We performed tympanoplasty on 39 patients with congenital cholesteatoma from September 1994 to November 2010. Using Potsic's staging system, we investigated the age of first examination, the reason for being examined, surgical procedure and auditory acuity at each stage of disease progression. There were 4 cases of stage I (10.2%), 4 cases of stage II (10.2%), 18 cases of stage III (46.3%), and 13 cases of stage IV (33.3%). The average age of the patients was 4 years at stage I, 5.2 years at stage II, 12.1 years at stage III and 12.7 years at stage IV. Their reasons for receiving otological examination included hearing impairment and the other than hearing impairment, such as vertigo, otitis media and otitis externa, and their congenital cholesteatoma was coincidentally identified. Minimally invasive surgery was possible because most of patients were young and mostly at stage I.

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The reconstruction of an open cavity using sliced cartilage, fascia and meatally based musculoperiosteal flap (Palva flap) was performed in 12 cases. All patients were followed for more than two years. The mastoid skin was elevated and trimmed, then sliced cartilage was transplanted to the mastoid side of the skin and covered using Palva flap. In the case of normal or shallow eardrum, ossicular reconstruction was performed in a single stage (10/12 cases, 83%). Two-staged surgery was planned for some of patients with adhesive eardrum (2/12 cases, 17%). The mastoid cavity was obliterated with bone chips in the poorly developed air cell groups (8 cases). However, we attempted to reconstruct a ventilated mastoid in the well-developed air cell groups (4 cases). Re-pneumatization of the mastoid was observed in 3 of these 4 cases. Overall success in hearing improvement was 67% (8/12 cases) 12 months postoperatively. In all cases, epithelialization was complete. None of the patients developed recurrent discharge, cholesteatoma or granulation. The reconstructed tympanum and posterior canal wall became a thick structure made of skin and sliced cartilage. This boot-shaped canal reconstruction was suited to staged ossiculoplasty because of the shape-memory effect that provides an adequate combination of stiffness and flexibility in the second stage. The structure was relatively stable over the long term. This method offers an advantage for adhesive cases that require secondary ossiculoplasty.

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The Vibrant Soundbridge (VSB), middle ear hearing implant, has been proven effective for patients who have moderate to severe sensorineural or mixed hearing loss, and a large number of patients mainly in Western countries have already been fitted.
A 71 year-old man with mixed hearing loss due to bilateral adhesive otitis media underwent VSB implantation on the round window membrane. Tympanopalsty had previously been performed twice in each ear, however, no significant hearing improvement was achieved. Hearing aids were uncomfortable, produced howling and therefore unsuitable for this patient. However, after surgery, his hearing was greatly improved.
We suggested that VSB can be effective for cases in which hearing was not significantly improved after middle ear surgery and/or after being fitted with hearing aid.

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Ceruminous adenoma is a rare benign neoplasm arising from the ceruminous grand of the external auditory canal. We reported a case of ceruminous adenoma mimicking squamous cell carcinoma in the external auditory canal. A 59-year-old woman presented with ear discharge in her left ear. She had no pain, hearing loss, tinnitus, nor vertigo. Physical examination showed a space-occupying lesion on the superior part of left external auditory canal. Biopsy was performed and squamous cell carcinoma was suspected. A local resection was performed with reconstruction of the external auditory canal using retroauricular flap. Postoperative pathological diagnosis was ceruminous adenoma. There was no evidence of recurrence at 18 months of follow-up.

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Usher syndrome is a major cause of genetic deafness and blindness. The standard classification of Usher syndrome recognizes three clinical types. The present state of Usher syndrome in Japan was evaluated by questionnaire including information on the number and the clinical types of patients diagnosed with the syndrome. We targeted 697 hospitals in which the Oto-Rhino-Laryngological Society of Japan approved as training facilities.
As a result, there were 111 cases reports. There were two possible reasons for this. The one was that the patients were not examined by any otolaryngologist, and the other was that they were not followed-up even if the patients presented themselves in otolaryngology department. In addition, the responders classified them into 15 patients with type 1, 27 patients with type 2, and 17 patients with type 3, but 52 patients (46.8%) were unclassified. We speculated that basis for the classification of Usher syndrome was ambiguous.
In order to achieve accurate classification, we thought that it is necessary to establish the datebase of Usher syndrome patients in cooperation with ophthalmologists.

