The Pathophysiology of Cholesteatoma

A typical presentation of congenital cholesteatoma (CC) is asymmetric conductive hearing loss (CHL). As CHL is usually associated with middle ear effusion, diagnosis of CC is frequently delayed. This study aimed to describe the clinical characteristics, treatment and outcomes of children with CC.

The medical files of children diagnosed with CC at a large tertiary pediatric medical center during 2000–2019 were reviewed. The primary outcome measures were: presenting symptoms, surgical findings, stage of disease, recurrence rate and hearing outcome. Imaging findings and the size of mastoid air cells were assessed in CT scans.

Thirty-nine children were diagnosed with CC. The presenting symptom was unilateral CHL in 85%, with an average speech reception threshold of 41.5 ± 13.7 dB in the affected ear. The mean time from first symptoms to diagnosis was 1.3 years. The surgical approach was exploratory tympanotomy in 25% and canal wall up mastoidectomy in 69%. Seventy percent of the children presented with Potsic stage III-IV. The mean postoperative speech reception threshold was 26.4 ± 12.2 dB (P = 0.002). Recurrence of cholesteatoma occurred in 38% of the patients, mostly in stage III-IV. Mastoid air cell size was significantly smaller on the affected than the unaffected side.

In children with persistent unilateral or asymmetric conductive hearing loss, CC should be suspected. Late diagnosis of CC is associated with a high recurrence rate. This highlights the need to promote awareness to the disease among primary physicians in the community health care system.

Cholesteatoma is a common occurrence in the middle ear, whereas cholesteatoma of the external auditory canal (EAC) is a rare condition. We report an unusual presentation of the cholesteatoma in the EAC.

We report a case of a 67-year-old male presented to the ENT casualty with a longstanding history of left sided squeaky type sound, aggravated whenever he talks or eats. He subsequently had a Computed Tomography (CT) scan of the left petrous bone which identified a left-sided EAC cholesteatoma. Clinical symptoms of EAC cholesteatoma are non-specific, and hence we recommend considering cholesteatoma when patients present with abnormal EAC symptoms and intact tympanic membrane.

His cranial nerves examination was normal, and the tympanic membrane was intact. His blood count and infective marker were normal. The CT scan of the brain showed a lesion in the left external auditory canal close to the tympanic membrane. The lesion was in contact with the anterior inferior canal wall which had features suggesting bony erosion. Gas bubble seen in the posterior part of the TMJ was in relation to bony erosion of the EAC.

The cholesteatoma of the EAC is very rare. CT scan can provide detailed information about the extent of external ear canal cholesteatoma, which can be used to identify complications of the disease, in addition to differentiating the external ear canal from the middle ear cholesteatoma. Early recognition of cholesteatoma and prompt treatment is essential to prevent catastrophic complications.

This study retrospectively compares diagnostic performance of 1.5 T versus 3 T non-echo planar diffusion weighted imaging with or without additional T1 and T2 sequences in the detection of residual and/or recurrent cholesteatoma.

Patients with clinically suspected recurrent cholesteatoma or postoperative routine survey MR who subsequently underwent surgical procedure were retrospectively included (135 patients, 164 operated ears) from a large database. Patients underwent 1.5 T (128 ears) or 3 T MRI (36 ears), with non-echo planar DWI, T1 and T2 acquisitions. Two radiologists independently reassessed the images. Definitive surgical diagnosis was used as gold standard. Sensitivity, specificity and diagnostic odds ratio were evaluated.

According to surgical diagnosis a cholesteatoma was present in 124 of 164 ears, corresponding with a prevalence of 75%. Sensitivity and specificity were lower for 3 T compared to 1.5 T, irrespective of whether additional T1 and T2-weighted sequences were used or not. Diagnostic odds ratios were higher for 1.5 T (34 and 12 for reader 1 and 2, respectively) compared to 3 T (3 and 4 for reader 1 and 2, respectively). Adding T1 and T2 sequences lowers sensitivity but increases specificity.

Non-epi DWI for the detection of residual/recurrent cholesteatoma is preferably performed on 1.5 T scanners over 3 T. The use of additional sequences regarding detection of cholesteatoma is debatable as it lowers sensitivity but increases specificity. However, these sequences may also be of use in diagnosing complications and planning surgical procedures in some hospitals.