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Conclusion: The results suggest that SOD1 rs4998557 could be associated with susceptibility to SSNHL in the Japanese population., Objectives: To assess the gene association with sudden sensorineural hearing loss (SSNHL)., Methods: A two-stage case control study was conducted to explore the relationship of the candidate genes to SSNHL. The 192 gene samples from SSNHL patients registered in the intractable inner ear disease gene bank were enrolled. As the candidate genes, 39 SNPs from 31 genes were selected for the first stage study. The second stage study examined whether the SOD1 gene polymorphisms, defined by significant differences between cases and controls in the first stage study, are associated with SSNHL., Results: Significant differences were observed in four SNPs from three genes, Glutathione-S-transferase pai 1 (GSTP1), proteine kinase C heta (PRKCH), and superoxide dismutase 1 (SOD1), in terms of allele frequency between SSNHL patients and HapMap controls. In the SOD1 gene, a significant difference was observed in the dominant model study of the SNP rs4998557 in the second stage study. Furthermore, as a result of dividing SSNHL patients based on the clinical data, the difference was more apparent in the case of the over 60 dB group and the tinnitus-positive group.

Conclusion: Bortezomib was effective in attenuating atrophy of the posterior cricoarytenoid (PCA) muscle, but not the thyroarytenoid (TA) muscle. This was probably due to differences in the fiber composition of the two muscles. The PCA muscle is composed of a combination of fast- and slow-twitch fibers, and therefore is more resistant to atrophy than the TA muscle, which is composed solely of fast-twitch fibers., Objectives: To investigate the preventive effects of bortezomib on denervation-induced atrophy of the TA and PCA muscles in the rat., Methods: Following transection of the left recurrent laryngeal nerve, bortezomib (100 μg/kg) was administered subcutaneously on post-denervation days 1 and 4, followed by a 10-day rest period every 14 days; each 2-week period constituted a single treatment cycle. In controls, saline was administered instead. Animals were killed for histological examination at 4 (n = 6), 8 (n = 7), and 12 (n = 7) weeks post-denervation. Muscle atrophy was assessed using three indices: wet muscle weight, muscle fiber cross-sectional area, and the number of muscle fibers/mm(2). The effects of bortezomib were evaluated by comparing the left (L) and right (R) muscles, with sequential changes in the L/R ratio assessed., Results: In saline-administered animals, atrophy of the left-sided TA and PCA muscles progressed rapidly during the first 4 weeks post-denervation, following which progression slowed. Atrophy was greater in the TA compared with the PCA muscle, although this difference was not statistically significant. In bortezomib-administered animals, atrophy of the PCA muscle was attenuated significantly at post-denervation weeks 8 and 12; no such reduction in atrophy was observed for the TA muscle.

Conclusion: An epidemiological survey of hospitals and private clinics in Japan regarding idiopathic sudden sensorineural hearing loss (SSNHL) revealed that the incidence of SSNHL was 60.9 per 100 000 population. There were more females than males in the younger generation., Objective: The incidence of SSNHL varies largely by country. Because the Japanese criteria for diagnosing SSNHL have changed in accordance with those widely used in other parts of the world, a clinicoepidemiological study was undertaken using the new criteria., Methods: Ehime, Aichi, and Iwate Prefectures were selected from the western, central, and northeastern regions of Japan, respectively. The subjects for this study were patients who suffered SSNHL between April 1, 2012 and March 31, 2013. Questionnaires were mailed to all hospitals and private clinics in which ENT doctors were working. Initial and final audiograms were requested for 10% of the patients., Results: In all, 78 of 90 hospitals (87%) and 303 of 407 private clinics (74%) responded. It was reported that 1663 patients visited hospitals and 3090 patients visited only private clinics. It was estimated that 6205 SSNHL patients visited hospitals or private clinics in 1 year from a population of 10 145 000. Also, 23% of patients suffered acute low-tone SNHL (female to male ratio; 3:1 in definite cases).

