Language：Japanese   Publisher：Japanese Society of Otorhinolaryngology-Head and Neck Surgery  

<p>　　Acute epiglottis can cause sudden upper airway obstruction and death, so that prompt and accurate diagnosis and timely treatment are essential. In previous years, tracheostomy was used to secure the airway in most cases, however, in recent years, endotracheal intubation using an endoscope (endoscopic intubation) has been increasingly adopted. Endoscopic intubation may be effective for securing the airway even in cases with relatively severe laryngeal edema.</p><p>　　We retrospectively reviewed the data of 73 patients diagnosed as having acute epiglottis at our hospital. The subjects were 48 men and 25 women aged 21 to 90 years. The patients were classified according to the endoscopic severity score of Katori et al., as grade 1 (slight swelling of the epiglottis, n=36), grade 2 (moderate swelling of the epiglottis, n=23), or grade 3 (severe swelling of the epiglottis, n=14). The endoscopic severity score of Tanaka et al. was rated as 1 (n=33), 2 (n=9), 3 (n=17), 4 (n=8), or 5 (n=6). Airway intervention was necessary in 13 of the 73 cases, including tracheostomy in 6 cases and endoscopic intubation in 7 cases. Not only tracheostomy, but also endoscopic intubation was confirmed to be useful for securing the airway, even in cases with relatively severe laryngeal edema in which intubation was predicted to be difficult. We believe that treatment of acute epiglottitis by endoscopic intubation requires close co-operation among otolaryngologists, anesthesiologists, and intensive care physicians. In all the 12 cases with grade 3B acute epiglottitis according to Katori's classification, worsening of the laryngeal edema and progression of airway stenosis were observed immediately before the airway intervention, but no significant decrease of the percutaneous arterial oxygen saturation (Sp0₂) or cyanosis was observed in any of the cases. In 7 of 12 cases, even aggravation of the subjective symptoms was not observed. In view of the high frequency of cases in which neither decrease of the percutaneous arterial oxygen saturation nor aggravation of subjective symptoms is observed until just before suffocation, it is considered safe to secure the airway as soon as possible in cases with grade 3B acute epiglottitis. The indications for airway intervention to secure were (1) orthopnea, (2) stridor, (3) dyspnea within 24 hours after the onset of symptoms, (4) severe swelling of the epiglottis and arytenoids (grade 3B according to Katori's classification, and/or a score of 4 or higher according to Tanaka's classification), and (5) grade 2 or greater swelling of the epiglottis according to Katori's classification associated with marked abscess formation. Any one of (1) to (5) is considered as an indication for airway intervention to secure the airway as early as possible.</p>

DOI： 10.3950/jibiinkotokeibu.127.2_100

CiNii Research

Language：English   Publisher：Laryngoscope Investigative Otolaryngology  

Objective: The pathophysiology and symptoms underlying Meniere's disease (MD) manifest as endolymphatic hydrops (EH), potentially impacting acoustic power absorbance in vestibular EH. The longitudinal effects of middle ear pressure therapy (MEPT) and conservative therapies for EH by magnetic resonance imaging (MRI) and on acoustic power absorbance on wideband acoustic immittance (WAI) were evaluated, and their changes were compared with clinical symptoms. Methods: Eleven patients with definite MD or delayed endolymphatic hydrops (DEH), resistant to conservative therapies and who continued MEPT for 1 year, were included. Vertigo scores, hearing levels, acoustic power absorbance on WAI, and degrees of EH on 3-T MRI were evaluated and compared before and after the treatments. Results: One year after the start of MEPT, all cases showed symptomatic improvement in vertigo score; however, the degrees of EH showed no improvements except in one case. In the affected ears with EH, their absorbances on WAI improved, particularly at 1580–1905 or 2400–2953 Hz (p <.05). Conclusion: Alleviation of vestibular symptoms with the therapy of MD was not necessarily associated with improved EH. Vestibular symptoms could be related to the change in the impedance of inner ear pressure, which was proven by the normalization of acoustic power absorbance. Assessments of acoustic power absorbance may provide useful information for physiological conditions and causative factors of vertigo in ears with EH. Level of evidence: 4.

