Osteoma of the malleus.
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Biomedical subjects
Publications and source records attributed to D B Kamerer.
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OBJECTIVE: To evaluate the efficacy and toxicity of intratympanic gentamicin therapy for the treatment of Meniere's disease. STUDY DESIGN: We compared retrospective case series with historical controls. SETTING: A tertiary referral center--the ambulatory clinic at The University of Pittsburgh Medical Center. PATIENTS: The study consisted of 28 patients with Meniere's disease who failed conventional medical therapy and were offered gentamicin treatment or surgical intervention. Patients had to be followed for at least 2 years to be eligible for review. INTERVENTION: Intratympanic gentamicin solution injections were given by serial titration to the involved ear on a weekly or biweekly schedule. MAIN OUTCOME MEASURES: Adhering to the American Academy of Otolaryngology--Head and Neck Surgery 1985 criteria for reporting treatment results in Meniere's disease, the hearing status, control of vertigo, and disability scores using intratympanic gentamicin therapy were reported and compared with historical controls. RESULTS: Complete or substantial control of vertigo was achieved in 91% of patients. Hearing loss occurred in approximately one third of patients. Historically, similar hearing loss occurs in patients who were treated successfully, either medically or surgically. Ototoxicity (high frequency hearing loss) was more likely to be seen in patients with pre-treatment 8,000 Hz hearing thresholds better than 65dB. CONCLUSIONS: Intratympanic gentamicin therapy given by serial titration injections provides significant control of vertigo without the significant cost and potential morbidity of a more invasive surgical procedure.
OBJECTIVE: To examine the utility of distortion-product otoacoustic emissions (DPOAEs) in the assessment of the nonorganic suspect because DPOAE analysis constitutes an objective test of hair-cell function that yields audiometriclike data. STUDY DESIGN: Retrospective study of clinical findings. SETTING: Audiology outpatient clinic of our university's medical center. PATIENTS: The study cohort comprised 30 patients who presented with a profile of suspicion for nonorganic hearing loss. Most cases were found, with the aid of DPOAE testing, to be nonorganic or to have nonorganic overlays to organic hearing loss. INTERVENTIONS: Interventions were diagnostic only. MAIN OUTCOME MEASURES: Observed audiometric findings and changes thereof. RESULTS: Statistically significant decreases in thresholds occurred in subgroups of those cases deemed to be truly nonorganic in origin. CONCLUSIONS: Especially considering test efficiency, the results support the inclusion of DPOAE analysis in the diagnostic management of the suspect-nonorganic patient.
Medical management of Meniere's disease is successful in approximately 70% of patients. Surgical intervention is the treatment option when medication fails. Middle ear installation of aminoglycosides provides significant control of vertigo. This article addresses the role of aminoglycoside ablation of vestibular function in Meniere's disease.
PURPOSE: The origin of acute/sudden hearing loss is multifactorial. The association of vestibular symptoms does not necessarily isolate the pathologic condition to the inner ear. The audiogram provides a screen for differentiating conductive from sensorineural loss but often fails to provide more localizing information. METHODS: Three unusual patients with a variety of retrocochlear presentations of hearing loss are presented. Along with conventional auditory brainstem response (ABR) testing, newer auditory tests, including otoacoustic emissions and three-dimensional ABR analysis, can facilitate site-of-lesion testing. Magnetic resonance imaging (MRI) also provides graphic documentation for sources of retrocochlear hearing loss. RESULTS: One patient had gamma-knife treatment of an arteriovenous malformation, incurring a localised lesion to the inferior colliculus contralateral to the side of hearing loss. This effectively eliminated wave V, as confirmed by three-dimensional ABR analysis. A second patient with human immunodeficiency virus developed sudden complete hearing loss with retained otoacoustic emissions, confirming a retrocochlear lesion. A third patient with acute otitis media with sudden hearing loss and vertigo had an abnormal ABR and "mass lesion" on MRI. Hearing subsequently returned to normal, as did a repeated scan. CONCLUSIONS: The unique aspects of each case of retrocochlear hearing loss and the applied auditory electrophysiologic tests are reviewed.
PURPOSE: Nimodipine is a highly lipophilic, centrally acting calcium channel blocker. It is similar in action to flunarizine, which has been studied for use in the medical treatment of Menière's disease. METHODS: Nimodipine was offered to patients with Menière's disease for whom first-line medical management failed (dietary restrictions and diuretics or vestibular suppressants). RESULTS: Our preliminary clinical experience using nimodipine in 12 patients with Menière's disease from December 1992 until March 1995 resulted in successful control of vertigo and hearing improvement or stabilization in seven (58%) of 12 patients. When hearing stabilization was not considered, eight (67%) patients had vertigo satisfactorily controlled (AAOO class A, B, or C). The four (33%) patients whose vertigo symptoms persisted despite treatment with nimodipine (AAOO class D) were surgically treated with successful vertigo control. Nimodipine was discontinued in one class D patient because of gastrointestinal intolerance. CONCLUSIONS: Nimodipine provides an alternative successful means for medical management of Menière's disease.
