Primary tumors of the facial nerve are relatively rare and have a variety of presenting symptoms. This article reviews 248 cases of facial neuroma in the world's literature and adds seven cases that were managed at the Baylor College of Medicine. Facial weakness was most common symptom, with facial spasm or tics, hearing loss, and masses in the external auditory canal also being frequently seen. At surgery, the tympanic, vertical, and labyrinthine segments were the most commonly involved areas. On the basis of this review, general principles have been drawn up to help the clinician in diagnosis and management of this (at times) difficult problem.
Thirty‐nine adults (34 male, 5 female) age 40 or under with squamous cell carcinoma of the head and neck (excluding nasopharyngeal carcinoma) were seen at the Baylor College of Medicine affiliated hospitals from 1964 to 1983. The average age of the patients was 36.3 years and they were nearly uniformly heavy smokers and drinkers. Twenty‐six of the 39 patients were black.
Lesions of the oral cavity and oropharynx were most frequent, followed by laryngeal lesions. Four patients presented with second primary tumors, and one patient had three separate lesions. Of the 39 patients, only 8 had cancers small enough to be considered curable by radiotherapy or organ‐sparing surgery. The remainder of the patients required radical surgery (26), were considered incurable at the time of presentation (8), or refused treatment (3).
Also noted was a relatively high incidence of prior trauma (gunshot wounds, laparotomies for trauma). Of 30 patients available for follow‐up more than one year after treatment, 19 have died of their tumors. Only one patient had a documented immune disorder (systemic lupus).
In contrast to previous authors, we conclude that the development of squamous cell carcinoma at a young age can be related to heavy smoking and drinking and that the poor survival in many patients is due to self‐neglect and failure to seek medical care early in the course of the disease.
\s=b\A mother and daughter both presented at age 5 years with the triad of right-sided congenital cholesteatoma, right preauricular pits, and bilateral sensorineural hearing loss. Twenty-six years apart, both were treated with middle ear exploration and removal of a cholesteatoma that filled the sinus tympani, facial recess, and middle ear. The sensorineural hearing losses were nonprogressive, and the preauricular pits were asymptomatic. These two cases may represent a unique variant of branchio-oto dysplasia. The mechanism of formation of these anomalies and the possible modes of inheritance are conjectural. This triad, however, supports genetic predisposition rather than aberrant epithelial rests during morphogenesis as a possible cause in congenital cholesteatoma.
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