Bilayer Hydrogel Composed of Elastin-Mimetic Polypeptides as a Bio-Actuator with Bidirectional and Reversible Bending Behaviors.

Biologically derived hydrogels have attracted attention as promising polymers for use in biomedical applications because of their high biocompatibility, biodegradability, and low toxicity. Elastin-mimetic polypeptides (EMPs), which contain a repeated amino acid sequence derived from the hydrophobic domain of tropoelastin, exhibit reversible phase transition behavior, and thus, represent an interesting starting point for the development of biologically derived hydrogels. In this study, we succeeded in developing functional EMP-conjugated hydrogels that displayed temperature-responsive swelling/shrinking properties. The EMP-conjugated hydrogels were prepared through the polymerization of acrylated EMP with acrylamide. The EMP hydrogel swelled and shrank in response to temperature changes, and the swelling/shrinking capacity of the EMP hydrogels could be controlled by altering either the amount of EMP or the salt concentration in the buffer. The EMP hydrogels were able to select a uniform component of EMPs with a desired and specific repeat number of the EMP sequence, which could control the swelling/shrinking property of the EMP hydrogel. Moreover, we developed a smart hydrogel actuator based on EMP crosslinked hydrogels and non-crosslinked hydrogels that exhibited bidirectional curvature behavior in response to changes in temperature. These thermally responsive EMP hydrogels have potential use as bio-actuators for a number of biomedical applications.

The characteristic of patulous eustachian tube patients diagnosed by the JOS diagnostic criteria.

OBJECTIVE: The objective is to describe characteristics of patients diagnosed with patulous Eustachian tube (PET) using the Diagnostic Criteria proposed by Japan Otological Society, and to evaluate the efficiency of objective tests to determine patent Eustachian tube. STUDY DESIGN: Retrospective. SETTING: Tertiary referral center. SUBJECTS: A retrospective survey of medical records in Sen-En Rifu Hospital identified 78 ears of 56 patients with "Definite PET" diagnosed by the JOS Diagnostic Criteria between January 2017 and December 2017. METHOD: Initial diagnosis, aural symptoms (voice autophony, aural fullness and breathing autophony), tubal obstruction procedures (posture change and pharyngeal orifice obstruction) and objective findings (tympanic membrane movement, Tubo-Tympano-Aerodynamic Graphy (TTAG) and sonotubometry) were evaluated. In addition, sonotubometry with postural change (Ohta's method), sitting CT and a newly devised PHI-10 score were also examined. RESULTS: Voice autophony, aural fullness, and breathing autophony were observed in 93.6%, 87.2%, 78.2%, respectively. In 91% of the ears, PET symptoms improved by postural change from sitting to the lying / forward-bending position. Synchronous movement of the TM upon respiration was observed in 69.1% of the ears. Positive findings of TTAG were observed in 75.6% of ears. Positive findings of sonotubometry were found in 55.1% of ears. Sonotubometry with postural change (Ohta's method), when the cut-off value of over 10dB was used, was positive in 45.2% of ears. Newly devised PHI-10 score representing severity of subjective symptoms classifying patients into no handicap, mild handicap, moderate handicap and severe handicap were observed in 12.2%, 10.8%, 18.9% and 58.1% of ears, respectively. The evaluation of the extent of patency of the ET by sitting CT indicated completely open, closed-short, and closed-long, in 68.6%, 11.4% and 21.4% of ears, respectively. Compared to the closed group, the completely open group had a significantly higher frequency of positive breathing autophony, positive sonotubometry, and positive Ohta's method. CONCLUSION: The characteristics of main symptoms and the efficiency of various tests in PET diagnosis were analyzed based on data obtained from "Definite PET" patients diagnosed by the JOS Diagnostic Criteria. The greater the availability of tests to evaluate PET, the greater the opportunities to diagnose "Definite PET". In particular, tests measuring pressure transmission between the nasopharynx and middle ear, such as TM observation and TTAG, are more sensitive than sonotubometry measuring sound transmission.

