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Neurofibromatosis 2

Introduction Neurofibromatosis 2 (NF2) is a rare syndrome characterized by bilateral vestibular schwannomas, multiple meningiomas, cranial nerve tumors, spinal tumors, and eye abnormalities. NF2 presents unique challenges to the otologist because hearing loss may be the presenting complaint leading to the diagnosis of the disorder. NF2 is quite invasive, requiring a multispecialist team approach for the evaluation and treatment of the disorder. The primary impairment is…

Petrosal Approach

Petroclival tumors arise from or involve the petroclival junction cephalad from the jugular tubercle, medial to the trigeminal nerve, and anteromedial to the internal auditory canal (IAC). , Tumors of the petroclival area are a particular challenge because they often involve the middle and posterior cranial fossae, cause significant brainstem compression, invade the cavernous sinus, and abut or surround the upper cranial nerves and basilar artery.…

Endoscopic Endonasal Approaches to the Skull Base and Paranasal Sinuses

Please access Videos to view the corresponding videos for this chapter. Introduction and Background The approaches to the skull base via the paranasal sinuses were introduced in the late 19th and early 20th centuries. Tumors of the sellar region were first approached through various incisions in the forehead to gain access to the sphenoid sinus via the ethmoid sinuses. In 1910, Cushing introduced a sublabial incision…

Anterior and Subtemporal Approaches to the Infratemporal Fossa

The infratemporal fossa (ITF) is a potential space bounded superiorly by the greater wing of the sphenoid and the temporal bone. Neurovascular foramina, including the carotid canal, jugular foramen, foramen spinosum, foramen ovale, and foramen lacerum, connect the ITF with the middle cranial fossa. Medially, the superior constrictor muscle, the pharyngobasilar fascia, and the pterygoid plates contain the ITF. Medially, the ITF communicates with the pterygopalatine…

Extended Middle Cranial Fossa Approach

Introduction Gaining access to the cerebellopontine angle (CPA) and prepontine cistern presents a formidable challenge. Approaches designed to expose infraclinoid basilar tip aneurysms, petroclival meningiomas, chondromas, chondrosarcomas, and chordomas involving the petrous apex and clivus must preserve vital neighboring neurovascular structures. , Risks encountered in the prepontine cistern during the management of aneurysms of the posterior circulation were best described by Drake in 1961: “the upper…

Endoscopic Internal Auditory Canal and Cerebellopontine Angle Surgery

Introduction and Background Approaches to the internal auditory canal (IAC) and cerebellopontine angle (CPA) require an intimate knowledge of the anatomy and its potential variations to obtain the best outcomes and to minimize complications. The operating microscope has long been the primary means used by the surgeon to visualizes the operative field and has the advantage of both providing depth perception and allowing the surgeon to…

Transcochlear and Transotic Approaches to the Cerebellopontine Angle

Please access Videos to view the corresponding videos for this chapter. The transcochlear (TC) approach was first described by William House and William Hitselberger in 1976 to treat midline clival lesions and cerebellopontine angle (CPA) masses arising anterior to the internal auditory canal (IAC) ( Fig. 46.1 ). Before the development of the transcochlear approach, these lesions were often considered inoperable due to reduced surgical visibility…

Retrosigmoid Approach to Tumors of the Cerebellopontine Angle

Please access Videos to view the corresponding videos for this chapter. The retrosigmoid approach is a versatile type of craniotomy that creates a panoramic view of the posterior fossa from the tentorium cerebelli to the foramen magnum. The indications for the retrosigmoid approach include (1) resection of extra-axial lesions, such as schwannoma, meningioma, and epidermoid; (2) cranial nerve neurectomy (e.g., CN V, VIII, and IX); (3)…

Translabyrinthine Approach

Please access Videos to view the corresponding videos for this chapter. The translabyrinthine approach for vestibular schwannoma resection was pioneered by Drs. William House and William Hitselberger in the 1960s. , Thousands of tumors have been removed at the House Clinic using this approach and it has become a standard tool for neurotologists and skull base surgeons around the world. The translabyrinthine approach allows excellent access…

Middle Fossa Approach

Please access Videos to view the corresponding videos for this chapter. Bullet-Point Summary The middle fossa approach, while technically challenging, is an ideal way to manage patients with small vestibular schwannomas and serviceable preoperative hearing surgically. Some of the principles of intraoperative success include: a craniotomy that is large enough to provide adequate temporal lobe retraction and visualization of the internal auditory canal (finding), finding the…

