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In a previous report! the anatomical findings were reviewed in all the cases of so-called congenital atresia of the ear where the temporal bones were sufficiently well described to permit a detailed developmental analysis of the findings. Altogether 59 temporal bones, belonging to 50 individuals, could be utilized. According to the severity of the malformation, the cases were divided into three groups.In the first group, the malformations of mild degree, either the external auditory meatus, although hypoplastic, or at least its inner part is present. The tympanic bone is hypoplastic and somewhat misshapen and the drum membrane smaller than normal. The tympanic cavity is normal in size or hypoplastic. Its contents show malformations of varying degree. This group comprised 17 temporal bones.The majority of the known cases belongs to the second group, the malformations of medium degree. The external auditory meatus is completely absent, the tympanic cavity is, as a rule, diminished in size and its contents deformed in varying degree. The lateral wall of the tympanic cavity forms the so-called atresia plate which is either completely or partly osseous. The tympanic bone is present in some cases and missing in others. If present, it is badly misshapen, often platelike; frequently it contains at its center a membrane-like mass of connective tissue which is continuous with the connective tissue occupying the place of the missing external auditory meatus.Regardless of whether a malformed tympanic bone is present or not, the upper end of Reichert's cartilage might show anomalies.

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In a previous paper the opinion was expressed by one of us" that the available histological evidence did not permit the assumption that osteogenesis imperfects and otosclerosis have the same basic etiology.In the meantime several papers were published dealing with the relationship of the diseases in question as well as with certain histochemical and biochemical aspects of otosclerosis which make a re-evaL uation of the whole problem advisable.Wullstein, Ogilvie and Ha1l 2 9 had come to the conclusion that otosclerosis and osteogenesis irnperfecta are based on a common genetic anomaly and that otosclerosis usually is a local manifestation of osteogenesis imperfecta. In order to elucidate the pathology of the temporal bone in proven cases of osteogenesis imperfecta and thereby, if possible, establish the similarity or even identity of the bony lesions in osteogenesis imperfecta and otosclerosis, Ogilvie and Hall'" studied the temporal bones of four cases of osteogenesis imperfecta, of a newborn child, a stillborn pig, a nine-week-old Alsatian dog, and a 16-year-old girl. Furthermore, bone chips obtained in the course of fenestration operations in three patients suffering from osteogenesis imperfecta tarda and otosclerosis were examined.From the

Deafness in leukemia was first described by Donne! and by Vidal. 2 Politzer" was the first to do histological studies on the temporal bones of a patient with deafness associated with leukemia. Subsequent cases were reported by Steinbrugge," Wagenhauser" and Schwabach.6 The latter studied histologically five cases of his own and reviewed ten cases from the literature. He stated that two of his own five cases had clinical ear symptoms without pathological changes in the temporal bones. Alexander,' after having studied 15 cases histologically, stressed the very high percentage of involvement of the middle and inner ear in leukemia. One other case was reported by Nishio."Alexander's? observations were confirmed by Druss" who studied 148 patients clinically and four temporal bones histologically. He found clinical involvement of the ear in 16.5 percent of the patients. He observed the following otologic signs: 1) ulceration of the skin of the external auditory meatus with and without hemorrhage, 2) hemorrhage into the middle ear and the mastoid with and without infection, and 4) mixed hearing loss and facial nerve palsy. Druss" observed that pathological findings in the temporal bones, such as infiltration of the middle ear mucosa, were not always associated with clinical symptoms. Other cases were histologically examined by Cawthorne;'? Hallpike," de Stefani'" and Schuknecht et aP3 Shambrom and Finch!' found otologic symptoms in 32 of 100