Recent progress in genetics is remarkable and the application of gene therapy to the inner ear disease is expected. Adenovirus has attracted a great attention as a vector for gene therapy but it has not clinically applied in ear diseases because the method of cochlear gene transfer has not established and the safety of the adenovirus in the ear has not been clear at present.
Our study was designed to confirm the feasibility of adenovirus vector into the cochlear of the gerbils and to determine whether adenovirus-mediated overexpression of one of neuronal growth factor: glial cell line-derived neurotrophic factor (GDNF) could prevent the profound cochlear damage iduced by transient ischemia in gerbil model. The injection of the adenovirus vector did not cause the increase of compound action potential (CAP) threshold. Furthermore, on the seventh day of ischemia the CAP threshold shift was remarkably suppressed in the GDNF adenovirus treated group comparing with control groups. Our results indicated that adenovirus-mediated GDNF might be useful for the treatment of human auditory degeneration.

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In this study, we performed clinical investigations for seventy-nine young patients affected with intractable otitis media including those who had otitis media as a complication from 1990 to 2000.
The subjects were 44 males and 35 females of the age from 3 months to 4 years and 3 months. The mean age was one year and two months. Of approximately 80% of the patients, the isolated bacteria from ear discharge were Haemophilus influenzae, Streptococcus pneumoniae or both. The number of young patients with intractable otitis media caused by H. influenzae who were required hospitalization has been increasing since 1999. Since the appearance of antibiotic-resistant H. influenzae (BLNAR) in 2000, therapy for otitis media has been more difficult.
In addition, Streptococcus pneumoniae were found to acquire the stronger resistance against antibiotics in 2000 than in 1999.
Although we have challenged intravenous administration of ABPC, SBT/ABPC, and CTRX to the patients with otitis media caused by H. influenzae and, of CTRX and MEPM to the patients with otitis media caused by BLNAR, therapeutic results are not yet satisfactory. Intravenous administration of PAPM/BP, however, was quite effective in treatment for otitis media caused by penicillin-resistant S. pneumoniae.
In nine of the 21 young patients to whom IgG2 levels were investigated in 2000, low levels of IgG 2 were detected. Globulin supplement therapy appeared to be effective in such cases. The ventilation tube in the middle ear was effective in treatment for recurrent otitis media resulting from secretory otitis media.

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Facial nerve paralysis caused by acute otitis media is not so rare in children. Recently, we treated three children with facial nerve paralysis complicated with acute otitis media. Penicillin-resistant or -intermediate Streptococcus pneumoniae were detected in all patients. Facial nerve paralysis was completely cured in all cases by use of antibiotics and surgical treatment such as myringotomy. Antibiotics-resistant bacteria are increasing in recent years, and the treatment against those bacteria seems to be more important. Misjudgement of the selection of antibiotics and the surgical treatment might lead a deterioration and prolongation of otitis media.
Facial nerve paralysis complicated with otitis media frequently occures in children of one year old or younger. Anatomical factors such as an immaturity of the facial canal and immunological factors such as IgG2 subclass immunodeficiency are considered to be associated with the pathogenesis. In conclusion, the occurrence of facial nerve paralysis should be paid attention in the treatment of acute otitis media in infants.

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To prevent ear infection many physicians advise avoidance of swimming after insertion of grommet tubes. This advice is a logical extension of the supposition that contaminated water entering the middle ear through the tube may cause an infection. This study was designed to examine the incidence of otorrhea after swimming with grommet tube or perforation of tymapanic membrane. Forty patients with grommet tubes or perforation of the tymapanic membrane were evaluated. The children were allowed to swim with precaution and were advised not to dunk the head. Out of 40 children, 11 had otorrhea in 11 ears. The infection was easily controlled in all cases with medication.
We conclude that swimming can be safely permitted for children with grommet tube or perforation of the tympanic membrane.

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Between November 1987 and April 1996, 110 cases with middle ear cholesteatoma were operated on in Kagawa Medical University Hospital. Seventy-three cases (66%) were followed more than 5 years after surgery with the average duration of 90 months (60 to 150 months).
Surgical procedures were classified according to surgical methods and approaches in tympanoplasty for chronic otitis media 2000 proposed by the Organizing Committee of Otology Japan. Thirteen-three cases were operated on by canal wall up tympanoplasty, and 60 cases by canal wall down tympanoplasty. Twentyone cases of them underwent staged tympanoplasty. Type I tympanoplasty was performed in 6 ears, type II tympanoplasty 1 ear, type III tympanoplasty 2 ears, modified type III 49 ears, and modified type IV 15 ears.
We evaluated the successful rate of hearing on 12 months and 60 months after surgery to investigate the change of the hearing level. The successful rate with modified type III tympanoplasty was stable for a longterm follow up. The successful rate with modified type IV tympanoplasty, however, was decreased 60 months after the surgery.
Recurrent cholesteatoma occurred in 5 ears of canal wall up tympanoplasty (31%), 3 ears of canal wall down with canal reconstruction and mastoid obliteration (21.4%) and one ear of canal wall down tympanoplasty (2%). The recurrence rate in children (33.3%) was significantly higher than that in adult (6.9%). Mastoid obliteration was a useful method to prevent the postoperative cavity problem.

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It is well known that the inner ear and stapes function are remained normal and the hearing improvement can be expected by meatoplasty, myringoplasty and ossiculoplasty in the patients with congenital atresia or stenosis of the ear canal. The patients who underwent meatotympanoplasty are expected for having good influence on daily life because of the patients being youths.
Recently, we have operated on 13 ears of 11 patients (two bilateral patients), aged from 5 to 21, 10 atresia, 2 stenosis, and one post-operative sequelae. Two dimensional target computed tomography was useful for surgical approach. We drilled the temporal bone at the external auditory meatus with the one tunnel method upto sufficient width for making the tympanic cavity and the external auditory canal.
Meatotympanoplasty for congenital auditory canal atresia or stenosis is good news for the patients suffering from this disease.