Frequency and clinical features of hearing loss caused by STRC deletions.

Sensorineural hearing loss is a common deficit and mainly occurs due to genetic factors. Recently, copy number variants (CNVs) in the STRC gene have also been recognized as a major cause of genetic hearing loss. We investigated the frequency of STRC deletions in the Japanese population and the characteristics of associated hearing loss. For CNV analysis, we employed a specialized method of Ion AmpliSeqTM sequencing, and confirmed the CNV results via custom array comparative genomic hybridization. We identified 17 probands with STRC homozygous deletions. The prevalence of STRC homozygous deletions was 1.7% in the hearing loss population overall, and 4.3% among mild-to-moderate hearing loss patients. A 2.63% carrier deletion rate was identified in both the hearing loss and the control population with normal hearing. In conclusion, our results show that STRC deletions are the second most common cause of mild-to-moderate hearing loss after the GJB2 gene, which accounts for the majority of genetic hearing loss. The phenotype of hearing loss is congenital and appears to be moderate, and is most likely to be stable without deterioration even after the age of 50. The present study highlights the importance of the STRC gene as a major cause of mild-to-moderate hearing loss.

Vibrant Soundbridge implantation via a retrofacial approach in a patient with congenital aural atresia.

OBJECTIVE: A method of Vibrant Soundbridge (VSB) placement to the round window (RW) via the retrofacial approach with preoperative evaluation of the relationship between the facial nerve (FN) and RW by 3D-CT reconstruction was proposed for the treatment of congenital aural atresia (CAA) patient. METHODS: A fenestration to the mesotympanum was made mastoid portion of the FN. During the approach, part of the stapedial muscle was encountered and removed. The RW niche was identified, and the floating mass transducer was placed from an inferior approach into the RW niche. RESULTS: There were no intra-operative or post-operative surgical complications. CONCLUSION: The VSB placement to the RW via the retrofacial approach with partial removal of the stapedial muscle can be feasible alternative in CAA cases associated with an anteriorly and laterally positioned aberrant FN. Preoperative assessment using 3D CT may facilitate in assessing the feasibility of the approach and implantation of VSB.

Surgical treatments for a case of superior canal dehiscence syndrome associated with patulous Eustachian tube.

OBJECTIVES: The patulous Eustachian tube (PET) and superior semicircular canal dehiscence syndrome (SCDS) have similarity in their symptoms and similar effects caused by positional changes, causing difficulty in the differentiation between the two disorders. This report describes a case of both SCDS and PET that was eventually successfully treated. METHODS: A 68-year-old man presented with hyperacusis to his own footsteps and gait disturbance. He had been diagnosed as PET two years before and had been treated by insertion of a silicone plug (Kobayashi plug) at the other hospital. Clinical case records, audiological data, cervical vestibular-evoked myogenic potential (cVEMP), Eustachian tube function tests and computed tomography (CT) were taken in the sitting position. RESULTS: While the CT confirmed superior semicircular canal dehiscence, the results of cVEMP was not typical of SCD likely due to preexisting hearing impairment in the right ear with a history of middle ear surgeries for the treatment of PET. He received round window reinforcement (RWR) and achieved relief from his symptoms but six months after the surgery, he visited again with complaints of autophony of his own voice and breathing. The tympanic membrane was found to move synchronous with respiration, and Eustachian tube function tests and the sitting CT confirmed the recurrence of severe PET. He had his silicone plug exchanged (increase in size of the Kobayashi plug) and achieved relief from symptoms. CONCLUSIONS: The present case was a rare instance showing that PET and SCDS can occur simultaneously in a patient. The patient achieved relief from symptoms after treatment with RWR and insertion of the Kobayashi plug.

Incidence of Functional Nasal Voice in Patients With Patulous Eustachian Tube.

