Abstract
We report a case of cholesteatoma in congenital aural atresia in which preoperative imaging analysis provided useful information. A 30-year–old man with total aural atresia had had ear symptoms on his right side for 10 years. Evaluation by computerized tomography and magnetic resonance imaging suggested that the mass in the atretic external auditory canal and the anterior part of the mastoid was cholesteatoma and that the mass in the posterior part of the mastoid was inflammatory granulation tissue and chronic osteomyelitis. Three-dimensional fluid-attenuated inversion recovery images showed increased signals in the labyrinth. The extent of the cholesteatoma revealed during surgery was consistent with the preoperative image findings. Imaging analysis provides useful information for management of congenital aural atresia, and combined examination with computerized tomography and magnetic resonance imaging could be beneficial for preoperative evaluation of state of ears in cases of congenital aural atresia.
1
Introduction
Complete aural atresia with accompanying cholesteatoma has been rarely reported . Diagnosis of cholesteatoma in congenital aural atresia tends to rely on radiological examination, especially in cases that present with no symptom other than hearing loss . In addition to computerized tomography (CT), magnetic resonance imaging (MRI) provides useful information regarding the nature of the disease. We report a case of cholesteatoma in congenital aural atresia in which preoperative imaging analysis provided useful information.
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Case report
A 30-year–old man came to visit our hospital with a continuous pus discharge from a fistula in a postaural lesion on his right side. He had first experienced swelling in his right temporal region 10 years earlier, which required intravenous administration of antibiotics in hospital. Thereafter, he occasionally had pain in the temporal portion of his right side. The external auditory canal on his right side was completely closed by bone, and his pure tone audiogram revealed conductive hearing loss of 70 dB. High-resolution CT showed a large mass between the atretic external auditory canal and the mastoid antrum; the posterior part of the mass showed partly geographical bone destruction and ground glass pattern ( Fig. 1 ). On MRI, the anterior part of the mass showed a hypersignal on diffusion-weighted images ( Fig. 2 A ); and the posterior part of the mass showed nonuniform contrast enhancement after gadolinium administration ( Fig. 2 B). These findings suggested that the anterior part of the mass is cholesteatoma and that the posterior part of the mass is inflammatory granulation tissue and chronic osteomyelitis. The disease had also caused thrombosis of the sigmoid sinus, which could be well visualized on the postcontrast T1-weighted image ( Fig. 2 C). Three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) images showed increased signals in the labyrinth, and further signal increase could be visualized on postcontrast ( Fig. 2 D).
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