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The most reliable head and neck position for cervical vestibular-evoked myogenic potentials (cVEMPs) measurements yet to be determined.To assess how four body positions used during clinical recordings of cVEMPs affect cVEMP parameters.cVEMPs of 10 healthy subjects (26-50 years old) were recorded in four body positions:Mean background sternocleidomastoid muscle (SCM) electrical activity was significantly higher in positions C and D than in positions A and B. The latencies of p13 and n23 differed significantly among the four positions. Raw p13-n23 complex amplitude was significantly greater in positions C and D than in A and B. These differences were reduced when amplitudes were corrected by SCM activity. For positions A and B, one and two subjects, respectively, had an abnormal raw asymmetry ratio (AR). After correction, all subjects had normal ARs in all positions.Body positions in which the head is elevated produce a quicker and larger cVEMP response compared to positions in which the head is not elevated. The difference in ARs among positions can be ignored as long as the correction is made.

Objective To investigate whether the long-term presence of a patulous Eustachian tube (PET) is associated with sensorineural hearing loss (SNHL). Study design Retrospective chart review. Setting Tertiary referral center. Patients Ears (n = 100) were classified into two groups based on duration of PET symptom(s), i.e., Short (≤3 mo; n = 47 ears) and Long (≥48 mo; n = 53 ears). Contralateral ears without PET (n = 28 ears) were classified as the Contralateral group. Main outcome measures We used ISO 7029 to calculate the hearing thresholds of an age- and sex-matched population at a given frequency. Hearing loss was defined as >25% of these calculated values. Results At 4 kHz, the Long PET group showed a higher prevalence of hearing loss (47%) at 4 kHz than did the Contralateral (21%) and Short PET (19%) groups (p = 0.0280 and 0.0043, respectively). Ears with breathing autophony or a sonotubometric low probe tone level showed a higher prevalence of hearing loss at 4 kHz than those without this symptom or with a high probe tone level (p = 0.0329 or 0.0103, respectively). At low frequencies, ≥89% of the ears in all groups showed mild hearing loss. Conclusion Chronic PET was associated with SNHL at 4 kHz. PET patients showed low-frequency hearing loss regardless of disease duration. Further studies are needed to better understand the pathophysiology of SNHL in patients with PET.

Objective Evaluating the prevalence of specific gene mutations associated with a certain audiometric configuration facilitates clinical assessment of patients with sensorineural hearing loss (SNHL). WFS1 is responsible for autosomal dominant nonsyndromic deafness 6/14/38 and is the most frequent genetic cause of low-frequency SNHL (LFSNHL); however, the exact prevalence of WFS1 mutations in LFSNHL is unknown. Therefore, we evaluated genetic mutations and clinical features in patients with nonsyndromic bilateral LFSNHL, focusing on the WFS1. Study Design Retrospective case series from 2002 to 2013 at the National Hospital Organization Tokyo Medical Center and collaborating hospitals. Methods WFS1, GJB2, and mitochondrial DNA mutation screening was carried out for 74 of 1,007 Japanese probands with bilateral LFSNHL. Results WFS1 and GJB2 mutations were identified in eight of 74 cases (10.8%). Four cases had heterozygous WFS1 mutations; one case had a heterozygous WFS1 mutation and a heterozygous GJB2 mutation; and three cases had biallelic GJB2 mutations. Three cases with WFS1 mutations were sporadic; two of them were confirmed to be caused by a de novo mutation based on the genetic analysis of their parents. In the case with mutations in both WFS1 and GJB2, a de novo mutation of WFS1 was confirmed in the proband's mother by genetic screening of the mother's parents. Conclusion Genetic screening focusing on LFSNHL has not been conducted. The present study first revealed the prevalence of specific gene mutations. WFS1 autosomal dominant mutations were identified even in sporadic cases. Our results also suggested a mutational hotspot in WFS1. Level of Evidence 4. Laryngoscope, 127:E324–E329, 2017

