Hearing impairment in Engelmann disease.

There are several hyperostosis diseases of the skull that are accompanied by hearing impairment. In this article a case of Engelmann disease is presented. Currently > 130 cases of Engelmann disease are reported in literature. About 18% of these cases reported hearing impairment, but it is possible that this figure may be higher, because many of the authors concentrated on other aspects of the disease and made no reference to hearing loss in their reports. The etiology of deafness is the narrowing of the internal auditory canals cause by bony encroachment on nerves and vessels. This lends to progressive perceptive hearing loss and narrowing of the tympanic cavities. The result is fixation or adhesion of ossicle to tympanic walls, and narrowing of the bony part of auditory tubes, which causes serous otitis media.

Coffin-Lowry syndrome with sensorineural deafness and labyrinthine anomaly.

A case of Coffin-Lowry syndrome is described. The child had the typical face and also soft and puffy hands with tapering fingers. His psychomotor development was retarded. Audiological tests revealed profound hearing impairment. Anomalous labyrinths were shown by CT scan of the temporal bones. It is inferred that deafness is one of the symptoms of this syndrome.

Supernumerary ring marker chromosome as a secondary rearrangement in a parapharyngeal lipoma with t(10;12)(q25;q15) as the primary karyotypic abnormality.

Cytogenetic analysis of a large parapharyngeal lipoma, a rare tumor at this site, showed the karyotype 46,XY,t(10;12)(q25;q15)/47,XY,t(10;12)(q25;q15),+r. The primary abnormality must have been t(10;12), whereas the +r developed secondarily as an additional aberration. Although a similar translocation between chromosomes 10 and 12 has not been described previously, that the tumor had a rearrangement of the 12q13-15 region, the most frequently involved genomic region in lipogenic tumors, indicates that the tumorigenetic mechanisms are identical in parapharyngeal lipomas and lipomas of other locations. Supernumerary ring markers are not usually detected in lipomas with 12q rearrangements but are common in atypical lipomas and well-differentiated liposarcomas. The parapharyngeal lipoma we describe recurred after initial resection, and we hypothesize that the more aggressive tumor phenotype thus demonstrated may be causally related to the clonal evolution it had undergone.

Aplasia of posterior semicircular canal in Waardenburg syndrome type II.

A case of Waardenburg syndrome type II is reported. The computed tomography (CT) revealed aplasia of the posterior semicircular canal and poor development of the vestible. Until now, including our case, only a few cases of Waardenburg syndrome with deafness were studied roentgenologically. Among them, at least 26% revealed aplasia of the posterior semicircular canal, which infers that it is a characteristic finding of Waardenburg syndrome.

[The clinical study of functional deafness].

Ninety eight cases with functional deaf who visited in recent 13 years were studied. The results were as follows; 1) Patients were likely to increase in the last 10 years. 2) Females seemed to be more frequently suffered from functional deafness rather than males. 3) It was found that there were two peaks of age when patient had functional deafness. 4) Chief complaints in most cases were hearing loss. 5) Pure tone audiograms in these cases showed bilateral in most cases, symmetrical curve, sensorineural in type, flat and sloping in shape (including saucer type), and its hearing level revealed in 30-40dB in most cases. 6) Békésy audiometry was useful for diagnosis. Ninety cases out of 98 showed the V type of Jerger classification. 7) Psychological problems related to troubles of school and friends were observed as causative factors, and episodes concerned otological problems were found to be as triggers. 8) It is possible to make sure a diagnosis by using ABR. For diagnosis, however, it was necessary to doubt the presence of this disease, and then it was important to find out the discrepancy in a battery of hearing test. 9) In some cases, the exactly the same, but higher threshold levels for hearing are always detected in repeated examinations of their audiograms, suggesting the actual disturbance of the loudness recognition in these patients.

[Acute low tone sensorineural hearing loss caused by inner ear barotrauma].

Three cases of inner ear barotrauma with subjective symptoms and hearing impairment which were similar to the low tone sudden deafness were reported. Case 1 was a 34-year-old man who developed a hearing loss in the next morning of taking an airplane and recovered four days after. Case 2 was a 42-year-old man who developed a hearing loss 2 days after flying in an airplane and hearing loss have recurred 4 times in his right ear for 3 months. Eight months after recovery of previous recurrent attack, a hearing loss occurred in his left ear without flying and recurred twice for 3 weeks. Case 3 was a 25-year-old woman who developed a hearing loss in the right ear after 24 meter depth scuba diving and recurred 4 times for 40 days. Those three patients complained of no vertigo at any attacks and were treated conservatively. From previous reports and the onset and the course of hearing disturbance, acute low tone sensorineural hearing loss in case 1 and case 2 was thought to be caused by circulatory disturbance of the inner ear and in case 3 thought to be caused by inner ear window rupture. But, endolymphatic hydrops was also needed to be take into account in those three cases as a common possible cause. Inner ear barotrauma and so called labyrinthine window rupture were considered to be one of the diseases needed to differentiate from low tone sudden deafness without reference to mono-attack type or recurrent type.

Novel antibiotics napyradiomycins. Production, isolation, physico-chemical properties and biological activity.

Novel antibacterial antibiotics napyradiomycins A, B1, B2, B3, C1 and C2 have been isolated from the culture broth of Chainia rubra MG802-AF1. In this paper, taxonomy of the producer, and production, isolation, physico-chemical properties and biological activities of napyradiomycins are reported. They contain the naphtopyran chromophore and halogens, and inhibit the growth of Gram-positive bacteria including drug-resistant strains.