Learning Objectives: Although relatively rare in childhood, cholesteatoma deserves a special attention by not only by general practitioners but also by specialists in different mutually related disciplines in the family of neurosciences and, particular, in neurootology.
Introduction. Cholesteatoma in childhood is difficult to diagnose in spite of substantial advances in diagnostic approaches. The delayed detection of this complicated pathology necessitates adequate surgical management.
Methods: We report a child aged eight years presenting with congenital cholesteatoma accompanied by peripheral facial nerve palsy. Management has been initiated by a neurologist and later on, an otorhinolaryngologist has been involved in the therapeutic team because of the failed drug therapy. According to parents' report, three years ago the child complained of gait disturbances attributed mainly to his overweight. A comprehensive physician's examination included preoperative audiometry and computer tomography, intraoperative monitoring system enabling safe cholesteatoma removal in the area of bone destruction at second genu level as well as pre- and postoperative electromyography.
Results: We successfully identified the degree of morphological damage and of the disturbed function. Besides, the prognosis concerning facial nerve restoration was clarified. On the seventeenth day after the surgical intervention, there was convincing electromyographic evidence of significant improvement of the facial nerve function and positive change of facial appearance as documented by pictures in 4-day intervals. A concise review of recent publications dealing with cholesteatoma diagnosis and surgery illustrated the socio-medical importance of this entity in childhood (e.g., A. T. Harris et al., J Laryngol Otol. 2016;130:235; M. S. Cohen et al., Laryngoscope. 2016;126:732; J. B. Hunter et al., Otolaryngol Head Neck Surg. 2016; Mar 1, etc.).
Conclusion: Scanty initial clinical symptoms along with poor otoscopic findings hamper considerably the early exact diagnosis of cholesteatoma. Interdisciplinary collaboration between neurologists, otorhinolaryngologists, radiologists and neurosurgeons could warrant the proper therapeutic behaviour in children with cholesteatoma.
