Faculty Bibliography

Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of growth and/or development, Genital and/or urinary abnormalities, and Ear abnormalities and deafness (CHARGE) syndrome is a disorder with multiple congenital anomalies seen on imaging. A retrospective review of 10 patients with CHARGE syndrome who underwent MR imaging of the brain as part of a preoperative evaluation for cochlear implantation was conducted. Structural abnormalities of the entire MR imaging of the head were evaluated, including the auditory system, olfactory system, face, skull base, and central nervous system. The most frequent MR imaging findings included dysplasias of the semicircular canals and hypoplasia of the frontal lobe olfactory sulci. Less frequent findings included cleft lip/palate and coloboma. Our study uncovered new findings of a J-shaped sella, dorsal angulation of the clivus, and absent/atrophic parotid glands, not previously described in patients with CHARGE. Our results emphasize the utility of MR imaging in the diagnosis and management of patients with CHARGE syndrome.

HYPOTHESIS: A novel smartphone-based software application can facilitate self-selection of frequency allocation tables (FAT) in postlingually deaf cochlear implant (CI) users. BACKGROUND: CIs use FATs to represent the tonotopic organization of a normal cochlea. Current CI fitting methods typically use a standard FAT for all patients regardless of individual differences in cochlear size and electrode location. In postlingually deaf patients, different amounts of mismatch can result between the frequency-place function they experienced when they had normal hearing and the frequency-place function that results from the standard FAT. For some CI users, an alternative FAT may enhance sound quality or speech perception. Currently, no widely available tools exist to aid real-time selection of different FATs. This study aims to develop a new smartphone tool for this purpose and to evaluate speech perception and sound quality measures in a pilot study of CI subjects using this application. METHODS: A smartphone application for a widely available mobile platform (iOS) was developed to serve as a preprocessor of auditory input to a clinical CI speech processor and enable interactive real-time selection of FATs. The application's output was validated by measuring electrodograms for various inputs. A pilot study was conducted in six CI subjects. Speech perception was evaluated using word recognition tests. RESULTS: All subjects successfully used the portable application with their clinical speech processors to experience different FATs while listening to running speech. The users were all able to select one table that they judged provided the best sound quality. All subjects chose a FAT different from the standard FAT in their everyday clinical processor. Using the smartphone application, the mean consonant-nucleus-consonant score with the default FAT selection was 28.5% (SD 16.8) and 29.5% (SD 16.4) when using a self-selected FAT. CONCLUSION: A portable smartphone application enables CI users to self-select frequency allocation tables in real time. Even though the self-selected FATs that were deemed to have better sound quality were only tested acutely (i.e., without long-term experience with them), speech perception scores were not inferior to those obtained with the clinical FATs. This software application may be a valuable tool for improving future methods of CI fitting.

Tympanic paragangliomas (TP) arise in the middle ear along the course of Jacobson's or Arnold's nerve. These lesions can vary from small masses on the cochlear promontory to tumors that extend into the mastoid and external auditory canal. Surgical excision is the treatment of choice for TP with very low-recurrence rates. The various surgical approaches for TP are adapted to the configuration of individual tumors to provide adequate exposure to the margins of the tumor in the mesotympanum, hypotympanum, and mastoid. Using these surgical approaches in combination with meticulous dissection and hemostasis with adjunctive tools such as laser and micro-bipolar instruments allows for safe and effective resection of these vascular tumors from within the confines of the tympanic cavity

