Many Japanese institutions use the Japan Otological Society criteria (2000) for the assessment of hearing gain in evaluating the postoperative hearing results following stapes surgery. However, there may be problems with high success rates, a lack of consideration of high frequency hearing, and differences in methods of measuring postoperative air-bone gap and assessment criteria between Japan and overseas. In this study, we compared postoperative results using the Japan Otological Society criteria (2000) and the American Academy of Otolaryngology (AAO-HNS) guidelines. We altered parameters such as the frequencies used, the method of calculation of the average hearing level, and the use of preoperative or postoperative bone conduction thresholds. As the result, we found that success rates were significantly higher using the Japan Otological Society criteria (2000) than the AAO-HNS guidelines. No influence was seen on postoperative results using the mean of 3 frequencies: 0.5, 1.0 and 2.0 kHz or the mean of 4 frequencies with the addition of 3.0 or 4.0 kHz. Furthermore, no difference was seen in the results between using the preoperative or postoperative bone conduction threshold to calculate the postoperative air-bone gap. We also displayed the audiology results using an Amsterdam hearing evaluation plot, which provided a useful visual presentation of each patient's results.

View full abstract

We presented 2 cases of congenital absence of the oval window. In the first case, the stapes and the oval window were absent, and the facial nerve was found to run inferiorly. Fenestration of the vestibule was performed superior to the malpositioned facial nerve, and the ossicular chain was reconstructed with a Teflon wire piston. In the second case, the facial nerve was normal, and fenestration of the vestibule was performed where the oval window was supposed to exist. The otic capsule was very thick at the fenestra. The hearing level improved, postoperatively, in both cases.
In congenital absence of the oval window, satisfactory postoperative hearing results can be obtained, even in the cases with facial nerve anomaly or thick otic capsule at the fenestra, sometimes making it difficult to perform the surgery.

View full abstract

A 64-year-old man presented with bilateral hearing impairment and vertigo since he was 24 years of age. He was treated as Méniere's disease, but the treatment was not effective. Blue sclera was noted and the patient was also suffered from bone fracture three times in the past, and he was diagnosed as having van der Hoeve syndrome. CT scan and MR imaging showed progressive otic capsule demineralization. Small fenestra stapedectomy was performed in his right ear. Otic capsule was significantly demineralized and thin. Finally, he recovered his hearing using a hearing aid without any complication. Stapes surgery for van der Hoeve syndrome is safe and useful.

View full abstract

A 69-year-old male presented with complaints of severe headache. MRI revealed that a mass located at the lateral cerebellum invading to the mastoid cavity of the petrous bone. The right external auditory canal (EAC) had no abnormality at the initial visit, but a small hole gradually appeared at the opening of EAC. During surgery, a cholesteatoma mass was removed from the mastoid cavity and the canal wall was reconstructed by a temporal bone fragment. This case could be classified as congenital cholesteatoma because the patient had no history of head injury.

View full abstract

The patients with patulous Eustachian tube presented with aural fullness, autophonia and audition of breathing sound in the ear, mainly due to the direct entry of sound created within the body to the ear through a patent Eustachian tube. In this study, we studied the patients visited our hospital during April 2004 to March 2010, according to the followings, age at first visit, symptoms, the cause of patulous Eustachian tube, auditory condition, and treatments.
A total of 41 patients visited and treated in our hospital and their ages ranged from 16 to 83 years (mean ± SD, 49.0 ± 20.2 years). They complained with aural fullness and autophonia in 76 % and 73 % of the patients, respectively. In contrast, 51% of the patients suffered from breathing sound in the ear. The causes of patulous Eustachian tube were as follows: weight loss in 14 patients, pregnancy in 2 patients and mixed connective tissue diseases in 2 patients. Fifteen patients were diagnosed with hearing loss including 6 patients with deterioration of hearing level in low frequencies. Regarding to the treatments, medication and nasal instillation of physiological saline were less effective than insufflation of the Lugol's solution or infusion of absorbable gelatin sponge solution into the pharyngeal orifice of the Eustachian tube. However the duration of effectiveness on the symptoms were short in the last 2 methods.
Four patients treated with conservative treatments described above were eventually underwent the insertion of a silicone plug in the patulous Eustachian tube. In three out of the four patients, their symptoms were remarkably reduced after this treatment, indicating insertion of the silicone plug was very effective for the patients with severe symptoms.

View full abstract

Idiopathic sudden hearing loss (ISHL) is defined as deafness of sudden onset and unknown origin. The clinical characteristics of ISHL in elderly people have not been well documented. We retrospectively investigated 61 cases of ISHL, whose age was older than 65 year. The average age of the subjects was 74.6 years. The pure tone average (PTA) of the affected ear, and the unaffected ear, was 74.6dB and 33.2dB, respectively. The PTA recovered to 56.2dB in the affected ear. The number of the complete recovery cases was 11 (18.0%), the marked recovery was 15 (24.6%), the slight recovery was 14 (23.0%), and the no recovery was 21 (34.4%). (By the diagnosis criterion of Health, Labour and Welfare Ministry of Japan.) The rate of improvement was lower than that in the previous reports. The prognosis of ISHL was analyzed in relation to the presence of vertigo and the underlying diseases, such as hypertension, diabetes mellitus, hyperlipidemia which are considered as microvascular risk factors for the prognosis of ISHL. The presence of vertigo was significantly related to the prognosis of hearing level. However, the other underlying diseases were not significantly related to the hearing recovery.

