Advances in developing new atraumatic concepts of electrode design as well as surgical technique have enabled hearing preservation after cochlear implantation surgery, and EAS (electric acoustic stimulation) accompanied with hearing preservation is a new trend for patients with residual hearing at the lower frequencies. In this study, based on the atraumatic concepts and using a round window insertion, an atraumatic electrode, and dexamethasone administration, hearing preservation was evaluated with full insertion of the electrode. Post-operative evaluation after full insertion of the electrodes showed hearing at low frequencies was well preserved in all 16 cases (16 ears) who were followed up more than one month. In our series of experiences with full insertion with a long electrode we were successfully able to preserve residual hearing at low frequencies. The present results indicated that development of atraumatic procedures, including fine flexible electrodes, surgical technique (round window insertion), as well as post-operative steroid application enabled successful preservation for hearing.

View full abstract

Objective: To evaluate the efficacy of Electric Acoustic Stimulation (EAS) for high frequency hearing loss patients in Japan, and to determine the effect of EAS in individuals with residual lower frequency hearing, on speech perception in quiet and noisy condition. Study Design: Prospective design. Patients and Methods: Nine patients with profoundly high frequency hearing loss were recruited. All patients were assessed with an audiological test battery, and underwent cochlear implantation with a round window approach using the MED-EL FLEXeas electrode. Residual hearing, Japanese monosyllable words and speech discrimination abilities were evaluated at the point of 1, 3 and 6 months after EAS activated. Results: There were no cases with complete hearing loss in residual hearing after implantation. Low frequency residual hearing was preserved in eight patients, and one patient with a loss of residual hearing benefited from electrical stimulation. All EAS patients showed significant efficacy on all audiological tests over time. Conclusion: At the lower frequency hearing preservation with cochlear implant can be possible by round window approach as atraumatically surgical technique and FLEXeas electrode. AS the results with EAS demonstrate that this newly devise is effective in Japanese speaker with high frequency hearing loss.

View full abstract

This study was to assess the effects of low dose immunosuppressive therapy on hearing in patients with steroid responsive autoimmune inner ear disease (AIED) and a Western blot assay positive for 68 kD inner ear antigen. We conducted retrospective analysis of 46 patients. 18 patients were treated with steroid alone and 28 patients were treated with steroid followed by a cytotoxic agent. Pure tone average (PTA) at 250Hz, 500Hz, 1 kHz, 2 kHz and 4 kHz were used as objective measures of outcome. At the completion of treatment, 20 of the 46 patients (43%) exhibited a positive response to therapy, 12 (26%) were stabilized and 14 (30%) were worsened. 26 of 69 affected ears (38%) were improved. 11 patients who were treated with steroid alone demonstrated improvement in 13 of 19 affected ears (68%). 9 patients who were followed by a cytotoxic-agent demonstrated improvement in 11 of 12 affected ears (82%).
We conclude that 24% of patients with steroid responsive AIED obtained a benefit from low dose corticosteroid therapy and that a cytotoxic medication appeared to improve in 32% of patients who fell responsive to corticosteroid therapy.

View full abstract

The aggressive resorption process of the temporal bone structure adjacent to the matrix is one of the most distinct clinical findings of middle ear cholesteatoma. Recently, the receptor activator of NF κ-B ligand (RANKL), a part of the combination of the ligand-receptor system, is known to be deeply involved in the maturation and activation of osteoclasts. In this study, we have semi-quantitatively assessed the degree of immunohistological expression of RANKL and Ki-67, a proliferation marker, in 26 chlesteatoma patients. Positive expression of RANKL was predominantly detected mainly in basal and para-basal layers of the matrix accompanying with severe inflammatory cell infiltration in the submucosal layer. The Ki-67 labeling index (LI) was 27.7 in the group with higher RANKL expression and 15.7 in the group with lower RANKL expression, and the difference was statistically significant. These results suggest that epithelial proliferative activities and inflammatory manifestation could be the possible factors responsible for the markedly increased bone resorption observed in cholesteatoma patients.

