We analyzed the results of 70 ears (54 cases) of the children with congenital ossicular malformation who was treated surgically between 1993 and 2003 in Nagoya National Hospital. The series included 18 male and 36 female patients. The age ditribution at the time of surgery ranged from 5 to 21 years, with an average of 10.1 years.
In exprolatory tympanotomy, there were various kinds of surgical findings. Epitympanic fixation found in 25 ears, ossicular detachment in 32 ears, stapes foot plate fixation in 43 ears, bony obliteration of the oval window niche in 2 ears, and the oval window niche covered with facial nerve in 1 ear. In these 70 ears, 27 ears had 2 or 3 ossicular malformation together.
We undertook various kinds of ossicular reconstructive surgery. We perfomed type I tympanoplasty in 7 ears, modified type III tympanoplasty in 17 ears, modified type IV tympanoplasty in 9 ears, modified type V tympanoplasty in 1 ear, stapedotomy in 22 ears, stapedectomy in 2 ears, stapedotomy with incus bypass method in 10 ears, and stapedectomy with incus bypass method in 2 ears. The postoperative hearing result showed that the mean air conduction at three frequencies (0.5kHz, 1kHz, 2kHz) was 29.7dB, the mean A-B gap was 17.7dB and the mean hearing gain was 23.7dB. The postoperative hearing improvement in air conduction within 30dBwas obtained in 48 ears (68.6%), the closure of A-B gaps within 15dB 43 ears (61.4%). 53 ears (75.7%) raised their hearing level at least 15dB after operation. 57 ears (81.2%) wereevaluated by the standard of the otological society of Japan as the improvement of hearinglevel.

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Between 1995 and 2003, we encountered 13 cases of congenital cholesteatoma, among which 5 were opentype. Most of congenital cholesteatoma belong to the closed-type while only a fewcases of the open-type have been reported. Of these 5, one was a mixed form where the open- and closed-types co-existed. All the others were found in adults (mean age, 21.6 years). The mean age of those with the closed-type found during the same period was 8.9 years, indicating that the age at the initial diagnosis was higher for those patients with the open-type. The major complaint of those with open-type cholesteatoma was hearing impairment, asymptom that has been experienced by all and recognized by the patients or others from early childhood. At the initial diagnosis, a conductive deafness of 32.5 to 52.5dB was recognized. In only 2 patients, was a white mass observed through the tympanic membrane, indicating a tendency toward a scarcity of characteristic findings related to this membrane. Four patients had a history of acute otitis media. In 3 of the 5 patients, the development ofmastoid cells was satisfactory on the affected side. Both air content and development of air cells were suppressed in 2, which were suspected to be the effects of otitis media suffered in early childhood. During surgery, cholesteatoma was recognized at the tympanic isthmus and the long process of the incus was absent in all. Three were also devoid of the suprastructure of the stapes. There was no complication of ossicular anomaly. The cholesteatoma, limited to the tympanic isthmus in only one patient, had extended to the attic and mastoid antrum in the others. Revision surgery has been conducted in 3, a remnant of the cholesteatoma appeared pearly in 1 and membranous in the other 2. In many instances, open-type cholesteatoma is invaded extensively by the epithelium, making extirpation difficult.

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We assessed the incidence of patients with postoperative otorrhea and the causes of postoperative otorrhea in 102 patients with cholesteatoma or non-cholesteatomatous chronic otitis media (OMC), undergone tympanoplasty during October 1998 to December 2003. Before the surgery, 73 patients had complained of otorrhea.
We usually perform simple mastoidectomy in patients with cholesteatoma or in the patients with OMC who had mastoid lesion detectedby computed tomography. The staged operation was performed in cholesteatoma cases.
Postoperative otorrhea appeared in nine ears, six of them showed an improvement following administration of antibiotics or local treatment. Other three ears had continuous otorrhea, one of which was caused by in flammation of the tympanic membrane, and others were due to recurrence of cholesteatoma.
Our results suggested that complete removal of the diseased mucosa by simple mastoidectomy is effective to avoid postoperative otorrhea. Furthermore, during the second stage operation, removal of recurrence or residual cholesteatoma was performed, and the result was successful for the prevention of late onset of otorrhea.

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In 2 years between January 2000 and December 2001, 4085 newborns underwent the universal hearing screening by automated auditory brainstem response (AABR; Natus ALGO2e). Twenty ninebabies were referred to the further examinations. Fifteen of 29 babies had a diagnosis ofhearing disorder by ABR. Eight of 15 patients had bilateral hearing impairment.
Six of 8 patients were followed up after the hearing test. Two patients had 21 trisomy with otitis media with effusion (OME) and their hearing disorders were improved after treatment of OME. A hearing loss of one patient was thought to be due to congenital cytomegalovirus infection. Two patients had hereditary hearing disorders and no etiology for hearing loss was found in other 2 patients. The last one patient had a cleft palate. One patient did not return to the hospital after hearing evaluation and a patient with cleft palate moved to another city, therefore we could not follow-up these patients. In this study, we reported 3 patients who changed their hearing thresholds during our follow-up care. The hearing level of patient with congenital cytomegalovirus infection became worse after newborn hearing screening as usually reported. The hearing levels of other 2 patients were improved during the following 2 years. One patient with hereditary hearing disorders with a hearing aid started to sing songs at 1 year 11 months old. One patient started to wear a hearing aid at her early period, but she did not try to wear a hearing aid after 1 year and 11 months old.These cases remind us that we must follow-up babies who are diagnosed as “refer” by AABR for the long period of time carefully.

