Wegener's Granulomatosis Presenting as a Parapharyngeal Mass.

BACKGROUND: Wegener's Granulomatosis (WG) is classically characterized by the triad of sinonasal, pulmonary, and renal manifestations. However, a growing body of research has demonstrated that atypical head and neck pathologies including retropharyngeal and parapharyngeal lesions are often the presenting feature of patients found to have WG. CASE: This report describes the hospital course of a patient who presented with a parapharyngeal mass with secondary superior cervical chain compression and Lemierre's Syndrome. We discuss how a stepwise approach involving a multidisciplinary team led to the diagnosis of WG. CONCLUSIONS: To our knowledge, this report is the first description of a patient presenting with a parapharyngeal mass causing superior cervical chain compression with simultaneous Lemierre's Syndrome who was ultimately diagnosed with WG. We highlight how the early consideration of WG in patients with atypical head and neck lesions refractory to multiple treatment regimens can led to an expeditious diagnosis and the coordination of appropriate short-term and long-term care.

3D Printed Pediatric Temporal Bone: A Novel Training Model.

OBJECTIVE: Temporal bone dissection is a fundamental element of otologic training. Cadaveric temporal bones (CTB) are the gold standard surgical training model; however, many institutions do not have ready access to them and their cost can be significant: $300 to $500. Furthermore, pediatric cadaveric temporal bones are not readily available. Our objective is to develop a pediatric temporal bone model. STUDY DESIGN: Temporal bone model. SETTING: Tertiary Children's Hospital. SUBJECTS: Pediatric patient model. METHODS: We describe the novel use of a 3D printer for the generation of a plaster training model from a pediatric high- resolution CT temporal bone scan of a normal pediatric temporal bone. RESULTS: Three models were produced and were evaluated. The models utilized multiple colors (white for bone, yellow for the facial nerve) and were of high quality. Two models were drilled as a proof of concept and found to be an acceptable facsimile of the patient's anatomy, rendering all necessary surgical landmarks accurately. The only negative comments pertaining to the 3D printed temporal bone as a training model were the lack of variation in hardness between cortical and cancellous bone, noting a tactile variation from cadaveric temporal bones. CONCLUSION: Our novel pediatric 3D temporal bone training model is a viable, low-cost training option for previously inaccessible pediatric temporal bone training. Our hope is that, as 3D printers become commonplace, these models could be rapidly reproduced, allowing for trainees to print models of patients before performing surgery on the living patient.

Unusual case of neonatal hypercalcemia.

We present a rare case of neonatal hyperparathyroidism secondary to a homozygous calcium sensing receptor (CASR) mutation, diagnosed by the genetics team. The CASR mutation is a homozygous inactivating mutation at the calcium sensing receptor. Inactivation of the receptor leads to hypercalcemia and activation leads to hypocalcemia. Heterozygous mutations can cause mild forms of asymptomatic hypercalcemia that often run in families. The homozygous mutation causes a rare form of neonatal severe hyperparathyroidism.

Does electrocautery damage cochlear implants?

OBJECTIVES: 1) Evaluate the effects of monopolar cautery on cochlear implant devices. 2) Determine whether voltage fluctuations within the cochlear implant adversely affect the cochlear implant devices STUDY DESIGN: Two Med-El cochlear implants modified to record voltage difference from the apical and proximal electrodes were implanted into an unembalmed, fresh cadaver. Cautery was applied to the ipsilateral pectoralis major muscle and ipsilateral temporalis muscle at bipolar, monopolar coagulation, and monopolar cut settings of 50 W. The intensity in each modality setting was increased by increments of 10 W to a maximum of 100 W. Integrity testing was performed before, during, and after each cautery setting. Voltage fluctuations were measured during cautery, and maximal voltage changes for each setting were noted. After explantation, devices were returned to the manufacturer for in-depth failure analysis to evaluate for any damage to the devices. SETTING: Tertiary medical center. SUBJECTS: Cadaveric study. METHODS: Basic science laboratory. RESULTS: No change in impedance or integrity testing occurred at any cautery setting when applied to either to pectoralis major or temporalis. The maximum voltage change was 22 V. Comprehensive device analysis showed no evidence of device damage from the study. CONCLUSIONS: The cochlear implant devices had no evidence of electrical damage by monopolar cautery, even up to levels of 100 W in the temporalis muscle. The maximum voltage change was 22 V, likely resulting from protecting diodes within the implant. Additional study is necessary, but more flexible recommendations regarding electrosurgery in cochlear implant recipients should be considered.

