including similar measurement angles and location. The mean Vs measured in the cadaveric ears under these conditions was similar to the mean Vs measurements in the 14 live ears. This indicates that middle-ear mechanics are similar in live and cadaveric ears. In addition, interspecies comparisons were made between our live human Vs and the Vs reported in different animal studies. There were some clear similarities in Vs across species, as well as differences. The primary interspecies differences were in the magnitude of the Vs as well as in the frequency of transitions in the magnitudes' frequency dependence from rising to flat or falling.

Research paper thumbnail of P32-17 Sources of amplitude variability in vestibular evoked myogenic potential (VEMP): a model-based study

Clinical Neurophysiology, 2010

Research paper thumbnail of The National Temporal Bone, Hearing, and Balance Pathology Resource Registry

Archives of Otolaryngology - Head and Neck Surgery, 1993

The National Temporal Bone, Hearing, and Balance Pathology Resource Registry has been established... more 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.

Research paper thumbnail of Ménière's disease

European Archives of Oto-rhino-laryngology, 1995

There now exists a vast literature on Ménìere's disease. In this review, we examine closely t... more There now exists a vast literature on Ménìere'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 paper thumbnail of Vestibular evoked myogenic potentials in patients with superior semicircular canal dehiscence

Otology & neurotology: official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology

To compare the audiometric and cervical vestibular evoked muscle potential (cVEMP) outcomes of ea... more To compare the audiometric and cervical vestibular evoked muscle potential (cVEMP) outcomes of ears with superior canal dehiscence (SSCD) to outcomes from normal ears and the unaffected ears of unilateral SSCD patients. Retrospective review of clinical testing outcomes. Tertiary care specialty teaching hospital. Three subject groups were used: 21 ears of healthy subjects, ages 21 to 52 years; 58 affected SSCD ears in patients, ages 15 to 65 years; and 26 unaffected ears of unilateral SSCD patients, ages 15 to 65 years. Results of pure-tone audiometry and cervical vestibular evoked muscle potential (cVEMP) testing. SSCD ears differed from normal ears or unaffected ears of unilateral SSCD patients in 2 ways: 1) they had significantly larger air-bone gaps at 250 and 500 Hz, resulting in poorer air-conduction thresholds at those frequencies, and 2) their cVEMP thresholds at 500 Hz were significantly lower. When the cVEMP threshold was adjusted for the size of the air-bone gap on the aud...

Research paper thumbnail of Vestibular evoked myogenic potentials (VEMP) can detect asymptomatic saccular hydrops

The Laryngoscope, 2006

The objective of this study was to explore the useful of vestibular evoked myogenic potential (VE... more The objective of this study was to explore the useful of vestibular evoked myogenic potential (VEMP) testing for detecting endolymphatic hydrops, especially in the second ear of patients with unilateral Ménière disease (MD). This study was performed at a tertiary care academic medical center. Part I consisted of postmortem temporal bone specimens from the temporal bone collection of the Massachusetts Eye & Ear Infirmary; part II consisted of consecutive consenting adult patients (n = 82) with unilateral MD by American Academy of Otolaryngology-Head and Neck Surgery criteria case histories. Outcome measures consisted of VEMP thresholds in patients and histologic saccular endolymphatic hydrops in postmortem temporal bones. Saccular hydrops was observed in the asymptomatic ear in six of 17 (35%) of temporal bones from donors with unilateral MD. Clinic patients with unilateral MD showed elevated mean VEMP thresholds and altered VEMP tuning in their symptomatic ears and, to a lesser degr...

Research paper thumbnail of Results after revision stapedectomy with malleus grip prosthesis

The Annals of otology, rhinology, and laryngology, 2006

Revision stapedectomy with a malleus grip prosthesis is a technically challenging otologic proced... more Revision stapedectomy with a malleus grip prosthesis is a technically challenging otologic procedure. The prosthesis is usually longer and extends deeper into the vestibule than a conventional stapes prosthesis, creating the potential to affect the vestibular sense organs. The prosthesis also bypasses the ossicular joints, which are thought to play a role in protecting the inner ear from large changes in static pressure within the middle ear. The prosthesis is in close proximity to the tympanic membrane, thus increasing the risk for its extrusion. We reviewed our experience with revision stapedectomy with the Schuknecht Teflon-wire malleus grip prosthesis in 36 ears with a mean follow-up of 23 months. The air-bone gap was closed to within 10 dB in 16 ears (44%) and to within 20 dB in 26 ears (72%). The incidence of postoperative sensorineural hearing loss was 8% (3 ears). There were no dead ears. Extrusion of the prosthesis occurred in 1 case (3%). Nearly 50% of patients reported va...