OBJECTIVE: Some patients with a patulous Eustachian tube (PET) complain of a nasal voice. This feature is often dismissed without further investigation. As such, there are only a few reports on this important symptom and scant studies have been conducted on a sufficiently large number of cases with PET. Therefore, this study was undertaken to investigate the characteristics of patients having a nasal voice and to examine whether this symptom can be an indication of the severity of PET. STUDY DESIGN: Retrospective. SETTING: Tertiary referral center. SUBJECTS AND METHODS: A retrospective survey of medical records in Sen-En Rifu Hospital identified 85 patients (40 men and 45 women) with PET between 2013 and 2016. Diagnosis of definite PET was based on the Proposal on Diagnostic Criteria of PET announced by the Otological Society of Japan (2017). The questionnaire inquired about the presence of a nasal voice and it was distributed to each patient at the first visit to the clinic. If a patient marked "yes" for the presence of nasal voice, he/she was later asked on the telephone to exclude nasal voice ascribable to causes other than PET, such as nasal diseases. Correlation between nasal voice and patient characteristics (age, sex, affected side, and PET symptoms such as autophony of own voice, aural fullness, and autophony of breathing sounds), subjective severity of PET evaluated by patulous Eustachian tube handicap inventory-10 (PHI-10), and that with the objective severity of PET evaluated by tubo-tympano-aerodynamic-graphy (TTAG) and sonotubometry were investigated. RESULTS: Seventy-six patients (36 men and 40 women) with definite PET were evaluated in this study. Thirteen patients (17.1%) (five men and eight women) reported a nasal voice coinciding with the occurrence of PET symptoms such as voice autophony, aural fullness, and breathing autophony. Age, sex, affected side, PET symptoms (autophony of their own voice, aural fullness, and autophony of their breathing sounds), and objective findings (TTAG and sonotubometry) were not significantly different between the two groups. The average total score of the PHI-10 in the "PET associated Nasal Voice Group" was 35.8 ±â€Š4.5, which was statistically higher than that of the "non PET associated Nasal Voice Group" 23.6 ±â€Š10.7 (p = 0.002). Out of 76 patients, 44 were treated surgically (Kobayashi Plug). In the "PET associated Nasal Voice Group," 85% (11 out of 13) were subjected to surgical treatment, whereas 52% (33 out of 63) underwent surgical treatment in the "non PET associated Nasal Voice Group." The rate of surgical treatment was significantly higher in "PET associated Nasal Voice Group" (p = 0.047). CONCLUSION: Nasal voice due to PET symptoms was observed in 17.1% of PET patients. It was generally found in patients with severe subjective symptoms. Nasal voice can be an indication of subjective severity. However, this study failed to show objective evidence of wider Eustachian tube in such cases. Patients with a nasal voice tended to seek vigorous treatment including surgery.

Duplicated internal auditory canal with inner ear malformation: Case report and literature review.

Internal auditory canal anomalies are rare. Narrow internal auditory canal is believed to occur as a result of aplasia or hypoplasia of the vestibulocochlear nerve. Narrow duplication of the internal auditory canal is considered to be very rare. Narrow duplication of the internal auditory canal with inner ear malformation has been reported in only 3 cases. We present 2 cases of narrow duplication of the internal auditory canal with inner ear malformation. The first case had inner ear malformation on only one side and the second case had inner ear malformation on both sides. The embryogenesis may be different between internal auditory canal and inner ear.

The efficacy of the Eustachian Tube Dysfunction Questionnaire (ETDQ-7) for patulous Eustachian tube patient.