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The purpose of this study was to determine the validity and reliability of a Japanese version of the Khalfa hyperacusis questionnaire (KHQ) and proposed a threshold KHQ score for classifying hyperacusis.In total, 112 patients with hyperacusis (group A) and 103 patients without hyperacusis (group B). The patients in group A were further classified into the following subgroups: subjects with hyperacusis as their chief complaint (n = 26, group A1) and subjects with hyperacusis accompanied by chief complaints of tinnitus and/or hearing loss (n = 86, group A2).The average total questionnaire score for patients in group A was 11.8 ± 9.7, which was statistically significantly higher than that of patients in group B, 5.7 ± 4.8. Cronbach's coefficients for internal consistency were high for the total score (0.92). The average total scores for groups A1 and A2 were 18.1 ± 11.1 and 9.9 ± 8.4, respectively, and the difference between the groups was statistically significant.We developed a Japanese version of the KHQ. It showed high reliability and validity; suggesting its usefulness in clinical practice. We propose that a total KHQ score of 16 is an appropriate cutoff for classifying hyperacusis.

A 36 y/o female presented with the chief complaint of diarrhea and vomiting which had lasted for four days, and with a family history of suicide. The first general examination showed severe dehydration with hyponatremia. After admission, she was diagnosed as having isolated adrenocorticotropic hormone (ACTH) deficiency and mixed connective tissue disease, and the steroid replacement therapy was started with the dose equivalent to 7.5 mg/day of prednisolone (PSL). Three days later, she had right sensorineural hearing loss (SNHL). She was given 40 mg/day PSL in addition to the steroid replacement therapy. On the next day, she developed a persecutory type of paranoid disorder, and then was given psychiatric medication. After tapering off PSL for SNHL, the delusion began to improve with psychiatric medication. Three weeks after the onset of SNHL, her hearing level had partially recovered. Ten months later, she did not show any psychic instability. A family history of psychosis and the present history of malnutrition and connective tissue disease are risk factors of steroid psychosis. It can develop even with 5 mg PSL if the patient has a risk factor. Careful medical history taking and knowledge about the steroid psychosis will prevent the severe side effects associated with steroid treatment.

We investigated whether inflammatory biomarkers and stress are involved in the pathophysiology of idiopathic sensorineural hearing loss (ISHL).Individual cohort study.Two tertiary centers.Forty-three ISHL and 10 non-ISHL patients seen in our ENT departments from 2004 to 2010 within a week from the onset of new symptoms and without steroid administration before visiting our departments.Multiple audiologic evaluations, blood tests including leukocyte counts, natural killer cell activity (NKCA), interleukin 6 (IL-6), tumor necrosis factor, high-sensitivity CRP (hCRP), and the General Health Questionnaire were used to evaluate the systemic stress and inflammatory response.Correlations between biomarkers and ISHL severity and prognosis were evaluated by statistical analysis.In the ISHL patients, a neutrophil count above the reference range was associated with severe hearing loss and poor prognosis, and was accompanied by low NKCA and high IL-6. In the non-ISHL patients, these associations were not present. The abnormal neutrophil count was independent of preexisting vascular diseases. The abnormal counts responded to treatment and decreased into the reference range.Neutrophil counts above the reference range of a facility will be a useful indicator of poor prognosis of ISHL. Synchronism of different types of NF-κB activation pathways could be required to cause severe ISHL. An NKCA decrease, an acute neutrophil count increase, and an IL-6 increase can induce NF-κB activation in the cochlea and cause severe ISHL. Further epidemiologic surveys should be conducted to evaluate whether stressful life events increase the risk of severe ISHL onset.

We used social and emotional approaches to psychologically evaluate hearing disorders, translating the Hearing Handicap Inventory for Adults (HHIA) into Japanese and using it to evaluate Japanese adults with sensorineural hearing loss. The HHIA is a 25-item self-assessment scale composed of 2 subscales, emotional and social/situational, which has been used to evaluate adult patients with hearing disorders in Europe and North America. The test-retest reliability of the Japanese version and its screening version (HHIA-S) were excellent (kappa coefficients were 0.912 and 0.842). Due to the limitation of social/situational items, test-retest reliability was only good (0.6 < kappa coefficient < 0.8), possibly because of problems in translating these items. We discovered that the average of scores of the HHIA Japanese version was higher in bilateral hearing disorder patients than in those with unilateral hearing disorder. This score peaked in 2 to 10 years after onset and decreased thereafter in bilateral hearing disorder patients. The correlation coefficient between the average hearing level of 7 frequencies and scores of the test was highest among the 4 audiological evaluations. The HHIA Japanese version is thus useful for following up patients with hearing disorders.

