Surgical Parameters for Minimally Invasive Trans- Eustachian Tube CSF Leak Repair: A Cadaveric Study and Literature Review

Background Cerebrospinal fluid rhinorrhea from a lateral skull base defect refractory to spontaneous healing and/or conservative management is most commonly managed via open surgery. Approach for repair is dictated by location of the defect, which may require surgical exploration. The final common pathway is the eustachian tube (ET). Endoscopic ET obliteration via endonasal and lateral approaches is under development. Whereas ET anatomy has been studied, surgical landmarks have not been previously described or quantified. We aimed to define surgical parameters of specific utility to endoscopic ET obliteration. Methods A literature review was performed of known ET anatomic parameters. Next, using a combination of endoscopic and open techniques in cadavers, we cannulated the intact ET and dissected its posterior component to define the major curvature position of the ET, defined as the genu, and quantified the relative distances through the ET lumen. The genu was targeted as a major obstacle encountered when cannulating the ET from the nasopharynx. Results Among 10 ETs, we found an average distance of 23 ± 5 mm from the nasopharynx to the ET genu, distance of 24 ± 3 mm from the genu to the anterior aspect of the tympanic membrane and total ET length of 47 ± 4 mm. Conclusions Although membranous and petrous components of the ET are important to its function, the genu may be a more useful surgical landmark. Basic surgical parameters for endoscopic ET obliteration are defined

Role of Electrode Placement as a Contributor to Variability in Cochlear Implant Outcomes

Suboptimal cochlear implant (CI) electrode array placement may reduce presentation of coded information to the central nervous system and consequently limit speech recognition

Phenotypic characteristics of early Wolfram syndrome

BACKGROUND: Wolfram Syndrome (WFS:OMIM 222300) is an autosomal recessive, progressive, neurologic and endocrinologic degenerative disorder caused by mutations in the WFS1 gene, encoding the endoplasmic reticulum (ER) protein wolframin, thought to be involved in the regulation of ER stress. This paper reports a cross section of data from the Washington University WFS Research Clinic, a longitudinal study to collect detailed phenotypic data on a group of young subjects in preparation for studies of therapeutic interventions. METHODS: Eighteen subjects (ages 5.9–25.8, mean 14.2 years) with genetically confirmed WFS were identified through the Washington University International Wolfram Registry. Examinations included: general medical, neurologic, ophthalmologic, audiologic, vestibular, and urologic exams, cognitive testing and neuroimaging. RESULTS: Seventeen (94%) had diabetes mellitus with the average age of diabetes onset of 6.3 ± 3.5 years. Diabetes insipidus was diagnosed in 13 (72%) at an average age of 10.6 ± 3.3 years. Seventeen (94%) had optic disc pallor and defects in color vision, 14 (78%) had hearing loss and 13 (72%) had olfactory defects, eight (44%) had impaired vibration sensation. Enuresis was reported by four (22%) and nocturia by three (17%). Of the 11 tested for bladder emptying, five (45%) had elevated post-void residual bladder volume. CONCLUSIONS: WFS causes multiple endocrine and neurologic deficits detectable on exam, even early in the course of the disease. Defects in olfaction have been underappreciated. The proposed mechanism of these deficits in WFS is ER stress-induced damage to neuronal and hormone-producing cells. This group of subjects with detailed clinical phenotyping provides a pool for testing proposed treatments for ER stress. Longitudinal follow-up is necessary for establishing the natural history and identifying potential biomarkers of progression

Clinical Considerations for Routine Auditory and Vestibular Monitoring in Patients with Cystic Fibrosis

Purpose Specific classes of antibiotics, such as aminoglycosides, have well-established adverse events producing permanent hearing loss, tinnitus, and balance and/or vestibular problems (i.e., ototoxicity). Although these antibiotics are frequently used to treat pseudomonas and other bacterial infections in patients with cystic fibrosis (CF), there are no formalized recommendations describing approaches to implementation of guideline adherent ototoxicity monitoring as part of CF clinical care. Method This consensus statement was developed by the International Ototoxicity Management Working Group (IOMG) Ad Hoc Committee on Aminoglycoside Antibiotics to address the clinical need for ototoxicity management in CF patients treated with known ototoxic medications. These clinical protocol considerations were created using consensus opinion from a community of international experts and available evidence specific to patients with CF, as well as published national and international guidelines on ototoxicity monitoring. Results The IOMG advocates four clinical recommendations for implementing routine and guideline adherent ototoxicity management in patients with CF. These are (a) including questions about hearing, tinnitus, and balance/vestibular problems as part of the routine CF case history for all patients; (b) utilizing timely point-of-care measures; (c) establishing a baseline and conducting posttreatment evaluations for each course of intravenous ototoxic drug treatment; and (d) repeating annual hearing and vestibular evaluations for all patients with a history of ototoxic antibiotic exposure. Conclusion Increased efforts for implementation of an ototoxicity management program in the CF care team model will improve identification of ototoxicity signs and symptoms, allow for timely therapeutic follow-up, and provide the clinician and patient an opportunity to make an informed decision about potential treatment modifications to minimize adverse events