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The otoferlin (OTOF) gene is known to be involved in autosomal recessive hearing impairment. OTOF mutations are considered to be a major cause of inherited auditory neuropathy (AN).
A total of 4 children with hearing impairments who were suspected of having AN based on audiological findings and language development delays were studied. All 4 had abnormal auditory brain-stem response (ABR) and normal distortion product otoacoustic emissions (DPOAE), at least on one side. None had inner ear malformations nor other complications or risk factors for AN (e.g., hyperbilirubinemia). Mutations in the OTOF gene were detected in 3 of the 4 cases: compound heterozygous mutations in 1 case and only 1 mutant allele in 2 cases.
The case with the compound heterozygous mutations had passed the newborn-hearing screening test (NHS) and had normal DPOAE at the first test, but an abnormal DPOAE at 5 years of age. Another case passed the NHS using an auto-ABR in the left ear and referred in the right ear, and had normal DPOAE in the left ear and abnormal DPOAE in the right ear. Profound hearing loss with little benefit from use of hearing aids was present in 2 cases. Mild to moderate hearing loss with some benefit received from hearing aids in language acquisition was present in 1 case.
It could be very helpful to investigate mutations in the OTOF gene in order to diagnose AN. Furthermore, detection of mutations in the OTOF gene should lead to appropriate management (such as cochlear implants). However, the recent report also suggested that AN-related mutations in the OTOF have case-by-case differences and that some cases of undiagnosed AN may exist due to abnormal DPOAE. Children with AN should be correctly diagnosed and managed in order to mitigate language development delay.

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Ramsay Hunt syndrome is a rare complication of herpes zoster in which reactivation of latent varicella zoster virus infection occurs in the geniculate ganglion, causing otalgia, auricular vesicle formation, and peripheral facial paralysis. In this syndrome, vesicles usually appear in the ipsilateral ear and/or soft palate. Some case reports of immunocompromised hosts with Hunt syndrome and generalized herpes zoster have been published.
We report the case of a 45-year-old man who developed otalgia, ear fullness, facial paralysis, pharyngitis, and generalized herpes zoster. These symptoms appeared in tandem. The characteristic generalized skin rash enabled early diagnosis. His medical history was not significant and a childhood history of varicella infection. The HIV screening test yielded negative results. The patient was diagnosed 13 days after he first visited our clinic and therapy with valacyclovir and prednisolone po was immediately initiated after diagnosis. Facial paralysis was detected on day 14 and completely resolved on day 27. The sensorineural hearing loss was partially remitted, but he was asymptomatic. The symptoms in Hunt syndrome do not always present at the onset and atypical cases like the one discussed in this report are not uncommon; therefore, this syndrome can be misdiagnosed and the patient may develop permanent facial paralysis. Therefore, it is important that otolaryngologists must have sufficient knowledge about infections caused by herpes group viruses, especially about skin rashes that appear in such cases.

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Hunt syndrome is a disease caused by an infection of the geniculate ganglion by the varicella -zoster virus reactivation. It considered to be one type of disease of herpes zoster which mainly produces facial nerve palsy.
One morning in May two years ago, the right side of facial nerve palsy suddenly appeared. Generally tiredness had arisen from ten days before the onset of facial nerve palsy. Moreover, there was displeasure of the parotid gland from the several days before, and an upper pharynx pain and ear pain appeared from 2 or 3 days before of the onset. Although it was partial facial nerve palsy at the onset of Hunt syndrome, it became complete facial palsy within 2 or 3 days. Medical treatment with an anti-viral medicine and steroid medicine was started. Six weeks afterward of the onset it became 8 points of 40 points (by Yanagihara method) and 8% in ENoG, and I was diagnosed as sever Hunt syndrome.
Now, it has improved even to such a degree that palsy is hardly conspicuous with a photograph, though partial facial nerve palsy, pathological synkinesia, facial contracture and facial spasm are remains as a sequel. The rehabilitation of the facial nerve palsy may be an effective therapeutic method for the sever Hunt syndrome.

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