Journal of neurosurgery | 2019
Editorial. Rear Admiral (Astronaut) Alan Shepard: De mortuis nil nisi bonum (say nothing bad of the departed).
Abstract
The article by Menger et al. “Rear Admiral (Astronaut) Alan Shepard: Ménière’s disease and the race to the moon” is a nice and engaging story to read.6 The protagonists of this drama as it unfolds were Alan Shepard (one of the original Mercury Seven astronauts) and Dr. William House (arguably the father of modernday neurotological surgery). Shepard’s Ménière’s disease threatened to abort his attempts toward further space travel, and in return he sought an experimental surgical procedure pioneered by Dr. House that thankfully was successful. The rest, as they say, is history. One can only imagine what screenwriters might do with a plot looking at all the other aspects of both lives (i.e., as a Christian Scientist, Shepard would have had to wrestle with his faith to have proceeded toward any form of surgery over prayer). Fast forward a half century later, and although much is known about Ménière’s disease it continues to confound all those who treat this condition. As a practicing neurotologist for more than 30 years I have studied histopathological specimens, done clinical research trials, been involved in meta-analyses and systematic reviews— yet I feel no further ahead in knowing what causes Ménière’s.1,3–5,7–12,14–16 Much of that terra incognita feeling revolves around whether the pathophysiological process of endolymphatic hydrops is the actual cause leading to membrane ruptures within the inner ear (resulting in the admixing of perilymph and endolymph and the temporary loss of the endocochlear potential—the so-called Na+-K+ intoxication theory) or whether we are looking at an endstage pathological reaction attributable to a number of different pathologies. The frequency of bilateral involvement (up to 50% in some series) certainly speaks for consideration of a systemic cause (possibly immune mediated) being involved at some point in the disease process. The overriding clinical feature of anyone with Ménière’s, however, is the presence of a condition associated with periods of remission and exacerbation. This feature undeniably makes it difficult to know whether any treatment (beyond that of a total osseous labyrinthectomy) completely provides a 100% durable cure (please bear in mind that the price of doing a labyrinthectomy may be worse than the cause in some instances). Those who argue otherwise might best look at the myriad of publications still searching for the Holy Grail of treatment that returns inner ear function to normal and prevents further attacks of episodic vertigo/loss of hearing/tinnitus from occurring. Without the ad fontes knowledge of all the facts surrounding Shepard’s presentation, it must however have been extremely gratifying for both individuals to have had such a successful outcome for a condition whose pathogenesis remains elusive and capricious. Surgical assaults on the endolymphatic sac and its variation on a theme (including bony decompression, endolymphatic sac–mastoid shunting, sac resection, formal sac obliteration, sac revascularization and, in the case of Alan Shepard, an endolymphatic sac–subarachnoid shunt) have all had their proponents in the past. Much of our conventional belief in these procedures seems to have been questioned, however, in what later became known as “the Danish Sham Study” by Thomsen et al. In this double-blind, prospective, randomized controlled surgical trial, sac decompression surgery (the active arm) was compared to a placebo surgical arm. When unblinded, the researchers found no significant difference ultimately between the two procedures in terms of efficacy. As expected, their findings created a maelstrom of reinterpretations, alternative explanations, and different statistical analyses (not to mention the Helsinki Declaration on Medical Ethics weighing into the fray) of the data. In the end, however, one could not escape the finding that the placebo arm had had significant improvement (and seemingly better long-term results at a 9-year follow-up when compared to the active arm).2,13 The reluctance to perform sac surgery in many Northern European countries and my native Canada remains based in part on these findings and our own collective experience. At the time, William House’s surgical treatment of EDITORIAL Rear Admiral (Astronaut) Alan Shepard: De mortuis nil nisi bonum (say nothing bad of the departed)