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In recent years, the multislice CT system has come into practical use that enables table movement of half mm, resulting in a significant improvement in resolution. The use of this CT system enables to depict the entire auditory ossicles, including the stapes. 3D reconstruction was performed using helical CT data in 5 patients with chronic otitis media and 5 patients with cholesteatoma.
An Aquilion Multi (Toshiba) multislice helical CT scanner and a Xtension (Toshiba) image workstation were used in this study. We demonstrated the 3D display with axial, coronal and sagittal images. Compared with the normal ears, it was necessary to set a higher threshold for the affected ears. It is important to select suitable threshold for demonstration of 3 D images optimally. Bone destruction of the stapes was confirmed at surgery in 2 ears. The stapes was observed at 3 D-CT imaging in other 18 ears. It was found that the 3 D images of the ossicular destruction in ears with cholesteatoma were consistent with surgical findings. It is therefore concluded that 3 D imaging of the middle ear using a multislice CT scanner is clinically useful.

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We experienced two patients with cholesterol granuloma who complained of bloody otorrhea. The first patient underwent a simple mastoidectomy and ventilation tube insertion, which allowed aeration of the middle ear spaces and hearing improvement. The second patient with total deafness in the offected ear under-went obliteration of the external ear canal, the middle ear space and mastoid cavity. Surgical exploration revealed complete obstruction of the Eustachian tube in both patients. We discussed the treatment of choice for cases of cholesterol granuloma caused by complete obstruction of the Eustachian tube.

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The diagnosis and treatment of carcinoma of the external auditory canal are thought to be difficult because of lack of the accepted staging system, rarity of disease and anatomical complications in the temporal bone. In this paper, five cases of carcinoma of the external auditory canal treated from 1999 to 2001 are presented. All cases were squamous cell carcinoma. Four cases were T 1 and T 2 group, and the other was T 3 group. All cases underwent lateral temporal bone resection and the anterior wall was reconstructed using the postauricular flap. One patient received postoperative chemotherapy and radiotherapy. The mean average period for epithelization of the ear cavity was 57 days and survival rate was 80% with a maximal three-year follow-up.
We suggested that the postauricular flap was useful for reconstruction of the anterior wall of the external auditory meatus and it accelerated the epithelization of the ear cavity, and that lateral temporal bone resection was useful in T 1 and T 2 cases.

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From October 1994 through September 2001 we had 24 cases of cochlear implant in children. Among them, twenty cases were congenitally deaf and four were perilingually or postlingually deaf. Thirteen of the children were between 2 and 4 years old at the time of surgery. Three were less than 2 years old. Most of children were found deaf between 1 and 2 years old but six were found deaf after 2 years old. Twelve children had been wearing hearing aids more than 24 months. The development of auditory performance was plotted as a function of month after surgery. The children between 2 to 4 years of ages showed the steady development reaching to the point they could understand 3 words sentence by 18 months after surgery. On the contrary it was very difficult for over 9 years old children to understand the words even after 2 years after the surgery.The hearing ability was limited to hear the sound around them or to hear their own name. The speech performance was also plotted as a function of month after surgery. The development rate was much slower than that of hearing development. Most of cases under 4 years old seemed to show the steady development of speech and they could speak sentences by 24 months after surgery. The results of the present study suggested us that if cochlear implant is performed in pediatric age under 4 years, the good results can be expected even in congenitally deaf children in terms of the hearing and speech.

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We investigated 90 patients with idiopathic sudden deafness treated in our hospital between 1996 and 2001. Forty-nine were treated with corticosteroids and stellate ganglion block (SGB) with lipo-prostaglandin E1 (Lipo-PGE1).These patients were compared with a control group consisting of 41 patients treated without Lipo-PGE1. The over all recovery rate of the patients treated with Lipo-PGE1 was 70% and this was significantly higher than that of the patients treated without Lipo-PGE1.

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Four high-risk infants who had normal auditory brainstem responses at the time of discharge from the neonatal intensive care unit were found on follow-up between 18 and 23 months later to have significant hearing loss. Birth weights ranged from 2422 to 3220g. Gestational ages ranged from 35 to 41 weeks. All of the infants required mechanical ventilation with severe respiratory difficulty. Three of the four had persistent pulmonary hypertension of the newborn (PPHN). Two infants received aminoglycoside, three pancuronium bromide, and four furosemide. We conclude that infants who had severe respiratory difficulty may remain at risk for development of significant hearing loss even though they have passed an initial auditory brainstem response screening test in their newborn period.

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Actual case report is very rare though it has been assumed that perilymph fistula can induce endolymphatic hydrops. We experienced a case with endolymphatic hydrops, in which the perilymph fistula was found by chance during exploratory tympanotomy combined with an endolymphatic sac decompression surgery.
surgery. A 22-year-old female was referred to our clinic for the treatment of the left hearing impairment after myringotomy. Though she was diagnosed as sudden deafness and treated, the improvement of hearing was not obtained. It was doubtful about endolymphatic hydrops because her severe headache and fluctuating hearing impairment, and an endolymphatic sac decompression was done. A perilymph fistula from the oval window was found during the surgery.
Endolymphatic sac decompression was done together with closing of the perilymph fistula. A headache disappeared postoperatively, and a hearing impairment was also improved.
Relative rise in the endolymph pressure was thought as a cause of the endolymphatic hydrops in this case due to the decline of the perilymph pressure.

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