Rehabilitation of Lower Cranial Nerve Deficits After Neurotologic Skull Base Surgery

Before the advent of modern skull base surgery, the treatment of cranial base lesions was associated with significant perioperative complications and long-term morbidity. Rapid advances in medical imaging have enabled the early detection of lesions and preservation of vital structures at the time of surgery. The development of advanced microsurgical techniques has made the removal of most skull base tumors not only possible, but also feasible.…

Surgery for Glomus Tumors and Other Lesions of the Jugular Foramen

Please access Videos to view the corresponding videos for this chapter. The therapy for glomus tumors of the temporal bone is controversial. Because the clinical characteristics and growth rates of these tumors vary, the full gamut of management has been recommended from observation alone through radiation therapy and surgical management. Although there are isolated case reports of prolonged survival without treatment, these lesions can be quite…

Drainage Procedures for Petrous Apex Lesions

Acknowledgments We wish to thank the co-authors of the previous version of this chapter, Drs. Courtney C.J. Voelker and Gregory P. Lekovic. Please access Videos to view the corresponding videos for this chapter. The petrous apex is one of the least accessible areas of the skull base. Located in the anterior-superior portion of the temporal bone, surgical approaches to lesions of the petrous apex require detailed…

Operations for Vascular Compressive Syndromes

Please access Videos to view the corresponding videos for this chapter. Introduction Surgical decompression for arteriovenous compression of the neural elements at the skull base was first described in the early 1900s. After significant refinement of surgical technique over several decades, it has become a popular and durable treatment for pain syndromes such as trigeminal neuralgia (TN), hemifacial spasm (HFS), and glossopharyngeal neuralgia (GN). The general…

Overview of Transtemporal Skull Base Surgery

Objective The objective of neurotologic skull base surgery is the exposure of the skull base through precise management of the temporal bone. In subsequent chapters, procedures are presented that accomplish ample surgical exposure and minimize brain retraction in posterior fossa, middle fossa, and lateral skull base lesions. An important consideration is the possibility of full three-dimensional approaches for certain lesions, including anterior endoscopic visualization and access.…

Superior Semicircular Canal Dehiscence Syndrome

Acknowledgment Dr. Heidi Nakajima kindly provided the summary of her laboratory’s recent work on the effects of SCD size on intracochlear pressures. Superior canal dehiscence syndrome (SCDS) was first described by Minor in 1998. It is a disease characterized by the clinical findings of sound-induced vertigo and eye movements, chronic disequilibrium, conductive hearing loss (CHL), and decreased hearing thresholds for bone-conducted sounds. Conductive hyperacusis may lead…

Intratympanic Pharmacotherapy

The origin of intratympanic (IT) therapy dates back thousands of years to the ancient Egyptians, Greeks, and Romans. Advances in anatomical sciences throughout the Renaissance and physiology through the 1700s paved the way for a more accurate understanding of otologic pathology. IT therapy was popularized in the late 18th century, and became widespread in otologic practice in the mid to late 20th century. Today, this method…

Transcanal Labyrinthectomy

Labyrinthectomy is an effective surgical procedure for the management of unremitting or poorly compensated unilateral peripheral vestibular dysfunction in the presence of ipsilateral, profound, or severe sensorineural hearing loss. The physiological rationale is that central vestibular compensation is more rapid and complete for the unilateral absence of peripheral vestibular function than it is for unilateral abnormal function, either episodic or chronic. Unilateral vestibular ablation has been…

Transmastoid Semicircular Canal Occlusion for Benign Paroxysmal Positional Vertigo and Other Disorders

Please access Videos to view the corresponding videos for this chapter. Benign paroxysmal positional vertigo (BPPV) is the most common vestibular end organ disorder; in one busy vestibular clinic, BPPV accounted for 17% of all diagnoses. Patients complain of brief vertigo spells, often accompanied by nausea, but rarely vomiting. The actual duration of the spells (5 to 15 seconds) is usually much shorter than what the…

Vestibular Neurectomy

Please access Videos to view the corresponding videos for this chapter. Surgical intervention may be considered when medical treatments and dietary adjustments fail to control spontaneous episodic vertigo of labyrinthine origin. The most common diagnosis in such patients is Ménière disease. Delayed secondary endolymphatic hydrops is a related disorder that may result from other types of inner ear injuries. Sometimes, ongoing vestibular symptoms after vestibular neuritis…