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Four sibs in a family on the Isle of Man, two brothers and two sisters ranging in age from 33 to 45 years, presented with low-grade malignant tumors of the submandibular gland in three cases and of the nasal cavities and maxillary sinuses in one. The neoplasms were all of the same histological type, apparently hitherto undescribed, showing well-differentiated neoplastic ducts, surrounded by neoplastic myoepithelial cells, together with sheets of epithelial cells expressing neuroendocrine markers by immunohistochemistry. Cervical neck node metastases have developed in all four cases. In the sib with a primary sinonasal neoplasm, widespread bloodstream metastases also became manifest and a single such metastasis in his brother. All four sibs have severe enamel hypoplasia and the same lesion is present in 5 of their 11 children. In the two male patients, severe sensorineural hearing loss has developed in adult life, unilateral in the left ear in one brother, bilateral in the other. In the brother with bilateral sensorineural hearing loss, magnetic resonance imaging revealed a vestibular schwannoma on the left side, which is currently under treatment. The inherited hearing loss is thought to be unilateral in this case also.

Patients with acquired immunodeficiency syndrome (AIDS) were investigated to determine the sites, nature, and severity of any otologic abnormalities. Tinnitus, "muffled" or "echoing" hearing, and vertigo were frequent complaints. Eight percent of the 155 patients studied showed evidence of chronic otitis media, usually with effusion. Mostly mild, but occasionally severe sensorineural hearing loss was found in many, affecting more severely the higher and lower frequencies than the middle range. Almost all patients showed diminished otoacoustic emissions, suggesting cochlear dysfunction resulting from infection or ototoxicity as the basis for the hearing loss. Impairment of the otoacoustic emissions by a subclinical otitis media with effusion cannot, however, be excluded. On the basis of this study it is suggested that the provision of otologic care to patients with AIDS may enhance their quality of life and, by the early detection and treatment of severe otitis media, may even prolong life.

All patients in a geriatric unit showed a hearing loss with a mean value of approximately 60 dB HL. Auditory brain stem evoked responses had normal amplitudes but, in most cases, waves I and II were absent. Extratympanic electrocochleograms produced well-marked action potentials with evidence of a type of recruitment. These results suggest a primary disturbance of cochlear function peripheral to the cochlear nerve. Examination of stained surface preparations of the organ of Corti showed complete atrophy of all hair cells at the end of the basal coil and severe outer hair cell loss in all three coils. This was accompanied by a severe alteration - giant stereociliary degeneration - in some surviving cells in all cases, but no other consistent alterations in the cochlea. These findings point towards degenerative change in the hair cells as the basis for hearing loss in the elderly.

A new technique of examining the organ of Corti in the perfused human cochlea is described. The fixed cochlea is microsliced and surface preparations are prepared and then stained for light microscopy. A method of assessing the distance/length ratio of samples is also described. Counts of inner and outer hair cells in such stained surface preparations were performed on elderly subjects and compared with those of infants. A marked loss of outer and a mild loss of inner hair cells was present in the elderly subjects. Complete atrophy of variable lengths of the terminal basal coil cells was also found in all elderly cochleas. Giant stereociliary degeneration takes place in hair cells (except basal coil outer hair cells) before they disappear.