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Endolymphatic sac tumors (ELST) are slow-growing, locally aggressive, low-grade malignancies that originate from the epithelium of the endolymphatic duct and sac. ELST often present with sensorineural hearing loss, tinnitus, and vertigo, which may mimic Meniere disease. Large tumors may present with additional cranial neuropathies. Management is primarily via microsurgical excision. Radiation therapy has a limited role for residual or unresectable disease. Early detection may enable hearing preservation techniques. ELST have an association with von Hippel-Lindau disease., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Ménière's disease (MD) is characterized by episodic vertigo, fluctuating hearing loss and tinnitus, and by the presence of endolymphatic hydrops on postmortem examination. This disease continues to be a diagnostic and therapeutic challenge. Patients with MD range from minimally symptomatic, highly functional individuals to severely affected, disabled patients. Current management strategies are designed to control the acute and recurrent vestibulopathy but offer minimal remedy for the progressive cochlear dysfunction. Recent research highlights the role of neurotoxicity in the pathogenesis of the cochleovestibular deterioration. This article discusses a patient with MD, and provides an algorithm for the management of this disease., (Published by Elsevier Inc.)

Glomus jugulare tumors arise from adventitial chemoreceptor tissue in the jugular bulb. Although histologically benign, these tumors can be locally aggressive because of their proximity to the lower cranial nerves and major vascular structures. Traditional treatment involves microsurgical removal with or without endovascular embolization, but morbidity following total resection can result in injury to the facial and lower cranial nerves. Radiosurgery has recently emerged as a promising alternative to older therapeutic strategies for treatment of glomus jugulare tumors. This article reviews the latest benefits of radiosurgery and demonstrates how this modality represents an effective treatment option for glomus jugulare tumors with excellent tumor control and low risk for morbidity. In addition, this article will detail the role of minimally invasive sub-total resection of glomus jugulare tumors as a surgical complement to gamma knife therapy.

Temporal bone fractures occur from high-energy mechanisms, typically but not limited to motor vehicle accidents. However, as the automotive industry continues to introduce improved safety measures, violence and falls account for a larger proportion of cases. Given the great forces involved, temporal bone fractures rarely occur in isolation and initial evaluation must focus on the fundamental ABCs of Advanced Trauma Life Support with special attention to Glasgow Coma Scale, intracranial injury, and cervical spine injury. Subsequent evaluation relies on physical examination, high-resolution CT, and electrodiagnostic testing to address the neurotologic consequences of temporal bone fracture, including cerebrospinal fluid leak, facial nerve injury, and injury to the peripheral hearing and balance organs. Management algorithms must address immediate (eg, ABCs, neurosurgical issues), short-term (eg, cerebrospinal fluid leak, facial nerve injury, hearing loss), long-term (eg, facial nerve injury, hearing loss, vestibular injury), and delayed (eg, encephalocele, cholesteatoma, late meningitis) issues. This article reviews the current state of temporal bone fracture evaluation and management with special attention to mechanisms of injury, clinical presentations and emergency evaluation, and diagnostic workup, including the evolution of radiographic fracture classification systems and electrodiagnostic testing. Discussion of treatment approaches address management of immediate, short-term, long-term, and delayed complications.

The endolymphatic sac tumor is a slow-growing, locally aggressive neoplasm that originates from the epithelium of the endolymphatic sac and duct. Disease progression can lead to profound sensorineural hearing loss, posterior fossa invasion, brainstem compression, drop metastasis, and eventual death. Early diagnosis and surgical attention are the primary objectives in the management of patients who have endolymphatic sac tumor. This article describes the latest rationale and techniques for hearing preservation surgery and a review of the latest developments surrounding this disease entity.

The pathogenesis of middle ear cholesteatoma continues to be highly debated. In recent years, there has been a substantial improvement in the understanding of the pathophysiology of this disease. This chapter provides a summary of the history and evolution of cholesteatoma and a review of the recent literature that pertains to the pathophysiology of congenital and acquired cholesteatoma. An emphasis is placed on the mechanism of osteolysis and the factors predictive of aggressiveness and recidivism.