Round window insertion of precurved electrodes is traumatic.

OBJECTIVE: Assess endocochlear trauma by adjusting: 1) location of cochleostomy or round window insertion, and 2) size of precontoured electrode array. STUDY DESIGN: Cadaveric temporal bone study. METHODS: Locations of electrode placement into the cochlea were as follows: 1) round window, 2) anterior inferior to the round window, 3) anterior inferior to the round window niche, 4) superior to the round window niche. Two types of electrode arrays were used: a larger precontoured electrode and thinner precurved research electrode. Histologic sections were made by a blinded third party. RESULTS: Fourteen bones were included in the study. Six (42.8%) of the bones were right ears. Seven bones had no endocochlear trauma. Seven bones have intracochlear trauma. Round window insertions had a high incidence of intracochlear trauma with precontoured electrodes (3/4 bones). Superior cochleostomies with electrode placement had significant intracochlear trauma (2/2 bones). Insertions made anterior inferior to the round window annulus had a 50% incidence of intracochlear trauma (2/4 bones). No endocochlear trauma observed for insertions through traditional cochleostomies (4/4 bones). Less endocochlear trauma was observed with the thinner electrode: 57% versus 42% in the larger electrode array. A higher incidence of tip fold-over was observed with the thinner electrode array (2/7 electrodes). No tip fold-over was noted in the larger electrode. CONCLUSION: The traditional cochleostomy had the least incidence of endocochlear trauma. The smaller electrode array did not significantly affect the incidence of endocochlear trauma, but the thinner array had a higher incidence of tip fold-over, which caused trauma distally. LEVEL OF EVIDENCE: 2C.

Monopolar cautery and adverse effects on cochlear implants.

IMPORTANCE: The use of monopolar cautery has been widely regarded as a contraindication in the setting of a cochlear implant because of presumed risk to the implant device. There are very limited data to support this contraindication. OBJECTIVES: (1) To evaluate the effects of monopolar cautery on cochlear implant devices. (2) To determine whether monopolar cautery changes the endocochlear temperature in an implanted cochlea. DESIGN: Sixteen cochlear implants from 3 manufacturers (Advanced Bionics LLC, Cochlear Americas Ltd, and Med-El Ltd) were implanted into 2 unembalmed, fresh cadavers. Monopolar cautery was applied to either the tongue or abdomen at coagulation settings of 10 W or 50 W for 30 minutes. Impedance and integrity testing were performed before, during, and after 30 minutes of cautery. The temperature in the endocochlear perilymph was measured during cautery. After explantation, devices were returned to the manufacturer for an in-depth "failure" analysis according to each manufacturer's current protocol evaluating explanted devices. SETTING: Basic science laboratory, tertiary medical center. PARTICIPANTS: Cadaveric study. INTERVENTION: Application of monopolar cautery to implanted cochlear implants in a cadaveric model. MAIN OUTCOME AND MEASURE: (1) Changes to the implanted devices either during electrocautery or following failure analysis. (2) Changes in the intracochlear temperature. RESULTS: No change in impedance, integrity testing, or failure analysis occurred at any cautery setting when applied to either the oral cavity or abdomen. The temperature of the cochlea did not increase with up to 30 minutes of cautery at a setting of 50 W. Comprehensive device analysis did not show any evidence of device damage at the conclusion of the study. CONCLUSIONS AND RELEVANCE: Monopolar cautery did not produce detectible damage to any of the cochlear implant devices or produce detectible temperature change in the cochlea at low or high levels of cautery in the oral cavity in this experimental model.