Research paper thumbnail of Temporal bone studies of the human peripheral vestibular system. Normative vestibular hair cell data

The Annals of otology, rhinology & laryngology. Supplement, 2000

Quantitative studies of the vestibular system with serially sectioned human temporal bones have b... more Quantitative studies of the vestibular system with serially sectioned human temporal bones have been limited because of difficulty in distinguishing hair cells from supporting cells and type I from type II hair cells. In addition, there is only a limited amount of normative data available regarding vestibular hair cell counts in humans. In this study, archival temporal bone sections were examined by Nomarski (differential interference contrast) microscopy, which permitted visualization of the cuticular plate and stereociliary bundle so as to allow unambiguous identification of hair cells. The density of type I, type II, and total numbers of vestibular hair cells in each of the 5 sense organs was determined in a set of 67 normal temporal bones that ranged from birth through 100 years of age. The mean total densities at birth were 76 to 79 cells per 0.01 mm2 in the cristae, 68 cells per 0.01 mm2 in the utricle, and 61 cells per 0.01 mm2 in the saccule. The ratio of type I to type II h...

Research paper thumbnail of Evaluating Inhibition of Motoneuron Firing From Electromyogram Data to Assess Vestibular Output Using Vestibular Evoked Myogenic Potentials

Ear and Hearing, 2015

Vestibular evoked myogenic potentials (VEMPs) are due to vestibular responses producing brief inh... more Vestibular evoked myogenic potentials (VEMPs) are due to vestibular responses producing brief inhibitions of muscle contractions that are detectable in electromyographic (EMG) responses. VEMP amplitudes are traditionally measured by the peak to peak amplitude of the averaged EMG response (VEMPpp) or by a normalized VEMPpp (nVEMPpp). However, a brief EMG inhibition does not satisfy the statistical assumptions for the average to be the optimal processing strategy. Here, it is postulated that the inhibition depth of motoneuron firing is the desired metric for showing the influence of the vestibular system on the muscle system. The authors present a metric called "VEMPid" that estimates this inhibition depth from the EMG data obtained in a usual VEMP data acquisition. The goal of this article was to compare how well VEMPid, VEMPpp, and nVEMPpp track inhibition depth. To find a robust method to compare VEMPid, VEMPpp, and nVEMPpp, realistic physiological models for the inhibition of VEMP EMG signals were made using VEMP data from four measurement sessions on each of the five normal subjects. Each of the resulting 20 EMG-production models was adjusted to match the EMG autocorrelation of an individual subject and session. Simulated VEMP traces produced by these models were used to compare how well VEMPid, VEMPpp, and nVEMPpp tracked model inhibition depth. Applied to simulated and real VEMP data, VEMPid showed good test-retest consistency and greater sensitivity at low stimulus levels than VEMPpp or nVEMPpp. For large-amplitude responses, nVEMPpp and VEMPid were equivalent in their consistency across subjects and sessions, but for low-amplitude responses, VEMPid was superior. Unnormalized VEMPpp was always worse than nVEMPpp or VEMPid. VEMPid provides a more reliable measurement of vestibular function at low sound levels than the traditional nVEMPpp, without requiring a change in how VEMP tests are performed. The calculation method for VEMPid should be applicable whenever an ongoing muscle contraction is briefly inhibited by an external stimulus.

Research paper thumbnail of Meniere's syndrome and endolymphatic hydrops. Double-blind temporal bone study

The Annals of otology, rhinology, and laryngology, 1989

A systematic double-blind assessment of case histories and histopathologic findings in temporal b... more 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.

Research paper thumbnail of Vestibular Evoked Myogenic Potential (VEMP) in Patients With M??ni??re???s Disease With Drop Attacks