DOI： 10.1002/lio2.1210

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Language：English   Publisher：Acta Oto-Laryngologica  

Background: Posterior expansion of the utricular endolymphatic space (ES) is a finding occasionally observed, and often occurs in bilateral ears. The clinical significance of posterior expansion of the utricular ES is not clear. Objectives: To investigate the clinical significance of posterior expansion of the utricular ES detected on magnetic resonance imaging (MRI). Materials and Methods: Among 957 patients who underwent contrast-enhanced MRI to investigate the presence of endolymphatic hydrops (EH), clinical findings in cases with posterior expansion of the utricular ES were evaluated and the significance of their existence is considered. Results: Posterior expansion of the utricular ES was detected in 30 ears from 20 cases (7 males, 13 females; mean age 48.7 years). The ears with posterior expansion had a significantly lower incidence of vestibular EH than those without, and this tendency was more prominent in cases in bilateral ears. Some cases with posterior expansion had vestibular symptoms and were diagnosed with Ménière’s disease, although EH was not detected in their vestibules. Conclusion and Significance: Posterior expansion of the utricular ES was associated with Ménière’s disease without vestibular EH. Posterior expansion of the utricular ES might not be associated with the formation of EH but may occur independently.

DOI： 10.1080/00016489.2024.2329783

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Language：Japanese   Publisher：The Society of Practical Otolaryngology  

<p>Syphilis is a systemic bacterial infection caused by <i>Treponema pallidum</i> that is exclusively transmitted through direct contact with contagious mucocutaneous lesions, with the exception of vertical transmission. The laryngeal mucosa serves as a vital portal of entry and manifests various signs and symptoms as the disease progresses through chronic stages.</p><p>A 26-year-old female was referred to our hospital with progressively enlarging neck swelling and sore throat that had persisted for a month. Palpable and painful neck masses were observable at levels II, III, and IV, and contrast-enhanced CT imaging confirmed pronounced bilateral cervical lymphadenopathy. Laryngoscopy revealed bulky lingual tonsils and an epiglottis covered in pus, as well as edematous changes in the arytenoids. We diagnosed laryngeal syphilis due to positive results from syphilis blood tests and her occupation as a commercial sex worker for several years. Laboratory tests for other possible venereal infections were all negative, and no genital lesions were found during the gynecological examination. We initiated treatment with intravenous ampicillin/sulbactam and adjuvant steroids. However, on the third day of admission, her respiratory status deteriorated due to airway obstruction. An emergency tracheostomy was required to alleviate the obstruction caused by supraglottic edema. She continued to receive ceftriaxone plus clindamycin and gradually recovered, ultimately being decannulated on the 20th day of admission.</p><p>Impaired neck lymph drainage, elicited by the swelling of pharyngeal lesions along with bilateral cervical lymphadenopathy, may have been the underlying etiology for the acute supraglottic edema in this case. Clinicians should be aware of the atypical presentation of early syphilis characterized by prominent swelling of neck lymph nodes and the absence of typical mucosal lesions.</p>

DOI： 10.5631/jibirin.117.523

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Language：Japanese   Publisher：The Society of Practical Otolaryngology  

<p>A 70-year-old female patient complaining of persistent pain in the left temporal region was referred to our university hospital by her physician. Audiometry and examination of the tympanic membrane yielded no unremarkable findings. Further diagnostic evaluation by computed tomography of the temporal bone revealed an infiltrative opacity and irregularity in the temporal bone, and on magnetic resonance imaging, both T1- and T2-weighted images revealed low signal intensity signals in the same area. Based on the presence of an infiltrative opacity in the left middle ear, elevated conductive thresholds in the temporal bone, and left facial nerve paresis, we scheduled a biopsy of the temporal bone lesion. A preoperative workup uncovered evidence of a primary lung neoplasm located in the middle lobe of the left lung. Histopathological examination confirmed the primary lung cancer, and consistent histological features in the temporal bone lesions. While the temporal bone is a relatively uncommon site of metastasis from malignant tumors, the most common primary sites are the lung, breast, and prostate. It is therefore recommended to conduct a systematic investigation of temporal bone lesions, including a search for primary tumors, given the possibility of malignant tumor metastasis in patients presenting with temporal bone lesions.</p>

DOI： 10.5631/jibirin.117.469

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CiNii Research

Language：English   Publisher：Otology and Neurotology  

DOI： 10.1097/MAO.0000000000003981

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Language：English  

CiNii Research

Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.66.318

CiNii Research

Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.66.404

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Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.66.446

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Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.66.325

