Nicholas L. Deep

Sinonasal phosphaturic mesenchymal tumor: Case report and systematic review.

We report a case of sinonasal phosphaturic mesenchymal tumor (PMT) and conduct a systematic review of the literature to highlight a unique paraneoplastic syndrome associated with PMT. We used English language publications from Medline and Cochrane databases (1970–2013) as data sources. A systematic review of the literature was conducted. All reported cases of head and neck PMTs were included. The presence or absence of the associated paraneoplastic syndrome was noted. We found 33 cases of PMT in the head and neck reported in the literature, 17 of which occurred in the sinonasal area. Approximately 5% of all PMTs are located in the head and neck. Just greater than half are concentrated in the sinonasal area, and the remaining involve various bony and soft tissue structures of the head and neck. PMT is sometimes associated with a paraneoplastic syndrome of tumor-induced (oncogenic) osteomalacia (TIO) causing bone pain, muscle weakness, and pathologic fractures. We present the 18th reported case of sinonasal PMT. A smooth mucosa-covered midline intraseptal mass filling the posterior nasal cavity with destruction and erosion of the skull base was found in an adult male. The patient underwent successful endoscopic resection with wide negative margins and is without recurrence at 24-month follow-up. PMT is a benign, locally aggressive tumor with rare malignant transformation. Knowledge of the bony invasion and destruction caused by this tumor is essential in planning surgical resection with wide negative margins. Familiarity with the associated TIO is essential to investigate for and manage any associated bony morbidity.

High-grade neuroendocrine carcinoma of the larynx: The mayo clinic experience

Objective: To report a single institutional series of high-grade neuroendocrine carcinoma of the larynx (NCL), a very rare yet aggressive tumor. To review the management of NCL, including discussion of clinical behavior, treatment outcome, and prognosis. Method: A retrospective chart review of high-grade laryngeal neuroendocrine carcinomas at a single institution, including small- and large-cell neuroendocrine carcinomas. A total of 8 patients with high-grade NCL treated at our institution from 1992 to 2014 were identified. Results: The median age at diagnosis was 65.5 years (range, 43-80). Five patients were male. Two patients had a known smoking history. Primary tumor location was supraglottic in 7 patients and glottic in 1 patient. Primary treatment consisted of surgery alone (3 patients), radiotherapy alone (1 patient), combination of chemotherapy and radiotherapy (1 patient), and surgery followed by postoperative chemoradiotherapy (3 patients). Locoregional recurrence followed by distant metastasis occurred in 6 patients. Median overall survival was 44.0 months (95% CI, 3-62.0). Conclusion: High-grade NCL is a rare diagnosis. Compared to well- and moderately differentiated NCL, high-grade NCL has a far more aggressive clinical course and associated with a worse prognosis. To our knowledge, this is the largest series of patients with high-grade NCL treated at a single institution. Prompt diagnosis and multimodality therapy including elective neck dissection may improve survival.

Teflon granulomas mimicking cerebellopontine angle tumors following microvascular decompression.

To report two patients with a history of microvascular decompression (MVD) for hemifacial spasm who presented with Teflon granulomas (TG) mimicking cerebellopontine angle (CPA) tumors and to perform a systematic review of the English‐language literature.

Measurement of cochlear length using the ‘A' value for cochlea basal diameter: A feasibility study†

Objectives: To determine whether the cochlea basal diameter (A value) measurement can be consistently and precisely obtained from high-resolution temporal bone imaging for use in cochlear length estimation. Methods: A feasibility study at a tertiary referral center was performed using the temporal bone CTs of 40 consecutive patients. The distance from the round window to the lateral wall was measured for each cochlea by two independent reviewers, a neuroradiologist and an otolaryngologist. The interrater reliability was calculated using the intraclass correlation coefficient (ICC) and the Bland–Altman plot. Results: Forty patients (19 males, 21 females) for a total of 80 cochleae were included. Interrater reliability on the same ear had a high level of agreement by both the ICC and the Bland–Altman plot. ICCs were 0.90 (95% CI: 0.82, 0.94) for the left ear and 0.96 (95% CI: 0.92, 0.98) for the right ear. Bland–Altman plot confirmed interrater reliability with all 96% of measurements falling within the 95% limits of agreement. Conclusions: Measurement between the round window and lateral cochlear wall can be consistently and reliably obtained from high-resolution temporal bone CT scans. Thus, it is feasible to utilize this method to estimate the cochlear length of patients undergoing cochlear implantation.