Electrocochleography (ECoG), an objective electrophysiologic test, is useful in the clinical diagnosis of endolymphatic hydrops. The purpose of this study was further to define the role of ECoG in the diagnosis of this disease. A retrospective chart review of 100 patients undergoing tympanic ECoG was undertaken comparing symptoms, degree of hearing loss, duration of disease, and diagnosis with ECoG results. The apparent sensitivity and specificity of ECoG in the diagnosis of endolymphatic hydrops were determined to be 57% and 94%, respectively. Three of 30 positive ECoG results were falsely positive. Fluctuating hearing loss and the degree of hearing loss (< 40 dB) and duration of disease (< 48 months) were statistically significant in predicting positive ECoG results. We conclude that a positive ECoG result is helpful in objectively confirming the disease. However, a negative result does not rule out hydrops.
The prostheses available for reconstruction of the ossicular chain have expanded to include a variety of synthetic materials. Hydroxyapatite contains the inorganic constituents found in human living bone and is currently being incorporated into many new prostheses. This study demonstrates the computed tomography and magnetic resonance image characteristics of eight middle ear prostheses, a block of dense hydroxyapatite, and a human incus. Imaging of ossicular prostheses is more informative with computed tomography.
Transtemporal approaches to the petrous apex and CP angle are standard procedures in the armamentarium of the neurotologist. In the majority of these cases, it is not possible to achieve a watertight suture closure of the dura following the procedure. Subsequently, cerebrospinal fluid leakage and potential meningitis are among the most troublesome complications for both patient and surgeon. Recent use of calcium phosphate cement (hydroxyapatite [HA]) has proved efficacious in animal studies and is now being used to close cranial defects in several medical centers, as part of an FDA-IDE study in human subjects. The use of this material is described in 11 neurotologic procedures. It is believed that hydroxyapatite cement (HAC) will become a standard tool in the management of cranial base and temporal bone defects following surgery.
Off-vertical axis rotation (OVAR) stimulates the otolith organs in a manner that is suitable for assessment of the otolith-ocular reflex. To further assess the potential clinical usefulness of OVAR, the eye movement responses of seven patients with surgically confirmed unilateral peripheral vestibular lesions were compared with the eye movement responses of a group of age-matched, healthy, asymptomatic control subjects. Patients and controls were tested with constant velocity rotations that followed a brief period of angular acceleration (velocity trapezoid) using either earth-vertical axis (EVA) rotation or OVAR. Both EVA and OVAR sinusoidal velocity profiles were also performed. Results indicated that each patient had 1) an asymmetric OVAR response, ie, a bias component whose direction was opposite normal when rotating toward the lesioned ear, and 2) a normal modulation component. Population data suggested that patients had 1) a more rapid decay of response than normal subjects during OVAR velocity trapezoids, 2) an increased phase lead as compared to normal subjects during sinusoidal OVAR, and 3) like normal subjects, a less rapid decay of response during OVAR velocity trapezoids than during EVA rotational velocity trapezoids. Taken together, these findings suggest that patients with unilateral peripheral vestibular deficits have abnormal otolith-ocular and semicircular canal-ocular reflexes but that a single labyrinth appears to provide an otolithic signal sufficient for qualitatively normal semicircular canal-otolith interaction.
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Revision stapedectomy operations performed over a 13-year period (1977 to 1990) for a conductive hearing loss are reviewed in terms of intraoperative findings and hearing results. All operations were performed in a conventional manner without use of laser techniques. A management algorithm based on intraoperative findings is described. Results are compared with previously reported series. The 66 cases include 20 males and 46 females, ranging in age from 8 to 73 years. Mean time between original and revision stapedectomy was 12.5 years. Prostheses encountered at time of revision included wireloop (29), Robinson (18), polyethylene (14), other (3), and two were not found. The most common cause of failure was displacement of the prosthesis. Incus erosion was found in 48% of wireloops, 35% of polyethylene, and only 11% of the Robinson prostheses. Revision resulted in closure of the pure-tone average (PTA) air-bone gap to within 10 dB in 46% and to within 15 dB in 76%. Sensorineural hearing loss (> 10 dB) occurred in 5 cases (7.6%), and a decline in speech discrimination (> 10%) occurred in 17%. Three of four cases requiring drillout had gap closure to within 10 dB. Findings suggest that our management technique produces results comparable to other large reported series. Drillout at the time of revision can be recommended.
