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IN RECENT years, much has been written about acoustic neurinomas. Most of these tumors are unilateral, but a few are bilateral. Bilateral tumors of the eighth nerve usually are manifestations of generalized neurofibromatosis (von Recklinghausen's disease), but this is not always the case. Cushing 1 and Fraser 2 believed acoustic neurinomas to be a mild form of neurofibromatosis. Penfield 3 felt there were histologic differences between the tumors of von Recklinghausen's disease and acoustic neurinoma. However, most observers 4,5 feel that by light microscopy it is impossible to distinguish histologic differences between these two groups of tumors. Gardner and Frazier 6 demonstrated hereditary transmission of von Recklinghausen's disease in a study of 217 members from five generations of one family with 38 cases of bilateral deafness. Moyes 7 studied a family with bilateral acoustic neurinomas affecting 14 members of four generations. The purpose of this paper is to present the

Aurists who have taken an acute interest in hearing tests and in their analysis are convinced that most of the tests cannot be considered singly as the sole criterion for a diagnosis. Thus the deductions from the findings in one test must be made only after collecting the data from the others. Findings will, of course, suggest the pathology present, and, vice versa, the pathology diagnosed should explain the test findings. For example, it has long been recognized that the Weber lateralization must be valued in the light of the following possibilities, namely, mixed deafness, unilateral or bilateral, and bilateral deafness of the same or of different types. It can nearly always be predicted what the Weber test will be if each ear is first tested for air conduction and bone conduction independently. For any testing instrument with which it is possible to make the Weber test, the sound

Hearing loss is a well-known complication of meningococcal meningitis and is commonly bilateral, severe, and permanent. 1A‚·2 A review of the literature revealed only three previously reported cases of meningitis with deafness at onset. 34 We describe a patient whose initial symptom was bilateral deafness, in whom signs of meningitis became obvious only later. REPORT OF A CASE A 20-year-old man was examined because of neck pain immediately following local trauma at work that day. Results of physical examination were unremarkable. The patient was afebrile, without meningitic signs. Roentgenograms of the cervical spine were normal, and he was sent home. The next day, the patient complained of continued neck pain, sore throat, and decreased hearing. He said he had no headache. His pharynx appeared inflamed, but he was afebrile and had no other significant physical findings. The patient was treated with a single dose of parenteral penicillin G sodium, followed

INNER ear complications occurring early and late following stapes surgery have been well documented. 1-10 A review of the articles would indicate the complications were always unilateral. The following case report illustrates an early postoperative complication in one ear and a later postoperative complication in the other ear. The result was bilateral or total deafness. Report of a Case The patient was an 18-year-old white girl who was referred to me Aug 6, 1968 at her parents' request. Stapes surgery had previously been performed in another city. The left ear was operated on in 1962 and the right ear in 1963. The patient and her parents were reconciled to the permanent deafness in each ear. They were seeking assistance to relieve the patient's recurrent vertigo and difficulty in walking. Complete medical records had been made available to the parents. The more important details were extracted for this report. History.

• Fulminant and progressive CNS disease developed in a young woman and was associated with bilateral deafness and sharply elevated CSF protein levels. Two months after onset, a severe sensorimotor neuropathy developed. Clinical improvement occurred with corticosteroid therapy.

Several years ago a 35-year-old Asian man went to the London Hospital with a two-year history of progressive bilateral deafness and tinnitus. Before otolaryngologists there could examine him, however, he was hospitalized for severe frontal headaches and underwent a full blood screen. Later, when otolaryngologist Gerald B. Brookes, MB, FRCS, looked at the results, he noticed that the plasma levels of inorganic phosphate, calcium, and alkaline phosphatase were abnormal, suggesting a bone disorder. "I didn't know what this meant, so I 'phoned up one of our metabolic physicians," recalls Brookes, who reported his work at the Sixth British Academic Conference in Otolaryngology in Bristol, England, during the summer. "He immediately asked me if the patient was Asian. It turns out, you see, that for some reason Asians in Britain are at particular risk for rickets and osteomalacia because of a vitamin D deficiency." Sure enough, when Brookes, senior registrar at

In the Neurosurgical Clinic of the University of Pennsylvania Hospital there are records of sixty-two verified cases of tumor of the cerebellopontile angle. Two of these are cases of bilateral acoustic neurofibromas, one of which was associated with generalized neurofibromatosis while the other was not. This survey was suggested by a statement in the history in the latter case which intimated deafness and blindness in a number of the family connections for five generations. It so happened that eleven of the twelve members of the second generation of this family migrated to another state, so that this survey covered chiefly the direct descendants of only one of the original twelve progenitors. In this family there were thirty-eight cases of bilateral deafness; fifteen of these persons subsequently became blind. In each case in which a history was obtainable it was found that headache and vomiting preceded the blindness. From the evidence

Of all the forms of neurologic syphilis, the one giving rise to the most sudden, the most profound and startling display of symptoms is gummatous meningitis. Existing throughout the central neural axis, the gravest manifestations naturally arise from interruption of nerve paths in the cerebrum. Coincident with the marked exudate of albumin and cells evident in the cerebrospinal fluid of such cases, there must be a similar perivascular exudate in the substance of the brain itself, producing, in certain cases, an ischemia of certain foci. Any cranial nerve may be suddenly put out of commission, and as a result the patient may exhibit some form of heterophoria, ptosis, single or double choked disk, unilateral or bilateral deafness, facial sensory or motor disturbances or respiratory difficulty. Still more bewildering are the symptoms following the blocking of the circulation in the subcortical vessels supplying the coordinating paths. An intensely interesting phenomenon results'when

The patient, a report of whose case follows, is a member of a family in which bilateral deafness has been transmitted as a true mendelian dominant character. The condition has been traced through five generations of the family, which includes 217 members. 1 Thirty-eight members have been affected. Of these thirty-eight, fifteen subsequently became blind, the blindness being preceded by headache and vomiting in each case in which information was available. Of the deaf and blind persons, four were examined prior to death, and were found to have choking of the optic disks with secondary atrophy. Of the deaf persons, seven were personally examined. Five of these had entire absence of vestibular responses in the Barany test. In the other two, a sluggish response was obtained from the left horizontal canal, but the remaining semicircular canals were non-functioning. In addition, four subjects were found who had little or no

The case of quinine amblyopia to be reported is considered of interest for several reasons. First, since the amblyopia occurred during a period of hospitalization for another condition, the opportunity arose for immediate, as well as continued, close ocular observation. Second, corticotropin (adrenocorticotropic hormone) was employed in the treatment of the quinine amblyopia. REPORT OF CASE A 48-year-old white man was admitted to the surgical service of the Veterans Administration Hospital, Jefferson Barracks, Mo., on Oct. 5, 1953, for treatment of a peripheral vascular disorder of the lower extremities, manifested by intermittent claudication and deficient pedal pulses. The patient was first seen by members of the ophthalmology department four days later, within 45 minutes after he developed sudden complete bilateral blindness, associated with dizziness, flushing, vomiting, tinnitus, and partial bilateral deafness. Review of the medication revealed that the patient had received a total of 0.8 gm. of oral quinine sulfate