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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

Glomus intravagale (paraganglioma of the nodose ganglion and/or vagus nerve) is an unusual tumor. There are approximately 45 cases reported in the literature; the authors report four more cases. This tumor may ba associated with other paragangliomas of the head and neck, such as carotid body tumors, and may metastasize to regional lymph nodes, although the histology always appears benign.

Posterior subglottic extension of glottic carcinoma has been a contraindication for partial laryngectomy because of the proximity of the cricoid cartilage. Resection of the upper portion of the posterior aspect of the cricoid is necessary for adequate tumor resection but is complicated by persistent aspiration. This paper presents a method of reconstruction following partial posterior cricoid resection.