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Biomedical subjects

G B Hughes

Publications and source records attributed to G B Hughes.

At least 19 recordsLinked to original sources

Retrolabyrinthine transtentorial approach to lesions of the anterior cerebellopontine angle.

Various surgical approaches to the cerebellopontine angle have been used for removal of acoustic neuromas. A retrolabyrinthine transtentorial approach has been developed that allows (1) access to the anterior cerebellopontine angle and all portions of the basilar artery, (2) extra dural retraction of the lateral sinus and cerebellum while avoiding the vein of Labbé, and (3) preservation of hearing. This approach allows good exposure of tumor and accurate visualization of cranial nerves. To avoid complications, control of spinal fluid is mandatory and great care must be taken to avoid injury of the cranial nerves. The retrolabyrinthine or translabyrinthine transtentorial approach enables skilled neurosurgeons and neurotologists to gain access to lesions that are located in areas difficult to approach.

Adult

The learning curve in stapes surgery.

Fewer stapes operations are available to train residents and to maintain individual competence. Most residents in the United States perform 0 to 10 cases during training, and produce results which are not as good as expert results, even with close supervision. After graduation, fewer cases are available to achieve expert results in private or academic practice. The authors' conclusions and recommendations are based on his own learning curve. Residents who have below average skills in middle ear surgery, and residents who do not wish to practice otology, should watch but not perform stapes surgery. Program directors, collectively or separately, should develop more formal guidelines for teaching stapes surgery during residency.

Clinical Competence

Is there consensus in perilymph fistula management?

A perilymph fistula is an abnormal communication between the inner ear fluids and the middle ear space. This article identifies areas of agreement in perilymph fistula management based upon 167 questionnaire responses from active surgeons of the American Otological Society and the American Neurotological Society. The average respondent performed 4.6 fistula explorations among 197 otologic surgeries (some of these were myringotomies) per year. The following statements represent areas of general agreement. The single most important feature of the history is previous trauma or barotrauma. At present no test can replace clinical judgment; however, despite sound judgment and sophisticated testing, it is very difficult to document the presence or absence of a fistula preoperatively. Most often the diagnosis is based on the total clinical picture: history, physical examination, and laboratory testing.

Ear Diseases

Practical management of Bell's palsy.

Bell's palsy is an acute unilateral weakness or paralysis of the face resulting from peripheral facial nerve dysfunction. While there is no readily identifiable cause, there is some recovery of function within 6 months. This article offers practical guidelines for diagnosing and treating Bell's palsy. These guidelines are based on the author's experience with 63 patients over the last 8 years. Physical examination should reveal diffuse nerve involvement, normal otoscopic findings, and no skin blebs or blisters and parotid masses. Other cranial nerve palsies may be present. Diagnostic testing should include basic audiometry if available, and computed tomography or magnetic resonance imaging scanning if paralysis is present. Facial photography is recommended. Topognostic testing is not helpful because it no longer guides the surgical approach, and prognostic tests are not necessary if the face is not paralyzed. The only sufficiently sensitive test to determine the need for possible surgery is electroneurography. Eye care is critical in all but the mildest cases, and steroids may also be helpful. Surgery is rarely needed and should consist only of middle fossa total decompression; simple decompression of the tympanic and mastoid segments is seldom helpful. The natural history of the disease and the limited role of surgery provide new guidelines for practical management of Bell's palsy.

Adolescent

Bilateral spontaneous perilymph fistulae: a diagnostic and management dilemma.

In this article, we present a case of bilateral perilymph fistulae. This entity is rare and always presents a diagnostic problem. Bilateral perilymph fistulae are more common in children and should be suspected when bilateral ear symptoms and congenital temporal bone radiographic anomalies are present. Previous trauma or barotrauma may be absent. When a patient manifests bilateral ear symptoms and a fistula has been identified and repaired in one ear, a second fistula should be suspected in the contralateral ear, especially if the fistula test is positive.

Adult

Objective tinnitus in benign intracranial hypertension: an update.

Previously, the authors reported that objective pulsatile tinnitus can be the major or only manifestation of benign intracranial hypertension. This report updates the authors' experience with 31 patients managed over the past 7 years. Benign intracranial hypertension should be suspected in all patients with pulsatile-objective tinnitus, especially when the patient is a young, obese female with headaches and/or visual disturbances. Papilledema and small ventricles or an empty sella on computerized tomography are almost diagnostic. The diagnosis is confirmed by elevated spinal fluid pressure on lumbar puncture. In such patients, angiography is not indicated. Furosemide and acetazolamide are very effective. Ligation of the internal jugular vein is contraindicated.