OBJECTIVE: To describe the clinical outcomes of patients undergoing serial observation for vestibular schwannoma (VS) and identify factors that may predict tumor growth or hearing loss. STUDY DESIGN: Retrospective review. METHODS: A retrospective review was conducted of patients seen at a tertiary care medical center between 2002 and 2013 with an International Classification of Diseases-9 diagnosis code of 225.1. Patients electing observation as initial management, with at least two documented imaging results, were included. Exclusion criteria comprised bilateral VS, diagnosis of neurofibromatosis type 2, and neoplasms other than VS. Decline in serviceable hearing, tumor growth, and changes in management strategy were recorded. Survival analysis to assess median time to outcomes and multiple logistic regression analyses were performed. RESULTS: A total of 94 patients met inclusion criteria. While undergoing observation, 22.3% of patients underwent a change in management strategy to microsurgical excision or stereotactic radiotherapy. For patients with initial serviceable hearing, 24.3% observed a decline to a nonserviceable level. No significant clinical factors were identified to predict changes in hearing. Survival analysis revealed that an estimated 69.1% of patients electing observation as initial management continued to do so at 5 years. Imbalance or disequilibrium at presentation was found to be associated with an increased adjusted odds ratio (OR) (OR 2.96; 95% confidence interval, 1.03-8.50; P = 0.04) for tumor growth. CONCLUSION: Serial observation of VS is a viable treatment strategy for selected patients, with two-thirds of patients electing to continue this management option after 5 years. Disequilibrium as a presenting symptom may be associated with subsequent tumor growth. LEVEL OF EVIDENCE: 4. Laryngoscope, 2015.

OBJECTIVES/HYPOTHESIS: This study investigated the differences between the standard guidelines and the practice patterns of otolaryngologists in managing "penicillin-allergic" patients. A major goal was to identify factors influencing an otolaryngologist's choice of antibiotic. STUDY DESIGN: Cross-sectional survey. METHODS: Four hundred seventy members of the American Society of Pediatric Otolaryngologists (ASPO) and 150 general otolaryngologists from the Florida Society of Otolaryngology (FSO) were surveyed. RESULTS: Ninety-six ASPO members (20.4%) and 22 members of FSO (14.6%) responded. When asked about the management of a pediatric patient with acute otitis media and a history of a nonsevere immunoglobulin E (IgE)-mediated amoxicillin allergy, 54% of ASPO respondents indicated they would initiate guideline-recommended cefdinir, whereas only 27% of FSO respondents chose cefdinir (P = .02). Otolaryngologists who are fellowship trained in pediatrics or have pediatric-focused practices were significantly more likely to prescribe cefdinir. Overall, 57% of respondents indicated that they were familiar with the literature regarding the cross-reactivity of beta-lactams, but only 25% of respondents felt that they could easily differentiate a potentially life-threatening IgE-mediated allergy from a non-IgE-mediated drug intolerance. CONCLUSIONS: The data show differences between the current recommendations and the behavior of otolaryngologists. Pediatric otolaryngologists were more familiar with the guideline-recommended therapy, likely from their frequent exposure to patients requiring a beta-lactam. Nevertheless, most otolaryngologists could benefit from increased awareness of the current literature. Patients may be receiving less than optimal medication management due to a misidentification of those at risk of life- threatening allergic cross-reactions. LEVEL OF EVIDENCE: NA Laryngoscope, 2015.

BACKGROUND: Spontaneous cerebrospinal fluid (CSF) leaks are caused by intracranial hypertension. Given this underlying etiology, patients may be at risk for developing multiple skull base defects. OBJECTIVE: The purpose of our study is to present the prevalence of multiple simultaneous skull base defects in patients with spontaneous CSF rhinorrhea. METHODS: We performed a retrospective chart review in a tertiary care practice of 44 consecutive patients with spontaneous CSF rhinorrhea who underwent endoscopic repair by the senior author (R.R.C.) to determine the prevalence of having multiple simultaneous skull base defects identified at the time of surgery. We defined this as two or more bony defects identified endoscopically with intact intervening bone with or without soft tissue prolapse into the nasal cavity or paranasal sinus cavity. RESULTS: Eight of 44 patients (18.2%) were found to have multiple simultaneous skull base defects. The average body mass index (BMI) of the study population was 34.5 (range, 22.7-59). CONCLUSION: A significant number of patients with spontaneous CSF rhinorrhea may have more than one skull base defect present at the time of presentation. The clinical significance of this finding in surgical and medical decision making is not clear at this time.

