Chronic invasive fungal sinusitis with orbital and olfactory cleft involvement secondary to indolent mucormycosis.

PURPOSE: Chronic invasive fungal sinusitis secondary to indolent mucormycosis is a rare clinical entity, and the ideal management is controversial. A case of indolent mucormycosis successfully managed with conservative debridement and retrobulbar amphotericin B is herein reported. OBSERVATIONS: A 42-year-old man with diabetes mellitus and kidney transplant presented with chronic invasive fungal sinusitis with left orbital involvement from indolent mucormycosis. The patient was treated with aggressive systemic antifungal therapy, left retrobulbar injection of liposomal amphotericin B, reduction in immunosuppression, and conservative surgical debridement. Although the left olfactory cleft was involved, the cribriform plate was not resected due to risk of seeding the intracranial space. Given mild orbital involvement, no orbital debridement was performed and the patient had resolution of his orbital findings with systemic and retrobulbar amphotericin B. The patient had clinical and radiographic stability at 6-month follow-up. CONCLUSIONS: Conservative resection with subsequent long-term antifungal treatment can be a successful regimen in indolent mucormycosis. Retrobulbar amphotericin B may be a prudent orbit-sparing adjuvant therapy in indolent mucormycosis.

Imaging Anatomy and Pathology of the Intracranial and Intratemporal Facial Nerve.

The facial nerve is one of the most complex cranial nerves, with motor, sensory, and parasympathetic fibers. A large spectrum of congenital, inflammatory, vascular, and neoplastic processes may affect one or more segments of the nerve. Customized use of computed tomography and magnetic resonance imaging combined with good understanding of the nerve anatomy and relevant clinical/surgical key points is crucial for appropriate assessment of facial neuropathy. This article reviews the anatomy of the intracranial and intratemporal facial nerve, and the imaging features of the most frequent disorders involving those segments of the nerve.

Diagnostic Protocol for Detecting Otosclerosis on High-Resolution Temporal Bone CT.

OBJECTIVES: To present a systematic checklist to improve diagnosing otosclerosis (OS) on high-resolution computed tomography (HRCT) of the temporal bones and review this protocol's efficacy in diagnosing OS on HRCT. METHODS: A retrospective case series was performed at a University Referral Hospital in urban Chicago, Illinois. High-resolution computed tomographies of the temporal bone were reviewed including 17 ears in the test group with surgically confirmed OS and 21 ears in the control group surgically confirmed to not have OS. Preoperative HRCTs were evaluated by a single neuroradiologist using a systematic protocol created to assist in diagnosing OS. This looked for radiolucency at the fissula ante fenestram and pericochlear region, and new bone formation around the oval and round windows. RESULTS: The radiologist accurately diagnosed OS in all 17 test group ears and ruled out OS in all 21 control group ears using the protocol. All 17 test ears were read to have lucency at the fissula ante fenestram, 9 (53.0%) to have new bone formation, and 8 (47.1%) to have cochlear lucency. The radiologist was more confident in diagnosing OS when cochlear lucency was present with the fissula ante fenestram lucency. CONCLUSIONS: This HRCT checklist is a highly accurate tool for evaluating the presence of OS when images are reviewed in the systematic fashion described. Imaging prior to surgery aids in counseling patients, preparing surgically, and excluding other pathologies.

Trigeminal nerve and pathologies in magnetic resonance imaging - a pictorial review.

A variety of conditions may affect the trigeminal nerve. Magnetic resonance imaging is the modality of choice when trigeminal nerve pathology is suspected, and this modality plays an essential role in detecting causes. This review illustrates some of the pathological conditions relevant to the trigeminal nerve in magnetic resonance imaging.

Stereotactic Radiosurgery and Fractionated Stereotactic Radiation Therapy for the Treatment of Uveal Melanoma.

PURPOSE: To evaluate treatment results of stereotactic radiosurgery or fractionated stereotactic radiation therapy (SRS/FSRT) for uveal melanoma. METHODS AND MATERIALS: We retrospectively evaluated 181 patients with 182 uveal melanomas receiving SRS/FSRT between 2007 and 2013. Treatment was administered with CyberKnife. RESULTS: According to Collaborative Ocular Melanoma Study criteria, tumor size was small in 1%, medium in 49.5%, and large in 49.5% of the patients. Seventy-one tumors received <45 Gy, and 111 received ≥45 Gy. Median follow-up time was 24 months. Complete and partial response was observed in 8 and 104 eyes, respectively. The rate of 5-year overall survival was 98%, disease-free survival 57%, local recurrence-free survival 73%, distant metastasis-free survival 69%, and enucleation-free survival 73%. There was a significant correlation between tumor size and disease-free survival, SRS/FSRT dose and enucleation-free survival; and both were prognostic for local recurrence-free survival. Enucleation was performed in 41 eyes owing to progression in 26 and complications in 11. CONCLUSIONS: The radiation therapy dose is of great importance for local control and eye retention; the best treatment outcome was achieved using ≥45 Gy in 3 fractions.

