Hidenobu Taiji

Evaluation of Hearing Recovery and Efficacy of Steroid Treatment in Sudden Deafness

In cases of sudden deafness (SD), hearing recovery is generally assessed by classifying recovery into four degrees. To evaluate factors that may affect the prognosis for hearing recovery (e.g., hearing level at initial examination, days elapsed from onset of hearing loss to start of treatment) or drug efficacy, however, a quantitative method would be more advantageous. In the present study, therefore, the recovery rate was used as an index in investigating the prognosis and the efficacy of steroid treatments in 183 patients with SD first examined within 14 days after the onset of hearing loss during the period 1980-85. As with the degree of hearing recovery, the recovery rate was found to be favorable in those patients with good hearing at the initial examination, no vertigo, little difference in high-tone and low-tone hearing loss, and only a short lag between onset of symptoms and the initiation of treatment. This indicated that the recovery rate could be used as an index for the evaluation of hearing recovery in cases of sudden deafness. The efficacy of steroid treatment was evaluated in patients in whom the average of the hearing levels at five frequencies from 250 to 4,000 Hz was 40-90 dB. No significant differences were found in either recovery rate or degree of recovery between those patients who received steroids and those who did not. This was believed to be attributable to the variety of pathological conditions involved in SD and to the influence of the other, non-steroidal drugs administered.

Clinical Practice Guidelines for the diagnosis and management of acute otitis media (AOM) in children in Japan – 2013 update

OBJECTIVE To (1) indicate methods of diagnosis and testing for childhood (<15 years) acute otitis media (AOM) and (2) recommend methods of treatment in accordance with the evidence-based consensus reached by the Subcommittee of Clinical Practice Guideline for Diagnosis and Management of AOM in Children (Subcommittee of Clinical Practice Guideline), in light of the causative bacteria and their drug sensitivity of AOM in Japan. METHODS We investigated the most recently detected bacteria causing childhood AOM in Japan as well as antibacterial sensitivity and the worldwide distinct progress of vaccination, produced Clinical Questions concerning the diagnosis, testing methods, and treatment of AOM, searched literature published during 2000-2004, and issued the 2006 Guidelines. In the 2009 and 2013 Guidelines, we performed the same investigation with the addition of literature, which were not included in the 2006 Guidelines and published during 2005-2008 and during 2009-2012, respectively. RESULTS We categorized AOM as mild, moderate, or severe on the basis of tympanic membrane findings and clinical symptoms, and presented recommended treatment for each degree of severity. CONCLUSION Accurate assessment of tympanic membrane findings is important for judging the degree of severity and selecting a method of treatment. Some of new antimicrobial agents and pneumococcal vaccination are recommended as new treatment options.

Subgroups of enlarged vestibular aqueduct in relation to SLC26A4 mutations and hearing loss

To investigate possible association of hearing loss and SLC26A4 mutations with the subgroups of enlarged vestibular aqueduct (EVA) morphology in Japanese subjects with hearing loss.

Unilateral cochlear nerve hypoplasia in children with mild to moderate hearing loss.

Abstract Conclusion: Even if hearing loss is mild to moderate, the presence of cochlear nerve (CN) hypoplasia associated with retrocochlear disorders should be considered. Objectives: CN hypoplasia is a term that refers to an absent cochlear nerve on high-resolution magnetic resonance imaging (MRI). Most cases of CN hypoplasia are associated with profound hearing loss. The present study reports six pediatric cases of unilateral CN hypoplasia with mild to moderate hearing loss. Methods: Between May 2008 and April 2011, pure-tone hearing tests were performed in 17 patients who were diagnosed with CN hypoplasia on high resolution for evaluation of unilateral sensorineural hearing loss at the National Center for Child Health and Development. Of these, six patients had average hearing levels in the affected ears of < 60 dB and were therefore included in this study. Results: All six ears with CN hypoplasia were associated with CN canal stenosis. DPOAEs were present in one (17%) of the six affected ears. The ABR thresholds of the ears with CN hypoplasia were significantly elevated compared with 1–4 kHz pure-tone hearing levels in one of three cases. In two of five cases, the maximum word recognition scores of the affected ears were poor compared with pure-tone hearing levels.

Evaluation of the Eustachian Tube in Normal Subjects and in Patients with Otitis Media with Effusion by High Resolution Computerized Tomography

In recent years, tests of Eustachian tube function have undergone remarkable development. In contrast, radiological techniques of examination of the Eustachian tube have not gained general popularity, as they require too much labor to be included in routine tests. For the past 2 years, we have investigated the use of high-resolution computed tomography (CT) in the examination of the normal anatomy of the Eustachian tube and its pathological state in patients with middle ear diseases, especially intractable otitis media with effusion (OME).

Langerhans cell histiocytosis with disequilibrium.