OBJECTIVE: To assess the efficacy of the Eustachian Tube Dysfunction Questionnaire (ETDQ-7) for patulous Eustachian tube (PET) patients. METHODS: A prospective survey of medical records identified 36 patients and 47 ears with PET, and 15 patients and 15 ears as control. The ETDQ-7, patulous Eustachian tube handicap inventory-10 (PHI-10) and Likert scale were evaluated. PET patients were divided into two groups based on severity of symptoms using the PHI score. RESULTS: The Cronbach α value of the PET group was 0.765. The average total score of the ETDQ-7 in the control group was 7.6 ± 1.1 and 22.5 ± 10.0 in the PET group (p < .01). No correlation was found between ETDQ-7 and Likert scale (r = 0.248, p = .09). The average total score of the ETDQ-7 in the mild or moderate PET group was 19.9 ± 9.0 and 25.3 ± 11.1 in the severe PET group and this was not statistically different (p = .08). CONCLUSION: The highest ETDQ-7 score was also observed in PET patients and in ET dysfunction patients. These findings necessitate careful discrimination between ET dysfunction and PET in balloon dilation Eustachian tuboplasty (BET) based on ETDQ-7.

POU4F3 mutation screening in Japanese hearing loss patients: Massively parallel DNA sequencing-based analysis identified novel variants associated with autosomal dominant hearing loss.

A variant in a transcription factor gene, POU4F3, is responsible for autosomal dominant nonsyndromic hereditary hearing loss, DFNA15. To date, 14 variants, including a whole deletion of POU4F3, have been reported to cause HL in various ethnic groups. In the present study, genetic screening for POU4F3 variants was carried out for a large series of Japanese hearing loss (HL) patients to clarify the prevalence and clinical characteristics of DFNA15 in the Japanese population. Massively parallel DNA sequencing of 68 target candidate genes was utilized in 2,549 unrelated Japanese HL patients (probands) to identify genomic variations responsible for HL. The detailed clinical features in patients with POU4F3 variants were collected from medical charts and analyzed. Novel 12 POU4F3 likely pathogenic variants (six missense variants, three frameshift variants, and three nonsense variants) were successfully identified in 15 probands (2.5%) among 602 families exhibiting autosomal dominant HL, whereas no variants were detected in the other 1,947 probands with autosomal recessive or inheritance pattern unknown HL. To obtain the audiovestibular configuration of the patients harboring POU4F3 variants, we collected audiograms and vestibular symptoms of the probands and their affected family members. Audiovestibular phenotypes in a total of 24 individuals from the 15 families possessing variants were characterized by progressive HL, with a large variation in the onset age and severity with or without vestibular symptoms observed. Pure-tone audiograms indicated the most prevalent configuration as mid-frequency HL type followed by high-frequency HL type, with asymmetry observed in approximately 20% of affected individuals. Analysis of the relationship between age and pure-tone average suggested that individuals with truncating variants showed earlier onset and slower progression of HL than did those with non-truncating variants. The present study showed that variants in POU4F3 were a common cause of autosomal dominant HL.

New Scoring System for Evaluating Patulous Eustachian Tube Patients.