Long-term Results of ABI in Children With Severe Inner Ear Malformations.

OBJECTIVE: To report the long-term outcomes of children who received auditory brainstem implant (ABI) because of severe inner ear malformations. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral otolaryngology clinic. SUBJECTS AND METHODS: Between July 2006 and October 2014, 60 children received ABI at Hacettepe University. Preoperative work up included otolaryngologic examination, audiological assessment, radiological evaluation together with assessment of language development and psychological status. The surgeries were performed via retrosigmoid approach with a pediatric neurosurgeon. Intraoperatively, electrical auditory brainstem response was utilized. Initial stimulation was done 4 to 5 weeks postoperatively. Outcomes were evaluated with Categories of Auditory Performance (CAP), speech intelligibility rate (SIR), functional auditory performance of cochlear implant (FAPCI) and Manchester Spoken Language Development Scale scores; receptive and expressive language ages were determined. RESULTS: Sixty children who received ABI were between ages of 12 and 64 months. Thirty-five patients with follow up period of at least 1 year, were reported in means of long-term audiological and language results. The most prevelant inner ear malformation was cochlear hypoplasia (n = 19). No major complication was encountered. Majority of the patients were in CAP 5 category, which implies that they can understand common phrases without lip reading. SIR was found out to be better with improving hearing thresholds. Children with ABI were performing worse than average cochlear implantation (CI) users when FAPCI scores were compared. Patients with the best hearing thresholds have expressive vocabulary of 50 to 200 words when evaluated with Manchester Spoken Language Development Scale. There was no relationship between the number of active electrodes and hearing thresholds. The type of inner ear anomaly with the best and the worst hearing thresholds were common cavity and cochlear aperture aplasia, respectively. Patients with additional handicaps had worse outcomes. Among 35 children, 29 had closed set discrimination and 12 developed open set discrimination above 50%. It was determined that, progress of the patients is faster in the initial 2 years when compared with further use of ABI. CONCLUSION: ABI is an acceptable and effective treatment modality for pediatric population with severe inner ear malformations. Bilateral stimulation together with CI and contralateral ABI should be utilized in suitable cases.

Oval window atresia: a novel surgical approach and pathognomonic radiological finding.

OBJECTIVES: The facial nerve usually occupies the oval window area in patients with oval window atresia. During exploration, if the facial nerve is discovered to lie in the oval window area, this is usually regarded as a contraindication for further surgical intervention. The aim of the present paper is to demonstrate the preoperative pathognomonic radiological sign and describe a new surgical approach for this difficult situation. METHODS: 3 patients and 4 ears were operated due to conductive hearing loss by the same surgeon in a tertiary referral center. Their clinical presentation, radiological findings, surgical findings and final outcomes were evaluated and correlated. RESULTS: Surgical findings were identical in all 4 ears: facial nerve was running over the oval window and tympanic portion was completely dehiscent. Incus long arm was medially displaced due to abnormal development of the stapes suprastructure. In each ear a successful vestibulotomy and teflon piston placement was achieved. Preoperative mean air-bone gap of 47.5dB was improved to 21.5dB. There were no complications. CONCLUSION: Oval window atresia is a rare middle ear anomaly usually regarded as a contraindication for surgical intervention. In this study we present a novel surgical approach with succesful results. However the best approach is to inform the family by showing the nerve on tomography, showing the operation video, informing the family about the sensorineural hearing loss and letting the family choose the treatment option.

Early computed tomography findings of the inner ear after stapes surgery and its clinical correlations.

OBJECTIVE: Pneumolabyrinth resulting from temporal bone trauma and stapes luxation has been associated with sensorineural hearing loss (SNHL). The principal purpose of this study was to determine the incidence and volume of pneumolabyrinth after stapedotomy in which iatrogenic perilymphatic fistula is created and to also correlate this with possible hearing loss and vertigo. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENTS AND METHODS: Fifty stapedotomy patients were operated on for otosclerosis, and of those 50, 20 underwent high-resolution computed tomography (CT) on the first day, 10 on the third day, and 20 on the seventh day. The patients followed up regarding SNHL and vertigo that could develop postoperatively, and the correlation of such complications with HRCT findings was examined. RESULTS: The 20 patients who had high-resolution CT (HRCT) on the first day all presented with pneumolabyrinth, and none of the 20 patients who underwent HRCT on the seventh day had pneumolabyrinth. Postoperatively, 92% of the patients had less than 20 dB and 62% had less than 10 dB air-bone gap. None of the patients had SNHL or persistent vertigo. There was no correlation between pneumolabyrinth and hearing loss or vertigo. CONCLUSION: Pneumolabyrinth is a radiological sign of perilymphatic fistula and has no effect on sensorineural hearing loss and vertigo. Observing pneumolabyrinth during the early postoperative stage should not necessarily implicate a complication; however, pneumolabyrinth after the first week supported with the clinical symptoms of perilymphatic fistula would be a meaningful finding.