Adult

Prognostic tests in acute facial palsy.

This issue of The American Journal of Otology includes the fourth of a series of articles on facial nerve disorders, which collectively are known as the "Facial Nerve Manual." In the early 1980s, Dr. Mark May chaired the AAO-HNS Facial Nerve Committee. One of the tasks he initiated was the creation of a Facial Nerve Manual, assigned to a small group of clinicians at the COSM Meeting in the spring of 1984. The purpose of this work was to disseminate practical information on the management of selected facial nerve problems, one of the primary charges of the Committee. Work began slowly, but surely. During Dr. Gale Gardner's tenure as Committee chairman, manuscripts were reviewed and edited by Dr. Nels Olson, and The American Journal of Otology agreed to publish the manual. Perhaps more than anyone else, Dr. Olson worked tirelessly to create a finished product of consistent style and content. Ever since the original writing and editing, controversial topics continue to emerge. As each chapter is published individually over this year, concepts may change, and the reader is encouraged to explore these issues in more depth. Drs. May, Gardner, Olson, and all the individual authors are to be congratulated for their efforts. The Committee sincerely thanks The American Journal of Otology for supporting this work.

Electromyography

Molecular and cellular biology of the inner ear. The next frontier.

Molecular and cellular biology is the study of cellular ultrastructure and function. Current research in immune regulation, delineation of receptor control function (signal translation at the cellular level), and nucleic acid manipulation (genetic engineering) illustrates fundamental relationships among biochemistry, immunology, and molecular and cellular biology. Our knowledge already includes significant discoveries in membrane permeability, ionic gradients, and mediators of homeostasis. To these are added newer discoveries in bone metabolism, vascular anatomy and physiology, trace element deficiency, and the broader field of nutrition. Biochemical research gradually is unlocking the mysteries of otosclerosis, Paget's disease, endolymphatic hydrops, ototoxicity, acoustic trauma, presbycusis, and other forms of sensorineural hearing loss. This paper presents some of the research topics of current interest. Relationships of molecular and cellular biology, immunology, and nutrition are discussed. Molecular and cellular biology of the inner ear is an exciting "new" frontier.

Adolescent

Autoimmune endolymphatic hydrops: five-year review.

This article reviews the authors' experience with autoimmune endolymphatic hydrops over the past 5 years. Twenty-seven of 52 patients with diagnosed autoimmune inner ear disease (52%) manifested endolymphatic hydrops, usually bilateral. Treatment consisted of low salt diet, diuretic, vestibular suppressant, and usually prednisone. Cytotoxic drugs and lymphocytoplasmapheresis were reserved for refractory cases. Audiometric followup (average, 23 months) was available in 12 patients. Using American Academy of Otolaryngology--Head and Neck Surgery guidelines, hearing improved or stabilized in at least one ear in nine patients and deteriorated bilaterally in three patients. Vestibular treatment results paralleled auditory treatment results. Autoimmune endolymphatic hydrops should be considered in the differential diagnosis when symptoms are bilateral and do not respond to conventional therapy or when immune laboratory tests are positive. In suspect patients, medical therapy with prednisone, and rarely cytotoxic drugs and lymphocytoplasmapheresis, should be recommended. Further research is needed to determine whether surgery should be performed in medically unresponsive cases.

Adolescent

Clinical diagnosis of immune inner-ear disease.

The purpose of this study was to develop a high-risk clinical profile of patients with autoimmune (immune-mediated) inner-ear disease. The records of 52 patients diagnosed over the past 5 years were reviewed. Age, sex, bilateral versus unilateral involvement, otologic symptoms, concomitant systemic immune disease, and presenting clinical diagnoses were recorded. The presenting diagnoses were Cogan's syndrome, Meniere's syndrome, Dandy's syndrome without hearing loss, or progressive sensorineural hearing loss without dizziness. Because Cogan's and Dandy's syndromes were relatively uncommon, the typical high-risk clinical profile was a middle-aged patient (often female) with bilateral, asymmetric, progressive sensorineural hearing loss, with or without dizziness, and occasional systemic immune disease such as rheumatoid arthritis. When a more common clinical diagnosis cannot be reached in suspicious patients, immune laboratory tests should be obtained and a trial of immunotherapy offered. Positive test results and beneficial response to therapy support a presumptive diagnosis of immune inner-ear disease.

Adult