OBJECTIVE: To assess the efficacy of patulous Eustachian tube handicap inventory (PHI) for patulous Eustachian tube (PET) patients. STUDY DESIGN: Prospective. SETTING: Tertiary referral center. SUBJECTS: A prospective survey of medical records in Sen-En Hospital identified 31 ears of 31 patients with definite PET who received insertion of the silicone plug as surgical treatment group, 29 ears of 29 patients treated with self-instillation of physiological saline solution as conservative treatment group, and 29 ears of 29 patients of sensorineural hearing loss without findings of PET treated between June 2015 and December 2015. METHOD: Diagnosis of definite PET was based on the proposal on PET diagnosis criteria announced by the Otological Society of Japan. The evaluation scale of PHI was modified from the Japanese version of the tinnitus handicap inventory-12 (THI-12). The classification for grading of severity is defined as follows: 1) no handicap (0-8), 2) mild handicap (10-16), 3) moderate handicap (18-24), and 4) severe handicap (26-40), matching the severity grades of tinnitus handicap inventory-25 (THI-25). The outcome measurement was modified from the previous scoring system and is defined as 1) complete relief, 2) significant improvement, 3) slight improvement, 4) unchanged, and 5) worse, and is applied according to the classification for grading of severity. The PHI was conducted at the first visit to our center for all patients in the three groups. For cases requiring surgery for plug insertion, patulous Eustachian tube handicap inventory 10 (PHI 10) was also conducted postsurgery after the treatment (postsurgery). RESULTS: The findings from questions 1 to 7 and 9 to 11 were significantly different between the surgical (presurgery) and conservative treatment groups (p < 0.05). Taking these results, we analyzed 10 questions excluding questions 8 and 12 (PHI 10). The total score of PHI 10 averaged 19.5 ±â€Š9.3 (n = 31) and 30.6 ±â€Š8.6 (n = 29) in the surgical treatment (presurgery) and conservative treatment groups, respectively, with a significant difference (p < 0.05). Internal consistency reliability testing of the PHI 10 yielded a Cronbach α of 0.887 for all questions. In the surgical treatment (presurgery) and conservative treatment groups, there were 0 (0%) and 3 cases (12%) of no handicap, 3 (10%) and 13 cases (50%) of mild handicap, 6 (19%) and 4 cases (15%) of moderate handicap, and 22 (71%) and 6 cases (23%) of severe handicap, respectively. There was a significant correlation between the PHI 10 and Likert scale (r = 0.796, p < 0.01). In the surgical group, the presurgery and postsurgery PHI 10 scores (n = 25) were 29.6 ±â€Š8.5 and 7.8 ±â€Š11.3, respectively. CONCLUSION: The PHI 10 is suitable for evaluating severity of PET if the patients have been diagnosed as definite PET. Furthermore, this scoring system could be suitable for surgical treatment assessment.

Computed tomography findings of the bony portion of the Eustachian tube with or without patulous Eustachian tube patients.

To investigate the bony segment of the Eustachian tube (ET) using sitting 3D-computed tomography (CT) scans in Patulous Eustachian tube (PET) patients. A retrospective survey of medical records in Sen-En Hospital identified 43 patients and 43 ears with PET and 30 patients and 30 ears with sensorineural hearing loss or vertigo patients as the control. Diagnosis of PET was based on the Proposal on PET Diagnosis Criteria announced by the Otological Society of Japan in 2012. Patients were examined by cone beam CT (Accuitomo; Morita, Kyoto, Japan) in the sitting position. The heights and widths at the tympanic orifice, the middle portion, and isthmus were measured. The lumen of the bony portion was divided into three shapes: peritubal cells (PTC) poor type, PTC good with prominence type, and PTC good without prominence type. In PET patients and the control group, the PTC poor type was identified in nine (21%) and seven ears (23%), PTC good with prominence type was identified in 14 (33%) and seven ears (23%), and PTC good without prominence type was identified in 19 (45%) and 16 ears (53%), respectively. There was no significant difference between the two groups. At the tympanic orifice portion, the average height of the ET lumen was 5.99 ± 1.29 and 6.04 ± 1.41 mm, and the average width of the ET lumen was 2.81 ± 0.82 and 2.78 ± 0.57 mm in the PET and control groups, respectively. The PTC good with prominence type had a significantly smaller width in the tympanic orifice portion than the other types in each group (p < 0.05). The width of the ET lumen in the tympanic orifice averaged 2.87 ± 0.38 and 3.10 ± 0.45 mm in the PTC poor type, 2.23 ± 0.70 and 2.22 ± 0.48 mm in the PTC good with prominence type, and 3.21 ± 0.87 and 2.90 ± 0.50 mm in the PTC good without prominence type in the PET and control groups, respectively. The shape of the bony portion of the ET in PET patients is almost identical to that of controls. Thus, the bony portion has no influence on the pathology of patulous Eustachian tube syndrome. The PTC good with prominence type has a significantly smaller tympanic orifice portion width than the other types. Preoperative evaluation of the bony portion of the ET could provide useful information for ET surgeons.

Hernia of the tympanic membrane.

