| ObjectiveCongenital middle ear cholesteatoma is a rare disease, because of the incidence of occult and aggressive, it is often shown as bone destruction and hearing impairment, and can even lead to intracranial complications, so early found and diagnosis is the key to treatment of congenital middle ear cholesteatoma. In order to investigate age distribution, clinical manifestation, characteristics of tympanic membrane of congenital middle ear cholesteatoma, and to reduce misdiagnosis, we intend to analyze and summary systemically the characteristics of it. MethodsAll clinical data from 6 cases of congenital middle ear cholesteatoma undergoing surgical treatment and confirmed by pathology in Department of Otolaryngology-Head and Neck surgery in Bethune Second Hospital of Jilin University from January 2013 to September 2013 were retrospectively analyzed. The age of onset, clinical manifestation, signs and the results of auxiliary examination [including electronic endoscope, pure tone audiometry, tympanometry and temporal bone high resolution computed tomography(HRCT)were evaluated. ResultsThere were 4 males and 2 females with an average age of 30.8 years. They suffered with hearing loss, accompanied by ear fullness with 5 cases, 5 cases of tinnitus, and dizziness in 1 case. The results of pure tone audiometry showed conductive deafness in 5 cases and 1 case of mixed deafness. In addition, the average air conduction threshold was 53 dBHL and the average air bone gap was 31 dB. Electronic ear endoscope showed that 6 cases with tympanic membrane but no retraction pocket formation. What is more, the medial to the tympanic membrane lumpy shadow mainly located in the posterior quadrant in 3cases and 3 cases in the tympanum. HRCT showed that ipsilateral mastoid was gasification type in 2 cases, sclerotic in 1 case, diploic type in 3 cases. The cholesteatoma in HRCT showed soft tissue density. The lesions of the gasification type confined to the tympanic cavity in 1 case and 1 case of violation of tympanic sinus. The lesions of the sclerosis and diploic type limited in tympanic cavity in 1 case, 3 cases of tympanic sinus and mastoid offenders, with destroyer of semicircular canal in 1 case, with facial nerve tube destroyer in 2 cases. According to the Kikuchi classification of congenital cholesteatome of middle ear, 1 case was closed type and 5 cases were open type. According to the Potsic grading standard, it showed that grade II in 1 cases, 2 cases of grade III, IV in 3 cases. All patients underwent surgical treatment, and according to the otitis media surgery classification standard made by The Chinese Medical Association in 2012, open mastoidectomy + type II tympanoplasty in 3 cases, tympanotomy + III tympanum shaping operation in 1 case, tympanotomy + II tympanum angioplasty in 1 case, tympanotomy + I tympanum angioplasty in 1 case. Patients were followed up for 6 months to 2 years, not currently found cholesteatoma recurrence. Conclusions1 The early clinical symptoms of congenital cholesteatoma are not obvious, or only showed unilateral conductive hearing loss, which is easy to be misdiagnosed, clinical diagnosis is often delayed for many years.2 For intact tympanic membrane and the inside of white or yellow lumpy shadow, with or without unilateral conductive hearing loss patients, otoscopy is crucial. And it suggests the possibility of congenital middle ear cholesteatoma.3 Temporal bone HRCT is one of the important methods to diagnose congenital cholesteatoma of middle ear. Once the diagnosis of congenital middle ear cholesteatoma, the operation should be underwent as soon as possible. |
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