Objective: Pathogenesis of middle ear cholesteatoma is unknown and how to surgically manage the disease is still controversial. We proposed in this study the surgical grading of attic cholesteatoma based on performing the canal wall up (CWU) tympanoplasty, and we showed the outcome of surgery.
Materials & Methods: 113 fresh cholesteatoma patients were operated on between July 1998 and April 2004 in our department. Non-attic types such as PSQ, congenital, and adhesive type were also included. The patients were divided into the following three groups according to the grading of the disease. The grade I group (38 ears) consists of the patients with small cholesteatoma not extending over the ossicles. The grade II group (54 ears) shows cholesteatoma involving the ossicles and extending into the mastoid cavity. The grade III group (21 ears) included large cholesteatoma destroying the bony part of the external ear canal 1/2 or more at least. For the grade I group, a one stage CWU tympanoplasty was applied. The grade II group underwent the planned staged CWU tympanoplasty. In the grade III group, canal wall down tympanoplasty was performed.
Results: Over one year after the operation, the microscopic otoscopy and CT scan revealed only one recurrence in a case in the grade I group. No recurrent case was seen in the grade II or the grade III group.
Conclusion: The very low recurrence rate (0.9%) in this study, although relatively short follow-up period, indicated that the surgical grading of cholesteatoma we proposed was appropriate and useful.

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We analyzed long term result of stapes surgery patients during the period of 1993 -2004 in our hospital, and were able to follow up 81 patients of 103 ears more than lyear among them. The mean follow up period after surgery was 33 months, ranging from 12 months to 132 months. Improvement of hearing level was evaluated by the standard of the Otological Society of Japan as the improvement of hearing level. The success rate of hearing improvement were 90.8%(89/98) at lyear, 86.4%(32/37) at 95%(19/20) at 5years, and 90%(9/10) at 10 years after operation, respectively. The long term result of stapes surgery was stable.
There ware 8 ears whose post-operative hearing level worsened more than 10dB. Three ears of these 8 ears showed air-bone gap increase, of which 2 out of 3 ears increased immediastely after operation, while the one increased one year after operation. Five ears of 8 ears showed progressive sensorineural hearing loss, of which 3 out of 5 ears progressed rapidly, which might be possibly caused by surgical trauma to the inner ear during stapes surgery.
The unoperated ear which were followed-up more than 5years were 22 ears. The mean hearing level and air-bone gap at three frequency of 0.5Hz, 1kHz, and 2kHz were stable in our outpatient office within 5 years. But beyond 5 years after, the mean hearing level worsend at 125Hz and 8kHz, and the air-bone gap increased at 250Hz clearly. Because the otosclerosis patient who has conductive or mixed hearing loss unilaterally has the possibility of progressive hearing loss and/or air bone gap in unoperated ear. In the long term we should follow up as long as possible after surgery in outpatient office.

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We have previously reported that surgical hearing improvement was demonstrated in 88% of all ears with minor congenital malformations. However, in cases of stapes fixation with inadequate incus, only 25% were successful. Recently, we encountered 4 ears in 3 patients underwent surgery using a malleus attachment piston with auditory ossicular malformations. In this study, the condition of the ossicles, the surgical procedure, the type of malleus attachment piston (Shea Malleus Piston ^® or Schuknecht Wire Malleus Attachment Piston ^®) and the postoperative hearing results were investigated.
One of the 4 ears showed absence of the footplate, deformity in the long process of the incus, and the facial nerve was inferior to the vestibule. The drilling was done superior to the facial nerve, then we connected the malleus handle and the new oval window using the all-teflon malleus attachment piston (Shea Malleus Piston ^®). Three of the 4 ears had stapes fixation and combined with the absence or deformity of the long process of the incus. Stapedotomy was performed in all of these ears and the ossicular chain using a malleus attachment piston made of wire and Teflon (Schuknecht Wire Malleus Attachment Piston ^®) or all Teflon.
Hearing improvement is usually designated successful when both an air-bone gap of within 15dB and a hearing gain exceeding 15 dB are satisfied. Three of the 4 ears using a malleus attachment piston obtained successful hearing improvement.
Two kinds of malleus attachment piston were usually prepared. One is made of all-Teflon, and the other is made of wire attachment and Teflon body. The advantage of the former is that postoperative extrusion is rear, but the disadvantage is that the length is short. In contrast, the latter has sufficient length and adequate angle, but it was reported that the wire was extruded through the tympanic membrane. In our last case, the wire extruded even though it was covered with connective tissue. Considering these aspects, the all-Teflon piston might be better than the wire-Teflon piston for long-term result.
We showed our results of surgery with auditory ossicular malformations using a malleus attachment piston. The improvement of the hearing level in this series was relatively high compared with that of previous reports in our department. At present, we recommend the all-Teflon malleus attachment piston for the case with stapes fixation or absence with inadequate incus.

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The prevalence of hearing loss, progressive hearing loss, tinnitus and dizziness, and age at onset of hearing loss were evaluated by questionnaire in subjects who were registered members of the Japanese Retinitis Pigmentosa Society (n=3200) in the year 2002. 834 questionnaires were returned (26.1% response rate), but 828 subjects (25.9%) who had been diagnosed of Retinitis Pigmentosa (RP) by the ophthalmologists were included in the analysis. The prevalence of cochlear symptoms (hearing loss: 29.5%, tinnitus: 30.4%) was 43.0%(n=356). Of 356 subjects with cochlear symptoms, progressive hearing loss and dizziness were 44.9% and 56.5%, respectively. The mean ages at onset of hearing loss and visual loss were 39.2 years old and 31.7 years old, respectively. 63.5% of the subjects with cochlear symptoms had a history of audiometric examination, and 22.1% of the subjects with hearing loss (n=244) used a hearing aid. Overall, the rate of occurrence of cochlear symptoms was calculated as 7.2 per 100, 000 in the general population (where subjects with an age at onset of cochlear symptoms of 61 or older were excluded from the calculation).

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This study was performed in order to estimate the prevalence of clinical otosclerosis in Japanese people. The subjects consisted of 850 people which were working in one company of Miyazaki. There were 664 males and 186 females with a median age of 44.7 years (45.8 years in male, 40.2 in female, age range 25-59 years). The results of either the questionnaire or simple hearing test suggested that 163 subjects may have had hearing loss. These subjects were given an inspection by three ENT doctors and each took a pure tone hearing test with two audiologists. Two (0.24%) of the subjects were suspected to have mild cases of otosclerosis. Despite the subjects being aware of mild hearing loss, they have never visited an ENT clinic. These results suggest that while the rate of clinical otosclerosis in Japanese people is lower than that in Caucasians, it might be more prevalent than previously believed.

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A 37-year-old man was struck by lightning on the beach during windsurfing. Although he lost consciousness and became cardio-respiratory arrest, fortunately, cardiopulmonary resuscitation was promptly performed by a nurse nearby, and he was restored. He was found to have the first and second grade linear burns i.e. lightning marks were observed on his right temporal, cervical, back, waist and inguinal region. After recovery from unconsciousness, he noticed right hearing loss and tinnitus. On examination, his right tympanic membrane was ruptured. A pure tone audiogram revealed averaged 20 dB conductive hearing loss. He was demonstrated neither any significant vestibular signs nor facial paralysis.
Five months after the injury, because the perforation was not closed by conservative treatment, we performed myringoplasty using an underlay temporalis fascia graft. Eight months postoperatively the perforation was closed. On a pure tone audiogram, hearing was found within normal range.

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