The Levy-Hollister syndrome is characterized by a highly variable expression of dysplasia in different organ systems. Autosomal-dominant inheritance is recognised, but most cases are sporadic. In the field of otorhinolaryngology, xerostomia can be found due to aplasia of the major salivary glands, as well as congenital sensorineural, conductive or combined hearing loss and dysplasia of the auricles, mostly appearing as cup-shaped ears. Here we report on a 2-year-old boy with severe xerostomia. The disease was found to be caused by bilateral aplasia of the parotid and submandibular glands. There were also slight dysplasias of both auricles and a bilateral inner ear malformation as the origin of sensorineural hearing loss. Due to its highly variable expression, Levy-Hollister syndrome is often difficult to distinguish from other diseases and syndromes, so that the cooperation of different departments is necessary for an accurate diagnosis. Because of its dominant inheritance, a genetic consultation should be recommended to the patient. Auricular dysplasia and conductive hearing loss can possibly be treated surgically.

Neurogenic neoplasms of the parotid gland are extremely uncommon. We present the case of a solitary intraparotid neurofibroma of the N. facialis occurring in a 55-year-old female who noted a painless left-side enlargement in the region of the parotid gland over a period of 2 years. Facial function was normal. Magnetic resonance imaging revealed a well-demarcated round mass within the left parotid. At surgery, a tumor was found involving the main trunk of the facial nerve, histopathologically representing a neurofibroma. Since solitary intraparotid neurofibromas are characterized by a very slow growth lacking the propensity for malignant transformation, the tumor was left to preserve facial nerve function. The clinical course over months or even years and the non-specific symptomatology are characteristic of intraparotid facial neurofibromas. Surgical management depends on the clinical setting in the individual case. Conservative treatment based on facial nerve preservation and leaving the tumor in situ is recommended, rather than radical tumor removal with resection of the segment of the N. facialis involved.

We present our results in national and international comparison, describe tendencies of resistance, give reasons for the low incidence of penicillin resistance in pneumococci in Germany and draw conclusions for empirical chemotherapy.

No abstract