Learning Objectives:
Introduction: At 6th Cholesteatoma and ear surgery meeting held at Canne, France in 2000, we had the discussion about the figure of congenital cholesteatoma in Japanese patients and in the patients of other countries. In the nineties most of the children with congenital cholesteatoma belonged to the severe cases. At the initial operation, cholesteatoma extended not only in the tympanic cavity, but to the mastoid in most of the Japanese children.
Recently the figure of the congenital cholesteatoma has changed. Introducing the endoscope and microscope into the ordinary tools of ENT office contributed to make diagnosis of congenital cholesteatoma in early stage. The hearing check up system was also useful to detect congenital cholesteatoma in Japan.
We present the recent congenital cholesteatoma cases in our hospital and describe the check up system for hearing from newborns to infant in Japan.
Study design: retrospective chart analysis of consecutive patients with congenital cholesteatoma.
Patients: Between September 2004 and August 2015 conclusive 47 patients underwent primary procedure.
Intervention: The diagnosis of congenital cholesteatoma with Potsic staging system and the therapeutic operation were performed.
Main outcome measures: The chance of detecting the congenital cholesteatoma, the patient age, the stage of the disease, the pathology of the ossicles and the hearing result of the surgery were studied.
Results: Twenty two percent of the patients belonged to the Stage I and II without ossicular involvement. They showed normal hearing. Eighty eight percent of the patients belonged to the Stage III or IV and 76% of the patients showed good hearing result postoperatively. Thirty percent of the patients had diagnosed by the hearing check up and 32 % of the patients had found accidentally with microscopic examination at the ENT office.