We report a 63-year-old man who presented with massive bleeding 23 months after irradiation for glottic cancer. He had undergone bilateral functional neck dissection without laryngectomy after irradiation. Pain in the left submandible suggested the site of bleeding. We found a hypopharyngeal fistula that communicated with the carotid artery lumen. The necrotic portion of the artery was ligated and resected with adequate replacement of blood volume : 20 units of blood. Bleeding occurred again on the 47th post-operative day and he developed a pharyngocutaneous fistula. We performed a laryngectomy with a pectoralis major myocutaneous skin flap (PMMF) following full nutritional management.Traditional ligation of the carotid artery still has an important role in the management of carotid rupture with radiation necrosis. One should not hesitate to perform laryngectomies with PMMF to avoid the risk of re-rupture, and we emphasize the need to perform early aggressive surgery with adequate nutritional management.

Hearing impairment can be the cause of serious socio-economic disadvantages. Recent studies have shown inflammatory responses in the inner ear co-occur with various damaging conditions including noise-induced hearing loss. We reported pro-inflammatory cytokine interleukin-6 (IL-6) was induced in the cochlea 6h after noise exposure, but the pathophysiological implications of this are still obscure. To address this issue, we investigated the effects of IL-6 inhibition using the anti-IL-6 receptor antibody (MR16-1). Noise-exposed mice were treated with MR16-1 and evaluated. Improved hearing at 4kHz as measured by auditory brainstem response (ABR) was noted in noise-exposed mice treated with MR16-1. Histological analysis revealed the decrease in spiral ganglion neurons was ameliorated in the MR16-1-treated group, while no significant change was observed in the organ of Corti. Immunohistochemistry for Iba1 and CD45 demonstrated a remarkable reduction of activated cochlear macrophages in spiral ganglions compared to the control group when treated with MR16-1. Thus, MR16-1 had protective effects both functionally and pathologically for the noise-damaged cochlea primarily due to suppression of neuronal loss and presumably through alleviation of inflammatory responses. Anti-inflammatory cytokine therapy including IL-6 blockade would be a feasible novel therapeutic strategy for acute sensory neural hearing loss.

After treatments, several patients with sudden deafness (SD) continued to have symptoms, including hearing loss, tinnitus and dizziness. These unresolved symptoms and their effect on the quality of life (QOL) in SD patients have not been studied. We evaluated QOL using the Hearing handicap inventory (HHIA) and an original questionnaire in SD patients who had been treated more than 6 months prior to the study. Compared to results in bilateral sensorineural hearing were significantly lower in SD patients (p0.01). In bil SNHL, this score peaked two to 10 years after onset of disease and decreased thereafter. The score peaked more than 10 years after onset of disease in patients with SD. While hearing and test scores were correlated in bil-SNHL, this was not observed in SD. About half of patients were embarrassed by hearing loss and tinnitus after treatment. Among patients who scored more than 44 points on HHIA, all reported hearing loss and tinnitus. When asked about subjective changes in hearing after treatment, 27% believed their hearing had improved, 60% believed there was no change, and 13% believed their hearing had deteriorated. Cases believing deterioration in hearing also had high scores on HHIA. Sequelae of SD may worsen QOL, driving embarrassed patients to visit other medical facilities in to improve their QOL. Even though hearing may not improve after initial treatment in ears affected by SD, informed consent about the clinical course and audiological follow-up should be done.

The stress response theory is a relatively new concept about the cause of idiopathic sudden sensorineural hearing loss (ISHL). A number of possible etiologies have been proposed in the literature, as discussed in this paper, but each proposed etiology has been both supported and refuted in the literature. However, the stress response theory can integrate hypotheses that have been advocated so far. The word “stress” refers to a constellation of physical and psychological stimuli including systemic viral and bacterial illness, systemic inflammatory disorders, and physical, mental or metabolic stress. Numerous studies have demonstrated adverse effects of systemic stress on health. Stress causes changes in the immune system and cytokine network through activation of the hypothalamus-pituitary-adrenal axis and the sympathetic nervous system. Several types of catecholamine and cytokine receptors are in the cochlea cells other than capillary cells, and then they can respond to systemic stressors. However, there are few studies examining how systemic stress is asFRONTIER