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Biomedical subjects

M J Ruckenstein

Publications and source records attributed to M J Ruckenstein.

12 recordsLinked to original sources

A prospective study of ABR and MRI in the screening for vestibular schwannomas.

The advent of magnetic resonance imaging (MRI) has significantly increased the clinician's ability to detect small vestibular schwannomas. This had led to controversy in the evaluation of patients with asymmetric sensorineural hearing loss, as some recent studies have suggested that the auditory brainstem response (ABR) does not adequately detect small tumors of the internal auditory canal and cerebellopontine angle. As these studies evaluated ABR results in patients already diagnosed with vestibular schwannomas, they could not determine the epidemiologic accuracy (validity) of the ABR as a screening test for retrocochlear pathology. We report on the preliminary results of an ongoing prospective study on the evaluation of patients with asymmetric sensorineural hearing loss. All patients with asymmetry in two or more pure-tone thresholds of > or = 15 decibels or asymmetry in speech discrimination scores of > or 15% or both entered the study and underwent both an ABR examination and an enhanced MRI scan. Based on preliminary results obtained from the first 47 patients entered in this study, the ABR screening test for retrocochlear pathology was determined to have a sensitivity of 63%, a specificity of 64%, a positive predictive value of 26%, and a negative predictive value of 89%. All patients in whom ABR failed to diagnose a vestibular schwannoma had unilateral hearing los. These results bring into question the validity of ABR as a screening test for retrocochlear pathology, particularly in cases of unilateral hearing loss. Continued patient enrollment in this study will allow the confirmation of these results.

Adolescent

Pain subsequent to resection of acoustic neuromas via suboccipital and translabyrinthine approaches.

Prolonged headache subsequent to excision of acoustic neuromas via a suboccipital approach has been cited as a significant complication of this procedure. However, few studies have sought to compare the incidence of postoperative headaches in patients undergoing either translabyrinthine or suboccipital approaches with surgical techniques designed to minimize this complication. We performed a retrospective survey of 52 patients having undergone either a suboccipital or translabyrinthine resection of acoustic neuromas. Cranioplasties were performed on all patients having undergone resections via a suboccipital approach. The survey asked patients to categorize headache severity based on a numeric scale at 1 month, 6 months, and 1 year after surgery. Medications required to control headaches were also recorded. At 1 and 6 months after surgery, headache severity was significantly less in patients having undergone a translabyrinthine resection (p < 0.05). However, by 1 year after surgery, headache severity in the two groups of patients was essentially equivalent (p = 0.6). Data concerning the strength of analgesics required to control postoperative headaches paralleled these results. These results indicate that within the first postoperative year, patients undergoing suboccipital craniotomies have significantly more postoperative pain than do those patients having undergone translabyrinthine resections, despite the performance of a cranioplasty. However, by 1 year after surgery, these differences are no longer significant. Thus the complication of long-term postoperative headache is no more prevalent in patients undergoing a suboccipital resection than in those having undergone translabyrinthine surgery. These results are important to both the surgeon and the patient during preoperative counseling regarding the choice of surgical approach for acoustic neuroma excision.

Cranial Nerve Neoplasms

Na,K-ATPase in the cochlear lateral wall of human temporal bones with endolymphatic hydrops.

Meniere's disease has traditionally been thought to arise from a disruption in longitudinal endolymphatic flow. This view has been brought into question by recent experimental studies that have focused attention on derangements of cochlear fluid and electrolyte homeostatic mechanisms in Meniere's disease, including abnormalities in Na,K-ATPase enzymes found in the cochlear lateral wall. The current study examined the immunohistochemical labeling pattern of the major ion-transporting enzyme of the stria vascularis, Na,K-ATPase, in archival sections of hydropic and nonhydropic human temporal bones for increased density of label that could indicate overproduction of fluid. The results showed good labeling of the stria vascularis in the celloidin sections. The hydropic ears tended to have darker label, but the difference was not statistically significant. The findings are consistent with normal functioning of the stria vascularis in cases of Meniere's disease.

Animals

Hearing loss. A plan for individualized management.

Because some cases of hearing loss demand urgent attention, an approach to patients with hearing loss should be adopted in the primary care setting that allows for systematic institution of an individualized and appropriate treatment plan. Workup is enhanced by categorizing the hearing loss as acute or chronic and conductive or sensorineural. The Rinne and Weber tuning fork tests are the most important tools in distinguishing between conductive and sensorineural hearing loss. The patient history and otologic examination also provide diagnostic clues. Treatment depends on the cause and the type of hearing loss.

