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L R Lustig

Publications and source records attributed to L R Lustig.

27 records · Page 2Linked to original sources

The history of Meniere's disease.

Prosper Meniere is widely credited with the first description of endolymphatic hydrops. Meniere's discovery was made possible through a combination of technical and scientific advances, a supportive scientific environment, and a keen intellect able to put the pieces of a puzzle together. Meniere's novel concept, however, that vertiginous disorders were caused by a pathologic process in the semicircular canals was not accepted overnight, but realized only after another 70 years of scientific debate. This article traces the story of endolymphatic hydrops from its anatomical beginnings to its current place in otolaryngology and the individuals who brought us to this point today.

History, 16th Century↗

The early history of the neurofibromatosis. Evolution of the concept of neurofibromatosis type 2.

Although neurofibromatosis (NF) became widely recognized as a pathologic entity in the late 19th century, only relatively recently has a clear distinction been made between its generalized form and the central variety. The latter form is typified by bilateral acoustic neuromas (ANs), which may be accompanied by other intracranial tumors, in particular, meningiomas. Up until almost the current era, confusion regarding the protean manifestations of the 2 types of NF existed in the minds of clinicians and in the literature. In 1987, a consensus panel of the National Institutes of Health differentiated the clinical manifestations associated with classic von Recklinghausen syndrome from those of the predominantly intracranial subtype and they were subsequently deemed NF type 1 (NF-1) and NF type 2 (NF-2), respectively. During the last few years, the genetic flaws that underlie these 2 syndromes have been elucidated, revealing that their origins lie in defects on separate chromosomes. The early literature on the subject included repeated descriptions of patients with manifestations typical of NF-2. The investigators, however, considered the intracranial lesions to be merely 1 facet of the generalized form of the disease. A few prescient individuals, however, demonstrated an appreciation for the distinguishing characteristics between these superficially similar, yet quite different, syndromes. The goals of this article are to trace the evolution of the concept of NF-2 as a distinct clinical entity from NF-1 and to assess the early awareness of and attitudes toward bilateral ANs, familial ANs, and ANs associated with other intracranial tumors.

Europe↗

The variable relationship between the lower cranial nerves and jugular foramen tumors: implications for neural preservation.

Tumors involving the jugular foramen (JF) have a variable relationship to the neurovascular structures (jugular vein, cranial nerves IX-XI) that traverse this conduit through the skull base. The surgeon familiar with the site of origin, growth pattern, and geometry of each of the common lesions affecting this region with respect to surrounding nerves and vessels is at a considerable advantage when undertaking a function-sparing procedure. Anatomically, the JF has two vascular compartments that may be affected by tumor: the jugular bulb laterally and a passage for the inferior petrosal sinus medially. Tumors may also penetrate the JF along the fibro-osseous diaphragm, which divides these two vascular channels. The lower cranial nerves lie on either side of this partition, which is connected to the posterior cranial fossa via a curved, funnel-shaped cone of dura. Tumors that arise within or penetrate the JF lateral to this neural plane displace the nerves medially, a position favorable for their preservation during tumor extirpation. By contrast, medially positioned tumors displace the cranial nerves onto the lateral tumor surface, where they interpose between surgeon and tumor-an unfavorable location. Glomus tumors consistently arise in the lateral aspect of the JF, displacing the lower cranial nerves medially. This positioning accounts for the high rate of neural preservation in small and medium-size glomus tumors that have not invaded the foramen's central partition. Meningiomas that arise lateral to the JF (e.g., the posterior petrous surface, sigmoid sinus) favorably displace the lower cranial nerves medially. By contrast, tumors that originate medial to the JF (e.g., clivus, foramen magnum) are unfavorable, laterally displacing the multiple small rootlets that coalesce into cranial nerves IX-XI into a vulnerable location. Schwannomas arise within the neural plane and have a variable geometry that depends, in part, upon the nerve of origin. Theoretically, tumors that arise from the ninth nerve, which is located on the lateral surface of the neural plane, should be more favorable than those originating from the tenth or eleventh nerves, which lie on its deep surface. The propensity of these three tumor types toward thrombosis of the jugulosigmoid complex also carries important surgical implications. Because glomus tumors arise from the jugular bulb, the jugulosigmoid complex is nearly always occluded. In both meningiomas and schwannomas, however, the jugular system may occasionally remain patent. This is important to recognize through angiography and/or magnetic resonance venography, since sacrifice of a patent, dominant system risks intracerebral venous infarction.

