From the Guidelines
Congenital cholesteatoma is staged using the Potsic classification system, which consists of four stages based on the extent of disease, and accurate staging requires thorough preoperative imaging with high-resolution CT scans of the temporal bone, as recommended by the American College of Radiology 1. The Potsic classification system is a widely accepted method for staging congenital cholesteatoma, and it helps determine the appropriate surgical management and predict outcomes. The stages are defined as follows:
- Stage I: single quadrant of the middle ear with no ossicular involvement or mastoid extension
- Stage II: multiple quadrants but still without ossicular erosion or mastoid extension
- Stage III: ossicular erosion but no mastoid extension
- Stage IV: mastoid extension, representing the most advanced disease This staging system is important because higher stages typically require more extensive surgery and have higher recurrence rates. According to the American College of Radiology, CT temporal bone without IV contrast is the recommended imaging modality for patients presenting with acquired conductive hearing loss without any mass lesion seen within the middle ear cavity, and it may also be useful for staging congenital cholesteatoma 1. The use of high-resolution CT scans of the temporal bone is crucial for accurate staging, as it allows for the assessment of the extent of disease and the involvement of surrounding structures. Early diagnosis and staging are essential, as congenital cholesteatomas tend to grow slowly but progressively, with potential for significant complications if left untreated, including hearing loss, facial nerve paralysis, and intracranial complications. The American College of Radiology recommends the use of CT temporal bone without IV contrast for evaluating patients with congenital hearing loss, total deafness, or cochlear implant, which may also be applicable to congenital cholesteatoma staging 1.
From the Research
Staging Systems for Congenital Cholesteatoma
There are several staging systems used to evaluate the extent of congenital cholesteatoma, including:
- Potsic's staging system, which categorizes the disease into four stages based on the location and extent of the cholesteatoma 2, 3, 4
- The Japan Otological Society (JOS) staging system, which classifies the disease into three stages based on the extent of the cholesteatoma and its location in the middle ear and mastoid 5
Potsic's Staging System
Potsic's staging system is a widely used classification system that categorizes congenital cholesteatoma into four stages:
- Stage I: disease confined to a single quadrant of the middle ear
- Stage II: cholesteatoma in multiple quadrants, but without ossicular involvement or mastoid extension
- Stage III: ossicular involvement without mastoid extension
- Stage IV: mastoid disease 2, 3, 4
Japan Otological Society (JOS) Staging System
The JOS staging system classifies congenital cholesteatoma into three stages:
- Stage I: limited to the anterior part of the tympanic cavity
- Stage II: extends to the posterior part of the tympanic cavity, but does not involve the mastoid
- Stage III: involves the mastoid 5
Clinical Characteristics and Treatment Outcomes
The clinical characteristics and treatment outcomes of congenital cholesteatoma have been studied in several research papers, including:
- A study of 71 patients with congenital cholesteatoma, which found that the disease was most commonly located in the anterior-superior quadrant of the tympanic membrane, and that the recurrence rate was 2.8% 2
- A study of 80 patients with congenital cholesteatoma, which found that the JOS staging system was more suitable for anatomical classification and surgical procedure selection 5
- A study of 38 patients with congenital cholesteatoma, which found that the size of the lesion was significantly correlated with residual or recurrent disease 3