Hearing Loss

A practical approach to dizziness. Questions to bring vertigo and other causes into focus.

Evaluation of a patient presenting with dizziness begins with and largely depends on the patient's history. The diagnosis often can be accurately determined in a primary care setting when a stepwise algorithmic approach is used. The first step is getting a detailed account of precisely what the patient means by "dizziness." This helps determine whether the cause is vertigo or another condition, such as orthostatic hypotension. Establishing whether the vertigo is central or peripheral in origin and, if peripheral, how long episodes last further focuses the investigation. Certain clues on physical examination and appropriate use of diagnostic tests help support the diagnosis. Referral should be contemplated when significant central disease is suspected and when vertigo of peripheral origin is persistent or atypical.

Dizziness

The MRL-lpr/lpr mouse: a potential model of autoimmune inner ear disease.

Most attempts at developing a model of autoimmune inner ear disease have focused on the immunization of healthy animals with cochlear tissue. We have chosen an alternate route of studying this entity utilizing the MRL-lpr/lpr (Lupus) mouse, an animal known to spontaneously develop multisystemic, organ nonspecific autoimmune disease. We report on the auditory pathology found in animals at early stages of this systemic disease. At the onset of clinical signs of illness (cachexia, weight loss, lethargy) animals were sacrificed and their cochleas and kidney prepared for morphologic analysis. Significant pathology was seen in the MRL/lpr animals involving the basal and middle turns of the cochlea which could not be correlated with the presence or degree of glomerulonephritis. Findings included outer and inner haircell degeneration, strial edema and degeneration, and an acellular infiltrate in the tunnel of Corti. Cochlear pathology was not found in control animals. Thus, at early stages of systemic disease, MRL/lpr mice manifest significant cochlear pathology not seen in control animals. The implications of these results with regard to the pathogenesis of these lesions as well as their clinical relevance are discussed.

Animals

Motion sickness. Helping patients tolerate the ups and downs.

The sensory conflict theory provides a useful framework for understanding motion sickness. Few modifications of this model have been proposed since its publication. The weak link in the study of motion sickness has been the failure to delineate the anatomic structures that correspond to the model's components. Effective pharmacotherapy is available for treatment of motion sickness. The choice should be based on the need to provide prophylaxis for or treatment of symptoms and on the side effect profile of the drug.

Adaptation, Physiological

Autoimmune inner ear disease: a review of basic mechanisms and clinical correlates.

Otolaryngologists have long sought to identify causes of sensorineural hearing loss that might be reversed by medical treatment. One such entity has become known as autoimmune inner ear disease. The potential improvement in auditory function in these patients subsequent to immunosuppressive therapy has created a desire in clinicians to better understand this disease. This paper begins by reviewing the basic concepts of autoimmunity. The experimental and clinical data concerning autoimmune inner ear disease are then described and analyzed. Finally, conclusions are drawn concerning our current state of understanding of this disease process.

Animals

The management of otolaryngological problems in the mucopolysaccharidoses: a retrospective review.

The mucopolysaccharidoses are rare, genetically transmitted metabolic disorders that affect children early in life. These potentially life-threatening diseases almost invariably involve the auditory apparatus and the upper respiratory tract. Thus, the otolaryngologist is frequently involved in the care of these patients. This paper presents a 10-year retrospective review of the management of these patients at the Hospital for Sick Children. Data concerning auditory and upper respiratory pathology are presented. Results indicate that persistent serous otitis, sensorineural hearing loss, and upper respiratory obstruction leading to sleep apnea, are frequent findings in these patients. Specific recommendations are made with regard to appropriate otolaryngologic intervention in children affected with these diseases.

Adenoidectomy

Angiolymphoid hyperplasia with eosinophilia: a case report and literature review.

Angiolymphoid hyperplasia with eosinophilia is a rare, benign vascular tumor affecting principally the head and neck region of young adult females. Microscopic analysis reveals hyperplastic blood vessels lined by a hypertrophic endothelium. An inflammatory infiltrate rich in eosinophils is also present. Etiology of the lesion is unknown. Various treatment modalities have been described. We describe a case successfully treated by extensive excision and reconstruction.

Adult