Brain↗

Lemierre's syndrome: two cases of postanginal sepsis.

Lemierre's disease consists of suppurative thrombophlebitis of the IJV in the presence of oropharyngeal infection and can be complicated by septic pulmonary emboli. If a patient has an oropharyngeal or deep neck infection and neck pain suspicious for IJV thrombosis, a CT or MRI is warranted to establish the diagnosis. Blood cultures should be obtained to establish the responsible organism. In most cases F. necrophorum, an anaerobic bacterium, is responsible for the sepsis. Once the diagnosis of Lemierre's disease is made, long-term, high-dose intravenous antibiotics with beta-lactamase anaerobic activity should be initiated. In cases with persistent sepsis and emboli despite appropriate medical management, ligation or excision of the IJV should be performed. Finally, if there is clinical or radiologic evidence of retrograde cavernous sinus thrombosis, the use of anticoagulants should be considered.

Adult↗

Changes in the cat cochlear nucleus following neonatal deafening and chronic intracochlear electrical stimulation.

The effects of chronic intracochlear electrical stimulation on the cochlear nucleus (CN) were studied in eight cats that were neonatally deafened by daily intramuscular injections of neomycin. Profound hearing loss was confirmed in each animal by auditory brainstem response (ABR) and frequency following response (500 Hz) testing. Five of the kittens were implanted unilaterally with a scala tympani electrode array at ages 8-16 weeks. These kittens were stimulated daily for four hours at 2 dB above the evoked ABR threshold, over a period of three months, and subsequently euthanized for histological analysis at 26-32 weeks of age. The three remaining deaf kittens were maintained without stimulation over prolonged periods in order to study the long-term consequences of neonatal deafening, and were euthanized at 66-133 weeks of age. This study compares the CN of these deafened experimental animals and the CN of normal adult cats. Three experimental parameters were examined: CN volume, cross-sectional area of spherical cells in the rostral anteroventral cochlear nucleus (AVCN), and spherical cell density in this same region. The CN in animals that received electrical stimulation showed significant bilateral degenerative changes in all three measured parameters. Total nuclear volume was reduced by 35-36%, spherical cell size was reduced by 20-26%, and spherical cell density decreased by 36-42%, as compared to the normal cat CN. Comparisons were also made in the stimulated animals between CN ipsilateral to the stimulated cochlea and the contralateral, unstimulated CN.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Repair of chronic tympanic membrane perforations using epidermal growth factor.

Perforation of the tympanic membrane (TM) is a frequent cause of conductive hearing loss. Persistent TM perforations often require surgical repair with an autologous tissue graft to restore hearing and prevent recurrent infection. While highly efficacious, this method of closure requires a relatively complex and expensive microsurgical procedure. We have recently developed a chronic TM perforation model in the chinchilla for use in the exploration of novel methods of TM repair.

Animals↗

Hyaluronan synthesis in the adult guinea pig endolymphatic sac.

The endolymphatic sac is believed to play a major role in membranous labyrinth homeostasis by controlling the volume of endolymph, removing debris, and participating in the immune response of the inner ear. The endolymphatic sac is postulated to absorb endolymph and to synthesize and secrete high-molecular-weight and osmotically active glycosaminoglycans (GAGs). The present study examines the ability of in vitro adult guinea pig endolymphatic sac cells to synthesize complex proteins and polysaccharides. The intent is to characterize the nature of these compounds by studying carbon-14 (14C) glucose incorporation in tissue cultured endolymphatic sac specimens using autoradiographic and specific enzymatic digestion techniques. Our results suggest that sac cells can synthesize GAGs and proteins in vitro in proportionately larger amounts than surrounding connective tissue and dura. The principal GAG synthesized by the endolymphatic sac appears to be hyaluronan.

Amylases↗