FDG PET/CT incidental diagnosis of a synchronous bladder cancer as a fourth malignancy in a patient with head and neck cancer.

A 74-year-old man with 40-year history of smoking and known history of chronic lymphocytic leukemia and cutaneous T-cell lymphoma underwent FDG PET/CT examination for a recent diagnosis of squamous cell carcinoma diagnosed from right frontal crown and left posterior ear skin biopsy. PET images revealed multiple FDG-avid lesions in the head and neck, highly suspicious for nodal metastases. Reviewing CT portion of PET/CT examination revealed a hyperattenuating density in the posterior bladder wall. This lesion was not noticed initially due to the intense physiologic bladder uptake. On lowering the intensity, this lesion showed intense FDG avidity on the PET portion of the examination. Cytoscopic biopsy revealed low-grade papillary urothelial cell carcinoma.

Otolaryngologic manifestations of mitochondrial cytopathies.

INTRODUCTION: Primary mitochondrial cytopathies include more than 40 different identified diseases that have varied genetic features. The common factor among these diseases is that the mitochondria are unable to effectively complete oxidative phosphorylation to generate energy. One in 5000 children per year in the United Sates are born with mitochondrial disease. The term primary mitochondrial disease refers specifically to mitochondrial dysfunction caused by genetic mutations directly impacting the composition and function of the respiratory chain. These defects impair mitochondrial oxidative phosphorylation, the process by which oxidation of the end products of metabolism in the electron transport chain is coupled to phosphorylation of adenosine diphosphate to produce energy in the form of adenosine triphosphate. The effects of mitochondrial disease can be quite varied, and the severity of the defect may be great or small. We sought to find the common otolaryngologic manifestations of this disease, to enable better treatment of the patients. We report on 41 pediatric cases of mitochondrial cytopathy diagnosed from 2001 to 2006 at our Center for Child Neurology (Cleveland, OH). We performed a retrospective review after obtaining institutional review board approval. Patients who had otologic manifestations including recurrent otitis media and hearing loss comprise 14.6%. Patients who were noted to have obstructive sleep apnea requiring adenotonsillectomy comprise 9.8%. Patients who had dysphagia comprise 22%; however, all of these patients were noted to have some developmental delay and seizures. It was also noted that there was a 17% incidence of gastroesophageal reflux, and 36.6% incidence of seizures in our patient population. In conclusion, mitochondrial cytopathies exhibit extreme clinical variability. This variability greatly increases the burden for affected families and impairs our ability to understand the pathogenesis of these disorders. In otolaryngologic examination, children appear to have recurrent otitis, dysphagia, and sleep apnea, at a much higher incidence than the general pediatric population. A comprehensive management approach to these patients including pediatric neurology, genetics, speech pathology, otolaryngology, and audiology is essential to their care.

Choanal atresia: embryologic analysis and evolution of treatment, a 30-year experience.

OBJECTIVES: Choanal atresia (CA) is a congenital obstruction of the posterior nasal apertures. Multiple surgical techniques have been proposed to repair the atresia. The purpose of this study is to review the basic science and embryology of CA with emphasis on the senior author's extensive experience, and refinement of the treatment of CA. In addition, we will review the outcomes after surgical correction of pediatric patients with CA. PATIENTS AND METHOD: : Retrospective review of 73 pediatric patients. Demographic information was recorded, including type of CA, concomitant medical problems, surgical method, and date of last follow-up by the surgeon. RESULTS: Our series show a 1.6:1 ratio of unilateral atresia to bilateral atresia. The incidence in males and females is statistically equal. High-arched palate and cross-bite deformities are particularly present if the patients undergo the transpalatal approach in the first year of life. The senior author has evolved to favoring the endoscopic approach, having a minimal long-term complication rate, and low stenosis rate (12%). CONCLUSIONS: The first repair of choanal atresia is the one most likely to succeed. The nasal endoscopic technique is the favored technique. It has the advantages of a low restenosis rate, does not violate the palate, and does not cause the cross-bite and palate arch deformities seen in the transpalatal technique.