View full abstract

Earwax genotype was investigated to prove whether it is related to the incidence of middle ear cholesteatoma by analyzing single nucleotide polymorphism (SNP). The subjects were 156 patients of middle ear cholesteatoma treated in our hospital. Control was 372 cases of normal volunteers. Wet earwax was found in 40 cases of cholesteatoma group (25.6%), while it was noted in 59 cases of control group (15.9%). Statistical analysis showed that the incidence of middle ear cholesteatoma was higher in wet earwax than in dry earwax (p<0.01). This finding indicates that wet earwax might be a strong risk factor of middle ear cholesteatoma.

View full abstract

We evaluated 38 patients (41ears) with auditory ossicle malformations without deformities of the external auditory meatus at our department between 2000 and 2009. There were 13 males and 25 females, and their mean age was 20 years. Classification of the pathologic condition based on surgical findings showed separation of incudostapedial joint in 20 cases, fixation of the malleus or incus in 10 cases, and fixation of the stapes footplate in 6 cases.
Auditory ossicular reconstruction was performed by type I in 4 cases (10%), modified type III in 20 cases (49%), modified type IV in 7 cases (17%) and stapes surgery in 6 cases (16%). Postoperative hearing success rate was 82% according to the criteria of the Japan Otology Society in 2010.
Improvement in hearing after surgery was relatively high compared with tympanoplasty for inflammatory disease. However, improvement in hearing after type I or stapes surgery was not satisfactory.

View full abstract

A 74-year-old woman complained of right aural fullness. She was diagnosed as secretory otitis media in her right ear and received a transtympanic ventilation tube insertion accompanied with antibiotic treatment. However, ear discharge through ventilation tube had been persisting and her symptoms were deteriorated. She was referred to our hospital because of mixed conductive-sensorineural hearing loss and dizziness. We suspected of bacterial labyrinthitis and treated with antibiotics and steroids (hydrocortisone). Although ear discharge and dizziness were improved after treatment, she had right facial nerve palsy and sever headache. Gadolinium-enhanced MRI revealed a thickening of dura mater in the right middle cranial fossa. Serum concentration of myeloperoxidase-antineutrophil cytoplasmic autoantibody (MPO-ANCA) was elevated. But proteinase 3 (PR3)-ANCA was negative. Histopathological examination of the specimen taken by right exploratory tympanomastoidectomy revealed non-specific inflammatory granulation tissue. Pulmonary and renal involvements were not detected. Thus, we diagnosed her disease as hypertrophic pachymeningitis associated with ANCA-associated vasculitis including a localized type of Wegener's granulomatosis (WG). After the administration of methylprednisolone and cyclophosphamide, her symptoms were sufficiently improved and the serum level of MPO-ANCA became normalized.
The treatment experience demonstrated that we have to make an early diagnosis of ANCA- associated vasculitis including WG and started steroids therapy along with immunosuppressant, even if the results of histopathological examination were not specific and MPO-ANCA was only positive.

View full abstract

We reported a case of MPO-ANCA-associated middle ear disease with sensorineural hearing loss and facial palsy. A 62-year-old female first complained of bilateral aural obstruction and the treatment for otitis media with effusion was ineffective. Then, bilateral sensorineural hearing loss developed, and after the transient effect of steroid therapy, left facial palsy appeared. CT demonstrated that the low density area in the middle ear cavity without bone destruction, and its granular tissue surgically resected showed non-specific findings by the histopathological examination. MPO-ANCA was positive, but PR3-ANCA was negative in the serum without pulmonary and renal lesions. Steroid treatment with cyclophosphamaide improved the hearing loss and facial palsy with a decrease of serum MPO-ANCA level.
Although it was reported previously that patients with MPO-ANCA-associated middle ear disease with sensorineural hearing loss and facial palsy were successfully treated by combination therapy with steroid and immunosuppressive drug in addition to our case, they could not meet the criteria of ANCA-associated vasculitides such as Wegener's glanulomatosis. Therefore, we proposed the diagnostic term and criteria of MPO-ANCA-associated middle ear disease with sensorineural hearing loss and facial palsy. The underlying pathophysiology of this disease may be MPO-ANCA-induced vasculitis, but review of literature showed that its clinical characteristics are different from those of ANCA-associated vasculitides.
The proposed diagnostic criteria were as follows: 1) the onset of otitis media-like symptom, 2) progressed sensorineural hearing loss, 3) complicating facial palsy, 4) lesion limited to the middle ear without pulmonary and renal lesions, 5) MPO-ANCA positive, but PR3-ANCA negative in the serum, 6) no findings of vasculitis in the middle ear lesion.

