Detection and management of childhood cholesteatoma

Childhood cholesteatoma is an aggressive disease that demonstrates higher rates of recidivism than its adult counterpart. The priorities in ideal management include total removal, followed by hearing restoration, followed by preserving the ear anatomy. Especially in children, one must endeavor to preserve ear anatomy if it does not jeopardize total removal of cholesteatoma. Absolute indications for CWD surgery include an only-hearing ear, a severely destroyed posterior canal wall, an extremely contracted mastoid, and matrix overlying a semicircular canal fistula. Reasons for staging childhood cholesteatoma include suspected residual disease, uncertainty about total removal of cholesteatoma, severe mucosal disease, and CWU procedures in which the cholesteatoma has diffusely invaded the bone. Adequate long-term follow-up is imperative. Patients and their families should be reminded frequently of the importance of close follow-up because recidivism is frequent. Successful management of cholesteatoma in children does not involve a rigid, "one-way" approach. The surgeon must be flexible and capable of employing the most appropriate procedure for the patient.