Conclusions: Potsic's staging system is a clinically useful procedure for evaluating the extent of congenital cholesteatoma (CC)., Objectives: We investigated the clinical features, presenting symptoms, and surgical results of CC according to Potsic's staging system., Methods: A total of 71 patients who had undergone surgery at our hospital were retrospectively analyzed for presenting symptoms, the location of cholesteatoma, and surgical results according to Potsic's staging system., Results: Of the 71 patients, 21 were classified as Potsic stage I, 9 as stage II, 31 as stage III, and 10 as stage IV. More than half of the patients with early-stage CC (stages I and II) were diagnosed asymptomatically by a chance visit to a clinic or on ear screening. Others were diagnosed following a complaint of hearing loss, acute otitis media, or otitis media with effusion. The location of CC varied somewhat by stage. In stage I CC, the most frequent location was behind the anterior-superior quadrant of the tympanic membrane; however, in stage III CC, it was behind the posterior-superior quadrant. All patients were treated surgically. Recurrence was detected in 2 of the 71 patients (2.8%); both had stage II CC. Recurrent lesions were removed during revision surgery.

Conclusion: An 18-mer peptide derived from the neurotrophic region of prosaposin (PS-pep) prevents hearing loss and cochlear damage due to transient cochlear ischaemia by activating an anti-apoptotic pathway. PS-pep is a potent candidate molecule for alleviating ischaemia-induced hearing loss., Objective: PS-pep was investigated for its protective effects against ischaemia-induced hearing loss and cochlear damage., Methods: Ischaemia was induced in both cochleae in Mongolian gerbils by pulling the ligatures around both vertebral arteries in an anterior direction using 5 g weights for 15 min. PS-pep was synthesized artificially and administered subcutaneously four times after the induction of transient cochlear ischaemia., Results: An increase in the auditory brainstem response threshold was alleviated in animals treated with 2.0 mg/kg PS-pep. Histological examinations conducted on day 7 showed that the loss of inner hair cells (IHCs) was more prominent than that of outer hair cells. Higher doses of PS-pep significantly alleviated IHC loss. An increase in the anti-apoptotic factor bcl-2 was also noted in the IHCs treated with PS-pep.

Conclusion: Severity of ipsilateral facial palsy (UFP) can be assessed objectively by use of OKAO Vision®. Facial symmetry percentage (FSP) values were well correlated with the results by two conventional grading systems., Objectives: To introduce the basic principle of facial motion analysis using OKAO Vision®, to represent the data measured in patients with UFP, and to show the correlation between the severity of facial palsy assessed by this method and conventional systems., Methods: Two independent facial motions, closing the eyes and grinning, were recorded with a video camera. By comparing the movement distances between right and left sides, the eye symmetry ratio (ESR) and grin symmetry ratio (GSR) were obtained. The degree of UFP was assessed by FSP, which was calculated by the formula: FSP = (ESR + GSR)/2 × 100 (%). FSP was measured in 12 normal volunteers and in 30 patients with UFP., Results: In patients with UFP, varying degrees of FSP were noted corresponding to the grade of facial palsy. The severity of facial palsy assessed by FSP was closely correlated with the Yanagihara score (r = 0.88, p < 0.05) and was inversely related to the House-Brackmann grade (r = -0.90, p < 0.05).

Conclusion: Topical application of basic fibroblast growth factor (bFGF) hydrogel facilitates faster healing from traumatic facial paralysis due to continuous release of bFGF., Objectives: bFGF is considered a potent agent to facilitate recovery from neuronal damage; however, exogenously applied bFGF does not work well because of its short acting time. To enhance the effects in vivo, we developed a new drug delivery system by embedding bFGF in a gelatin hydrogel that degrades slowly. In this study, the effects of bFGF-hydrogel on traumatic facial nerve paralysis were investigated in guinea pigs., Methods: The intratemporal facial nerve was exposed and clamped at the vertical portion using micro needle forceps. The animals were then subjected to one of the following three procedures: group A, no further treatment; group B, one-shot application of bFGF to the nerve; and group C, application of bFGF-hydrogel instead. Six weeks later, facial nerve functions were evaluated by three test batteries: observation of facial movements, electrophysiological testing, and histological study., Results: The results for groups A and B were similar in the three tests, indicating that one-shot application of bFGF did not benefit facial nerve recovery. In contrast, group C achieved better results in all tests.

Conclusion: Although both T-cell subsets are essential for inhibiting HSV-1 reactivation in the GG, CD4 + T cells play a more important role in host defense against virus replication., Objective: To elucidate the host immunological factors that participate in herpes simplex virus type 1 (HSV-1) reactivation in the geniculate ganglia (GG) and lead to facial paralysis, we developed a mouse model of facial paralysis that involved the reactivation of HSV-1 following general immune suppression., Material and Methods: Eight weeks after recovery from primary facial paralysis caused by inoculating the auricle with HSV-1 the auricle was scratched and mice (n = 69) were given an i.p. injection of either anti-CD4 (n = 46) or anti-CD8 (n = 23) monoclonal antibody to deplete specific T-lymphocyte subsets. Following this reactivation procedure, the rate of recurrent facial paralysis was compared between the two models. The GG were examined histopathologically and using polymerase chain reaction to detect HSV-1 DNA., Results: Facial paralysis developed in 42% of mice in the anti-CD4 model and in 13% in the anti-CD8 model. HSV-1 DNA was detected in 50% of the mice in both models. Histopathologically, neurons were destroyed in parts of the GG and numerous virus particles were seen in the surviving neurons.