CiNii Research

Language：English   Publisher：Otology and Neurotology  

Objective To evaluate the listening conditions of bilateral cochlear implant (CI) users in their daily living environment. Study Design Retrospective study. Setting University hospital. Patients Thirty-one adult CI users 16 years or older; 18 underwent sequential surgery, and 13 underwent simultaneous surgery. Inclusion criteria included the availability of CI data logging features and a minimum duration of binaural use of 6 months. Intervention Retrospective analysis of data obtained from the automatic scene classifier data logging system. Main Outcome Measure Comparison of data logging and maximum speech discrimination scores of the two surgery groups (sequential vs. simultaneous) to investigate the potential influence of these factors on the listening conditions of CI users. Results The maximum speech discrimination score of the second CI in the sequential group was significantly worse than that of any other CI in the sequential and simultaneous CI groups. Additionally, the longer the interval between surgeries, the more significant the difference in "time on air"between the first and the second CIs. The second CI in sequential CI surgery had a shorter "time on air"than the first or the bilateral simultaneous CIs; the second CI was also used more frequently in noisy and speech with background noise environments. Conclusions A second CI may be more frequently used in challenging listening environments because of its binaural auditory effect, despite its lower speech discrimination performance. The timing of sequential implantation and the potential impact of binaural hearing should be considered when developing rehabilitation strategies for individuals with bilateral CIs.

DOI： 10.1097/MAO.0000000000003955

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Language：English  

Objective: To evaluate the listening conditions of bilateral cochlear implant (CI) users in their daily living environment. Study Design: Retrospective study. Setting: University hospital. Patients: Thirty-one adult CI users 16 years or older; 18 underwent sequential surgery, and 13 underwent simultaneous surgery. Inclusion criteria included the availability of CI data logging features and a minimum duration of binaural use of 6 months. Intervention: Retrospective analysis of data obtained from the automatic scene classifier data logging system. Main Outcome Measure: Comparison of data logging and maximum speech discrimination scores of the two surgery groups (sequential vs. simultaneous) to investigate the potential influence of these factors on the listening conditions of CI users. Results: The maximum speech discrimination score of the second CI in the sequential group was significantly worse than that of any other CI in the sequential and simultaneous CI groups. Additionally, the longer the interval between surgeries, the more significant the difference in “time on air” between the first and the second CIs. The second CI in sequential CI surgery had a shorter “time on air” than the first or the bilateral simultaneous CIs; the second CI was also used more frequently in noisy and speech with background noise environments. Conclusions: A second CI may be more frequently used in challenging listening environments because of its binaural auditory effect, despite its lower speech discrimination performance. The timing of sequential implantation and the potential impact of binaural hearing should be considered when developing rehabilitation strategies for individuals with bilateral CIs.

CiNii Research

Language：English   Publisher：Audiology Research  

Clinical findings on cartilage conduction hearing aids (CCHAs) have gradually become clear; however, few reports include a large number of cases. This study included 91 ears from 69 patients who underwent CCHA fitting in our hospital. Their ears were divided into six groups (i.e., bilateral aural atresia or severe canal stenosis, unilateral aural atresia or severe canal stenosis, chronic otitis media or chronic otitis externa with otorrhea, sensorineural hearing loss, mixed hearing loss, and conductive hearing loss) according to their clinical diagnosis and type of hearing loss. Most clinical diagnoses were aural atresia or meatal stenosis (bilateral, 21.8%; unilateral, 39.6%). The purchase rate of CCHAs was higher in the closed-ear group (bilateral, 77.3%; unilateral, 62.5%). In the bilateral closed-ear group, air conduction thresholds at 1000, 2000, and 4000 Hz and aided thresholds with CCHAs at 4000 Hz were significantly lower in the purchase group than the non-purchase group. No significant difference was observed between the purchase and non-purchase groups in the unilateral closed-ear group. In the bilateral closed-ear group, air conduction thresholds and aided thresholds were associated with the purchase rate of CCHAs. In the unilateral closed-ear group, factors other than hearing might have affected the purchase rate of CCHAs.

DOI： 10.3390/audiolres13040049

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Language：English   Publisher：Nagoya University Graduate School of Medicine, School of Medicine  

The potential mechanism of augmented response on cervical vestibular evoked myogenic potential (cVEMP) testing and its decrease following treatment in a patient with Meniere’s disease (MD) are discussed. Changes of static posturography and cVEMP testing before and after a glycerol drip in a 69-year-old man with unilateral MD, in which significant endolymphatic hydrops (EH) was confirmed on magnetic resonance imaging (MRI) on the diseased side, were evaluated. Values of total locus lengths, areas of postural sway, and their Romberg ratios were decreased after the glycerol drip. On cVEMP testing, the diseased ear demonstrated a 375% larger amplitude than the contralateral ear before treatment, but both ears showed almost the same responses after treatment. An augmented response on cVEMP testing and a decrease following treatment for MD reflect the diversity of clinical findings in MD. Responses on cVEMP testing may relate not only to the degree of EH, but also be due to abnormal acoustic energy absorbance transmitted into the saccule.