Nonsteroidal anti-inflammatory drugs and postoperative bleeding following adenotonsillectomy in pediatric patients.

OBJECTIVE: To assess the effects of nonsteroidal anti-inflammatory drugs (NSAIDs) on bleeding for pediatric adenotonsillectomy in a retrospective study, based on the common practices at 2 different tertiary care facilities. DESIGN: A retrospective study. SETTING: Two different tertiary care facilities. PATIENTS: Children up to 16 years of age, who underwent elective adenotonsillectomy or tonsillectomy, were included in the study. All indications for adenotonsillectomy, and all surgical techniques were included. Children with a bleeding tendency, and those with contraindications to the use of NSAIDs (eg, because of allergy), were excluded from the study. INTERVENTIONS: Nonsteroidal anti-inflammatory drugs. MAIN OUTCOME MEASURE: Postoperative bleeding in patients. RESULTS: A total of 1160 patients were selected who met the criteria: 673 patients underwent an adenotonsillectomy or tonsillectomy and did not receive any preoperative and postoperative ibuprofen, and 487 patients underwent routine adenotonsillectomy or tonsillectomy and were given postoperative ibuprofen. We noted a 0.7% postoperative bleeding rate in patients who were not allowed to take ibuprofen perioperatively. There was a 1.0% postoperative bleeding rate in patients who were allowed to take ibuprofen perioperatively (P = .75). CONCLUSION: Ibuprofen is not a contraindication to adenotonsillectomy or tonsillectomy and should be used in the control of postoperative pain if it is indicated in the patient.

The prevalence and clinical course of patients with 'incidental' acoustic neuromas.

CONCLUSIONS: This study suggests that among patients diagnosed with 'incidental' acoustic neuromas (ANs), a substantial portion are discovered incidentally. Small and medium-sized ANs that are found incidentally may have a more benign nature, and may be less likely to require interventions. OBJECTIVE: To estimate the prevalence of ANs, and to compare the prognosis and progression of the disease between those diagnosed incidentally verse symptomatically with an AN. PATIENTS AND METHODS: This was a retrospective evidence-based case series of patients with AN treated at a tertiary medical center between November 1999 and January 2005. An MRI with gadolinium was performed on all patients to establish the diagnosis of AN. A medical chart review of these patients was searched for sex distribution, age, presenting symptoms, hearing loss, speech discrimination scores, tumor characteristics by imaging, intervention performed, and time between diagnosis and intervention. The studied population was divided into those patients with pre-imaging audiovestibular symptoms provoking a clinical suspicion of AN (symptomatic group) and those without a pre-imaging suspicion of AN (incidental group). RESULTS: The charts of 120 patients with ANs were analyzed and categorized as either incidentally or symptomatically discovered. Incidentally discovered ANs accounted for 12% of patients with the diagnosis of AN in our population. The average age at diagnosis was 55.7 and 52.8 years (p = 0.50) in the symptomatic and incidental groups, respectively. The gender distribution was not different between the groups (p = 0.08). Audiometry revealed a speech discrimination score asymmetry greater in the symptomatic group (p < 0.0001). Tumor size by imaging performed at diagnosis in the incidental population was 1.09 cm on average, compared with 1.5 cm in the symptomatic patients (p = 0.08). A greater proportion of patients with symptomatically discovered AN underwent intervention by surgical resection, stereotactic radiosurgery, or radiation compared with patients with incidentally discovered AN (76% versus 47%, p = 0.02).

Effectiveness of amitriptyline versus cough suppressants in the treatment of chronic cough resulting from postviral vagal neuropathy.