What is the best imaging modality for diagnosing a large vestibular aqueduct

BACKGROUND A large vestibular aqueduct (LVA) is the most common inner ear anomaly found on the imaging evaluation of children with sensorineural hearing loss (SNHL). It is frequently associated with syndromic and nonsyndromic forms of SNHL or as an isolated finding. Large vestibular aqueduct syndrome can be characterized by delayed-onset, fluctuating, asymmetric, unilateral or bilateral SNHL or mixed hearing loss. Knowledge of an LVA is useful for practitioners to counsel their patients on avoiding contact sports or other activities with risk of head trauma. Others use the diagnosis to prompt testing for mutations in the EYA and PDS genes, looking for associated conditions such as Pendred syndrome. Currently there is debate between the best initial imaging modality to use when evaluating patients who have passed the newborn hearing screen but are presenting with idiopathic delayed-onset SNHL and are suspected of having an LVA.

Facial Nerve Schwannomas: Review of 80 Cases Over 25 Years at Mayo Clinic

OBJECTIVE To elucidate the long-term clinical behavior, treatment, and outcomes of sporadic facial nerve schwannoma (FNS) in a large cohort of patients managed in the post-magnetic resonance imaging era. PATIENTS AND METHODS Retrospective review at a single tertiary health care system (January 1, 1990, through December 31, 2015), evaluating 80 consecutive patients with sporadic FNS. RESULTS Ninety-eight patients with FNS were identified; 10 with incomplete data and 8 with neurofibromatosis type 2 were excluded. The remaining 80 patients (median age, 47 years; 58% women) were analyzed. Forty-three (54%) patients presented with asymmetrical hearing loss, 33 (41%) reported facial paresis, and 21 (26%) reported facial spasm. Seventeen (21%) exhibited radiologic features mimicking vestibular schwannoma, 14 (18%) presented as a parotid mass, and 5 (6%) were discovered incidentally. Factors predictive of facial nerve paresis or spasm before treatment were female sex and tumor involvement of the labyrinthine/geniculate and tympanic facial nerve segments. The median growth rate among growing FNS was 2.0 mm/y. Details regarding clinical outcome according to treatment modality are described. CONCLUSION In patients with FNS, female sex and involvement of the labyrinthine/geniculate and tympanic segments of the facial nerve predict a higher probability of facial paresis or spasm. When isolated to the posterior fossa or parotid gland, establishing a preoperative diagnosis of FNS is challenging. Treatment should be tailored according to tumor location and size, existing facial nerve function, patient priorities, and age. A management algorithm is presented, prioritizing long-term facial nerve function.

Diagnosing Large Vestibular Aqueduct: Radiological Review of High-Resolution CT Versus High-Resolution Volumetric MRI

OBJECTIVES To compare the diagnostic yield of high-resolution volumetric T2-weighted MRI (HRT2-MRI) with high-resolution computed tomography (HRCT) for diagnosis of large vestibular aqueduct (LVA). STUDY DESIGN Three board-certified neuroradiologists performed an independent, blinded radiological review for diagnosing LVA with 2:1 age-matched controls on patients with both HRCT and HRT2-MRI imaging. SETTING Tertiary referral center. PATIENTS All patients between 2002 and 2016 with hearing loss who underwent both HRCT and HRT2-MRI and were diagnosed with LVA on either modality. MAIN OUTCOME MEASURES Concordance rate for LVA between HRCT and HRT2-MRI. RESULTS Concordance rate for HRCT and HRT2-MRI for diagnosing LVA was 88% (124/141) when assessing both the midpoint and external aperture diameters. Fifteen ears had LVA on computed tomography (CT), but not on magnetic resonance imaging (MRI); in comparison, two ears had LVA on MRI, but not on CT (p = 0.002). Excellent inter-rater reliability among the three radiologists was demonstrated. CONCLUSION Historically, HRCT has been the imaging modality of choice for diagnosing LVA. Although a higher concordance rate of HRT2-MRI was found compared with previous studies utilizing earlier MRI technology, HRCT still detected a larger number of patients with clinically significant hearing loss compared with MRI. Given the high concordance rate and efficacy of both modalities in diagnosing LVA, the ultimate decision of which modality to choose may depend on other patient-specific and clinical factors.