DOI： 10.18999/nagjms.85.2.375

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DOI： 10.1016/j.anl.2022.01.0040385-8146

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Background: Listening difficulties (LiD) present difficulties in listening and paying attention to spoken information despite normal pure tone audiometry. Endolymphatic hydrops (EH) is a common inner ear condition associated with Ménière’s disease but may also be present in the asymptomatic ear. Objectives: Using magnetic resonance imaging, we investigated EH in patients with LiD and assessed whether the severity of EH was related to the results of auditory processing tests (APTs). Materials and Methods: 111 patients with no abnormalities on pure tone audiometry, but displaying difficulties in listening, underwent evaluation through APTs and questionnaires. Upon obtaining informed consent, the inner ears of 20 consenting patients were evaluated utilizing a 3-Tesla magnetic resonance imaging. Results: A higher percentage of patients diagnosed with LiD by APTs had significant EH in the cochlea and vestibule than in previously reported control cases. The percentage of correct answers in the speech-in-noise test was significantly lower in patients with than in those without significant EH. Conclusion and significance: In this study, significant EH of the cochlea was associated with poor listening to noise. The presence of EH affects the functioning of the auditory processing system, even in ears that test normally on standard audiometric tests.

CiNii Research

Language：English   Publisher：Acta Oto-Laryngologica  

Background: Listening difficulties (LiD) present difficulties in listening and paying attention to spoken information despite normal pure tone audiometry. Endolymphatic hydrops (EH) is a common inner ear condition associated with Ménière’s disease but may also be present in the asymptomatic ear. Objectives: Using magnetic resonance imaging, we investigated EH in patients with LiD and assessed whether the severity of EH was related to the results of auditory processing tests (APTs). Materials and Methods: 111 patients with no abnormalities on pure tone audiometry, but displaying difficulties in listening, underwent evaluation through APTs and questionnaires. Upon obtaining informed consent, the inner ears of 20 consenting patients were evaluated utilizing a 3-Tesla magnetic resonance imaging. Results: A higher percentage of patients diagnosed with LiD by APTs had significant EH in the cochlea and vestibule than in previously reported control cases. The percentage of correct answers in the speech-in-noise test was significantly lower in patients with than in those without significant EH. Conclusion and significance: In this study, significant EH of the cochlea was associated with poor listening to noise. The presence of EH affects the functioning of the auditory processing system, even in ears that test normally on standard audiometric tests.

DOI： 10.1080/00016489.2023.2182450

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Language：English   Publisher：Laryngoscope Investigative Otolaryngology  

Objective: The increased endolymph volume affects a shift in the organ of Corti and basilar membrane in ears with endolymphatic hydrops (EH), which might affect distortion-product otoacoustic emissions (DPOAE) by altering the operating point of the outer hair cells. We investigated how changes in DPOAE are related to the distribution site of EH. Study Design: Prospective study. Methods: Among 403 patients with hearing or vestibular symptoms who underwent contrast-enhanced magnetic resonance imaging (MRI) for the diagnosis of EH and subsequent DPOAE testing, subjects whose hearing levels on pure tone audiometry were ≤35 dB at all frequencies were included in this study. In patients with EH on MRI, the presence and amplitude of DPOAE were evaluated between groups with hearing levels of ≤25 dB at all frequencies versus hearing levels of >25 dB at one or more frequencies. Results: There were no differences in the distribution of EH between groups. The amplitude of DPOAE had no clear correlation with the presence of EH. However, in both groups, there was a significantly higher probability of the presence of a DPOAE response from 1001 to 6006 Hz in cases with EH in the cochlea. Conclusion: Among patients whose hearing levels were ≤35 dB at all frequencies, better responses on DPOAE testing were found in subjects with EH in the cochlea. Alteration of DPOAEs in the early stages of hearing impairment could indicate morphological changes in the inner ear with altered basilar membrane compliance due to EH. Level of Evidence: 4.

