Histopathologic and ultrastructural changes in the temporal bones of HIV-infected human adults.

Acquired immunodeficiency syndrome (AIDS) is a devastating disease that is affecting the human population in epidemic numbers. Patients with AIDS are known to have a significant incidence of otologic disease, including hearing loss, vertigo, tinnitus, otalgia, and infection with unusual pathogens. There has been no previous work on the histopathology of this disease. Ten temporal bones from five patients who were seropositive for the human immunodeficiency virus (HIV), the causative retrovirus of AIDS, were obtained. Seven specimens were analyzed using light microscopic techniques. Electron microscopy was performed on selected areas of pathology. A myriad of pathologic findings was seen, including severe petrositis with marrow replacement, mastoiditis, otitis media, ossicular destruction, precipitations in the perilymphatic and endolymphatic spaces of the vestibule and of the semicircular canals, and subepithelial elevation of the neurosensory epithelium of the saccule and utricle. The organ of Corti was relatively free of pathologic change. Many of the otologic symptoms encountered in these patients can be explained by the findings in this study. Further investigation using light and electron microscopy, and immunohistochemical techniques, is urged.

Temporal bone histopathologic findings in drowning victims.

The human temporal bones of five drowning victims, the largest such series, to our knowledge, were evaluated to determine what histopathologic changes occurred. Thickening of the periosteal epithelium, especially on the surgical dome of the otic capsule, was evident in all cases. There was also hemorrhage in the middle ear cavity in four of the cases. In the fifth case, a cholesteatoma and ruptured tympanic membrane were observed, but there was no evidence of hemorrhage. It is proposed that an intact tympanic membrane is needed to create sufficient negative pressure in the middle ear cavity to cause rupture of the blood vessels and hemorrhage. Such bleeding is indicative of drowning when the tympanic membrane is intact.

Hanhart's syndrome with special reference to temporal bone findings.

We report two cases of Hanhart's syndrome. The first patient was a male who died in the neonatal period, and whose temporal bones were removed, processed, and histologically examined; the findings are presented in this article. The second case concerns a living patient with the typical characteristics of the syndrome. An attempt is made to show that a gross first and second branchial arch anomaly exists in this syndrome and not just the micrognathia that is obvious to the onlooker. Possible causes and classifications of facial dysmorphia are discussed.

The Campomelic syndrome. Temporal bone histopathologic features and otolaryngologic manifestations.

The campomelic syndrome is characterized by dwarfism, craniofacial anomalies, bowing of the tibiae and femora, cutaneous dimpling overlying the tibial bend, respiratory distress, and early death. Otolaryngologic manifestations include flat facies with a broad nasal bridge, low-set ears, cleft palate, mandibular hypoplasia, and tracheobronchial malacia. The underlying pathologic feature appears to be disturbance in cartilage growth involving the affected bones and the respiratory tract cartilage. The cause is unknown. We report clinical and histopathologic features in two cases of this syndrome. The endochondral layer of the otic capsule contained no cartilage cells. The cochlea was short and flattened, presenting a scala communis. The vestibule and the canals were deformed by bone invasion. Defective endochondral ossification of the petrooccipital synchondroses possibly explains the shortened skull base seen in this syndrome. The tracheobronchial malacia significantly contributes to respiratory distress and neonatal death.

Temporal bone findings in hemifacial microsomia.

The temporal bone findings in a case of hemifacial microsomia are described with photographs. The right facial hypoplasia was associated with anophthalmia and microtia on the same side. The right petrous bone was hypoplastic and showed total superior dehiscence of the internal acoustic meatus. Th otic capsule was deformed with an underdeveloped cochlear modiolus grossly deficient in spiral ganglion population. The spiralling cochlear shell showed partial deficiency of the interscalar septum between the middle and apical coils. The cochlear duct was shorter than that on the normal side; the organ of Corti however was normal. The vestibular system did not show any structural abnormality except for the degeneration and reduction of the Scarpa's ganglion cells and nerve fibers. An additional interesting fact was that the facial nerve was totally absent in the temporal bone except for its nervus intermedius component.

Familial dysautonomia (Riley-Day syndrome). Temporal bone findings and otolaryngological manifestations.

Familial dysautonomia, or Riley-Day syndrome, is inherited in an autosomal recessive fashion and occurs almost exclusively in Jewish families. This disorder is characterized by a smooth tongue devoid of fungiform papillae and of taste buds, and is clinically associated with poor taste discrimination. An unsteady gait and dizziness on change in position are also common presenting symptoms. This study reports the histopathological findings of eight temporal bones from four patients with documented familial dysautonomia. For the control series, 13 normal temporal bones were also studied. The most striking finding in the dysautonomic patients was an extreme paucity of geniculate ganglion cells (P less than 0.001). A statistically significant reduction in the number of neurons was also found both in the superior and in the inferior divisions of the vestibular nerve (P less than 0.001). The paucity of the geniculate ganglion cells correlates well with the impairment of the taste in dysautonomic individuals, since the afferent fibers leaving taste buds of the anterior two-thirds of the tongue run via the chorda tympani and have their cell bodies in the geniculate ganglion. Furthermore, the reduction in the number of Scarpa's ganglion cells observed in the dysautonomic patients studied here could account for a poor response to caloric test, positional vertigo and an unsteady gait in this condition.

Mondini defect in association with multiple congenital anomalies.

A case of bilaterally symmetrical genetic aplasia conforming to Mondini type of congenital deformity in a 12-day-old child is presented with the help of temporal bone sections. Cochlear changes include a stunted modiolus, deficient interscalar septum between the middle and upper coils forming a scala communis cochleae, a degenerated organ of Corti and reduced spiral ganglion cells and dendrites. The vestibule is malformed, with membranous labyrinth being deficient. The utricle and semicircular canals are absent. There is no oval window or stapedial footplate, and the facial nerve is hypoplastic. An interesting feature is the unusual association of bilateral bony choanal atresia, atrial septal defect, cleft lip, absence of olfactory bulbs in the brain, and congenital ophthalmic anomalies.