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Biomedical subjects

Steven D Rauch

Publications and source records attributed to Steven D Rauch.

13 recordsLinked to original sources

Using audiometric thresholds and word recognition in a treatment study.

OBJECTIVES: First, to examine a possible limit on significant results imposed by a progressive floor effect for hearing threshold improvement in a treatment study. This floor effect for hearing recovery suggests that if inclusion criteria are not set sufficiently high, the superiority of a treatment group may not be detectable. Second, to examine the outcomes when using two different types of criteria for significant change in a subject's word recognition score. METHODS: Several single-number criteria (e.g., 15 percentage points) are compared with the 95% (p=0.05) criteria from the binomial critical difference table for monosyllables. Critical differences for binomial variables change depending on whether the starting value lies in the middle (near 50% correct) or at either extreme of the range of scores (0 or 100%). Different judgments of significant word recognition improvement (or decrease) using binomial versus single-value criteria are presented. DATA SOURCE: A recent treatment study of sudden sensorineural hearing loss (n=318) is used to illustrate these effects. CONCLUSION: First, there is a progressive floor effect of presenting severity that co-varies with the outcome measure hearing threshold recovery. In some designs, this may act to constrain the ability to detect a significant difference. Second, in the example data set, the use of single-value criteria for significant within-subject change in word recognition (e.g., 15 percentage points) introduced a miscategorization error rate of approximately 9% when compared with the result of the binomial 95% critical difference table.

Audiometry, Pure-Tone↗

Vestibular evoked myogenic potential (VEMP) in patients with Ménière's disease with drop attacks.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Acoustic Stimulation↗

Vestibular evoked myogenic potentials (VEMP) can detect asymptomatic saccular hydrops.

OBJECTIVE: 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). METHODS: 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. RESULTS: 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 degree, in their asymptomatic ears. Specific VEMP frequency and tuning criteria were used to define a "Ménière-like" response. This "Ménière-like" response was seen in 27% of asymptomatic ears of our patients with unilateral MD. CONCLUSIONS: Bilateral involvement is seen in approximately one third of MD cases. Saccular hydrops appears to precede symptoms in bilateral MD. Changes in VEMP threshold and tuning appear to be sensitive to these structural changes in the saccule. If so, then VEMP may be useful as a detector of asymptomatic saccular hydrops and as a predictor of evolving bilateral MD.

Acoustic Stimulation↗

Vestibular evoked myogenic potentials.

PURPOSE OF REVIEW: Vestibular evoked myogenic potential testing is the only clinically feasible way to measure function of the saccule. Interest in this test has exploded in recent years because of its potential utility in diagnosing third-window disorders and in diagnosing and monitoring Ménière's disease. RECENT FINDINGS: Recent literature on vestibular evoked myogenic potential covers a wide range of topics. Review articles, method articles and many case reports and uncontrolled case series are seeking new applications for vestibular evoked myogenic potential in diagnosis and monitoring of neurotologic disease, and in shedding light on inner ear diseases by mapping anatomic sites of involvement. The most informative work is still in the areas of superior semicircular canal dehiscence and in Ménière's disease. Also, many aspects of vestibular evoked myogenic potential and its use have not yet been adequately studied or described. SUMMARY: Vestibular evoked myogenic potential is a new test of saccular and inferior vestibular nerve integrity. It holds great promise for diagnosing and monitoring Ménière's disease and some other neurotologic disorders. It is still an evolving field, however. The methods, equipment, and applications for vestibular evoked myogenic potential testing are not yet standardized. It is not yet time for this test to be widely applied, but that time is not far off.

Acoustic Stimulation↗

Results after revision stapedectomy with malleus grip prosthesis.

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 various degrees of vertigo or disequilibrium during the first 3 weeks after surgery. These vestibular symptoms resolved by 6 weeks in all but 1 case. We did not find evidence of damage to the inner ear due to the length of the prosthesis or due to the potential for direct transmission of changes in static pressures within the middle ear to the labyrinth. Our results are similar to those published in the literature for malleus attachment stapedectomy and conventional revision incus stapedectomy.

Adult↗

Serial audiometry in a clinical trial of AIED treatment.