Langerhans cell histiocytosis (LCH) is a very rare disease in which granulation tissue forms in various organs and the central nervous system (CNS) due to monoclonal proliferation of Langerhans cells. Some patients develop ataxia, tremor, or neurodegenerative abnormalities (such as personality changes and mental deterioration) several years after the onset as the late effects of LCH. We report a case of a 4-year-old boy with LCH, showing speech disorder, truncal ataxia and a wide-based gait with abnormal findings of central nervous system in CT and MRI image. The results of auditory brain stem response revealed a conduction block in the auditory conduction pathway, suggesting an axonopathy of the brain stem. Disequilibrium may be due to brainstem dysfunction associated with paraneoplastic syndrome because an anti-GluRε2 antibody was seen. Paraneoplastic syndrome is a neuropathy induced through an autoimmune mechanism caused by an antibody directed against the nervous system. Neuro-otological examination is helpful for the assessment of CNS neurodegeneration associated with LCH.

Risk factors for elevation of ABR threshold in NICU-treated infants

OBJECTIVE Several risk factors for hearing impairment among infants treated in the neonatal intensive care unit (NICU) have been reported, but there have been few studies that show the correlation strength between the risk factors in NICU-treated infants and hearing impairment in childhood. The aim of this study was to clarify the relationship between risk factors in NICU-treated infants and a deteriolation of auditory brainstem response (ABR) threshold in their childhood. METHODS One hundred one NICU-treated infants with ABR threshold of 50 dBnHL or more underwent 2nd ABR test at 20 months after delivery. Multiple regression analysis was performed with ABR threshold change as an objective variable and risk factors as explanatory variables. RESULTS Two ABR tests of the 101 infants resulted in that 7 showed an elevation of ABR threshold by 20 dB, 70 showed a drop of ABR threshold by 20 dB, and 24 showed no significant change. Multiple regression analysis revealed that the factors contributing to the elevation of ABR threshold were congenital diaphragmatic hernia, severe respiratory disease, and a high C-reactive protein (CRP) level. CONCLUSIONS In the infants treated in NICU, an incidence of ABR threshold of 50 dBnHL or more was 9.0%, and 6.9% of the infants with the ABR threshold abnormality showed a significant elevation of ABR threshold in their childhood. Factors significantly related to an elevation of ABR threshold were a history of congenital diaphragmatic hernia, severe respiratory disease, and elevation of CRP. In infants with such factors, periodical examination of hearing is required.

Treating Pediatric Psychogenic Dizziness with a Japanese Herbal Medicine

OBJECTIVE No conventional pharmacotherapy is available for the treatment of psychogenic dizziness in pediatric patients. Adults with psychogenic dizziness are treated with psychiatric medicines as the standard treatment. In children, this treatment is not widely accepted because of the potential for adverse reactions. Instead, Kampo, an alternative medicine, is probably the best choice of treatment. DESIGN We report herein three of four cases of pediatric psychogenic dizziness successfully treated with Yoku-kan-san-ka-chimpi-hange (YKCH), a traditional Japanese Kampo medicine known to ameliorate psychiatric symptoms. SUBJECTS YKCH was prescribed to four patients (two boys and two girls) with psychiatric dizziness whose ages ranged from 11 to 15 years. Four weeks after the initiation of treatment, clinical improvement was assessed by the Clinical Global impression-Improvement scale. RESULTS In three of the four patients, the drug showed some significant effects. The remaining patient could not continue the medication. It is reasonable to assume that YKCH was effective because of its serotonergic mechanism and the improvement of sleep in our patients. CONCLUSIONS This report is the first to describe the beneficial effects of YKCH in the treatment of pediatric psychogenic dizziness. We conclude that administration of YKCH may be a suitable complementary therapy for pediatric psychogenic dizziness.

Evaluation of vestibulocochlear nerves in cases with acoustic schwannoma by direct sagittal oblique 3D-FIESTA MR imaging

Oblique sagittal MRI with 3D-FIESTA (3-D fast imaging employing steady-state acquisition) sequences can be obtained through the internal auditory canal (IAC) without relying on multi-planar reconstructions. The high spatial resolution direct sagittal oblique imaging technique is useful for the identification of the four intracanalicular nerves. This work included one case of IAC tumor and two cases of cerebellopontine angle (CPA) tumors. MR imaging using a 3D-FIESTA sequence was performed in the oblique sagittal plane perpendicular to the course of the IAC. The direct sagittal oblique imaging technique allowed clear delineation of the individual nerves of the IAC. In a case of acoustic schwannoma, which was surgically proven to originate from the inferior vestibular nerve (IVN), the results of vestibular assessment indicated an IVN pathology. Acquired cochlear nerve hypoplasia with moderate hearing loss was found in a case of CPA tumor. Correspondence to: Hidenobu Taiji, MD, Department of Otorhinolaryngology, Saiseikai Central Hospital, 1-4-17, Mita, Minato-ku, Tokyo 108-0073, Japan, Tel: +81 3 3451 8211; Fax: +81 3 3451 6102; E-mail: taijih5@gmail.com