OTORHINOLARYNGOLOGICAL COMPLICATIONS OF PROGRESSIVE FACIAL HEMIATROPHY (ROMBERGS DISEASE)

Progressive facial hemiatrophy (PFH) is characterized by slowly progre ssive atrophy of subcutaneous tissue. Bone, muscles, nerves, the eye, and the brain may be affected by atrophy. Four patients suffering from various otorhinolaryngological complications of PFH or Romberg's dise ase are reported. Unilateral hearing loss could be located in the inne r ear of one patient by audiologic examination. Localized bone destruc tion and disintegration of a portion of the anterior wall of the front al sinus were observed in a patient after more than three decades. Mar ked shrinking of the homolateral parotid gland and homolateral mastica tory spasm are reported as further otorhinolaryngological manifestatio ns. The various complications of PFH call for close interdisciplinary cooperation.