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Middle ear implants (MEIs) such as the Vibrant Soundbridge (VSB) are attractive and alternative treatments for patients with conductive, sensorineural, and mixed hearing loss who do not benefit from, or who choose not to wear, conventional hearing aids (HAs). Recent studies suggest that MEIs can provide better improvements in functional gain, speech perception, and quality of life than HAs, although there are certain risks associated with the surgery which should be taken into consideration, including facial nerve or chorda tympanic nerve damage, dysfunctions of the middle and inner ears, and future device failure/explantation. In Japan, a multi-center clinical trial of VSB was conducted between 2011-2014. A round window vibroplasty via the transmastoid approach was adopted in the protocol. The bony lip overhanging the round window membrane (RWM) was extensively but very carefully drilled to introduce the Floating Mass Transducer (FMT). Perichondrium sheets were used to stabilize the FMT onto the RWM. According to the audiological criteria, the upper limit of bone conduction should be 45 dB, 50 dB, and 65 dB from 500 Hz to 4, 000 Hz. Twenty-five patients underwent the surgery so far at 13 different medical centers. The age at the surgery was between 26-79 years old, and there were 15 males and 10 females. The cause of conductive or mixed hearing loss was middle ear diseases in 23 cases and congenital aural atresia in two cases. The data concerning on the effectiveness and safety of VSB was collected before the surgery and 20 weeks after the surgery. Significant improvements of free-field Pure Tone Audiogram (PTA) from 250 Hz to 8, 000 Hz were confirmed (p < 0.001). Hearing gain up to 40 dB was achieved in the 1, 000 Hz to 4, 000 Hz range. No deterioration in either air conduction or bone conduction at PTA was noted at 20 weeks after the surgery. Monosyllable speech perception in both quiet and noisy conditions improved significantly (p < 0.001). The speech discrimination score in both quiet and noisy conditions improved significantly too (p < 0.001). In the future, it is likely that there will be an increasing population even in Japan that will meet the criteria for MEIs such as VSB. However, the long-term efficacy and safety of these devices should be established.

The Vibrant Soundbridge (VSB) is an active middle ear implant with the Floating Mass Transducer (FMT). We performed a multicenter study to study the efficacy of the VSB by means of "the 10 Questionnaire on Hearing 2002" and "the APHAB questionnaire" at 13 hospitals between 2011 and 2013. In all, 23 patients with mixed or conductive hearing loss received VSB implantation by the round window placement technique. These individuals were generally unable to use, or gained little from conventional hearing aids or bone conduction hearing aids. Two questionnaires were administrated before the surgery and 20 weeks after the VSB implantation. Scores on every item of "the 10 Questionnaire on Hearing 2002" showed significant improvement under noise after VSB implantation. On the APHAB, the scores for Ease of Communication, Reverberation, and Background subscales improved significantly after the VSB implantation, while the score for the Aversiveness subscale alone failed to show a positive improvement from the inexperience to the new sound. Analysis of the responses to these subjective questionnaires revealed better results after VSB implantation as compared to the preoperative data. In conclusion, RW vibroplasty with the use of VSB provided subjective benefit in patients with conductive and mixed hearing loss.

Mycotic pseudoaneurysm is a rare complication of deep neck infection. We report herein on a case of mycotic pseudoaneurysm of the external carotid artery following myelodysplastic syndrome and a peritonsillar abscess. The patient was a male in his 60s, who complained of a sore throat and swelling of the left side of his neck. CT scan of the neck showed a left peritonsillar abscess and a pseudoaneurysm of the left external carotid artery. To correspond to airway obstruction in the event that the pseudoaneurysm might rupture to the pharyngeal space, the patient was transferred to our hospital. Surgical management was not selected, since MRI taken just after the admission to our hospital demonstrated remission of the pseudoaneurysm by organization of the structure and decrease of the blood flow. An antibacterial drug and an antifungal drug, SBTPC and VRCZ, were administered to the patient, the inflammatory signs reduced and the size of the pseudoaneurysm gradually diminished without sequela. These findings strongly suggest the importance of controlling the initial neck infection by administration of appropriate antibacterial and/or antifungal drugs to avoid the expansion of the mycotic pseudoaneurysm. Furthermore, MRI was useful to assess the state of the lesion and to determine the way of management.