Objective: The aim of this experimental study was to examine whether recombinant human bone morphogenetic protein-2 (rhBMP-2) could induce new bone formation in a small hole drilled through the temporal bone near the external auditory canal wall in guinea pigs., Material and Methods: A 4-mm diameter hole was made in the center of the temporal bone and a piece of collagen sheet infiltrated with rhBMP-2 was placed in the hole. New bone formation was confirmed by means of X-ray examination and the bone mineral density was quantified using dual-energy X-ray absorptiometry 1, 2 and 4 weeks after treatment., Results: The bone mineral density in the rhBMP-2-treated group was significantly increased compared to the control group., Conclusion: These results suggest that rhBMP-2 induces remarkable new bone formation in the temporal bone and will be useful in clinical ear surgery.

Although Ramsay Hunt syndrome is one of the most important diseases causing peripheral facial palsy, the detailed pathology of the disease in the intratemporal facial nerve remains unclear. The purpose of this study was to increase knowledge of the pathogenesis of the syndrome by means of surgical findings. Between April 1976 and March 1997 we performed subtotal decompression of the facial nerve in 74 patients with severe Ramsay Hunt syndrome. The grade of nerve swelling was assessed using a microscope and recorded in a standardized form. The relationships between nerve swelling, the timing of surgery and the swelling of each segment were analyzed. Pronounced neural swelling, involving the geniculate ganglion and the horizontal segment, was consistent finding in the acute phase. Although the incidence of pronounced swelling of the horizontal segment gradually declined with time after onset, in most cases nerve swelling persisted even beyond the 16th week after onset. These data suggest that diffuse viral neuritis occurs throughout the intratemporal facial nerve. We assume that the viral inflammatory swelling involving the geniculate ganglion and horizontal segment is mostly responsible for the acute facial palsy in the acute phase.

The effects of lidocaine on basilar membrane (BM) vibration and compound action potential (CAP) were studied in guinea pigs in order to elucidate the site of lidocaine action in the cochlea. BM vibration was measured with a laser Doppler vibrometer through an opening made in the lateral bony wall of the scala tympani at the basal turn. Ten min after local administration of lidocaine (250 microg) into the scala tympani, the velocity of BM vibration and the CAP amplitude decreased significantly at around the characteristic frequency of the stimulus sound (p < 0.05). The maximum decreases were 4 dB in the velocity of the BM vibration and 40 dB in the CAP amplitude. In contrast, such changes were not observed after i.v. injection of lidocaine (1.5 mg/kg). These results suggest that when lidocaine is administered locally in the cochlea it acts not only on the cochlear nerve but also on the outer hair cells.

The pathogenesis of facial nerve paralysis and vestibulo-cochlear dysfunction of Ramsay Hunt syndrome remains unclear as varicella-zoster virus (VZV) has not been demonstrated in the lesions. Using the polymerase chain reaction, we detected VZV genomes not only in the vesicles on the auricles or oral cavity but also in the facial nerve sheath, middle ear mucosa and cerebrospinal fluid from patients with Ramsay Hunt syndrome. The VZV genome was undetectable in the same kinds of clinical samples obtained from control patients with facial nerve paralysis of other etiologies. The results indicated that VZV spreads widely in the neural components, mucocutaneous tissue and cerebrospinal fluid. The present study will facilitate better understanding of the pathogenesis of facial nerve paralysis, vertigo, hearing impairment and other cranial nerve dysfunction of Ramsay Hunt syndrome.

