Faculty Bibliography

BACKGROUND: Oncogenic osteomalacia is a rare paraneoplastic syndrome characterized by osteomalacia, which occurs as a result of excess renal phosphate excretion caused by fibroblast growth factor-23 secreted by mesenchymal tumors. This entity is rare in head and neck cancers. We report a rare case of oncogenic osteomalacia in a patient with an anterior skull base giant cell tumor. METHODS AND RESULTS: A 34-year-old woman presented with a 5-year history of progressive weakness in both lower limbs and the trunk. Hypophosphatemia and hypocalcemia had been noted by a local physician, but her symptoms persisted despite receiving calcium and vitamin D supplements. A recent onset of epistaxis and nasal blockage led to referral to the head and neck services. Nasal endoscopy revealed a left nasal cavity mass. Further evaluation with imaging studies revealed a mass in the nasal cavity with intracranial extension. Biopsy of the lesion suggested a neurogenic tumor. A putative diagnosis of anterior skull base neurogenic tumor with paraneoplastic hypophosphatemia was made. After the biochemical parameters were corrected, the patient underwent craniofacial resection. The final histopathologic study suggested the lesion as a "giant cell tumor." During the postoperative period the patient's biochemical and clinical symptoms improved dramatically, allowing her to regain normal mobility. CONCLUSIONS: Clinicians and pathologists must be aware of the clinical symptoms, laboratory abnormalities, and pathologic features of oncogenic osteomalacia, which may be caused by tumors in the head and neck and thus make an exhaustive effort to diagnose the same.

OBJECTIVES/HYPOTHESIS: Glottic insufficiency (GI) can be either grossly obvious or subtle in its presentation. Subtle GI is demonstrated by various Laryngovideostroboscopic (LVS) clues, including complete but "short" phase closure of the true vocal folds (VFs) during the glottic cycle. We used the frame by frame analysis (FBFA) technique to evaluate its effectiveness in objectively contributing to the diagnosis of subtle GI in patients with atrophic and/or paretic VFs. This article intends to formally present the methods and intentions of the FBFA technique and report our findings using FBFA on subjects with clinically diagnosed GI and normal volunteers. STUDY DESIGN: Retrospective review and demonstration of technique. METHODS: Forty-four subjects with a prior clinical diagnosis of true VF atrophy (25/44) and/or paresis (19/44) and five normal volunteers were identified. Using the FBFA technique, each subject's average percentage of closed frames per glottic cycle was recorded. RESULTS: Subjects with atrophy spent 32.4% of the frames of the glottic cycle in the closed phase, subjects with paresis spent 35.7% of the frames closed, and normal subjects spent 50.2% of the frames closed. CONCLUSIONS: FBFA appears to be a simple objective method for the novice or experienced LVS interpreter, by which one can suspect subtle GI. Because of the inherent physical properties by which LVS gives an "illusionary" representation of the glottic cycle, the FBFA technique remains a theoretical tool. Future studies using high-speed digital imaging are needed to validate this useful technique.

BACKGROUND: The aesthetics of facial structure are used by humans to measure one's beauty, character, and overall "goodness." Individuals born with cleft lip and/or palate are often stigmatized and face much psychosocial adversity. Social attitudes and beliefs have a direct impact upon the psychological development of these individuals. Such social norms are in large part shaped by the physical representations of "good" and "attractive" in various art media including films, advertisements, and paintings. OBJECTIVE: Individuals born with a cleft have been portrayed in the artworks of different eras. The light in which they are portrayed stems from the prevalent beliefs of each period and sheds light on the social attitudes of each epoch toward clefts. Here we discuss the social and psychological ramifications of these works. We then review several artworks representing cleft lip and/or palate and propose an active role for the artist in shaping social attitudes regarding facial deformities. METHODS: Numerous articles and works of arts were examined and inspected for signs of facial deformity, with particular attention to cleft lip and/or palate. CONCLUSION: Social media have an important role in defining the norms of society. Much of the art of the past has depicted negatively individuals born with cleft lip and/or palate deformity, thus excluding them from the norm. In order to decrease the negative social stigmas of cleft lip and/or palate, it is now the responsibility of society to widen its range of norms to include individuals born with these deformities through "normal" representations in the various media.

