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OBJECTIVE: Conduct a comparison study between conventional audiometry and a tablet-based hearing screening application, Kids Hearing Game (KHG). If KHG measures hearing at levels comparable with conventional audiometry, it could be used to screen hearing in children. METHODS: Prospective equivalence study where measurements of pure tone hearing via KHG are compared with measurements of pure tone hearing via conventional audiometry in patients aged 6-11 years over a 4-month period. Eighteen patients completed the study. Results from 36 ears were included in the data for analysis. Decibel measurements from each frequency measured with KHG for each ear were compared with conventional audiometry. Mean measurements were calculated for each ear and frequency as well as mean differences in measurements at each ear and frequency. Tests of equivalence were used to assess mean within-subject differences in decibel measurements using a 10-dB zone of indifference. RESULTS: Mean decibel measurements using KHG for the right ear at 500, 1000, 2000, 4000, and 8000 Hz and the left ear at 1000, 2000, 4000, and 8000 Hz were found equivalent to conventional audiometry (p < 0.050). The mean decibel measurement using KHG for the left ear at 500 Hz was found not equivalent (p = 0.101). However, when left and right ear data were analyzed together, KHG was found to be equivalent to conventional audiometry across all frequencies. Eight patients having hearing loss greater than 25 dB on conventional audiometry were also identified by KHG to have hearing loss. CONCLUSION: KHG is comparable to conventional audiometry and may be used as a screening tool for children.

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OBJECTIVE: To develop a three-dimensional study tool of the membranous labyrinth in order to study the pathophysiology, diagnostic workup and treatment of benign paroxysmal positional vertigo (BPPV). BPPV is the most common cause of peripheral vertigo. Its diagnosis and treatment depend on an understanding of the anatomy of the vestibular labyrinth and its position relative to the head. To date, many illustrations have been made to explain principals of diagnosis and treatment of BPPV, but few have been based on anatomical studies of the membranous labyrinth. METHODS: A cadaveric human membranous labyrinth was axially sectioned at 20 µm resolution, stained and segmented to create a high-resolution digital model. The model was cloned to create an enantiomeric pair of labyrinths. These were associated a 3D model of a human skull, segmented from MRI data, and were oriented according to established anatomic norms. Canal markers representing otoliths were created to mark canalith position during movement of the model within the 3D environment. RESULTS: The model allows visualization of true membranous labyrinth anatomy in both ears simultaneously. The dependent portion of each semicircular duct and of the utricle can easily be visualized in any head position. Moveable markers can mark the expected progress of otolith debris with changes in head position and images can be captured to document simulations. The model can be used to simulate pathology as well as diagnostic maneuvers and treatment procedures used for BPPV. The model has great potential as a teaching tool. CONCLUSION: A simple model based on human anatomy has been created to allow careful study of BPPV pathophysiology and treatment. Going forward, this tool could offer insights that may lead to more accurate diagnosis and treatment of BPPV.

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OBJECTIVES: We describe the histopathologic findings in the temporal bones of a patient who had, during life, received a diagnosis of superior canal dehiscence (SCD) syndrome. METHODS: The patient was found to have SCD syndrome at 59 years of age. She became a temporal bone donor, and died of unrelated causes at 62 years of age. Both temporal bones were prepared in celloidin and examined by light microscopy. RESULTS: The patient developed bilateral aural fullness, pulsatile tinnitus, and difficulty tolerating loud noises after minor head trauma at 53 years of age. The symptoms were worse on the right. She also had Valsalva-induced dizziness and eye movements, as well as sound-induced dizziness (more prominent on the right). Audiometry showed a small air-bone gap of 10 dB in the right ear. Vestibular evoked myogenic potential testing showed an abnormally low threshold of 66 dB on the right, and a computed tomography scan showed dehiscence of the superior canal on the right. Histopathologic examination of the right ear showed a 1.4 x 0.6-mm dehiscence of bone covering the superior canal. Dura was in direct contact with the endosteum and the membranous duct at the level of the dehiscence. No osteoclastic process was evident within the otic capsule bone surrounding the dehiscence. The left ear showed thin but intact bone over the superior canal. Both ears showed focal microdehiscences of the tegmen tympani and tegmen mastoideum. The auditory and vestibular sense organs on both sides appeared normal. No endolymphatic hydrops was observed. CONCLUSIONS: The findings were consistent with the hypothesis put forth by Carey and colleagues that SCD may arise from a failure of postnatal bone development, and that minor trauma may disrupt thin bone or stable dura over the superior canal.