OBJECTIVE: The objective of this prospective, randomized, controlled study (N = 28) was to evaluate the effectiveness of amitriptyline versus cough suppressants in the treatment of chronic cough resulting from postviral vagal neuropathy. METHODS: Patients were selected based on a clinical history consistent with postviral vagal neuropathy and a history of an antecedent upper respiratory tract infection. All patients had been tried on antireflux medication (proton pump inhibitors) and had a negative chest x-ray before presentation. All were nonsmokers without a history of asthma. Patients on angiotensin-converting enzyme inhibitors were excluded from the study. All patients completed a pretreatment, validated cough-specific quality-of-life (QOL) survey. Patients were randomized by chart numbers to either 10 mg amitriptyline at bedtime or 10 to 100 mg/5 mL, 10 mL codeine/guaifenesin every 6 hours standing dose while awake. Both groups were instructed to complete 10 days of therapy and then asked to subjectively rate the reduction in the frequency and severity of their cough by 100%, 75%, 50%, 25%, or 0% as well as completing the posttreatment cough QOL questionnaire. Those patients experiencing a 75% to 100% reduction were recorded as having a complete response, 25% to 50% a partial response, and 0% as having no response. Final results and the cough QOL survey were recorded and used for statistical analysis. RESULTS: A majority of patients in the amitriptyline group achieved a complete response on the initial dose of 10 mg. None of the codeine/guaifenesin group achieved a complete response. The data were analyzed using a logistic regression model, and amitriptyline was found to be a highly significant predictor of a greater than 50% response when compared with codeine/guaifenesin (P = .0007). The same data were analyzed using a proportional odds model and similar results were noted. CONCLUSIONS: Chronic cough can have a profound impact on the psychosocial function of patients. The most common causes of a persisting cough in the absence of infection or chronic smoking are laryngopharyngeal reflux, asthma, particularly the cough variant, allergy, rhinosinusitis, bronchitis, and medications, in particular angiotensin-converting enzyme inhibitors. Currently, there are few effective treatments for cough with an acceptable therapeutic ratio and more selective drugs with a more favorable side effect profile are needed. This is this first prospective, randomized, controlled study comparing the effectiveness of amitriptyline versus codeine/guaifenesin for select cases of chronic cough resulting from suspected postviral vagal neuropathy.

Exploratory tympanotomy revealing incus discontinuity and stapedial otosclerosis as a cause of conductive hearing loss.

OBJECTIVE: To describe a unilateral progressive conductive hearing loss caused by incus discontinuity (without erosion of the long process of the incus), and otosclerosis with fixation of the stapedial footplate. STUDY DESIGN: Case report. SETTING: Department of Otolaryngology, Head and Neck Surgery of the University of Rochester Medical Center, which is a regional tertiary referral center. PATIENT: A 54-year-old woman with multiple otologic complaints including tympanic membrane perforations, otalgia, tinnitus, and hearing loss. Audiography demonstrated 100% speech discrimination bilaterally and a significant conductive right-sided hearing loss. INTERVENTION: The patient underwent a stapedectomy, during which a discontinuity between the long process of the incus and the stapes with no bony erosion was identified. The stapedectomy was completed and an ossicular piston prosthesis was inserted to reestablish ossicular continuity with the tympanic membrane. MAIN OUTCOME MEASURE: Improved subjective hearing confirmed objectively by audiography. CONCLUSION: This is the third reported case of an unusual combination of otosclerosis and ossicular discontinuity, and the first such case report in a patient without head trauma. In addition, it adds a unique item to the differential diagnosis of the pathologic features implicated in an ear with a conductive deficit and normal tympanogram.

Review of nasopharyngeal carcinoma.

We review the literature on nasopharyngeal carcinoma that has been published within the past 5 years. Nasopharyngeal carcinoma is a highly morbid disease, and survival is poor. Its management remains extremely difficult, not just for otolaryngologists but for radiation oncologists and medical oncologists, as well. A clear understanding of its etiology is still lacking, but nasopharyngeal carcinoma is widely suspected to be the result of both a genetic susceptibility and exposure to environmental factors or Epstein-Barr virus infection. With no clear cause, treatment is controversial. For example, an optimal radiation regimen has not been determined, reports in the literature regarding the role of chemotherapy for advanced disease are conflicting, and treatment of local recurrences is unsettled. Still, advances in immunologic research and chemotherapy offer hope for better control of the disease. We hope that our assessment of the recent literature will provide otolaryngologists with a more clear understanding of the etiology and management of nasopharyngeal carcinoma.