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Four cases suffering from intractable vertiginous attacks with Meniere's disease were treated by a middle ear pressure device, i. e. Meniett^®, fo2ry ears. In allc ases, v ertigow as controlledb v 4 months aftert he start of the pressure treatment. Three cases had reappearance of the vertigo when the tympanostomy tube was troubled in one case, when the device was used less frequently than recommended in one case and when the pressure pulse was weakened because of the device trouble in one case. These finings suggested that the middle earpressure treatment should be continued for at least 3months, and otolaryngologists shouldpay attention to both tympanostomy tube and device trouble at the time of reappearance of vertigo.

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Stapes-pyramidal fixation with normal stapedius ligament is very rare. We present one casewith the stapes-pyramidal fixation with normal stapedius ligament in both ears. Stapes head and pyramidal bone were connected with a bony bar and stapes footplate was not scleroticbut mobile. Preoperative diagnosis was otosclerosis. At first operation, removal of the bony bar from the pyramidal bone using a chisel was attempted and consequently a total stapedectomy was resulted. The opposite ear has same stapes-pyramidal fixation by a bony bar with normal stapedius ligament.
Using SKEETER^® drill (Medtronic XOMED, Inc.) only the bonybar was removed between the pyramidal bone and the stapes head, then stapes mobility was achieved. In each side of the ear the stapes showed monopodal superstructure.

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A 3-year-old female was born as the second child of twins in 34 weeks pregnancy. Comparedwith twins' elder sister, the delay of lingual development has been conspicuous at one anda half years old. She had no response to auditory brainstem response (ABR). Computed tomography (CT) of the inner ear showed very narrow internal auditory canals bilaterally. Magnetic resonance imaging (MRI) revealed the internal auditory canal was very narrow and the cochlear nerve bunch was not delineated.
Under general anesthesia electrophysiologic tests including distortion product otoacoustic emission (DPOAE), auditory steady-state response (ASSR), electrocochleogram (ECoG), electrically evoked auditory brain stem response (EABR) were performed. EABR testing showed that there was response only in the left side. Therefore, cochlear implantation was performed in the left side using CI24M. Neural response telemetry (NRT) during the operation showed observable response from all electrodes. The mapping with ACE strategy was possible using all electrodes at the time of switch-on. Afterswitch-on, Speech performance is developing.
MRI is not totally reliable to delineate the thin cochlear nerve in the narrow internal auditory canal. EABR should be indicated to decide candidate for cochler implant even in cases with very poor sound responses and an uncertain image of cochlear nerve. Therefore, it is necessary to perform adequate electrophysiologic tests.

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A 7-year-old girl with no otological symptoms was incidentally found a blue mass protruding from the hypotympanum in the right ear by an otologist, and she was referred to our clinic for further examination. Her audiogram showed a mixed hearing loss in the right ear.
CT scan revealed a dehiscent jugular bulb bulging into the right middle ear space, but did not contact to the ossicular chain. Sigmoid sinus was dilated and cortical bone attachedto the sigmoid sinus became very thin. MRI and MRA revealed remarkable dilatation of rightsigmoid sinus and transverse sinus. Furthermore, the left transverse sinus. sigmoid sinus and jugular vein were not detected by MRI and MRA. Neck ultrasonic examinations revealed the left jugular vein, but there was no blood flow detected by pulse doppler mode. We discuss some important points on this patient with these pathologies.

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Intraoperative facial nerve monitoring is essential for acoustic neuroma surgery to preserve postoperative facial nerve function. The monitoring is now adopting for otologic surgeries such as cochlear implant and middle ear cholesteatoma. Several kinds of insulated dissector have been developed for identification and/or dissection of the facial nerve from tumor. However, the size and shape of the functional edge differ in each dissector. Here, wedeveloped new insulated dissectors by modification of Kanzaki model (Nagashima Medical Instrument). Our new model is Teflon-coated up to its functional edge, and consists of 11 dissectors having four different edges of pin, ball, knife and ring. The dissectors can be used by connecting with NIMPulse^TMIntegrity Monitor (Medtronic XOMED).

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The Na⁺/H⁺exchanger (NHE, SLC9) is a membrane transporter that catalyzes the electroneutral counter-transport of Na⁺for H⁺.NHE is involved in various physiological and pathological aspects such as maintenance of ionic homeostasis, cell volume regulation, transepithelial transport of electrolytes, cell growth and apoptosis. To date more than 10 different NHE isoforms have been identified and their molecular and cellular biochemistry has been extensively studied. In this report, we will briefly summarize the structurefunction of NHE, particularly focusing on our recent findings.

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