DOI： 10.1002/lio2.998

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Language：English   Publisher：Frontiers in Neurology  

Objective: To summarize the pathophysiological analysis of idiopathic sudden sensorineural hearing loss (ISSNHL) by magnetic resonance imaging (MRI), focusing on the findings of high signal or endolymphatic hydrops (EH) in the inner ear. Methods: We summarize the published studies of our research group regarding the pathophysiological analysis of ISSNHL on MRI and review related clinical articles that have reported significantly high signal or the existence of EH in ears with ISSNHL. Results: Pre-contrast high signal on MRI may indicate minor hemorrhage or increased permeability of surrounding vessels to the perilymph, whereas post-contrast high signal indicates breakdown of the blood–labyrinth barrier, in which irreversible changes would lead to poor prognosis. In some cases of ISSNHL, primary EH could be pre-existing and may be a risk factor for the onset of ISSNHL. Conclusion: Analysis of ISSNHL by cutting-edge MRI evaluation could provide useful information for elucidating its pathophysiology and for predicting prognosis in this disease.

DOI： 10.3389/fneur.2023.1193104

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Language：Japanese   Publisher：The Society of Practical Otolaryngology  

<p>We report a case of bilateral cochlear implant surgery performed for profound sensorineural hearing loss in a 2-year-old girl with Wolfram syndrome and osteogenesis imperfecta type I. Wolfram syndrome is a rare autosomal-recessive genetic disorder characterized by juvenile diabetes mellitus and visual impairment. The hearing loss in Wolfram syndrome is typically a slowly progressive sensorineural hearing loss, primarily in the high-frequency range. Genetic testing of our patient showed mutations in <i>WFS1</i> and <i>COL1A1</i>. Bilateral cochlear implant surgery was performed, because adequate wearing thresholds could not be achieved with hearing aids. This report is the first case of a patient with Wolfram syndrome with profound congenital hearing loss who benefited from cochlear implant surgery. The patient had a visual-auditory dual disability and lacked external stimuli. The cochlear implant provided valuable auditory compensation to ensure auditory stimulation. In the future, it is necessary to continue comprehensive rehabilitation for the patient with collaboration among the parents, speech-language pathologists, and rehabilitation facilities.</p>

DOI： 10.5631/JIBIRIN.116.415

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Language：English   Publisher：Nagoya University Graduate School of Medicine, School of Medicine  

Primary laryngeal cryptococcosis is an extremely rare infection and presents with non-specific symptoms such as hoarseness or sore throat, resulting in delayed diagnosis. Here, we report the patient of a 56-year-old female patient with primary laryngeal cryptococcosis, who was being treated with oral and inhaled steroids for rheumatoid arthritis and bronchial asthma. The patient suffered from prolonged hoarseness and sore throat, and endoscopic biopsy was performed several times under local anesthesia, demonstrating only inflammatory cell infiltration. Considering the possibility of laryngeal malignancy, a third biopsy was performed by endoscopic laryngomicrosurgery under general anesthesia. Intraoperative frozen section revealed non-neoplastic laryngeal mucosa with erosion and severe inflammatory cell infiltration. However, we could not confirm the definite diagnosis of the lesion in the intraoperative consultation. Postoperative histopathological examination revealed a small number of yeast-type fungi and a definitive diagnosis was established by special stains including Alcian blue stain. Finally, the patient was diagnosed as primary laryngeal cryptococcosis. Daily oral administration of fluconazole (400 mg/day) was performed for 6 months according to the treatment protocol for pulmonary cryptococcosis. The symptoms gradually improved, and endoscopy revealed no recurrence 6 months post-treatment. Clinicians should consider the possibility of laryngeal cryptococcosis when severe inflammation is found in the larynx and discuss the disease history and pathological results with pathologists more closely.

DOI： 10.18999/nagjms.84.4.900

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Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.65.417

CiNii Research

Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.65.428

CiNii Research

Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.65.431

CiNii Research

Language：English   Publisher：Nagoya University Graduate School of Medicine, School of Medicine  

We summarize the presence of endolymphatic hydrops (EH) in otological disorders evaluated with magnetic resonance imaging (MRI) of temporal bones, and propose a classification of EH based on its pathological significance. A search of the literature published in English-language journals was performed using electronic databases, especially focusing on EH-related otological disorders. Clinical articles that contained the terms EH and contrast-MRI published from 2007 to the present, with relevant human temporal bone studies, were included. The following three main points are discussed based on the results in the relevant articles: i) otological disorders that present EH, ii) current grading for evaluation of EH on MRI, and iii) a proposed classification of EH based on its pathological significance. MRI evaluation revealed that EH exists extensively not only in ears with typical Meniere’s disease (MD), but also in those with various other otological disorders. The etiological classification of EH helps to summarize ideas for determining the pathophysiology of otological disorders, while a therapeutic classification provides clues to their management. MRI evaluations of EH have led to breakthroughs in investigations of EH in otological disorders. Precise grading for evaluation and clarification of EH on MRI based on its pathological significance could provide keys to elucidating the pathophysiology of EH-related otological disorders.