Although tympanic bulging is commonly encountered, tympanic herniation occupying the external auditory canal is extremely rare. A 66-year-old man was presented to our hospital with left aural fullness, bilateral hearing loss and otorrhea. Preoperative findings suggested tympanic membrane (TM) hernia located in the left external auditory canal. We performed total resection of the soft mass by a transcanal approach using endoscopy. Ventilation tubes were inserted into bilateral ears. Histopathological findings confirmed diagnosis of TM hernia. Passive opening pressure of this patient was higher than normal condition of the Eustachian tube, where active opening was not observed. Hernia of the TM most likely resulted from long-term excessive Valsalva maneuver.

Tubal function tests with optional myringotomy detect Eustachian tube closing failure in acquired pars flaccida retraction cholesteatoma.

OBJECTIVE: To re-evaluate the incidence of Eustachian tube closing failure in acquired middle ear cholesteatoma. METHOD: Thirty-one cases with acquired middle ear cholesteatoma who received surgery were enrolled. Presence of Eustachian tube closing failure was determined through two Eustachian tube function tests. First Step Tests: Test 1: Positive sniff test identified by retraction of the tympanic membrane upon sniffing was observed. Test 2: The pressure in the external auditory meatus was found to change synchronously with that of the nasopharynx during respiration or upon sniffing. Second step test: For cases with negative First Step Tests, myringotomy was performed and Test 2 was repeated. RESULTS: Test 1 was positive in six (19.4%) and Test 2 was initially positive in nine (29.0%) out of 31 cases. Twelve out of 31 cases (38.7%) were positive for either one of the tests. The remaining 19 cases with initial negative test results subsequently received myringotomy and were subjected to Test 2 again. Positive results were obtained in five (13.9%) additional cases, and a final total of 17 (54.8%) out of 31 cases were positive for Eustachian tube closing failure. CONCLUSION: Sniff test with optional myringotomy may be useful for preoperative diagnosis of Eustachian tube closing failure.

The gastric H,K-ATPase in stria vascularis contributes to pH regulation of cochlear endolymph but not to K secretion.

BACKGROUND: Disturbance of acid-base balance in the inner ear is known to be associated with hearing loss in a number of conditions including genetic mutations and pharmacologic interventions. Several previous physiologic and immunohistochemical observations lead to proposals of the involvement of acid-base transporters in stria vascularis. RESULTS: We directly measured acid flux in vitro from the apical side of isolated stria vascularis from adult C57Bl/6 mice with a novel constant-perfusion pH-selective self-referencing probe. Acid efflux that depended on metabolism and ion transport was observed from the apical side of stria vascularis. The acid flux was decreased to about 40 % of control by removal of the metabolic substrate (glucose-free) and by inhibition of the sodium pump (ouabain). The flux was also decreased a) by inhibition of Na,H-exchangers by amiloride, dimethylamiloride (DMA), S3226 and Hoe694, b) by inhibition of Na,2Cl,K-cotransporter (NKCC1) by bumetanide, and c) by the likely inhibition of HCO3/anion exchange by DIDS. By contrast, the acid flux was increased by inhibition of gastric H,K-ATPase (SCH28080) but was not affected by an inhibitor of vH-ATPase (bafilomycin).  K flux from stria vascularis was reduced less than 5 % by SCH28080. CONCLUSIONS: These observations suggest that stria vascularis may be an important site of control of cochlear acid-base balance and demonstrate a functional role of several acid-base transporters in stria vascularis, including basolateral H,K-ATPase and apical Na,H-exchange. Previous suggestions that H secretion is mediated by an apical vH-ATPase and that basolateral H,K-ATPase contributes importantly to K secretion in stria vascularis are not supported. These results advance our understanding of inner ear acid-base balance and provide a stronger basis to interpret the etiology of genetic and pharmacologic cochlear dysfunctions that are influenced by endolymphatic pH.

Relationship Between Clinical Test Results and Morphologic Severity Demonstrated by Sitting 3-D CT in Patients With Patulous Eustachian Tube.

