Congenital Cholesteatoma with Facial Nerve Palsy: Pediatric Case

Abstract

Congenital cholesteatoma is commonly diagnosed through examination of the eardrum for hearing difficulty. We unexpectedly found a congenital cholesteatoma in a boy with acute mastoiditis while performing mastoidectomy. On 15 June 2003, left earache and high fever appeared in a 3-year-old boy. His symptoms did not recover despite oral antibiotic administration at a pediatric clinic. The boy and his mother visited an otorhinolaryngologic clinic on 17 June, where left facial nerve palsy was suspected. On 18 June, because left otorrhea and left facial nerve palsy were rapidly aggravated, the patient was referred to our out-patient clinic of University Hospital as a suspected case of acute mastoiditis. We observed otorrhea, high fever and complete facial nerve palsy at the first examination, and hospitalized him for conservative treatment. The treatment did not improve his facial nerve palsy score of 0 points on admission. To drain the leakage from affected organs, on 21 June we performed a left mastoidectomy, and found a white mass in the left mastoid. Making the diagnosis of left cholesteatomatous tympanitis, we extirpated the mass, but not totally, because our preoperative explanation did not cover the complications of tympanoplasty. On 7 July, we performed the so-called type-0 tympanoplasty and facial nerve decompression, and made good the previous insufficiency. Thereafter, vital data indicative of inflammation recovered, and the facial nerve palsy score improved through 16 points after the first operation to 18 points after the second operation. The boy lived favorably without recurrence of facial nerve palsy as of 23 October 2003. It is quite difficult to make the diagnosis of congenital cholesteatoma before surgical operation of patients with acute tympanitis combined with acute mastoiditis. In cases of treating infants with acute mastoiditis or tympanitis as reported here, we should be aware of the probability of congenital cholesteatoma.