DOI： 10.18999/nagjms.84.3.497

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Language：Japanese   Publisher：東京 : へるす出版  

CiNii Research

Language：English   Publisher：Otology and Neurotology  

DOI： 10.1097/MAO.0000000000003328

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Language：Japanese   Publisher：Japan Otological Society  

<p>The Slim Modiolar electrode (CI532/632), a pre-curved electrode for cochlear implants released by Cochlear^®, can effectively stimulate the nerves in its proximity and reduce the risk of cochlear injury and the likelihood of electrode translocation from the scala tympani to scala vestibuli in comparison with the Slim Straight electrode (CI522/622). In this study, 31 ears underwent implantation of the CI532/632 electrode. The neural response telemetry (NRT) thresholds and impedances for CI532/632 electrodes around the cochlear cupula were lower than those for CI522. No difference was observed in the operative time and the short-term postoperative hearing and clinical performance of the two electrodes. Due to the complexity of the sheath and electrode configuration, insertion of the electrode can be challenging, and tip folding can occur. A broad extension of posterior tympanotomy is required while inserting the CI532/632 electrode. The round-window niche overhang (the ridge of bone around the round window fossa) has to be drilled out following exposure of the round window fossa. If electrode insertion via the round window is difficult, an extended round-window approach should be employed inserted. The NRT threshold and impedance of the Slim Modiolar electrodes were better in the short term. However, Slim Modiolar electrodes showed no significant difference from Slim Straight electrodes in terms of cochlear implant performance after six months. Thus, Slim Modiolar electrodes are options for cases with a normal cochlear study, and long-term evaluations of cochlear implant performance with these electrodes are essential.</p>

DOI： 10.11289/otoljpn.32.136

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Language：English   Publisher：Frontiers in Neurology  

We evaluated the inner ear distribution of 17O-labeled saline administered to the human tympanic cavity. Magnetic resonance imaging was performed after intratympanic administration in five healthy volunteers and one patient with cochlear endolymphatic hydrops. In all volunteers, 17O-labeled water permeated the cochlear basal turn and vestibule at 30 min and disappeared gradually within 2–4 h. All participants experienced positional vertigo lasting a few hours to a few days. Visualization of 17O-labeled water distribution in the endolymphatic space of the posterior ampulla showed indistinct separation of endolymph and perilymph in the cochlea and most of the vestibule in all participants. Intralabyrinthine distribution of 17O-labeled water differed from that in previous reports of intratympanically administered gadolinium-based contrast agent. 17O-labeled water in the endolymphatic space may cause heavier endolymph and positional vertigo. These results of this study may add new insights for investigating the distribution and the effects of molecules in the inner ear after the intratympanic administration in living humans.

DOI： 10.3389/fneur.2022.1016577

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Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.64.391

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Language：English  

Background: Otological diseases including Meniere’s disease (MD) involve endolymphatic hydrops (EH), which can be visualized by magnetic resonance imaging (MRI) with gadolinium contrast agents, but the temporal changes of contrast in the inner ear have not been evaluated. Objectives: We investigated the permeability of the blood–perilymph barrier (BPB) in ears with EH to evaluate the severity of the inner ear disturbances. Materials and methods: The study included 32 ears from 16 patients with EH or related diseases who underwent MRI. The permeability of the BPB was assessed by the signal–intensity ratio (SIR) at four-time points: before and at 10 min, 4 h, and 24 h after administration of gadolinium for assessing EH. Results: Cochlear EH was found in 25 of the 32 ears, and vestibular EH in 11. The rate of EH was significantly higher in symptomatic ears; however, the existence of EH was not related to SIR values. Nevertheless, SIR values in the basal turn were significantly higher 4 and 24 h after injection of gadolinium in patients aged ≥50 years. Conclusion and significance: Higher SIR values observed in older patients with EH indicate severe disturbances of the BPB in the cochlea, which may account for intractable inner ear disturbances in older patients.