OBJECTIVE: To investigate the correlation of sitting 3-D computed tomography (CT) scans of the Eustachian tube (ET) with subjective and objective findings in patients with patulous Eustachian tube (PET). STUDY DESIGN: Retrospective. SETTING: Tertiary referral center. SUBJECTS: A retrospective survey of medical records in Sen-En Hospital identified 40 patients and 62 ears with PET between September 2014 and June 2015. METHOD: Diagnosis of PET was based on the presence of three characteristic aural symptoms (autophony of voice or breathing sounds, and aural fullness), as well as verification of synchronous movement of the tympanic membrane in response to forced breathing under an endoscope. Any pressure changes in the external auditory canal (EAC) elicited by deep breathing and sniffing were detected by tubotympanoaerodynamography (TTAG). In addition, sonotubometry was performed where two parameters were used determined to evaluate ET function. Patients were examined by 3-D CT (Accuitomo; Morita, Kyoto, Japan) in the sitting position. The length of the closed ET lumen section was measured. Ears were divided into three groups as follows: completely open, closed-short (3 mm or less), and closed-long (longer than 3 mm). RESULTS: The median length of the closed section of the ET lumen was 1.85 ±â€Š2.69 mm in positive findings of PET. The three groups were significantly different in both aural fullness (p = 0.023) and, similarly, the difference in tympanic membrane movement (p = 0.032) among these three groups was also significantly different (p = 0.032). However, for autophony of breathing sounds, there was no significant difference with regard to autophony of breathing sounds among these three groups (p = 0.324). Although TTAG findings were did not reveal any significantly difference among these three groups (p = 0.589), the difference was significant (p = 0.001) in degree of EAC pressure change in TTAG. The difference among the three groups was significant (p = 0.001) based on sonotubometry findings. CONCLUSION: Under resting conditions, the lengths of the closed area of the ETs in PET groups are clearly shorter than in groups without PET based on sitting position CT scans in resting condition. Among the symptoms and clinical test findings including the ET function test results, the presence of tympanic membrane movement induced by respiration, the high degree of EAC pressure change in TTAG, as well as the positive results of sonotubometry are significantly correlated with the positive findings of sitting CT revealing the open ET.

Hammer sound elicited tinnitus in car body repair worker cured by stapedial tenotomy - A case report.

Abnormal auditory sensations or tinnitus caused by abnormal middle ear muscle contraction are extremely rare and uncomfortable for patients. A 67-year-old man who performed paint and body work for cars presented at our hospital with complaint of an audible and annoying abnormal sound that was synchronous with the striking of his hammer against the metal of the car body during his work. The patient reported that the sound was audible of left ear with a split-second delay after his hammer struck the metal. Preoperative subjective and objective testing failed to reveal any abnormal findings in our case. The patient's symptom was successfully cured by selective transection of the stapedius tendon. The characteristic nature of tinnitus with a split-second delay after striking the metal helped our diagnosis and method of intervention in this case.

Factors Affecting the Variation of Maximum Speech Intelligibility in Patients With Sensorineural Hearing Loss Other Than Apparent Retrocochlear Lesions.

OBJECTIVES: To examine the relationship between speech intelligibilities among the similar level of hearing loss and threshold elevation of the auditory brainstem response (ABR). METHODS: The relationship between maximum speech intelligibilities among similar levels of hearing loss and relative threshold elevation of the click-evoked ABR (ABR threshold - pure tone average at 2,000 and 4,000 Hz) was retrospectively reviewed in patients with sensorineural hearing loss (SNHL) other than apparent retrocochlear lesions as auditory neuropathy, vestibular schwannoma and the other brain lesions. RESULTS: Comparison of the speech intelligibilities in subjects with similar levels of hearing loss found that the variation in maximum speech intelligibility was significantly correlated with the threshold elevation of the ABR. CONCLUSION: The present results appear to support the idea that variation in maximum speech intelligibility in patients with similar levels of SNHL may be related to the different degree of dysfunctions of the inner hair cells and/or cochlear nerves in addition to those of outer hair cells.

Audiological evidence of therapeutic effect of steroid treatment in neuromyelitis optica with hearing loss.