Facial Nerve Meningioma: A Cause of Pediatric Facial Weakness

OBJECTIVE To present an unusual case of a temporal bone meningioma with intrafascicular spread throughout the temporal facial nerve from cerebellopontine angle (CPA) to stylomastoid foramen. PATIENT Four-year-old female with progressive facial weakness and normal hearing. MAIN OUTCOME MEASURE Clinical, radiological, and histopathological findings of temporal bone meningiomas. RESULTS A patient presented with progressive facial weakness and normal hearing. Imaging demonstrated a mass within the left internal auditory canal radiologically consistent with a schwannoma. Asymmetric enlargement with enhancement of the left facial nerve from CPA to the stylomastoid foramen suggested facial schwannoma. At surgery, gross tumor was noted in the internal auditory canal, the fallopian canal seemed expanded and the facial nerve was enlarged and had an irregular contour. Resection of the facial nerve from the CPA to just proximal to its exit at the stylomastoid foramen was necessary to achieve negative margins. Cable grafting was performed. The histopathologic diagnosis was transitional meningioma with intraneural growth throughout the length of the resected facial nerve segment. CONCLUSION Meningiomas involving the temporal bone are exceedingly rare. We report a rare case of a child presenting with progressive facial weakness due to a presumed facial schwannoma spreading along the facial nerve throughout its intratemporal course that at surgery was found to be an intrafascicular CN VII meningioma.

Transmastoid Endoscopic-Assisted Eustachian Tube Packing After Translabyrinthine Tumor Resection: A Cadaveric Feasibility Study

HYPOTHESIS Endoscopically assisted packing of the Eustachian tube (ET) will improve visualization of the protympanic space compared with standard techniques with the microscope. BACKGROUND Postoperative cerebrospinal fluid (CSF) leakage after translabyrinthine tumor resection remains a problem. Current techniques of packing the ET are limited by inadequate visualization. The objective of this study is to demonstrate the feasibility and usefulness of transmastoid endoscopic-assisted ET packing during translabyrinthine tumor resection. METHODS Eight human cadaveric temporal bone dissections were performed on four heads to test the visualization that could be obtained of the ET orifice with an endoscope via transmastoid-facial recess approach. The incus body and incus buttress were removed, the aditus ad antrum enlarged, and tensor tympani muscle was cut. The scope was placed where the incus buttress had previously resided. The ET orifice was visualized and subsequently packed. RESULTS In all eight temporal bones, the endoscope was successfully able to visualize the ET orifice, with improved visualization of the orifice compared with standard techniques. Surgical technique and potential pitfalls are discussed. CONCLUSIONS The transmastoid endoscopic approach for packing the ET improves visualization of the ET orifice. Packing under direct visualization provided greater reassurance that the material entered the true ET lumen as opposed to a false passage. The technique can be performed without any significant changes to standard surgical technique, allowing for a fast and accurate closure of the ET orifice and has application in potentially decreasing postoperative CSF leak rates.

The Natural History of Jugular Paraganglioma

Jugular paragangliomas (JP) are uncommon, slow growing, highly vascular primary neoplasms of the temporal bone that develop from chief cells located in the adventitia of the jugular bulb [1–5]. The great majority of extra-adrenal paraganglioma reside in the abdomen, while only 10% are located in the head and neck—carotid body tumors (CBT) being most common, followed by JP and vagal paraganglioma (VP). Most JP are solitary, nonsecreting, and benign, while less than 5% are associated with catecholamine release or malignant character, and up to 17% of patients display multiple paragangliomas [6]. JP exhibit a female predominance and most commonly manifest during the fourth to fifth decades of life with symptoms of unilateral pulsatile tinnitus, conductive hearing loss, and less commonly lower cranial neuropathy. Owing to the rare prevalence and insidious growth, many patients endorse a long duration of preceding symptoms and are found to have advanced disease at diagnosis [1, 2].