The Laryngoscope, 2006

In this retrospective study, we tested the hypothesis that vestibular evoked myogenic potential (... more In this retrospective study, we tested the hypothesis that vestibular evoked myogenic potential (VEMP) thresholds are more often elevated or absent in patients with Ménière's disease experiencing Tumarkin drop attacks than in other patients with Ménière's disease. Subjects included normal subjects (n = 14) and patients with unilateral Ménière's disease by AAO-HNS (1995) diagnostic criteria with (n = 12) and without (n = 82) Tumarkin drop attacks at a large specialty hospital otology service. VEMP threshold testing was conducted using 250, 500, and 1,000 Hz tone burst stimuli. VEMP responses were present in at all frequencies in both ears of all normal subjects. In unaffected ears of patients with unilateral Ménière's disease, VEMPs were undetectable in 13% of measurements attempted. This number rose to 18% in affected ears of patients with unilateral Ménière's disease and to 41% in Meniere ears with Tumarkin drop attacks. Frequency tuning of the VEMP response in normal subjects showed lowest thresholds at 500 Hz. In Meniere ears, the tuning was altered such that the 500-Hz thresholds were higher than the 1,000-Hz thresholds. There was a gradient of threshold elevation and altered tuning that corresponded to the gradient of worsening disease. Our findings support the hypothesis that Tumarkin drop attacks arise from advanced disease involving the saccule and that VEMP may be a clinically valuable metric of disease severity or progression in patients with Ménière's disease.

Research paper thumbnail of Chronic Otitis Media

Evidence-Based Otolaryngology, 2008

ABSTRACT A computerized PubMed search of MEDLINE 1966-December 2005 was performed. The medical su... more ABSTRACT A computerized PubMed search of MEDLINE 1966-December 2005 was performed. The medical subject heading “otitis media, suppurative” was exploded and the resulting articles were cross-referenced with those mapping to the subject headings “anti-bacterial agents,” “macrolides,” “fluoroquinolones,” “clindamycin,” or “lactams,” yielding 227 publications. These articles were then reviewed to identify those that met the following inclusion criteria: 1) patient population without cholesteatoma with discharging ears through a chronic perforation, 2) intervention with topical antibiotic drops versus placebo or antiseptic drops, 3) outcome measured in terms of cessation of otorrhea. Because of the richness of the literature, randomized controlled trials (RCTs) were specifically extracted as the best evidence available. Articles in which random allocation could not be confirmed or in which other interventions were allowed were excluded. The bibliographies of the articles that met these inclusion criteria and relevant reviews were manually checked to ensure no further relevant articles could be indentified.

Research paper thumbnail of Efficacy of Tympanomastoid Surgery for Control of Infection in Active Chronic Otitis Media

The Laryngoscope, 1997

The efficacy of surgery in controlling infection in 272 tympanomastoidectomy procedures for chron... more 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. Fortyseven 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 andor 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.

Research paper thumbnail of Nucleus 22 Cochlear Implantation Results in Postmeningitic Deafness

The Laryngoscope, 1997

Cochlear implant surgery was performed on 13 patients with postmeningitic deafness (seven adults,... more 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.

Research paper thumbnail of Revision Stapedectomy: Intraoperative Findings, Results, and Review of the Literature

The Laryngoscope, 1997

Seventy-four revision stapedectomies performed consecutively over 10 years (1986 to 1995) were re... more 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.

Research paper thumbnail of Neuropathological changes in transgenic mice carrying copies of a transcriptionally activated Mos protooncogene

Proceedings of the National Academy of Sciences, 1990

Independent transgenic mouse lines carrying the mouse Mos protooncogene linked to a retroviral tr... more Independent transgenic mouse lines carrying the mouse Mos protooncogene linked to a retroviral transcriptional control sequence display behavioral abnormalities including circling, head tilting, and head bobbing. This dominant phenotype shows various degrees of penetrance in different transgenic founder animals and lines. Neuronal and axonal degeneration, gliosis, and inflammatory infiltrates are found in all transgenic mouse lines in which behavioral traits are present. Recordings of auditory-evoked potentials in mice of one of these lines demonstrate that transgenic mice are deaf; in these mice spiral ganglia degenerate and most of the cochlear hair cells are absent. By using an S1 nuclease protection assay, we have detected RNA expression of the transgene in all tissues examined and, in particular, at high levels in brain. In situ hybridization experiments show that Mos expression can be detected in specific areas of the central nervous system. Lesions are present in areas with demonstrable overexpression ofMos.