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OBJECTIVE: To describe the successful surgical treatment of 5 cases of superior semicircular canal dehiscence via a transmastoid middle fossa craniotomy using a soft tissue graft. DESIGN: Case report. SETTING: Private practice otologic referral center. RESULTS: All patients have experienced reduction in auditory and vestibular symptoms. Pulsatile tinnitus and autophony are now absent in the operated ears. Chronic disequilibrium is subjectively improved. Patients with sound evoked eye movements no longer have sound sensitivity on the operated side. Head thrust testing indicates no obstruction of the operated superior canal in all patients with normal head thrust preoperatively. Audiometry is unchanged from preoperation, and cervical vestibular-evoked myogenic potential thresholds have increased on the operated side in 4 of 4 patients. Three patients had dehiscence at the superior petrosal sinus inaccessible to standard middle fossa repair. All patients were discharged to home the morning after surgery. CONCLUSION: Transmastoid craniotomy repair of the superior semicircular canal dehiscence using a soft tissue graft offers numerous advantages over traditional surgical approaches and can be performed safely in the outpatient setting. The strategy is particularly useful in patients with dehiscence at the superior petrosal sinus. This article will review our strategy and discuss the advantages and disadvantages of the different surgical treatments used for patients with severe symptoms from superior canal dehiscence.

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OBJECTIVE: To investigate the association of otalgia and migraine. STUDY DESIGN: Retrospective survey with evaluation of otalgia response to migraine treatment. Only patients with minimum symptom duration of 3 months, who accepted migraine treatment and had a minimum follow-up of 3 months, were included. SETTING: Single neurotology practice. SUBJECTS: All patients with otalgia in whom other causes of otalgia had been excluded and who were treated with migraine therapies. INTERVENTION: Standard first-line abortive and prophylactic migraine therapies. MAIN OUTCOME MEASURES: Specific clinical data, as well as pretreatment and posttreatment severity scores, were gathered. Response to treatment was assessed by comparing pretreatment and posttreatment symptom scores using paired t test. RESULTS: A total of 26 patients were included. Ninety-two percent responded to migraine therapy with improved symptom frequency, severity, and duration (p < 0.001). Median duration of symptoms was 5 years. Mean delay to response was 2.3 weeks, and mean follow-up was 20 months. Otalgia was the chief complaint in 77%. Pain was dull in 35%, sharp in 19%, throbbing in 19%, and mixed in 27%. Sixty-five percent demonstrated triggerability of otalgia. A total of 65% had International Headache Society migraine. Patients responded to many classes of migraine preventive and abortive medications. CONCLUSION: Otalgia of unclear cause can be related to migraine mechanisms. Our group showed a high prevalence of migraine characteristics, including headache, migraine-associated symptoms, patterns of triggerability, and response to migraine treatment. Clinical criteria for diagnosis of migraine-associated otalgia are suggested for future prospective study.

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Most cases of primary hyperparathyroidism (80 to 90%) are caused by a parathyroid adenoma; most of the rest are caused by either parathyroid gland hyperplasia or multiple adenomas. Parathyroid carcinoma can be the cause of primary hyperparathyroidism in 1 to 5% of patients. When the appropriate clinical scenario is presented, it must be considered in the differential diagnosis. We describe the case of a 72-year-old woman with a long history of hypercalcemia and arthritic shoulder and neck pain who was admitted with an elevated calcium level and acute renal failure secondary to bilateral obstructing ureteral calculi. A sestamibi scan and magnetic resonance imaging detected the presence of what appeared to be a right inferior parathyroid adenoma. Her intact parathyroid hormone (PTH) level was 2,257 pg/ml. Following placement of bilateral ureteral stents and adequate hydration, the patient was taken to the operating room for a neck exploration and removal of a parathyroid adenoma with rapid intraoperative PTH monitoring. A 4 x 2.5-cm, 10-g mass was removed from the right inferior pole of the thyroid gland. Following excision, her intraoperative PTH level dropped from 1,103 to 110 pg/ml. Her ionized calcium levels fell from a high of 8.4 mg/dl preoperatively to 4.7 mg/dl. On final pathologic examination, the mass was confirmed to be a parathyroid carcinoma.

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Surgical orbital decompression is indicated for patients with compressive optic neuropathy, exposure keratopathy, an uncontrolled elevation of intraocular pressure, globe subluxation, and disfiguring proptosis secondary to Graves ophthalmopathy. Controversy exists, however, regarding the selection of surgical technique to achieve orbital decompression. We compared the results of our combined transnasal endoscopic and transconjunctival approach with those of our combined transnasal endoscopic and transantral approach to orbital decompression. We conducted a retrospective chart review of patients who had undergone medial- and inferior-wall orbital decompression from January 1994 through January 2004. During that time, 189 combined medial- and inferior-wall orbital decompressions were performed on 124 patients; 51 combined endoscopic and transantral decompressions were performed on 28 patients, and 138 combined endoscopic and transconjunctival decompressions were performed on 96 patients. Patient demographics and the degree of preoperative proptosis were statistically equal in the 2 groups. The incidence of optic neuropathy in the transantral group was significantly higher than the incidence in the entire group (p = 0.03), and the incidence of exposure keratopathy was significantly lower in the transantral group than in the entire group (p = 0.03). Postoperatively, the reduction in proptosis in the 2 groups was statistically equivalent, but the transconjunctival group had a significantly lower incidence of both infraorbital hypesthesia (p< 0.0001) and early rhinosinusitis (p = 0.008). Three cases of globe ptosis and 2 of infraorbital neuralgia occurred. No cases of visual loss, worsened optic neuropathy, diplopia in patients without preexisting diplopia, cerebrospinal fluid leak, significant epistaxis, or periorbital hematoma were noted. We conclude that combined endoscopic and transconjunctival orbital decompression offers equivalent efficacy with less postoperative infraorbital hypesthesia and early rhinosinusitis than does combined endoscopic and transantral orbital decompression.