Arterial tortuosity and aneurysm in a case of Loeys-Dietz syndrome type IB with a mutation p.R537P in the TGFBR2 gene.

We report a 13-year-old girl with Loeys-Dietz syndrome (LDS) caused by a known transforming growth factor beta receptor II (TGFBR2) gene mutation, who developed aortic root dilatation and saccular aneurysm of the internal carotid artery. LDS is a rare, autosomal dominant aortic aneurysm syndrome with multisystem involvement. The disease is typically characterized by the triad of arterial tortuosity and aneurysms, hypertelorism, and bifid uvula/cleft palate. The characteristic LDS symptoms observed in the reported case included craniofacial dysmorphism (hypertelorism, cleft palate, blue sclerae, malar hypoplasia, retrognathia), skeletal deformities (scoliosis, talipes equinovarus, pectus deformity, arachnodactyly), congenital heart defects (patent ductus arteriosus, PDA), and arterial tortuosity and aneurysms. Molecular genetic testing revealed a heterozygous mutation (c.1610 G>C, p.R528C) in the serine-threonine kinase domain of the TGFBR2 gene. Magnetic resonance (MR) angiography showed aortic dilatation, tortuosity of bilateral supraaortic arteries, and saccular aneurysm on the right cervical internal carotid artery. LDS resembles Marfan-related disorders (Marfan, Shprintzen-Goldberg and vascular Ehlers-Danlos syndrome), but arterial tortuosity and aneurysms are characteristic for LDS, so a timely diagnosis of LDS is important for early diagnosis and intervention of aneurysms to prevent vascular events. Here, we describe a LDS patient who presented with arterial tortuosity and saccular aneurysm.

Suppurative cervical adenopathy and pharyngeal mass due to tularemia unresponsive to medical treatment.

Tularemia is a zoonotic disease caused by Francisella tularensis. Tularemia presents with various clinical forms, such as ulceroglandular, glandular, oculoglandular, oropharyngeal, pneumonic, and typhoidal tularemia forms. As an intracellular pathogen, F. tularensis causes granulomatous and suppurative lesions especially in the affected regional lymph nodes and various organs. Tularemia is seen most commonly in the Black Sea and Marmara regions of Turkey. Herein, we describe a girl with tularemia who presented with right cervical lymphadenopathy and left nasopharyngeal mass. To the best of our knowledge, this is the first reported case of tularemia with deep neck infection and also the first tularemia case from Corum, a city in the central Anatolian region of Turkey.

Comparative evaluation of three nickel-titanium instrumentation systems in human teeth using computed tomography.

This study investigated several parameters of root canal preparation with three different rotary NiTi systems: Hero Shaper, ProTaper, and RaCe. The parameters evaluated were: changes in root canal volume and cross-sectional area, canal transportation, and working time. Curved, mesial molar roots were scanned in a computed tomography (CT) unit before and after instrumentation. Cross-sectional views of each canal were obtained at 1 mm intervals. Postinstrumentation volume and cross-sectional area of root canals and the extent of transportation were calculated using image analysis software and, thereafter, subjected to statistical analysis. ProTaper removed significantly more dentin than Hero Shaper (p < 0.05). Instrumentation increased the cross-sectional area at all cutplanes, while the difference between test groups was not significant (p > 0.05). RaCe files significantly transported the canals at the coronal level (p < 0.05). There was no difference between any rotary systems in regards to working time.

Computed tomography technique for evaluation of the nasal valve.

OBJECTIVE: To compare measurements of the nasal valve on computed tomographic images obtained in the traditional coronal imaging plane with those obtained using the Nasal Base View (NBV) for evaluation of the sinonasal cavities. METHODS: Thirty computed tomograms of the sinuses were evaluated retrospectively. Coronal re-formations were performed in a plane perpendicular to the hard palate at the most anterior aspect of the nasal bones at the nasal dorsum. Re-formations of the NBVwere performed in a plane perpendicular to the anterior aspect of the estimated acoustic axis. Measurements of the nasal valve angle were performed for both imaging planes in each patient. RESULTS: Nasal valve angles measured in the traditional, coronal plane were found to have an angle of 8.3 degrees +/- 2.0 degrees (mean +/- SD). Nasal valve angles measured in the NBV had an angle of 11.4 degrees +/- 2.6 degrees. A significant difference was demonstrated (P<.001). CONCLUSIONS: The traditional coronal computed tomograms of the sinonasal cavities may underestimate the true nasal valve angle. The NBV may provide a more accurate assessment of the nasal valve, as the measured angles of the nasal valve in this plane were found to be more consistent with classic descriptions of 10 degrees to 15 degrees.