DOI： 10.1080/00016489.2021.1957500

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Language：English  

Objectives: To investigate the clinical features of ears with dilatation of the endolymphatic space in the ampulla of the posterior semicircular canal on magnetic resonance imaging. Study Design: Retrospective study. Setting: A university hospital. Methods: This study included 1,842 ears from 934 patients who underwent 3-T magnetic resonance imaging with gadolinium to investigate the presence of endolymphatic hydrops. Age, sex distribution, hearing thresholds on pure-tone audiometry, and vestibular symptoms were compared between cases of unilateral and bilateral dilatation of the endolymphatic space in the ampulla of the posterior semicircular canal. Results: Forty-eight ears (17 men and 14 women; mean age 49.9 yrs) showed dilatation of the endolymphatic space in the ampulla of the posterior semicircular canal. Age and the rate of chronic sensorineural hearing loss were significantly higher in the unilateral group (14 ears) than in the bilateral group (34 ears). The average hearing thresholds and rates of vestibular symptoms reported did not differ between unilateral and bilateral cases, but some patients showed positional nystagmus. Conclusions: Dilatation of the endolymphatic space in the ampulla was observed selectively in the posterior semicircular canal, though its pathogenesis was not clear. Such dilatation is not usually accompanied by vestibular endolymphatic hydrops, and it may be a cause of vertigo and dizziness.

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Language：English  

Two patients with auditory neuropathy spectrum disorder (ANSD) considered to be associated with Charcot-Marie-Tooth (CMT) are reported. In case 1, a 23-year-old man presented with progressive bilateral sensorineural hearing loss (SNHL) from 10 years of age and was diagnosed with ANSD. He was later diagnosed with CMT by neurological testing. In case 2, a 16-year-old girl, the younger sister of the patient in case 1, presented with progressive SNHL with similar auditory findings since 6 years of age as those of her brother. Both cases underwent bilateral cochlear implantation. In case 1, the maximum discrimination score improved to 45% 24 months after the first side of cochlear implantation from 5% before the surgery. In case 2, the score was 5% 10 months after cochlear implant (CI) surgery from 0% before the surgery. CI treatment for CMT patients has been considered difficult because of both failure in synchronization of nerve conduction due to demyelination and axonal failure of the auditory nerve. Though slower progress compared to the average subset of patients receiving CI was seen, significant improvement was gradually observed in the present patients after bilateral cochlear implantations. CI is thus a viable option for rehabilitation of SNHL in CMT patients.

DOI： 10.1016/j.anl.2020.03.003

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Language：Japanese   Publisher：Acta Oto-Laryngologica  

Background: High signals in the inner ear of idiopathic sudden sensorineural hearing loss (ISSNHL) on magnetic resonance imaging (MRI) have been reported, but no quantitative evaluation has yet been done. Objectives: To evaluate hearing outcomes and cochlear signal intensities on 3-T heavily T2-weighted three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging (hT2W-3D-FLAIR) in patients with ISSNHL. Materials and methods: Twenty-nine patients with ISSNHL were included. Patients underwent hT2W-3D-FLAIR with intravenous gadolinium injection and pure tone audiometry (PTA) at initial visits and 3 months later. Signal intensity ratios (SIRs) were measured in the basal or apical-middle turns of the affected cochlea. A statistical analysis of relationships between SIRs and the average hearing levels (HLs) at low (125, 250, and 500 Hz) and high (2, 4, and 8 kHz) tone frequencies was performed. Results: Hearing improvements at high-tone frequencies in ears with HLs ≥60 dB were significantly worse in those with high SIRs at the basal turns on pre-contrast images. Similarly, hearing improvements at low-tone frequencies in ears with HLs ≥60 dB were significantly worse in those with high SIRs at the apical-middle turns on post-contrast images. Conclusions and significance: High SIRs on hT2W-3D-FLAIR indicate cochlear disturbances with severe ISSNHL and could provide lesion-specific prognostic information.

DOI： 10.1080/00016489.2020.1827159

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PubMed

Language：Japanese   Publisher：Acta Oto-Laryngologica  

Background: High signal intensity in the endolymphatic duct (ED) is occasionally observed on magnetic resonance imaging (MRI) in ears that have otological disorders. Objective: The signal intensity (SI) in the ED on post-contrast MRI was investigated in subjects with various otological disorders, and the meaning of high SI in the ED was evaluated. Material and methods: 392 patients with otological disorders and 21 controls without otological symptoms underwent 3 T MRI. The SIs of the ED and the cerebellum were measured, the SI ratio (SIR) was calculated, and ears with SIR ≥4 were identified. Results: A high SIR was identified in the ED of 3.7% of ears affected by definite Meniere’s disease (dMD), 100% of ears affected by large vestibular aqueduct syndrome (LVAS), and 7.1% of ears with no otological symptoms. On the whole, a significant relationship was found between the existence of vestibular or cochlear EH and the SIR in the ED. Conclusion: The MRI finding of high SI in the ED may indicate the mechanism of inner ear disturbances in ears with otological disorders, especially in those with LVAS, and it may suggest an underlying disorder in some ears in which otological symptoms are not apparent.

