• Clinical and Experimental Otorhinolaryngology
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• v.11 no.4
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• pp.233-241
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• 2018

Objectives. As endoscopic instrumentation, techniques and knowledges have significantly improved recently, endoscopic ear surgery has become increasingly popular. Transcanal endoscopic ear surgery (TEES) can provide better visualization of hidden areas in the middle ear cavity during congenital cholesteatoma removal. We aimed to describe outcomes for TEES for congenital cholesteatoma in a pediatric population. Methods. Twenty-five children (age, 17 months to 9 years) with congenital cholesteatoma confined to the middle ear underwent TEES by an experienced surgeon; 13 children had been classified as Potsic stage I, seven as stage II, and five as stage III. The mean follow-up period was 24 months. Recurrence of congenital cholesteatoma and surgical complication was observed. Results. Congenital cholesteatoma can be removed successfully via transcanal endoscopic approach in all patients, and no surgical complications occurred; only one patient with a stage II cholesteatoma showed recurrence during the follow-up visit, and the patient underwent revision surgery. The other patients underwent one-stage operations and showed no cholesteatoma recurrence at their last visits. Two patients underwent second-stage ossicular reconstruction. Conclusion. Although the follow-up period and number of patients were limited, pediatric congenital cholesteatoma limited to the middle ear cavity could be safely and effectively removed using TEES.

• Journal of Yeungnam Medical Science
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• v.35 no.2
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• pp.244-247
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• 2018

Congenital intratympanic membrane cholesteatoma (ITMC) is a rare type of congenital cholesteatoma located within the tympanic membrane. This lesion tends to increase in size over time. The development of ITMC can cause several complications such as hearing impairment, dizziness, facial palsy, and intracranial complications, similar to any other cholesteatoma. The treatment of congenital cholesteatoma requires the removal of the lesion through surgery, because disease progression induces bony destruction of the nearby tissue. Most patients presenting with this cholesteatoma type are also treated with primary surgical removal. However, we recently experienced a case of an ITMC that showed a natural transition to an external auditory canal cholesteatoma.

• Korean Journal of Otorhinolaryngology-Head and Neck Surgery
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• v.61 no.12
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• pp.710-713
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• 2018

A congenital cholesteatoma is a benign mass formed from the keratinizing stratified squamous epithelium. It usually occurs in young children's anterosuperior part of the middle ear. A congenital cholesteatoma which originates from mastoid temporal bone or expands to posterior cranial fossa is rare. Standard treatment of an intracranial cholesteatoma is surgical removal with craniotomy. A 69-year-old woman was diagnosed with a congenital cholesteatoma of mastoid temporal bone that expanded to the posterior cranial fossa, which was successfully treated with transmastoid marsupialization without craniotomy. This is a first documented case of a congenital cholesteatoma of mastoid temporal bone that expanded to posterior cranial fossa, which was successfully treated with transmastoid marsupialization without craniotomy.

• Annals of Clinical Neurophysiology
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• v.9 no.2
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• pp.81-84
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• 2007

Vernet's syndrome is a complex of multiple cranial nerve palsy including ninth, tenth, and eleventh cranial nerves which results from various lesions involving the jugular foramen. There are several kinds of lesions that can cause Vernet's syndrome. It includes congenital cholesteatoma, vascular lesions such as protruded jugular bulb, infections such as external otitis or abscess, and tumoral lesions such as schwannoma or paraganglioma. We present a rare case of Vernet's syndrome caused by non-specific inflammatory mass lesion in the neck area sparing jugular foramen.