View full abstract

We treated 3783 patients who had acute otitis media and mastoiditis from April 1997 to May 2007. In those patients, we had encountered five cases with intractable otitis media caused by antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis syndrome. The major symptoms of ANCA-associated vasculitis syndrome were interstitial pneumonia, subacute glomerulonephritis and upper respiratory symptoms. On the other hand, the initiation of treatment was often delayed in the patients who had no symptoms such as the above, because it was difficult to make the definite diagnosis. As the earlier treatment is likely to achieve better prognosis in terms of hearing, the early detection is crucial. We reported the cases treated in our department and consider the important points for early initiation of treatment.

View full abstract

It is well known for a long time that various drugs and substances injected in the tympanic cavity could enter perilymph through the round window membrane, and many experimental studies have been reported concerning to uptake of steroid in the inner ear by intratympanic instillation. Summing up these studies, 1) concentration of perilymph steroid by intratympanic injection is 24.7-189.6 times higher as compared with intravenous injection, 2) immunohistochemical studies revealed steroid can diffuse and distribute to the spiral ligament, organ of Corti and spiral ganglion in all turns of the cochlea, 3) uptake of steroid in the inner ear can reach the peak level 1 to 3 hours after instillation and, it is not detected 24 hours later. From these reports, intratympanic steroid injection has the advantage that highly-concentrated inner ear uptake can be obtained by a small dose of steroid administration. This is the reason why intratympanic steroid injection becomes to attract attention more recently as a treatment of acute sensorineural hearing loss such as idiopathic sudden sensorineural deafness.

View full abstract

Intratympanic steroids therapy has been widely used to treat inner ear diseases such as sudden sensorineural hearing loss and Ménière's disease. We administered gadolinium contrast agents (Gd) intratympanically and performed magnetic resonance imaging (MRI) to investigate how the drug distributes in the inner ear. Gd signal intensity in the inner ear showed wide individual difference after the intratympanic Gd injection. The individual difference may depend on the degree of the permeability through the round window membrane. Two patients underwent exploratory tympanotomy because the Gd movement into the inner ear was poor. In these patients, we removed the overhanging bone to visualize the round window membrane and observed good round window reflex when the stapes was touched. In one patient, however, Gd movement from the middle ear into the inner ear could not be obtained. These results indicated that evaluation of the permeability through the round window membrane was difficult even though accessibility to the round window membrane could be established by the surgery. Intratympanic Gd injection and MRI provide useful information for intratympanic drug therapy for the treatment of inner ear diseases.

View full abstract

Intratympanic injection of drugs into the inner ear has been developed for the treatment for sudden sensorineural hearing loss and Meniere's disease. However, adhesion covering the round window membrane (RWM) was observed in 10-30% of all the cases. To remove the adhesion prior to injection of drugs we developed and tested two types of new otoendoscopy device that allowed visualization of the round window (RW) membrane. The first otoendoscope for inner ear drug delivery has a fine needle with catheter can be used to remove or perforate RW niche (RWN) mucosal adhesions. The second one is thinner and more flexible to perform less traumatic for tympanic membrane and middle ear. The two types of otoendoscope we developed can be used for application of drugs directly onto the surface of the RWN, and to verify the correct placement of an inner ear drug delivery system.

View full abstract

Recently, local drug application has gained particular attention as a therapeutic strategy for inner ear diseases. In local treatment, therapeutic agents are applied on the round window membrane (RWM) in the middle ear, and transferred into the cochlear fluid through the RWM by diffusion. Sustained delivery of drugs is included critical issues for the efficiency of local therapy. We have performed a clinical trial to test the safety and efficacy of local application of insulin-like growth factor 1 (IGF1) using gelatin hydrogels, which enables sustained release of growth factors, for patients with sudden deafness resistant to systemic steroids. The results demonstrate hearing improvement in a half of patients and no occurrence of serious adverse events, indicating the safety and efficacy of local IGF1 treatment via gelatin hydrogels for sudden deafness.

View full abstract

Although the systemic steroids have been considered as gold-standard treatment for sudden sensorineural hearing loss, overall recovery is poor in those patients who fail initial therapy. For those patients, we used intratympanic steroids therapy. We have evaluated the effect in 83 patients. As clinical outcomes, 26 patients (31.3%) demonstrated complete recovery, 5 patients (6.0%) marked recovery, 21 patients (21.5%) slight recovery and 31 patients (37.3%) non recovery. The average hearing level before the treatment was under 60dB and the treatment was started within 8 weeks in most of the patients who showed complete recovery and marked recovery. There were no severe side effects and complications.
Intratympanic steroids therapy should be considered as an effective treatment in the case who failed in systemic steroids.

View full abstract

Musical activities promote many aspects of child development. In particular musical activities offer opportunities to engage young CI users in listening. Musical pitch is poorly transmitted through CI's indicating the need for music to be presented in a sequential and logical way. A music training programme was implemented at a rehabilitation centre in Turkey and 25 young CI users' progress through the programme was monitored. Children's performance on musical activities improved overtime with training. Necessary adaptations were made to the programme before publishing MusicalEars®. MusicalEars® with detailed information and video clips should make inclusion of musical activities into habilitation programmes easier for non-specialist teachers.

View full abstract