Functional Implications of Ubiquitous Semicircular Canal Non-Orthogonality in Mammals

The ‘canonical model’ of semicircular canal orientation in mammals assumes that 1) the three ipsilateral canals of an inner ear exist in orthogonal planes (i.e., orthogonality), 2) corresponding left and right canal pairs have equivalent angles (i.e., angle symmetry), and 3) contralateral synergistic canals occupy parallel planes (i.e., coplanarity). However, descriptions of vestibular anatomy that quantify semicircular canal orientation in single species often diverge substantially from this model. Data for primates further suggest that semicircular canal orthogonality varies predictably with the angular head velocities encountered in locomotion. These observations raise the possibility that orthogonality, symmetry, and coplanarity are misleading descriptors of semicircular canal orientation in mammals, and that deviations from these norms could have significant functional consequences. Here we critically assess the canonical model of semicircular canal orientation using high-resolution X-ray computed tomography scans of 39 mammal species. We find that substantial deviations from orthogonality, angle symmetry, and coplanarity are the rule for the mammals in our comparative sample. Furthermore, the degree to which the semicircular canals of a given species deviate from orthogonality is negatively correlated with estimated vestibular sensitivity. We conclude that the available comparative morphometric data do not support the canonical model and that its overemphasis as a heuristic generalization obscures a large amount of functionally relevant variation in semicircular canal orientation between species.Funding for this research was provided by grants NSFIIS-0208675 (http://www.nsf.gov/cise/iis/hcc_pgm.jsp), and EAR-0948842 (http://www.nsf.gov/awards/award_visualiz​ation.jsp?org=EAR). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.Geological SciencesAnthropologyEmail: [email protected]

Responses of the ear to low frequency sounds, infrasound and wind turbines.'

a b s t r a c t Infrasonic sounds are generated internally in the body (by respiration, heartbeat, coughing, etc) and by external sources, such as air conditioning systems, inside vehicles, some industrial processes and, now becoming increasingly prevalent, wind turbines. It is widely assumed that infrasound presented at an amplitude below what is audible has no influence on the ear. In this review, we consider possible ways that low frequency sounds, at levels that may or may not be heard, could influence the function of the ear. The inner ear has elaborate mechanisms to attenuate low frequency sound components before they are transmitted to the brain. The auditory portion of the ear, the cochlea, has two types of sensory cells, inner hair cells (IHC) and outer hair cells (OHC), of which the IHC are coupled to the afferent fibers that transmit "hearing" to the brain. The sensory stereocilia ("hairs") on the IHC are "fluid coupled" to mechanical stimuli, so their responses depend on stimulus velocity and their sensitivity decreases as sound frequency is lowered. In contrast, the OHC are directly coupled to mechanical stimuli, so their input remains greater than for IHC at low frequencies. At very low frequencies the OHC are stimulated by sounds at levels below those that are heard. Although the hair cells in other sensory structures such as the saccule may be tuned to infrasonic frequencies, auditory stimulus coupling to these structures is inefficient so that they are unlikely to be influenced by airborne infrasound. Structures that are involved in endolymph volume regulation are also known to be influenced by infrasound, but their sensitivity is also thought to be low. There are, however, abnormal states in which the ear becomes hypersensitive to infrasound. In most cases, the inner ear's responses to infrasound can be considered normal, but they could be associated with unfamiliar sensations or subtle changes in physiology. This raises the possibility that exposure to the infrasound component of wind turbine noise could influence the physiology of the ear

The Role of 3-Canal Biomechanics in Angular Motion Transduction by the Human Vestibular Labyrinth

The present work examines the role of the complex geometry of the human vestibular membranous labyrinth in the process of angular motion transduction by the semicircular canals. A morphologically descriptive mathematical model was constructed to address the biomechanical origins of temporal signal processing and directional coding in determining the inputs to the brain. The geometrical model was developed based on shrinkage-corrected temporal bone sections using a segmentation/data-fitting procedure. Endolymph fluid dynamics within the 3-canal labyrinth was modeled using an asymptotic form of the Navier–Stokes equations and solved to estimate endolymph and cupulae volume displacements. The geometrical model was manipulated to study the role of major morphological features on directional and temporal coding. Anatomical results show that the bony osseous canals provide reasonable estimates of the orientation of the delicate membranous canals—the two differed by only 3.48 ± 1.89°. Biomechanical results show that the maximal response directions are distinct from the anatomical canal planes, but can be closely approximated by fitting a flat plane to the centerline of the canal of interest and weighting each location along the centerline with the inverse of the cross-sectional area squared. Vector cross-products of these maximal response directions, in turn, determine the null planes and prime directions that transmit the direction of angular motion to the brain as three independent directional channels associated with the nerve bundles. Finally, parameter studies indicate that changes in canal cross-sectional area and shape only moderately affect canal temporal and directional coding, while three-canal orientation is critical to directional coding