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Certain biologic data assume clinical importance The highest incidence is in the fth and sixth decades, and the sexes are equally affected Familial occurrence is a mark, usually, of von Recklinghausen disease The earliest symptom reported by the 46 patients in the series of Ojemann and coworkers was loss of hearing (33 of 46 patients); headache (4 patients); disturbed sense of balance (3 patients); unsteadiness of gait (3 patients); or facial pain, tinnitus, and facial weakness each in a single case Some patients sought medical advice soon after the appearance of the initial symptom, some later, after other symptoms had occurred Usually, by the time of the rst neurologic examination, the clinical picture was quite complex One-third of the patients were troubled by vertigo associated with nausea, vomiting, and pressure in the ear The vertiginous symptoms differed from those of Meniere disease in that discrete attacks ` separated by periods of normalcy were rare The vertigo coincided more or less with hearing loss and tinnitus (most often a unilateral high-pitched ringing, sometimes a machinery-like roaring or hissing sound, like that of a steam kettle) By then, many of the patients were also complaining of unsteadiness, especially on rapid changes of position (eg, in turning), and this may have interfered with work and other activities Some of our patients ignored their deafness for many months or years; often the rst indication of the tumor in such patients has been a shift to the unaccustomed ear (usually right to left) in the use of the telephone Others neglected these symptoms to a point where they presented with impaired mentation, imbalance, and sphincteric incontinence due to brainstem compression and secondary hydrocephalus The neurologic ndings at the time of examination in the series mentioned above were as follows: eighth nerve involvement (auditory and vestibular) in 45 of 46, facial weakness including disturbance of taste (26 patients), sensory loss over the face (26 patients), gait abnormality (19 patients), and unilateral ataxia of the limbs (9 patients) Inequality of re exes and 11th and 12th nerve palsies were present in only a few patients Signs of increased intracranial pressure appear late and have been present in fewer than 25 percent of our patients These ndings are comparable to those reported by House and Hitselberger and by Harner and Laws The contrast-enhanced CT scan will detect practically all acoustic neuromas that are larger than 20 cm in diameter and project further than 15 cm into the cerebellopontine angle Much smaller intracanalicular tumors (ie, restricted to the acoustic canal in the petrous bone) can be detected reliably by MRI with gadolinium enhancement (Fig 31-17), a procedure that in general is the most useful in determining size and anatomic relationships Audiologic and vestibular evaluation includes the various tests described in Chap 15, the brainstem auditory evoked response probably being the most sensitive In combination, they permit localization of the deafness and vestibular disturbance to the cochlear and vestibular nerves rather than to their end organs The CSF protein is raised in two-thirds of the patients (over 100 mg/dL in one-third); a clinically inevident acoustic schwannoma is one of the causes of an unexpectedly high CSF protein when a lumbar puncture is performed for other reasons Treatment The preferred treatment in most cases has been surgical excision Most neurosurgeons who have had a large experience with these tumors favor a microsurgical suboccipital transmeatal operation (Martuza and Ojemann) The facial nerve can usually be preserved by intraoperative monitoring of brainstem auditory responses and facial nerve electromyography (EMG); in experienced hands, hearing can be preserved in approximately one-.

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third of patients with tumors smaller than 25 cm in diameter If no attempt is to be made to save hearing, small tumors can be removed safely by the translabyrinthine approach An alternative to surgery is focused gamma or proton radiation, which controls the growth of many of the smaller tumors In a large series of patients treated with radiosurgery, facial motor and sensory functions were preserved in three-quarters of cases and, after 28 months of observation, no new neurologic de cits were detected (Kondziolka et al) This approach is favored in older patients with few symptoms but is being adopted increasingly for others The rates of hearing loss and facial numbness and weakness are comparable or lower than with surgery but the follow-up period in most series is less than 5 years (Flickinger et al) Focused radiation with the gamma knife or linear accelerator also appears to be preferable to surgery in cases of recurrent tumor Other Tumors of the Cerebellopontine Angle Region Neurinoma or schwannoma of the trigeminal (gasserian) ganglion or neighboring cranial nerves and meningioma of the cerebellopontine angle may in some instances be indistinguishable from an acoustic neuroma Fifth nerve tumors should always be considered if deafness, tinnitus, and lack of response to caloric stimulation ( dead labyrinth ) are not the initial symptoms of a cerebellopontine angle syndrome A true cholesteatoma (epidermoid cyst) is a relatively rare tumor that is most often located in the cerebellopontine angle, where it may simulate an acoustic neuroma but usually causes more severe facial weakness Spillage of the contents of the cyst may produce an intense chemical meningitis Other disorders that enter into the differential diagnosis are glomus jugulare tumor (see below), metastatic cancer, syphilitic meningitis, arachnoid cyst, vascular malformations, and epidural plasmacytoma of the petrous bone All these disorders may produce a cerebellopontine angle syndrome, but they are more likely to cause only unilateral lower cranial nerve palsies and their temporal course tends to differ from that of acoustic neuroma Occasionally, a tumor that originates in the pons or in the fourth ventricle (ependymoma, astrocytoma, papilloma, medulloblastoma) or a nasopharyngeal carcinoma may present as a cerebellopontine angle syndrome Craniopharyngioma (Suprasellar Epidermoid Cyst, Rathke s Pouch or Hypophysial Duct Tumor, Adamantinoma) This is a histologically benign epithelioid tumor, generally assumed to originate from cell rests (remnants of Rathke s pouch) at the junction of the infundibular stem and pituitary By the time the tumor has attained a diameter of 3 to 4 cm, it is almost always cystic and partly calci ed Usually it lies above the sella turcica, depressing the optic chiasm and extending up into the third ventricle Less often it is subdiaphragmatic, ie, within the sella, where it compresses the pituitary body and erodes one part of the wall of the sella or a clinoid process; seldom does it balloon the sella like a pituitary adenoma Large tumors may obstruct the ow of CSF The tumor is oval, round, or lobulated and has a smooth surface The wall of the cyst and the solid parts of the tumor consist of cords and whorls of epithelial cells (often with intercellular bridges and keratohyalin) separated by a loose network of stellate cells If there are bridges between tumor cells, which have an epithelial origin, the tumor is classed as an adamantinoma The cyst contains dark albuminous uid, cholesterol crystals, and calcium deposits; the calcium can be seen in plain lms or CT scans of the suprasellar region in 70 to 80 percent of cases The sella beneath the tumor tends to be attened and enlarged The majority of the patients are.

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