Motion sickness can be a limiting factor for sea and air missions. We report the experience of a Pararescue (PJ) team on a Pacific Ocean rescue mission in which motion sickness was prevalent. Cinnarizine, an antagonist of H1-histamine receptors, was used to treat affected PJs. We also report findings of a survey of PJs regarding motion sickness. A family of four on a disabled sailboat 900 miles off the coast of Mexico sent out a distress call because their 1-year-old daughter became severely ill with fever and diarrhea. Four PJs were deployed on a C-130, performed a free-fall parachute insertion into the ocean, and boarded the sailboat. All four PJs experienced onset of motion sickness at some point during the early part of the mission and symptoms persisted through the first 24 hours. Three PJs experienced ongoing nausea, vomiting, dizziness, and sensory imbalances. The captain of the sailboat offered the three sick PJs approximately 18mg of cinnarizine two or three times a day with relief of symptoms and improvement on operational effectiveness. A new, anonymous, voluntary survey of Air National Guard PJs and combat rescue officers revealed that 78.4% of Operators have experienced motion sickness at sea. We discuss the current theories on motion sickness, the effect of motion sickness on operational effectiveness, and research on treatment of motion sickness, including the medication cinnarizine.

OBJECTIVE: To determine the prevalence of otolith dysfunction in patients with refractory benign paroxysmal positional vertigo (BPPV). STUDY DESIGN: Unmatched case control. SETTING: Tertiary care institution. SUBJECTS AND METHODS: Patients included were diagnosed with BPPV, failed initial in-office canalith repositioning maneuvers (CRMs), and completed vestibular testing and vestibular rehabilitation (n = 40). Refractory BPPV (n = 19) was defined in patients whose symptoms did not resolve despite vestibular rehabilitation. These patients were compared with a control group of those with nonrefractory BPPV (n = 21) for results of a caloric test, cervical vestibular evoked myogenic potential (cVEMP), and subjective visual vertical (SVV). RESULTS: Forty-six of 251 patients failed initial treatment with in-office CRM. Forty patients met inclusion criteria. There was no significant difference between the cases (refractory BPPV) (n = 19) and controls (nonrefractory BPPV) (n = 21) in terms of age, duration of symptoms, laterality of BPPV, and BPPV symptoms. There was no difference in the prevalence of caloric weakness and cVEMP abnormalities (P > .05), with odds ratios (ORs [95% confidence interval (CI)]) of having abnormal results among cases vs controls of 1.1818 (0.3329-4.1954) and 4.3846 (0.7627-25.2048), for caloric and cVEMP, respectively. Abnormal eccentric SVV was more prevalent in refractory BPPV cases (58%) than in controls (14%) (P < .0072). The OR (95% CI) of having abnormal SVV was 8.25 (1.7967-37.8822) higher among patients with refractory BPPV than those with nonrefractory BPPV. CONCLUSION: Patients with refractory BPPV are more likely to have abnormal eccentric SVV and thus underlying utricular dysfunction. This finding is important to take into account when designing rehabilitation strategies for patients with BPPV who fail CRM.

OBJECT: The resection of glomus jugulare tumors can be challenging because of their inherent vascularity. Preoperative embolization has been advocated as a means of reducing operative times, blood loss, and surgical complications. However, the incidence of cranial neuropathy associated with the embolization of these tumors has not been established. The authors of this study describe their experience with cranial neuropathy following transarterial embolization of glomus jugulare tumors using ethylene vinyl alcohol (Onyx, eV3 Inc.). METHODS: The authors retrospectively reviewed all cases of glomus jugulare tumors that had been treated with preoperative embolization using Onyx at their institution in the period from 2006 to 2012. Patient demographics, clinical presentation, grade and amount of Onyx used, degree of angiographic devascularization, and procedural complications were recorded. RESULTS: Over a 6-year period, 11 patients with glomus jugulare tumors underwent preoperative embolization with Onyx. All embolization procedures were completed in one session. The overall mean percent of tumor devascularization was 90.7%. No evidence of nontarget embolization was seen on postembolization angiograms. There were 2 cases (18%) of permanent cranial neuropathy attributed to the embolization procedures (facial nerve paralysis and lower cranial nerve dysfunction). CONCLUSION: Embolizing glomus jugulare tumors with Onyx can produce a dramatic reduction in tumor vascularity. However, the intimate anatomical relationship and overlapping blood supply between these tumors and cranial nerves may contribute to a high incidence of cranial neuropathy following Onyx embolization.