Search

• A neonate had a destructive, fibrous lesion of the temporal bone. There was x-ray evidence of disseminated, lytic disease. A histologic diagnosis of malignant fibrous histiocytoma was made. No treatment was given, yet the child survived with spontaneous resolution of her lesions. We briefly discuss malignant fibrous histiocytoma and propose congenital generalized fibromatosis as an alternative diagnosis to explain the child's recovery. ( Arch Otolaryngol 105:215-219, 1979)

An attempt was made to produce viral labyrinthitis in the rhesus monkey. Rhesus monkeys are susceptible to the mumps virus. Nine animals were used. After removal of the stapes, the left oval windows were plugged with Gelfoam® soaked in a culture of live mumps virus; right oval windows were plugged with Gelfoam® soaked in killed cultures. Animals were sacrificed at different time intervals, postinfection. In eight of the nine animals there was seroconversion from negative to positive; however, none of the animals developed the histologic changes of viral labyrinthitis.

Three patients are presented who developed reversible sensorineural hearing losses during treatment with intravenous erythromycin lactobionate. A fourth patient treated with erythromycin gluceptate did not develop hearing loss. Ototoxicity with erythromycin lactobionate has been previously reported in only three patients, one of whom had medication orally. Withdrawal of the antibiotic resulted in prompt improvement in every case with return of hearing to pretreatment levels.

Presentation of Wegener's granulomatosis as an otologic problem is unusual and leads to incorrect diagnosis and unsuccessful therapy. In seven years, five patients with a diagnosis of otitis media failed to respond to the usual therapeutic regimens. All had surgical manipulations on the middle ear or mastoid, or both. Wegener's granulomatosis was suspected because of persistence of the otologic problem. All patients developed pulmonary lesions and had positive diagnoses by biopsy of either the lung, kidney, or upper respiratory mucosa. Withe timely treatment, all patients have survived.

The histologic findings in the temporal bones of three patients who died from viral encephalopathy are presented. Pathology was restricted to the scala media, vestibular labyrinth, and internal auditory canal and was considered to be expressions of viral labyrinthitis. The changes were different degrees of degeneration of the organ of Corti, early encapsulation of the tectorial membrane, degeneration of the stria vascularis, and round cell infiltration of the modiolus and contents of the internal auditory canal. A new finding in the organ of Corti and early stages of cystic degeneration of the stria vascularis are documented. In all cases, the saccule was degenerated with sloughing of the otolithic membrane and vestibular labyrinth was involved in varying degrees.

Twenty-seven subjects with a family history of Waardenburg's syndrome were examined with respect to 18 specific characteristics of the syndrome, with particular emphasis in identifying the spectrum of the phenotypic expression of affected persons. Our results indicate that patients with the syndrome may have a variety of clinical signs, and an accurate diagnosis will therefore depend on a thorough and pertinent family history and physical examination.

OVER THE past two decades investigators from different parts of the world have reported that 30% to 40% of sensorineural congenital deafness is inexplicable in the light of modern knowledge. Congenital deafness is recognized as one of the more common sequelae of clinical first trimester maternal rubella, but investigators have largely ignored the part played by subclinical maternal infection. The object of this paper is to discuss the role of subclinical or asymptomatic maternal rubella in the etiology of congenital sensorineural deafness. This investigation was carried out in the island of Trinidad in the West Indies in early 1966 and is confined to children with severe sensorineural hearing loss, and does not include any child with evidence of middle ear disease. The study is based upon 139 children, 10 years old and younger, who are congenitally deaf. All of these children were examined personally by me and all of their

At present 40 per cent of congenital deafness is inexplicable. The effect of rubella on the frequency of congenital deafness was studied five years after an epidemic of rubella in an island population. Of 87 congenially deaf children born in 1961, the year after the epidemic, 86 had deafness as the only demonstrable congenital abnormality. Only 20 gave a history of first-trimester rubella. By modern classification the remaining 67 cases should be labeled as idiopathic congenital deafness, all known causes for congenital deafness having been ruled out. Serologic tests for rubella antibodies on 30 of the idiopathic deaf children were positive in 74 per cent, as compared to 30 per cent in a control group born within the same year. This study suggests asymptomatic rubella infection in the first trimester as an etiologic factor in congenital deafness.

RECENT reports from most parts of the world show a definite progressive increase in the incidence of early syphilitic infection. 1 Moore et al 2 state that there were 124,000 cases of syphilis reported in the USA in 1962 and also conclude that this figure represents but ⅕ of the total number. The most disturbing factor in the recent upsurge is the large number of young people involved with the possibility of transmission of the disease to their progeny. In 1962 there was more than a 100% increase in reported congenital syphilis compared to 1957, 3 and the upward trend continues. In 1963, a total of 4,140 cases were reported in the USA compared to 4,085 for 1962. 4 We are concerned in this paper with a clinical and pathological study of the deafness which is such a common symptom of congenital luetics and present our findings of a

• Tetanus is an infectious disease affecting the neuromuscular system. Two of its most common symptoms, trismus and dysphagia, fall within the expertise of the otolaryngologist, and may result in the patient with the disease seeking his attention initially. Since the diagnosis of tetanus is a purely clinical one and does not involve diagnostic laboratory tests, it is of paramount importance for the practicing otolaryngologist to be thoroughly familiar with the clinical presentation of the disease. ( Arch Otolaryngol 1985;111:342-343)

Sclerosis of the mastoid portion of the temporal bone is often seen on radiographs of patients who have or have had chronic otitis media. Occasionally lesions are seen that cause sclerosis of the entire temporal bone. The otolaryngologist must be alert to the fact that these findings may signify important pathology beyond that of chronic otitis media and indeed may be indicative of life threatening problems in adjacent or distant areas. In this paper the differential diagnoses of sclerotic lesions of the temporal bone will be discussed. These include fibrous dysplasia, osteopetrosis, meningioma, ossifying fibroma and metastatic lesions to the temporal bone. Three representative cases with sclerotic temporal bones are presented. The first is a patient with a conductive hearing loss secondary to stenosis of the external auditory canal. He was found to have a fibrous dysplasia of the temporal bone. The second case is an elderly male with a draining ear and a facial nerve paresis. He was found to have adenocarcinoma of the prostate gland metastatic to the temporal bone. The third case is that of a woman with a meningioma who demonstrated a sclerotic temporal bone on routine radiographs of the skull.