Exostoses are benign bony excrescences of the external auditory canal, commonly encountered in people who avidly engage in aquatic activities, hence the name "surfer's ear". Exostoses are more prevalent in cold water surfers, and additional years of surfing increase one's risk not only for developing an exostosis but also for developing more severe lesions. Exostoses remain clinically silent until they become large enough to impair the egress of epithelial debris and water from the canal, in which case there may be an associated external otitis and fluctuating hearing loss. Histologically, they demonstrate a laminated structure consistent with a periodic growth pattern. They may also cause a hearing loss by impinging upon the tympanic membrane and manubrium. Symptomatic relief is attained by surgical removal and skin grafting of the epithelially denuded areas of the bony walls of the external auditory canal. We report herein on 3 cases of exostoses which developed in patients who had a habit of taking a cold water bath after a hot sauna for more than 15 years: in spite of the limited time of exposure to cold water stimulation, alternating exposure to the hot environment of the sauna and cold water baths seemed to have acceralated the formation of the exostoses.

Usher syndrome is an autosomal-recessive disorder that causes bilateral sensorineural hearing loss, retinitis pigmentosa (RP), and occasionally vestibular dysfunction. Usher syndrome types 1, 2, and 3 can be distinguished by differences in audiovestibular features. The objectives of this retrospective study were to evaluate 26 patients with Usher syndrome clinically. The 26 patients (male: 12 cases, female: 14 cases) with Usher syndrome, with a clinical diagnosis based on symptoms of bilateral sensorineural hearing loss and RP, had been registered from 13 hospitals as a multicenter study. We assessed the clinical history and performed audiovestibular and ophthalmologic examinations, and genetic testing. Eleven of the patients were classified as having Usher type 1 (38.5%), 6 with Usher type 2 (23.1%), and 9 with Usher type 3 (38.5%). However, many patients with atypical Usher type 1 (70%) and type 2 (83.3%) were found compared with Usher type 3 (10%). The conductive rate of vestibular examinations including the caloric test (50%) was low. There were many variations in the clinical symptoms in Usher syndrome patients, therefore the classification of Usher types 1, 2, and 3 has been complicated. We have proposed a flowchart for the diagnosis of Usher types 1, 2, and 3.

Between 1992 and 2010, we studied audiometric findings obtained from the annual hearing examination for senior high-school students who belonged to an active Kendo team. The subjects comprised 140 males and 88 females with ages between 15 and 18 years. The pure tone audiometry showed that 69 ears of 45 students (19.7%) had sensorineural hearing loss with the highest threshold shift at 2000 Hz, followed by 4000 Hz. Frequent audiometric patterns included a 2000 Hz-dip, a 4000 Hz-dip and a combined loss of 2000 and 4000 Hz. Some of these affected subjects had shown a completely normal audiogram at the previous examination. Moreover, small-dips with a depth less than 25 dB were found to be limited either at 2000 Hz or at 4000 Hz, suggesting early audiometric changes from a temporary or a permanent threshold shift caused by noise and/or head blows during Kendo practice. The incidence of such abnormal audiograms among this Kendo club members appears to be decreasing year by year owing to the annual check-ups over the 18-year study period, and student counseling regarding the possible adverse effect of Kendo on the inner ear function.

A 4-month-old healthy female infant presented with rapid onset of subaural swelling over a three-month period. A head and neck exam demonstrated a subaural elastic hard mass with a red birthmark below the left auricle. MRI of the neck demonstrated a well-defined parotid mass consistent with a haemangioma. We treated this infant with 1 mg/kg of propranolol, which was gradually increased over two months to a dose of 2 mg/kg daily. The tumor began to reduce in size within three days after drug administration, and became less prominent in one month, and had almost totally disappeared within four months. On ten-month follow-up, the patient was asymptomatic and repeated MRI demonstrated further regression of the tumor. Propranolol could be the first-line choice for treating haemangioma rather than simple

The effectiveness of bone anchored hearing aid (BAHA) for the patients with congenital aural atresia was evaluated by multicenter clinical study in Japan. Twenty patients (17 bilateral and 3 hemilateral) of congenital auricular atresia were registered for this study and finally, 18 of them (15 bilateral and 3 unilateral) were subjected to further evaluation. Primary endpoint of this study was free sound-field pure-tone audiometory and speech threshold hearing test in quiet and noisy circumstances. Secondary endpoint of this study was patient's satisfaction based upon APHAB (Abbreviated Profile of Hearing Aid Benefit) questionnaire survey. These results were compared between before and 12 weeks after BAHA surgery. Both hearing level of pure tone and speech threshold significantly improved after BAHA surgery. APHAB scores also suggested the improvement of the QOL after BAHA usage, except for the scores that concerned with unpleasantness of noisy sound. BAHA is one of the useful options for the treatment of congenital auricular atresia.