OBJECTIVE: We analyzed pure-tone and speech audiometric results from a prospective trial of anti-inflammatory treatment of subjects with active autoimmune inner ear disease (AIED). We sought to characterize the pattern and size of the treatment effect as reflected in clinical audiometry and to identify audiometric predictors of response to steroid treatment of AIED. SUBJECTS: Adult participants demonstrated clinically established criteria for AIED (n = 116). Eligibility required audiometric evidence of active AIED as indicated by idiopathic sensorineural hearing loss with threshold elevations within 3 months of enrollment. METHODS: We evaluated audiometric changes after 4 weeks of treatment with pharmacologic doses (60 mg/day) of prednisone. We examined the relationship between audiometric pure-tone thresholds at baseline and changes in word intelligibility score (WIS) using parametric and nonparametric analyses. Magnitudes of change were assessed using independent or paired t-tests. Separate analyses were performed on subgroups that did or did not show improved WIS score with steroid treatment. RESULTS: Overall mean pure-tone averages improved from baseline to closeout of prednisone treatment in better hearing ears from 52.4 to 48.3 dB (p < .0001). Mean WIS improved in the better ear from 71.4% to 78.1% (p < .0001). Of pure-tone measures, only the six-tone average showed significant correlation with both the absolute improvements in WIS and with the percentage change in WIS after treatment. Individual frequencies at baseline showed no significant relationship with changes in WIS score after treatment. In 69 (59.5%) of 116 subjects, WIS improved (range, 2-80%) in the better ear. In these subjects, the baseline pure-tone thresholds and pure-tone averages correlated significantly and positively with improvement in WIS. CONCLUSIONS: Steroid treatment in AIED-mediated hearing loss produce variable but significant hearing gains. Neither a focal, cochleotopic region of greatest vulnerability to AIED nor frequency-specific amenability to treatment were evident. We did observe that analysis of predictors and the degree of treatment effect vary with different approaches to measuring change in the WIS. Depending on the approach adopted, the size of the treatment effect may be greatest across intermediate hearing levels at baseline. These observations offer an audiometric database that may enable greater precision in judging clinically meaningful parameters for future studies of AIED treatment and other interventions for sensorineural hearing loss.

Adolescent↗

Intratympanic steroids for sensorineural hearing loss.

Corticosteroids are standard treatment for a number of inflammatory and immune-mediated inner ear diseases. In recent years there has been growing interest in intratympanic administration of steroids as a means of achieving high inner ear drug concentration and low risk of systemic side effects. Idiopathic sudden sensorineural hearing loss seems to be the condition with the greatest potential for both study and clinical use of intratympanic steroid treatment. Despite promising preliminary observations, there re-main many unanswered questions about this treatment modality.

Administration, Oral↗

Superior semicircular canal dehiscence presenting as conductive hearing loss without vertigo.

OBJECTIVE: The objective of this study was to describe superior semicircular canal dehiscence (SSCD) presenting as otherwise unexplained conductive hearing loss without vestibular symptoms. STUDY DESIGN: Retrospective. SETTING: Tertiary referral center. PATIENTS: The study comprised 8 patients (10 ears), 5 males and 5 females aged 27 to 59 years. All 10 ears had SSCD on high-resolution computed tomography scan of the temporal bone. DIAGNOSTIC TESTS AND RESULTS: All 10 ears had significant conductive hearing loss. The air-bone gaps were largest in the lower frequencies at 250, 500, and 1000 Hz; the mean gaps for these 3 frequencies for the 10 ears were 49, 37, and 35 dB, respectively. Bone-conduction thresholds below 2000 Hz were negative (-5 dB to -15 dB) at one or more frequencies in 8 of the 10 ears. There were no middle ear abnormalities to explain the air-bone gaps in these 10 ears. Computed tomography scan and laboratory testing indicated lack of middle ear pathology; acoustic reflexes were present, vestibular evoked myogenic potentials (VEMPs) were present with abnormally low thresholds, and umbo velocity measured by laser Doppler vibrometry was above mean normal. Middle ear exploration was negative in six ears; of these six, stapedectomy had been performed in three ears and ossiculoplasty in two ears, but the air-bone gap was unchanged postoperatively. The data are consistent with the hypothesis that the SSCD introduced a third mobile window into the inner ear, which in turn produced the conductive hearing loss by 1) shunting air-conducted sound away from the cochlea, thus elevating air-conduction thresholds; and 2) increasing the difference in impedance between the oval and round windows, thus improving thresholds for bone-conducted sound. CONCLUSION: SSCD can present with a conductive hearing loss that mimics otosclerosis and could explain some cases of persistent conductive hearing loss after uneventful stapedectomy. Audiometric testing with attention to absolute bone-conduction thresholds, acoustic reflex testing, VEMP testing, laser vibrometry of the umbo, and computed tomograph scanning can help to identify patients with SSCD presenting with conductive hearing loss without vertigo.