The effects of obliteration of the mastoid cavity on the growth of residual cholesteatoma were histologically studied in an animal model. A dermal cyst was produced by grafting a piece of autologous auricular skin in the otic bulla of 11 guinea-pigs. Three weeks after grafting, part of the cyst wall facing the cavity was removed and the debris accumulated inside was inserted into the surrounding granulation using a micropick. This procedure simulates the growth mechanism of cholesteatoma residue which sometimes occurs after middle ear surgery in human subjects. In six animals, the bulla was then obliterated with plaster of Paris. The remaining five animals were used as controls. Animals were killed for histological study at 2, 4 or 8 weeks postoperatively. Microscopic examinations revealed that in the obliteration group, severe inflammatory reactions were induced in the otic bulla, although the graft epithelium survived there; dermal cyst reformed in only one of six animals. In the controls, cyst reformation was recognized in all animals. This indicated that severe inflammation induced by plaster prevented growth of the graft epithelium in the otic bulla.

The effect of graded variations in middle ear pressure on ossicular vibration was measured in 15 normal human temporal bone specimens. The displacement amplitude of the umbo and stapes head was measured at 16 frequencies between 0.2 kHz and 3.5 kHz at a constant sound pressure of 134 dB SPL at the tympanic membrane (TM) using a non-contacting video measuring system. Both negative and positive pressures decreased umbo and stapes vibration at low frequencies and slightly increased the vibration at higher frequencies. The effects were greater for negative pressure than for positive pressure. The change in stapes vibration was less than that of the umbo at low frequencies, but increased at higher frequencies. In some temporal bones, a small positive pressure produced improvement in stapes vibration at all frequencies. These effects were thought to be primarily due to an increased stiffness of the TM and a damping of ossicular vibration, due to stretching of the ossicular suspensory ligaments and the annular ligament of the footplate.

An autologous free skin graft taken from the upper or the lower external ear canal or the auricle was implanted in the otic bulla of 28 guinea pigs. An epidermal cyst simulating middle ear cholesteatoma was successfully produced in 25 (89.3%) animals 8 weeks after grafting. Using this model, the histological reaction of middle ear granulation tissue to the debris was studied. Cysts originated from the auricular skin showed the strongest activity with regard to growth rate, amount of debris produced, and inflammatory reaction to the exposed debris. We conclude that the exposed debris plays an important role in chronic granulomatous inflammation in association with cholesteatoma.

A platinum-iridium electrode with a Teflon cover tube was chronically implanted through the round window into the scala tympani of the guinea pig. Animals were sacrificed at 30 to 40 weeks after electrode implantation, and the cochleae were observed by scanning electron microscopy or with light microscopy. The results revealed that the electrode was firmly enclosed with fibrous tissue on the inside of the round window and with epithelial tissue on the outside. These tissues were considered to act as a barrier to the entry of infection while preventing leakage of the perilymph.

The healing mechanism of experimentally produced ruptures of the round window was histologically studied in guinea pigs. Surgically created rupture of the round window resulted in spontaneous closure within 2 weeks. Light microscopical examination indicated that the rupture closed with proliferation of connective tissue which usually appeared to protrude into the middle ear cavity, each case showing a unique shape. In one case, the wound was covered with granulation tissue at the site of closure on the middle ear surface. Although the rupture was closed by connective tissue, the elastic fibres of the middle layer remained disrupted at the early stage of healing.

Vibration modes of the ossicles and the lever function were studied in human cadaver temporal bones with an intact cochlea. After placing tiny steel spheres on the ossicles, ossicular vibration to a sound stimulus was measured by observing the displacements of the spheres under a microscope with strobe illumination by means of a video measuring system. The lever ratio varied from 1.9 at 0.6 kHz (minimum) to a peak of 6 near 2 kHz. This relatively high lever ratio at higher frequencies was considered to be caused by a shift of the malleus-incus rotation axis secondary to the loading of the cochlear fluid on the ossicular system. Dependence of the lever ratio on frequency indicated that the rotation axis of the ossicles was not fixed, but variable according to frequency due to a relative increase in the translational movements of the rotation axis of the malleus and incus with frequency.

Brain stem evoked responses (BSERs) to acoustic and electrical stimulations of the cochlea were compared in guinea pigs. The electric stimuli evoked an averaged response of five to six successive waves within 10 msec, which was similar to that elicited by acoustic stimuli, though the latency of the electrical response was markedly shorter. Moreover, direct electrical stimulation of the auditory nerve evoked the same pattern of response as that elicited by electrical stimulation of the cochlea. On the other hand, stimulation of the vestibular nerve evoked a distinctly different pattern of response that lasted at most for 1.5 msec. Facial nerve stimulation evoked no brain stem response by this procedure. These results suggest that the waves of longer latency over 1.5 msec are available as indicators of auditory activity, even if the response elicited by electrical stimulation of the cochlea might be the sum of auditory and vestibular activities.