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Conclusions: Potsic's staging system is a clinically useful procedure for evaluating the extent of congenital cholesteatoma (CC)., Objectives: We investigated the clinical features, presenting symptoms, and surgical results of CC according to Potsic's staging system., Methods: A total of 71 patients who had undergone surgery at our hospital were retrospectively analyzed for presenting symptoms, the location of cholesteatoma, and surgical results according to Potsic's staging system., Results: Of the 71 patients, 21 were classified as Potsic stage I, 9 as stage II, 31 as stage III, and 10 as stage IV. More than half of the patients with early-stage CC (stages I and II) were diagnosed asymptomatically by a chance visit to a clinic or on ear screening. Others were diagnosed following a complaint of hearing loss, acute otitis media, or otitis media with effusion. The location of CC varied somewhat by stage. In stage I CC, the most frequent location was behind the anterior-superior quadrant of the tympanic membrane; however, in stage III CC, it was behind the posterior-superior quadrant. All patients were treated surgically. Recurrence was detected in 2 of the 71 patients (2.8%); both had stage II CC. Recurrent lesions were removed during revision surgery.

The first reported case of facial paralysis due to an inflammatory pseudotumor (IPT) of the facial nerve as a complication of epidermal nevus syndrome (ENS) is herein presented. A 10-month-old female patient was diagnosed with ENS at 3 months of age. She was referred to us because of moderate left facial paralysis. Epidermal nevi of her left auricle extended deep into the external ear canal. Otoscopy revealed polypous nevi and cholesteatoma debris filling the left ear. Computed tomography showed a soft mass filling the ear canal, including the middle ear, and an enormously enlarged facial nerve. Surgical exploration revealed numerous polypous nevi, external ear cholesteatoma, and tumorous swelling of the facial nerve. The middle ear ossicles were completely lost. The facial paralysis was improved after decompression surgery, but recurred 5 months later. A second operation was conducted 10 months after the first. During this operation, facial nerve decompression was completed from the geniculate ganglion to near the stylomastoid foramen. Histological diagnosis of the facial nerve tumor was IPT probably caused by chronic external ear inflammation induced by epidermal nevi. The facial paralysis gradually improved to House-Blackmann grade III 5 years after the second operation., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

The rate of residual disease after surgery for acquired middle ear cholesteatoma was investigated in 167 ears of 164 patients who had undergone planned second-look tympanoplasty by the intact canal wall technique. Overall, operative findings at the second stage revealed 65 cases of residual disease in 48 ears (29%). These consisted of 50 squamous pearls, 11 cases of the flat, open type, and 4 cases of the extensive type. The configuration of residual disease is closely related to the technical difficulty of eradication, since en bloc removal is much easier in the squamous pearl than in the open or extensive type, mainly because of the unclear margin with the surrounding tissues. The proportion of cases of the open type was greater in children than in adults, in pars tensa cholesteatoma than in pars flaccida cholesteatoma, and in severe primary middle ear disease than in moderate or mild disease, although these differences were not statistically significant. The extensive type occurred in 4 ears with severe primary disease, 3 of which were in children. These results support the value and importance of the staged procedure for middle ear cholesteatoma, particularly when operated on by the intact canal wall technique.

Surgical treatment of petrous cholesteatoma that involves the petrous pyramid and extends beyond the internal auditory canal is often difficult because of the site and extent of the cholesteatoma. Various problems pertaining to the operation are discussed from our experience with fourteen patients who underwent surgery in our hospital. When accompanied with otorrhea, the middle ear was eradicated by radical mastoidectomy before total removal of the cholesteatoma in order to avoid intracranial infection. Staging operation was conducted in 6 patients in which otorrhea could not be stopped by conservative treatment. The surgical approach was translabyrinthine in 10 patients, middle cranial fossa plus transmastoid in 2, and a combination of these methods in 2. The postoperative large cavity was totally exteriorized in 5 cases in which eradication of the cholesteatoma was deemed too risky, although the exposed internal auditory canal and the denuded dura were supplemented locally. In the other 9 cases, the cavity was obliterated with abdominal fat and/or muscle flap. Facial nerve palsy was present preoperatively in 11 patients. They were treated by decompression in 7 cases, nerve-anastomosis in 2 and nerve grafting in one. In one patient, atrophy of the nerve was too severe to perform nerve grafting. Plastic surgery, such as suspension of the eyelid and masseter muscle transfer, was additionally carried out in 6 of the above patients. Postoperative follow-up study with CT and MRI is very important. In 3 of our cases, the cholesteatoma recurred and the reoperation was needed.