Objective: This study, was conducted to determine the clinical value of diffusion-weighted MR imaging (DWI) in detecting the presence of cholesteatoma., Subject and Methods: Fifty-six patients (21 female and 35 male patients; mean age, 43 years) who underwent middle ear surgery were referred to the radiology department for a preoperative DWI study., Results: DWI depicted 41 out of 48 cholesteatomas involving the middle ear cavity (sensitivity, 85.4%). Seven patients with middle ear cholesteatoma who showed negative DWI findings (false-negative cases) had limited keratin accumulation due to simple atelectasis or meticulous evacuation of keratin debris before the MRI study. No falsepositive cases were found in this study (specificity, 100%). The positive predictive value and negative predictive value were 100% and 53.3%, respectively. The minimum size of middle ear cholesteatoma detected by the current MRI system was 5mm., Conclusion: Diffusion-weighted MR imaging was useful for the detection of middle ear cholesteatoma.

Enlarged vestibular aqueduct (EVA) is an inner ear anomaly occasionally associated with sensorineural hearing loss (SNHL) and/or dizziness. Recent genetic studies indicate that mutations in the PDS gene may cause EVA. A 10-year-old EVA patient who had undergone annual hearing tests for 7 years had an aunt and cousin who also had hearing loss and EVA, so genetic examinations were conducted for a possible genetic link. Two new PDS gene mutations, S610X and S657N, were found in all 3, including the proband. We discuss the importance of genetic analysis, which offers new insight into SNHL diagnosis and treatment in children.

A partially implantable middle ear implant using an ossicular vibrator of a piezoelectric ceramic bimorph has recently been developed in Japan as a new rehabilitative method for hearing. Four patients at the Miyazaki Medical College Hospital were implanted with this device between September 1994 and October 1996. Implantation of the internal component was performed under local anesthesia after confirmation of sufficient sensitivity to the ossicular vibrator in an intraoperative vibratory hearing test. All patients regained socially useful hearing with the middle ear implant without any foreign body reactions or recurrence of otitis media. None of the cases showed any elevations of bone conduction thresholds 2 to 4 years postoperatively. Natural and clear sounds without interference noise and howling due to acoustic feedback were highly satisfactory in all patients. However, the first case, who had an implant in an ear following cholesteatoma surgery using the intact canal wall technique, experienced about a 10dB deterioration of the average hearing level with the implant 2 years after implantation. Gradual deterioration appeared to be caused by the retracted tympanic membrane interfering with the mobility of the ossicular vibrator. The following three cases were implanted in ears previously operated on by radical mastoidectomy, showing stable or improving hearing results with the implant. Closure of the external canal skin at the cartilaginous portion at the time of implantation seemed appropriate to maintain a sufficient middle ear space for the ossicular vibrator to work properly. Our case study confirms that the partially implantable middle ear implant is a safe and useful tool for patients with mixed deafness which cannot be satisfactorily rehabilitated by tympanoplasty and/or a conventional hearing aid.

Extratympanic recording of electrocochleography (ECochG) has played an important role in the differential diagnosis of inner ear diseases. We used a special electrode, which was wrapped in a cotton ball at the tip and covered with a silicon sheath over the entire length, and recorded ECochG from the tympanic membrane (tympanic ECochG). Our method was found to be more convenient and less traumatic than recording with an ear canal electrode. Tympanic ECochG records from 10 normal volunteers showed no influence of iontophoretic anesthesia on the tympanic membrane. The effects on the conductive hearing mechanism were negligible. The input-output curve of the action potential (AP) by click stimuli was fairly stable and comparable to that obtained with transtympanic recording. We performed tympanic ECochG in patients with Meniere's disease or other sensory hearing loss, and compared the amplitude ratios of the summating potential (SP) and AP (SP/AP ratio) with those in normal hearing subjects. The SP/AP ratios in patients with Meniere's disease were significantly increased, an observation consistent with the results of other studies. The SP/AP ratio was also elevated in patients with autoimmune sensory hearing loss or perilymphatic fistula. Based on the results of the present study, we speculate that it is possible to diagnose an inner ear disorder by comparing the tympanic ECochG findings with not only records from normal subjects, but also the contralateral record of tympanic ECochG from the same subject. We conclude that tympanic ECochG using disposable electrodes can provide useful information on inner ear function, because of its convenience, non invasiveness and safety in clinical use. We found tympanic ECochG to be useful in the glycerol dehydration test and for monitoring inner ear function during acoustic neurinoma surgery.