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The thyroid gland is a relatively uncommon site for a secondary malignancy; even less common is a case of malignant melanoma metastatic to the thyroid. We describe the case of a 68-year-old man who presented with a neck mass in the posterior triangle. Fine-needle aspiration biopsy (FNAB) identified the mass as a malignant melanoma. The patient had had no known primary skin melanoma. He underwent a left modified radical neck dissection, and the mass was discovered to be a positive lymph node. Postoperatively, he declined to undergo radio- and chemotherapy. Eighteen months later, he returned with a diffusely enlarged thyroid. FNAB again attributed the enlargement to malignant melanoma. Soon thereafter, the patient began experiencing seizures, and on magnetic resonance imaging, he was found to have metastatic disease to the brain. He developed ventilator-dependent respiratory failure and required a subtotal thyroidectomy for the placement of a tracheostomy tube. Patients who present with a thyroid nodule and who have a history of malignancy present a diagnostic dilemma: Is the nodule benign, a new primary, or a distant metastasis? The findings of this case and a review of the literature strengthen the argument that any patient with a thyroid mass and a history of malignancy should be considered to have a metastasis until proven otherwise.

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OBJECTIVE: To discuss the range of signs and symptoms of patients with superior canal dehiscence syndrome identified in a single neurotologic practice. STUDY DESIGN: Retrospective chart review of 35 patients diagnosed with superior canal dehiscence from April 2000 to June 2007. RESULTS: All patients had evidence of superior canal dehiscence on computed tomography. These 35 patients represented 0.56% of new patients seen over a five-year period. The incidence of clinical symptoms and signs is compared to other published series. The mean vestibular evoked myogenic potential thresholds of affected ears are significantly lower than the mean vestibular evoked myogenic potential thresholds of unaffected ears. To date, 5 of 35 patients have undergone transmastoid occlusion of the superior semicircular canal with significant improvement in clinical symptoms in 4 of 5 cases. CONCLUSION: Not all patients with a diagnosis of superior canal dehiscence syndrome will have classic symptoms and signs. A high index of suspicion with careful clinical examination and properly performed ancillary testing is required to confirm this diagnosis.

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Malignant melanoma of the nose and paranasal sinuses can be a devastating disease, typically presenting at an advanced stage, with a 5-year survival rate ranging between 20 and 30%. It is an uncommon process, often misdiagnosed both clinically and pathologically. We present the case of an 80-year-old man who had a 2-month history of progressively worsening left-sided epistaxis and nasal obstruction. Radiographic evidence indicated the presence of soft tissue in the left maxillary sinus and nasal cavity resembling massive nasal polyposis and chronic fungal sinusitis. Magnetic resonance imaging was not performed because the patient had a pacemaker. After endoscopic debridement of the soft-tissue mass, frozen-section analysis detected no evidence of tumor. The final pathologic diagnosis was malignant melanoma. Otolaryngologists should be familiar with the difficulties inherent in the diagnosis and management of sinonasal melanomas.

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PURPOSE: This study evaluated the strategy of performing neck dissection (ND) without primary tumor resection prior to definitive chemoradiotherapy (CRT) for N2+ oropharynx cancer. METHODS: We analyzed records of 25 patients who underwent ND before concurrent CRT with weekly low-dose concurrent paclitaxel and a platinum compound. The extent of ND was highly customized (1 to 39 nodes) and median radiotherapy dose was 70 Gy. RESULTS: Median follow-up was 36 months. Two-year and 3-year actuarial locoregional control rates were 95% and 88%. No patient had regional neck nodal failure. Two-year rate of freedom from distant metastases was 91%. The 2- and 3-year event-free survival rates were 88% and 75%. Fifteen percent had Grade 3+ late toxicity; none had permanent gastrostomy tube dependence. CONCLUSIONS: Neck dissection without primary tumor resection before definitive chemoradiotherapy for oropharynx cancer is a safe and effective management program and warrants further exploration.

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Benign Paroxysmal Positional Vertigo (BPPV) is a common cause of dizziness caused by debris, which has collected within the semicircular canals of the inner ear. Stereoscopic representations of the human labyrinth are constructed and incorporated into a downloadable viewing platform to allow visualization of straightforward and complex variations of BPPV.

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Perilymph fistulae are difficult to diagnose because they present with a wide variety of signs and symptoms, they are associated with many etiologies, and they often mimic other conditions. In this article, we describe a case of perilymph fistula that featured one of its more rare causes: acoustic trauma--specifically, damage from a loud blast from the siren of a fire engine. We also review the literature and discuss the difficulties of diagnosis and treatment and the possible mechanisms by which acoustic trauma and other etiologies cause perilymph fistulae.

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