Surgical treatment of petrous cholesteatoma is difficult, especially in infected cases, since it often involves vital structures. We report the case of a patient successfully treated for an infected petrous cholesteatoma by staging the surgical procedures to reduce the risk of intracranial complications. The patient, a 53-year-old man, was referred to our hospital because of vertigo during coughing or strenuous effort. The left side mastoid cavity was open to the external ear canal and wholly covered with cholesteatoma epithelium with purulent discharge. The superior basal turn of the cochlea, superior and posterior semicircular canals, and roof of the internal auditory canal were eroded. Conservative treatment was not effective in eradicating the otorrhea. Four weeks after the first operation (radical mastoidectomy), the second operation was conducted following a combined middle cranial fossa and transmastoid approach. The postoperative course was uneventful. Normal facial nerve function was preserved and unsteadiness disappeared, but hearing could not be preserved. The MRI examination, performed one year after surgery, did not reveal any evidence of residual cholesteatoma.

A variety of solutions were tested in vitro to find a suitable solvent which could be used in clinical practice for cholesteatoma debris. Though a little weak as a solvent, a liquid soap composed mainly of plant oil did not cause irritation of the middle ear mucosa, and was thought to be a promising solvent with which to rinse away tenacious debris, especially when used in combination with hydrogen peroxide.

In the treatment of cholesteatoma employing intact canal wall tympanoplasty, staging the operation and re-establishment of aeration of the tympanic cavity are required to eradicate possible causes of recurrence, cholesteatoma residue, and retraction pocket. The planned staged tympanoplasty with preventive measures for recurrence has evolved. At the second-stage operation, one of the following three types of operations was performed according to the grade of aeration and healing of tympanic cavity: type S1, only ossiculoplasty; type S2, ossiculoplasty and scutumplasty; and type S3, ossiculoplasty, scutumplasty, and mastoid obliteration. The surgical concept, indication, and technique are described in detail. The recurrence rate in the 134 patients without previous surgery, 95 adults and 39 children, operated on between 1987 and 1991 was 2.2 percent (7.6% in the children and 0% in the adult). Although the rate of the recidivism was significantly reduced, deep retraction pocket developed in 15 percent of adults and in 23 percent of children. The incidence of deep retraction pocket formation was lowest in the adults with type S1 operation and highest in the children with type S3 operation.

Incidence of retraction pocket and recurrent cholesteatoma in the attic after surgery for middle ear cholesteatoma using the staged intact canal wall technique were investigated in 95 ears of 91 patients, all of which had various degrees of bone defect in the tympanic scutum. Surgical procedures employed in the second stage for prevention of attic retraction were classified into three types: Type I, no scutumplasty; Type II, scutumplasty; Type III, scutumplasty plus mastoid obliteration. In 83 ears followed up for more than 1 year after the second stage, such retraction troubles occurred in 2 of 13 ears (15%) in Type I, 8 of 20 ears (40%) in Type II, and 24 of 50 ears (48%) in Type III. Incidence of retraction troubles was higher in Types II and III, probably because these procedures were indicated in ears with large scutum defects. Dislocation and atrophy of the graft materials, together with bone resorption around the bone defect were the main reasons for failure in scutumplasty. Dysfunction of the eustachian tube and traction of the eardrum by the scar tissue behind it may have also contributed to attic retraction. Mastoid obliteration with small blocks of hydroxyapatite was more effective in prevention of retraction troubles than that with pedicled temporalis muscle flap.

A variety of solutions were tested in vitro to find a suitable solvent of cholesteatoma debris for use in clinical practice. The specimens were taken during surgery from the patients of otitis media with cholesteatoma. They were divided in pieces and put in test tubes. Each tube was then admixed with one of the test solutions and incubated at 37 degrees C for 48 hours. Hydrochloric acid (1N) and sodium hydroxide (1N) had no substantial effect to solve the debris. Urea (10N), acetylcysteine (20%) and chymotrypsin (1%) had a weak such effect. Proteolytic agents such as diiodosalitylic acid (0.1N), sodium dodecyl sulfate (0.1N) and cholic acid (0.1N) showed a stronger effect but not enough for clinical use. In contrast, a detergent which contains interfacial active agents and a proteolytic enzyme (alkaline cellulase), such as Attack and Hi-Top, proved to be more effective to solve the debris. However, biological effect of such detergent on the ear is not clear. Further study will be necessary before actual application in the patients.