We present a 76-year-old male patient with adhesive-type cholesteatoma and with metal foreign bodies which were shown to be located in the bony eustachian tube by computed tomography. He sustained a burn injury of the left tympanic membrane when he was struck by a bomb 52 years ago, during World War II. The cannonball fragments that entered the tympanic cavity were apparently transported to and stuck in the eustachian tube isthmus by mucociliary action after spontaneous closure of the tympanic membrane perforation. Persistent tubal obstruction due to the impacted foreign bodies and surrounding granulation tissue seems to have caused chronic adhesive otitis, leading to cholesteatoma which developed in the attic and mastoid antrum. No foreign bodies became visible after cholesteatoma removal by an intact canal wall technique in conjunction with anterior tympanotomy for wide exposure of the supratubal recess and the tympanic osteum of the eustachian tube. Therefore, anterior tympanotomy was further extended anteriorly to open the enlarged bony eustachian tube, allowing visualization and safe removal of two cannonball-fragments firmly impacted within it. We call this surgical approach to the bony eustachian tube "extended anterior tympanotomy". The transmastoidal accessibility of the bony eustachian tube produced by this technique should be assessed by preoperative computed tomography.

Between 1987 and 1993, 71 ears of attic type cholesteatoma and 20 ears of adhesive type were operated on using the intact canal wall technique combined with anterior tympanotomy. Pre- and postoperative pure tone hearing after the surgery were analyzed, especially to determine whether anterior tympanotomy is harmful to the inner ear. Mean values of 500, 1000 and 2000Hz of air conduction threshold immediately, before and about 1 year after the operation were compared. In attic cholesteatoma, type 1, type 3, and type 4 of ossiculoplasty revealed significant improvement of 8.4dB, 9.3dB, and 8.0dB respectively. In adhesive cholesteatoma, results obtained were 8.4dB, 0.8dB, and -3.0dB. Differences between pre- and postoperative bone conduction threshold at 4000Hz were 1.5dB in type 1, 1.3dB in type 3, and 5.0dB in type 4 in attic cholesteatoma. Those in adhesive cholesteatoma were -2.5dB, -0.2dB, and -10.0dB respectively. From the results obtained, we concluded that (1) our results were satisfactory with regards to hearing ability following all types of ossiculoplasty in attic cholesteatoma and in type 1 in adhesive cholesteatoma, (2) anterior tympanotomy itself is not a harmful procedure to the inner ear in both types of cholesteatoma, (3) an increase in bone threshold in adhesive cholesteatoma is due to the surgical procedure at the stapes and/or the oval window.

This study was designed to show MRI findings of postoperative middle ear pathologies and to discuss the usefulness of Gadolinium-enhanced MRI in evaluating the postoperative state of cholesteatoma. Thirty-eight ears which underwent intact canal wall tympanoplasty for cholesteatoma were examined. Recurrent cholesteatoma was detected as an iso-intensity area on T1-weighted images with negative enhancement. Notably, residual cholesteatoma were generally depicted as a round iso-intensity area with negative enhancement. Residual cholesteatoma less than 5mm in diameter were, however, not generally detectable with our MRI scanner. Granulation tissue can be separated from cholesteatoma as an area with positive enhancement. Cholesterol granuloma shows a characteristic high signal pattern on both T1 and T2-weighted images. Hypovascular fibrous tissue and fluid collection may be depicted as a pattern similar to that of cholesteatoma. However, the signal is usually more homogeneous than that of cholesteatoma. We conclude that Gadolinium-enhanced MRI is useful for detecting postoperative cholesteatoma and avoiding unnecessary second-look operations after cholesteatoma surgery, by the canal-up procedure.