Acoustic Impedance Tests↗

Vestibular evoked myogenic potentials show altered tuning in patients with Ménière's disease.

OBJECTIVE: Acoustic stimulation of the saccule gives rise to a vestibulocollic reflex, the output of which can be measured in the neck as inhibition of activity in the ipsilateral sternocleidomastoid muscle. This vestibular evoked myogenic potential has been promoted as a means of assessing integrity of saccular function. In this study, we test the hypothesis that the cochleosaccular hydrops of Ménière's syndrome leads to alterations in saccular motion that change the dynamics of the vestibular evoked myogenic potential. STUDY DESIGN: Prospective cohort study. SETTING: Large specialty hospital, department of otolaryngology. SUBJECTS: Fourteen normal adult volunteers and 34 consecutive consenting adult patients with unilateral Ménière's disease by American Academy of Otolaryngology-Head and Neck Surgery diagnostic criteria. INTERVENTIONS: All subjects underwent vestibular evoked myogenic potential testing using ipsilateral broadband click and short tone-burst stimuli at 250, 500, 1,000, 2,000, and 4,000 Hz. MAIN OUTCOME MEASURES: Threshold, amplitude, and latency of vestibular evoked myogenic potential responses in normal and Ménière's affected and unaffected ears. RESULTS: Vestibular evoked myogenic potential was present in all ears tested. Normal subjects show a frequency-dependent vestibular evoked myogenic potential threshold, with best response ("frequency tuning") at 500 Hz. Compared with normal subjects and unaffected ears of Ménière's subjects, affected Ménière's ears had significantly increased vestibular evoked myogenic potential thresholds. Affected Ménière's ears showed threshold shifts at all frequencies and there was less tuning apparent at 500 Hz. Unaffected ears of Ménière's subjects also showed significantly elevated vestibular evoked myogenic potential thresholds compared with normal subjects. Analyses of vestibular evoked myogenic potential thresholds for effects of age, hearing loss, and audiometric configuration showed no significant differences. CONCLUSIONS: Ménière's ears display alterations in vestibular evoked myogenic potential threshold and tuning, supporting our hypothesis of altered saccular motion mechanics arising from hydropic distention. Unaffected ears of unilateral Ménière's subjects show similar changes, though to a lesser degree. This finding may be because of occult saccular hydrops in the asymptomatic ear or binaural interactions in the vestibular evoked myogenic potential otolith-cervical reflex arc.

Acoustic Stimulation↗

Vestibular evoked myogenic potentials versus vestibular test battery in patients with Meniere's disease.

OBJECTIVE: The present study was undertaken to assess the sensitivity of vestibular evoked myogenic potentials testing to side-of-disease in Meniere'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 Meniere'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 vestibular evoked myogenic potentials using 250-Hz toneburst stimuli, achieving 80% correct assignment. The least accurate method was caloric asymmetry using a traditional 30% interaural difference limen, achieving 55% correct assignment. Comparison of 5% interaural difference criterion and vestibular evoked myogenic potentials using 250-Hz toneburst stimuli showed discordant results, but in no case did both 5% interaural difference criterion and vestibular evoked myogenic potentials using 250-Hz toneburst stimuli make an incorrect assignment. CONCLUSION: Vestibular evoked myogenic potentials threshold was shown to be highly sensitive to side-of-disease in unilateral Meniere's disease. We observed instances of discordance in side-of-disease assignment by caloric asymmetry and vestibular evoked myogenic potential methods but no case in which both methods were incorrect. This supports the hypothesis that vestibular evoked myogenic potentials supplies information complementary to that provided by other components of the vestibular test battery.

Adult↗

Treatment of corticosteroid-responsive autoimmune inner ear disease with methotrexate: a randomized controlled trial.