Neuromyelitis optica (NMO) is an autoimmune astrocytopathy caused by anti-aquaporin 4 antibody. Only two patients with NMO have been reported presenting with hearing disorders to our knowledge. We recently treated a 40-year-old woman with NMO complaining of right hearing loss. Audiometry showed minimal asymmetry, but the auditory brainstem responses (ABR) were severely attenuated on the right. The attenuated ABR and her aural symptoms (hearing loss and fullness) improved after steroid treatment. The present case shows that the retrocochlear-type hearing loss may be associated with NMO.

Effects of contralateral noise on the 20-Hz auditory steady state response--magnetoencephalography study.

The auditory steady state response (ASSR) is an oscillatory brain response, which is phase locked to the rhythm of an auditory stimulus. ASSRs have been recorded in response to a wide frequency range of modulation and/or repetition, but the physiological features of the ASSRs are somewhat different depending on the modulation frequency. Recently, the 20-Hz ASSR has been emphasized in clinical examinations, especially in the area of psychiatry. However, little is known about the physiological properties of the 20-Hz ASSR, compared to those of the 40-Hz and 80-Hz ASSRs. The effects of contralateral noise on the ASSR are known to depend on the modulation frequency to evoke ASSR. However, the effects of contralateral noise on the 20-Hz ASSR are not known. Here we assessed the effects of contralateral white noise at a level of 70 dB SPL on the 20-Hz and 40-Hz ASSRs using a helmet-shaped magnetoencephalography system in 9 healthy volunteers (8 males and 1 female, mean age 31.2 years). The ASSRs were elicited by monaural 1000-Hz 5-s tone bursts amplitude-modulated at 20 and 39 Hz and presented at 80 dB SPL. Contralateral noise caused significant suppression of both the 20-Hz and 40-Hz ASSRs, although suppression was significantly smaller for the 20-Hz ASSRs than the 40-Hz ASSRs. Moreover, the greatest suppression of both 20-Hz and 40-Hz ASSRs occurred in the right hemisphere when stimuli were presented to the right ear with contralateral noise. The present study newly showed that 20-Hz ASSRs are suppressed by contralateral noise, which may be important both for characterization of the 20-Hz ASSR and for interpretation in clinical situations. Physicians must be aware that the 20-Hz ASSR is significantly suppressed by sound (e.g. masking noise or binaural stimulation) applied to the contralateral ear.

Severe acute otitis media caused by mucoid Streptococcus pyogenes in a previously healthy adult.

Streptococcus (S.) pyogenes is well recognized as the most common pathogen causing pharyngotonsillitis in school-age children. In Japan, mucoid Streptococcus pneumoniae is well known as a causative agent of severe acute otitis media (AOM); however, mucoid S. pyogenes has rarely been reported. To the best of our knowledge, this is the first report of an AOM patient caused by mucoid S. pyogenes in Japan. A 36-year-old previously healthy female was referred to our hospital with suspicion of cerebrospinal otorrhea due to increasing otalgia accompanied by headache following myringotomy. Bacterial cultures of middle ear secretions were performed, and mucoid-form colonies surrounded by zones of complete ß-hemolysis were produced on sheep's blood agar. Antigen-agglutination test results were positive for S. pyogenes, and thus the patient received treatment with panipenem-betamipron 2.0 g/day for 10 days, which resolved nearly all symptoms. The bacteriological features of this strain were then investigated. The M-protein genotype encoded by the emm gene, the major virulence factor of S. pyogenes, was determined to be emm75. Generally, S. pyogenes forms colonies having non-mucoid matt appearances based on ß-hemolysis of sheep's blood agar. The mucoid phenotype results from abundant production of hyaluronic acid capsular polysaccharide, a key virulence determinant. emm75 is common in noninvasive, but less common in invasive disease. In conclusion, mucoid S. pyogenes can cause severe infection even in previously healthy persons. Emergence of mucoid S. pyogenes and drug resistance trends should be monitored in the future.