DOI： 10.1080/00016489.2020.1781927

Web of Science

Scopus

PubMed

Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.63.367

CiNii Research

Language：Japanese   Publisher：Acta Oto-Laryngologica  

Background: The presence of endolymphatic hydrops (EH) may cause hearing loss and affect the transmission of acoustic energy to the inner ear. Objectives: Acoustic energy absorbance on wideband acoustic immittance (WAI) was evaluated, focusing especially on EH in the vestibule. Material and methods: A total of 32 ears from 16 patients who underwent 3-T magnetic resonance imaging (MRI) to evaluate the presence of EH were examined, retrospectively. The degree of EH in the vestibule was classified into three grades (no, mild, and significant), and pure tone audiometry (PTA) and WAI were measured before and after a glycerol drip. Results: Ears with significant EH showed significantly higher hearing levels and air-bone gaps (ABG), and higher absorbance values on WAI at low frequencies (560–600 Hz) than ears with mild or no EH. Changes in absorbance values were observed in some ears without threshold change on PTA. Conclusions and Significance: This study showed significantly higher absorbance values of acoustic energy with significant vestibular EH at low frequencies. Considering ABGs observed in ears with significant EH, the presence of EH in the vestibule might cause an obstacle to the transmission of acoustic energy to the inner ear.

DOI： 10.1080/00016489.2020.1754460

Web of Science

Scopus

PubMed

Language：Japanese   Publisher：Otology and Neurotology  

Objective: The objective of this study was to investigate the relationship between endolymphatic hydrops (EH) and hearing level or vestibular symptoms by focusing on EH herniation into the semicircular canal (SCC). Study Design: Retrospective study. Setting: University hospital. Methods: The study included 1,548 ears of 775 patients who underwent magnetic resonance imaging (MRI) examination in our university hospital to investigate possible EH. MRI was performed 4 hours after intravenous injection of a standard dose of gadodiamide hydrate and/or 24 hours after intratympanic injection of gadopentetate dimeglumine diluted eightfold. The hearing threshold and vestibular symptoms were compared between ears with unilateral and bilateral EH herniation into the SCC and between ears having vestibular EH adjacent to the stapes footplate with or without EH herniation. Results: Forty-four ears (19 men and 25 women, mean age 53.6 yr) showed EH herniation into the SCC. The average hearing thresholds at 500 to 4000 Hz and presence of vestibular EH adjacent to the stapes footplate were significantly higher in ears with unilateral herniation than in those with bilateral herniation. The average hearing thresholds at 500 and 1000 Hz were significantly higher in the group of ears having adjacency with herniation than in those without herniation. Vestibular symptoms did not differ significantly between groups. Conclusion: Unilateral herniation occurs with EH progression. Bilateral herniation may occur regardless of EH progression and might be influenced by other factors that alter the membranous labyrinth. Key Words: Endolymphatic hydrops—Herniation—Magnetic resonance imaging—Menière’s disease.

DOI： 10.1097/MAO.0000000000002022

Web of Science

Scopus

PubMed

Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.61.494

CiNii Research

Language：Japanese   Publisher：JIBI TO RINSHO KAI  

<p>Thyroplasty type Ⅰ and Ⅲ (TP Ⅰ + Ⅲ) has improved dysphonia with severe vocal fold atrophy in cases of oculopharyngeal muscular dystrophy (OPMD). The patient was a 68-year-old man who had been diagnosed with OPMD 10 years before the operation. He underwent cricopharyngeal myotomy three years after the diagnosis, and his swallowing function improved. Three years later, he became annoyed by his altered voice. Autologous fat injections twice and collagen injections twice to the right vocal cord were performed over the subsequent few years;however, his voice quality was not improved due to the absorption of fat and collagen and the marked progression of vocal fold atrophy. TP Ⅰ + Ⅲ was performed to improve his breathy voice, while an endoscopic examination of swallowing showed that his swallowing function had deteriorated steadily. After the operation, each vocal cord was shifted toward the midline successfully, although the vocal cord closure was not perfect. TP Ⅰ + Ⅲ seems to be an effective phonosurgical technique for the treatment of dysphonia due to severe vocal fold atrophy.</p>

DOI： 10.11334/jibi.64.5_189

CiNii Research

Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.64.489

CiNii Research

Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.63.484

CiNii Research

Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.63.342

CiNii Research

Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.63.430

CiNii Research

Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.62.406

CiNii Research

Language：Japanese   Publisher：一般社団法人 日本聴覚医学会  

DOI： 10.4295/audiology.61.344

CiNii Research