Preoperative counseling of patients, regarding facial nerve function following cerebellopontine angle (CPA) surgery, has remained a difficult task. Facial nerve electroneurography (ENoG) or evoked electromyography can detect subclinical neural degeneration. This test was used in 44 patients undergoing CPA surgery, and facial nerve function was followed postoperatively. A normal preoperative ENoG study appeared to predict an 81 percent and 84 percent chance of good (House grade I-II) early and late postoperative facial function, respectively. An abnormal test is not as reliable. The relation of preoperative ENoG results with tumor size and tumor adherence to the facial nerve is investigated. Electroneurography appears to be a useful preoperative test when counseling patients regarding facial function following CPA surgery.
Intraoperative stimulation of the facial nerve during surgery of the cerebellopontine angle greatly aids the surgeon in identification of the nerve. The preservation of the ability to stimulate the facial nerve, at the brain stem following tumor removal, has been shown to correlate with good postoperative facial outcome. Thresholds of facial nerve stimulation recorded in our series, using constant voltage stimulation, showed statistically significant relevance to facial nerve outcome. When the difference between thresholds before and after tumor removal was 0.2 V or less or when the threshold after tumor removal was less than or equal to 0.2 V, a good postoperative facial outcome could be expected. This paper discusses the relevance of stimulation threshold prior to tumor removal to the size of tumor.
The large vestibular aqueduct syndrome describes an abnormally large endolymphatic duct and sac with associated sensorineural hearing loss. This entity was originally reported in 1978 and has since been identified as a finding in children with progressive hearing loss. The original description of the large vestibular aqueduct employed hypocycloidal polytomography of temporal bone. Subsequent reports studied patients identified with this syndrome using computed tomographic scans. We report magnetic resonance imaging of two patients diagnosed with the large vestibular aqueduct syndrome. The magnetic resonance imaging and computed tomographic scans are compared and the significant findings on magnetic resonance imaging are reviewed. This should assist the otolaryngologist and radiologist with establishing the appropriate diagnosis.
Surgical management of chronic otitis media with cholesteatoma can be performed in a single-stage procedure, with revision surgery necessary because of recurrent disease or electively with unsatisfactory hearing results. We retrospectively reviewed 164 cases with cholesteatoma surgically managed from 1980 through 1986. In total there were 11 tympanoplasties, 36 canal wall-up mastoidectomies, 81 canal wall down modified radical mastoidectomies, and 36 radical mastoidectomies. The recidivistic rate for cholesteatoma in patients available for 5-year followup was 11% for tympanoplasties, 19% for canal wall-up mastoidectomies, 5% for canal wall-down modified radical mastoidectomies, and 0% for radical mastoidectomies. Hearing results were best in patients who required tympanoplasty alone, followed by those who underwent canal wall-down procedures. Single-stage management of cholesteatoma with modified radical mastoidectomy (canal wall-down) required fewer revisions for recidivistic cholesteatoma and achieved better hearing results than canal wall-up procedures. We conclude, therefore, that staging is not necessary. Improved hearing may likely be achieved with a second procedure, but this is at the patient's discretion.
The diagnosis and management of perilymphatic fistula has received considerable attention in recent years. Despite the use of sophisticated technology, the diagnosis of perilymphatic fistula continues to rest primarily upon clinical suspicion and the exclusion of other disorders. In addition, the confirmation of a perilymphatic fistula during surgical exploration is usually based upon the subjective observation of fluid pooling in niches of the middle ear. A sensitive and objective laboratory test for identifying perilymph in the middle ear would be a useful adjunct for the diagnosis and management of perilymphatic fistula. The objective of this paper is to demonstrate the potential utility of beta 2 (beta 2) transferrin assay in the diagnosis of perilymphatic fistula. To accomplish this objective, we confirmed that beta 2 transferrin is present in living human perilymph and is absent in the normal or inflamed middle ear. In addition, the utility of beta 2 transferrin assay in the diagnosis of cerebrospinal fluid otorrhea is presented.
A multidisciplinary approach to the study of a family with autosomal dominant sensorineural hearing loss is presented. The affected family members underwent extensive clinical and laboratory evaluation. They were found to have various degrees of bilateral congenital inner ear anomalies as imaged by computed tomography. The degree of structural abnormalities in the inner ears correlated with the severity of hearing impairment. Vestibular testing revealed nonspecific abnormalities generally correlating with audiologic and radiographic findings. This report presents a unique form of sensorineural hearing loss not previously described and is the first in-depth clinical study of nonsyndromal "Mondini dysplasia" occurring in a family.