INTRODUCTION: : Glutamic acid decarboxylase (GAD) is the rate-limiting enzyme converting glutamate into gamma-aminobutyric acid. Impaired GAD function can alter motor, cognitive, and behavioral function. Anti-GAD antibodies (GADAbs) can cause several neurological disorders. However, the association between anti-GADAbs and pure psychosis, without seizures or focal neurological deficits, is not well defined. CASE REPORT: : A 19-year-old woman with recent-onset psychotic disorder was diagnosed with schizophrenia. Brain magnetic resonance imaging and cerebrospinal fluid analysis were normal. Serum anti-GADAb titers were elevated. Brain biopsy showed subcortical gliosis and microglia-macrophage infiltration. The clinical syndrome improved with immune therapy. CONCLUSIONS: : Severe psychosis and mild cognitive decline without other neurological features, meeting the Diagnostic and Statistical Manual of Mental Disorders, 4th edition, text revision diagnostic criteria for schizophrenia, can result from brain inflammation associated with elevated serum anti-GADAbs.

Although functional status serves as a major predictor of morbidity, researchers and clinicians use different terms and measures, limiting comparisons across studies. To demonstrate how differing measures may generate varied findings, we compared and contrasted data from the SF-12 Health Survey Physical Component Summary Scale (SF-12 PCS) and the Enforced Social Dependency Scale (ESDS). The sample consisted of 49 women aged 65 and older recovering from gynecological cancer surgery with data collection at baseline (postoperative period) and then at 3 and 6 months. Analysis of the relationship between SF-12 PCS and ESDS over time using generalized estimating equations (GEE) demonstrated the relationship was less than 1.0, signaling less than perfect agreement between measures (beta = 0.16, P = .002). These findings suggest that that the 2 measures are not interchangeable and may produce conflicting evidence. This highlights the importance of researchers' and clinicians' careful conceptualization and operationalization of functional status before measure selection.

Cochlear implants have become a viable treatment option for individuals who present with severe to profound hearing loss. While there are several parameters that affect the successful use of this technology, quality programming of the cochlear implant system is crucial. This review chapter focuses on general device programming techniques, programming techniques specific to children, objective programming techniques, a brief overview of programming parameters of the currently commercially available multichannel systems, and managing patient complaints and device failures. The chapter also provides what the authors believe the future may hold for new programming techniques

This study investigated the integration of place- and temporal-pitch cues in pitch contour identification (PCI), in which cochlear implant (CI) users were asked to judge the overall pitch-change direction of stimuli. Falling and rising pitch contours were created either by continuously steering current between adjacent electrodes (place pitch), by continuously changing amplitude modulation (AM) frequency (temporal pitch), or both. The percentage of rising responses was recorded as a function of current steering or AM frequency change, with single or combined pitch cues. A significant correlation was found between subjects' sensitivity to current steering and AM frequency change. The integration of place- and temporal-pitch cues was most effective when the two cues were similarly discriminable in isolation. Adding the other (place or temporal) pitch cues shifted the temporal- or place-pitch psychometric functions horizontally without changing the slopes. PCI was significantly better with consistent place- and temporal-pitch cues than with inconsistent cues. PCI with single cues and integration of pitch cues were similar on different electrodes. The results suggest that CI users effectively integrate place- and temporal-pitch cues in relative pitch perception tasks. Current steering and AM frequency change should be coordinated to better transmit dynamic pitch information to CI users.

Implantation of the ossified and dysplastic cochlea presents many unique challenges to both the surgeon and programming team. Altered embryology and physiology of these labyrinthine dysplasias may result in forms and functions unfamiliar to those casually involved with cochlear implants. Remarkable developments in diagnosis, surgical technique, electrode design, processing strategies, and programming have all contributed to the ability to successfully implant patient populations previously excluded from this life-changing intervention

Since the first cochlear implant approved by the US Food and Drug Administration in the early 1980s, great advances have occurred in cochlear implant technology. With these advances, patient selection, preoperative evaluation, and rehabilitation consideration continue to evolve. This article describes the current practice in pediatric candidacy evaluation, reviews the medical and surgical considerations in pediatric cochlear implantation, and explores the expanding criteria for cochlear implantation within the pediatric population