CONTEXT: A number of therapies have been proposed for the long-term management of corticosteroid-responsive, rapidly progressive, bilateral sensorineural hearing loss (autoimmune inner ear disease [AIED]). Methotrexate has emerged as the benchmark agent but has not been rigorously evaluated for hearing improvement in patients with AIED. OBJECTIVE: To assess the efficacy of long-term methotrexate in maintaining hearing improvements achieved with glucocorticoid (prednisone) therapy in patients with AIED. DESIGN, SETTING, AND PARTICIPANTS: A randomized, double-blind, placebo-controlled trial conducted from February 3, 1998, to November 5, 2001, of 67 patients with rapidly progressive, bilateral sensorineural hearing loss at 10 tertiary care centers in the United States. INTERVENTION: Randomization to either oral methotrexate (15 to 20 mg/wk; n = 33) or placebo (n = 34), in combination with an 18-week prednisone taper. Follow-up examinations, including audiometric evaluation, were performed at 4, 8, 12, 24, 36, 48, and 52 weeks, or until hearing loss was documented. MAIN OUTCOME MEASURE: Maintenance of hearing improvement achieved from prednisone treatment. RESULTS: Sixty-seven patients (57.8%) enrolled in the prednisone challenge experienced hearing improvement. Twenty-five patients (37%) experienced hearing improvements in both ears. Of the individuals who reached study end points, 24 (80%) of 30 end points were because of measured hearing loss in the methotrexate group and 29 (93.5%) of 31 end points were because of measured hearing loss in the placebo group (P =.15). Methotrexate was no more effective than placebo in maintaining the hearing improvement achieved with prednisone treatment (hazard ratio, 1.31; 95% confidence interval, 0.79-2.17; P =.30). CONCLUSION: Methotrexate does not appear to be effective in maintaining the hearing improvement achieved with prednisone therapy in patients with AIED.

Audiometry↗

A practical assessment algorithm for diagnosis of dizziness.

OBJECTIVE: We sought to test a 3-parameter model for diagnosis of dizziness based on the type and temporal characteristics of the dizziness and on hearing status. STUDY DESIGN AND SETTING: We conducted a prospective blinded study at a tertiary referral neurotology practice. Before examination, patients completed a questionnaire reporting type and timing of dizziness symptoms and hearing status. Clinical diagnoses were compared with questionnaire results. RESULTS: Fifty-seven patients completed the questionnaire. We were able to correctly classify 21 (60%) of the 35 subjects who had a common otogenic cause of vertigo by the diagnostic algorithm. CONCLUSION: A simple classification of dizziness by type, timing, and hearing status can be self-reported by patients using a brief questionnaire. This classification scheme is as good as others of much greater complexity. SIGNIFICANCE: The simple classification scheme reported here is based on history alone and facilitates triage of dizzy patients into diagnostic groups for work-up and management.

Adult↗

Oral steroid treatment of sudden sensorineural hearing loss: a ten year retrospective analysis.

OBJECTIVE: To describe ten years of experience with Sudden Sensorineural Hearing Loss and compare the outcomes with and without treatment with oral corticosteroids. STUDY DESIGN: Retrospective review of medical records. SETTING: Large specialty hospital, Department of Otolaryngology. PATIENTS: Patients presenting with sudden onset (72 hours) unilateral sensorineural hearing loss, with no evidence of Ménière's Disease, acoustic injury, retrocochlear disease, and other specifiable disorders. INTERVENTIONS: The majority of patients received a standard course of oral corticosteroids (Prednisone 60 mg and taper). A smaller group declined treatment. MAIN OUTCOME MEASURES: Recovery of hearing sensitivity was measured using standard audiometry and reported as change in Pure Tone Average. Word recognition scores were also analyzed. RESULTS: When severe-to-profound cases are analyzed, a significant improvement (p <.01) in Pure Tone Average is seen in cases treated with steroids versus those untreated. When milder cases are included, a statistical floor effect prevents differentiation of these groups. Word recognition scores were significantly improved (p <.05) in the treated group. CONCLUSIONS: Application of steroid medication significantly improves the recovery outcomes in cases of Severe Sudden Sensorineural Hearing Loss.

Administration, Oral↗