Research paper thumbnail of Normalization Reduces Intersubject Variability in Cervical Vestibular Evoked Myogenic Potentials

Otology & Neurotology, 2014

Objective: Cervical vestibular evoked myogenic potentials are used to assess saccular and inferio... more Objective: Cervical vestibular evoked myogenic potentials are used to assess saccular and inferior vestibular nerve function. Normalization of the VEMP waveform has been proposed to reduce the variability in vestibular evoked myogenic potentials by correcting for muscle activation. In this study, we test the hypothesis that normalization of the raw cervical VEMP waveform causes a significant decrease in the intersubject variability. Study Design: Prospective cohort study. Setting: Large specialty hospital, department of otolaryngology. Subjects: Twenty healthy subjects were used in this study. Intervention: All subjects underwent cervical vestibular evoked myogenic potential testing using short tone bursts at 250, 500, 750, and 1,000 Hz. Both intersubject and intrasubject variability was assessed. Main Outcome Measures: Variability between raw and normalized peak-to-peak amplitudes was compared using the coefficient of variation. Intrasubject variability was assessed

Research paper thumbnail of Vestibular Evoked Myogenic Potentials Show Altered Tuning in Patients with M??ni??re???s Disease

Otology & Neurotology, 2004

Research paper thumbnail of Vestibular Evoked Myogenic Potentials versus Vestibular Test Battery in Patients with Ménière’s Disease

Otology & Neurotology, 2004

The present study was undertaken to assess the sensitivity of vestibular evoked myogenic potentia... more The present study was undertaken to assess the sensitivity of vestibular evoked myogenic potentials testing to side-of-disease in Ménière's disease patients and to test the hypothesis that information supplied by vestibular evoked myogenic potentials is complementary to that provided by a conventional vestibular test battery. Study Design: Prospective cohort study. Setting: Large specialty hospital, department of otolaryngology. Subjects: Twenty consenting adults (9 men and 11 women) with unilateral Ménière's disease by American Academy of Otolaryngology-Head and Neck Surgery diagnostic criteria. Interventions: All subjects underwent bilateral vestibular evoked myogenic potentials testing using ipsilateral broadband click and short-toneburst stimuli at 250, 500, and 1,000 Hz. All subjects also underwent electronystagmography and sinusoidal vertical axis rotation testing. Main Outcome Measures: Accuracy of side-of-disease assignment by vestibular evoked myogenic potentials, caloric asymmetry, and multivariate analysis. Results: Side-of-disease assignment was most accurate using caloric asymmetry with a 5% interaural difference criterion, achieving 85% correct assignment. The next best method was

Research paper thumbnail of NATIONAL DEAFNESS AND OTHER COMMUNICATION DISORDERS ADVISORY COUNCIL

____________________________________ 1For the record, it is noted that members absent themselves ... more ____________________________________ 1For the record, it is noted that members absent themselves from the meeting when the Council is discussing applications (a) from their respective institutions or (b) in which a real or apparent conflict of interest might occur. This procedure applies only to individual discussion of an application and not to "en bloc" actions.

Research paper thumbnail of Measurements of stapes velocity in live human ears

Hearing Research, 2009

Sound-induced stapes velocity (Vs) was measured intraoperatively in 14 patients undergoing cochle... more Sound-induced stapes velocity (Vs) was measured intraoperatively in 14 patients undergoing cochlear implantation. All 14 patients had no history of middle-ear pathology, and their ossicular chains appeared normal on intraoperative inspection and palpation. The magnitude of the mean Vs (normalized by simultaneously-measured ear-canal sound pressure) was stiffness-dominated at frequencies below 1 kHz, increased up to ~4 kHz, and then decreased at higher frequencies. The phase of the mean velocity was +0.2 periods at 0.3 kHz, and gradually became a phase lag at higher frequencies. The mean Vs measured in this study was similar to that of seven ears reported in the only other published study of live human measurements . We also made measurements of Vs in fresh cadaveric temporal bones using a technique identical to that used in live ears, including similar measurement angles and location. The mean Vs measured in the cadaveric ears under these conditions was similar to the mean Vs measurements in the 14 live ears. This indicates that middle-ear mechanics are similar in live and cadaveric ears. In addition, interspecies comparisons were made between our live human Vs and the Vs reported in different animal studies. There were some clear similarities in Vs across species, as well as differences. The primary interspecies differences were in the magnitude of the Vs as well as in the frequency of transitions in the magnitudes' frequency dependence from rising to flat or falling.

Research paper thumbnail of P32-17 Sources of amplitude variability in vestibular evoked myogenic potential (VEMP): a model-based study

Clinical Neurophysiology, 2010

Research paper thumbnail of The National Temporal Bone, Hearing, and Balance Pathology Resource Registry

Archives of Otolaryngology - Head and Neck Surgery, 1993

The National Temporal Bone, Hearing, and Balance Pathology Resource Registry has been established... more 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.