View full abstract

A 55-year-old woman presented with the chief complaint of breathing difficulty and hearing loss. She visited a local physician on April 24, 2007 because of fever and coughing for about 6 days. Pneumonia was found by chest x-rays, and she was referred to our hospital for the further examinations and treatment. On pure tone audiometry, a sensorineural hearing loss was found in both ears. In the respiratory medical department, mycoplasma pneumonia was diagnosed, and cephem and macrolide antibiotics were administered. The intravenous drip infusion of steroids was started in our department. When she was discharged hospital on May 3, her hearing level was improved up to 43dB in the right ear and 46dB in the left ear. The antibody high value was thought to due to infection of mycoplasma. The cochlear canaliculus theory, the autoimmunity theory, and the round window theory were convincing for mechanism of her sensorineural hearing loss. It may be said that an autoimmunity theory was reasonable in our case. It seemed that it was necessary to keep a possibility of mycoplasmal infection in mind when sensorineural hearing loss was accompanied with pneumonia.

View full abstract

Syphilis used to be a common cause of sensorineural hearing loss. The incidence has decreased markedly after the introduction of penicillin, however, there was recent increase in parallel with HIV infection. Otosyphilis, characterized by cochleovestibular dysfunction, is one of the clinical presentations in syphilis. We describe the unique otologic findings and clinical outcomes of a case diagnosed as otosyphilis in HIV-infection. A 55-year-old man was referred to our department of Kagoshima University Hospital for evaluation of hearing loss in his right ear. He had suffered from right ear fullness and the rash on his face for one month and half, and hearing loss in his right ear occurred suddenly one week ago. The patient showed moderate sensorineural hearing impairment in the right ear on pure tone audiometry. Because his facial skin lesion was diagnosed as seborrheic dermatitis by dermatologist and the patient was treated for having Entamoeba histolytica infection eight years ago, HIV infection was highly suspected. Serological examinations revealed HIV and syphilis infection and the patient was diagnosed as otosyphilis with HIV-infection. Other neurologic tests and imaging study were normal. Hearing impairment in the right ear improved with a 9-week course of amoxicillin of 1500mg/day orally. This case provides evidence that auditory findings arise as the initial manifestation of shyphilis in HIV-coinfected patient. Therefore, careful evaluation is needed in diagnosis of patients with hearing loss.

View full abstract

Paget's disease of bone is the second most common metabolic bone disease in European countries, although Paget's disease is uncommon in Japan. Paget's disease can affect one or multiple bones in the systemic skeleton, including the temporal bone. Paget's disease of the temporal bone is often associated with a mixed hearing loss or hemifacial spasm or expansion of head circumference. But the facial nerve palsy has rarely been reported.
We reported the case of a 52-year-old woman with left facial palsy and auditory disturbance due to Paget's disease at the left temporal bone.
We had chosen steroids treatment at first, and bisphosphonate was given, but her facial palsy was not improved. Three months later, the patient underwent operation of the facial nerve decompression and meatoplasty because the level of ENoG was deteriorated. The facial nerve was decompressed from bone and swelling. After the operation, her severe facial palsy and auditory disturbance were recovered.
Paget's disease at the temporal bone rarely accompanied with facial nerve palsy. We review the literature and discuss clinical features and management of this rare case.

View full abstract