Guidelines for the evaluation of hearing aid fitting (2010).

OBJECTIVE: The methods to evaluate the efficacy of the adjusted hearing aid for a hearing-impaired person are fitting tests. The tests include those presently carried out for evaluating hearing aid fitting, and the methods of testing and evaluation have been published as "Guidelines for the evaluation of hearing aid fitting (2010)" by the Japan Audiological Society. METHODS: Guidelines for the following 8 test methods are presented. (1) Measurements of speech performance-intensity functions and speech recognition scores; (2) Assessment of hearing aid fitting from the aspect of tolerance of environmental noise; (3) Measurement of real-ear insertion gain (measurement of sound pressure levels at the eardrum); (4) Measurement of the hearing threshold level and the uncomfortable loudness level (UCL) in sound pressure level (SPL) with an inserted earphone; (5) Aided threshold test in a sound field (functional gain measurement); (6) Prediction of insertion gain and aided threshold from hearing aid characteristics and the pure tone audiogram; (7) Measurement of speech recognition in noise; (8) Assessment of hearing aid fitting using questionnaires. In the above tests, (1) and (2) are mandatory tests, and (3) to (8) are informative tests. RESULTS: By performing test combinations properly selected from the above 8 tests, the benefits of a hearing aid could be determined. CONCLUSION: The above test methods were useful and valuable in determining the efficacy of the adjusted hearing aid for a hearing-impaired person during clinical practice.

[Complications Resulting from Taking Ear Impressions].

In 2012, we carried out a study in a large sample to understand the secondary injuries caused during the taking ear impressions for hearing aids. This study is a follow-up of previous research conducted in 1986 (285 medical institutions) and 1999 (98 medical institutions). We posted a questionnaire survey to the otolaryngology departments of 3,257 medical institutions. The response rate to the questionnaire was 62.9% (2,050 of the 3,257 institutions), and the results indicated that 301 of the 2050 institutions (14.7%) had experience with secondary injuries, with a total of 460 cases reported. In 342 of the 460 cases (74.3%), the secondary injuries occurred at hearing-aid dealerships, followed by 67 cases (14.6%) at affiliated medical institutions, and 51 cases (11.1%) in other locations, including other medical institutions, rehabilitation counseling centers, and educational institutions. The most common type of secondary injury (298 cases, 64.8%) was caused by the presence of foreign bodies in the ear, which in turn was a result of complications occurring during the removal of residual ear impression material. Of these 298 cases, 32 required excision of the foreign bodies and surgical intervention under general anesthesia. The remaining 10 cases exhibited isolated tympanic membrane perforation without foreign body-related complications. Furthermore, 146 cases (31.7%) developed bleeding and otitis externa following removal of the ear impression, and there were reports of cases with bleeding that required long-term outpatient care and treatment. Therefore, since retention of a foreign body in the ear and tympanic membrane perforation can occur even in patients without a history of surgery or prior otologic history, adjustment of hearing aids requires prior otorhinolaryngological examination. Furthermore, because of the risk of secondary injury when taking ear impressions, this procedure must be performed with caution under the guidance of an otolaryngologist.

Multiple system atrophy manifesting as complex sleep-disordered breathing.

A 56-year-old female presented with multiple system atrophy (MSA) and pure obstructive sleep-disordered breathing (SDB) with an apnea hypopnea index of 29.6/h. However, after the elimination of obstructive events, central apneas emerged with a central apnea index (CAI) of 45.3/h as determined by continuous positive airway pressure titration. Adaptive servoventilation titration decreased the CAI; however, it remained at 14.2/h. There are two different underlying causes of SDB in MSA patients, one is upper airway obstruction at the glottic level, and the other is central components owing to the degeneration of the pontomedullary respiratory centers. Clinicians should recognize that whether a patient has central apnea cannot be determined by diagnostic polysomnography but only after the elimination of upper airway obstruction in MSA patients.

Three-dimensional morphological analyses of positional dependence in patients with obstructive sleep apnea syndrome.

BACKGROUND: It is generally considered that patients with obstructive sleep apnea syndrome (OSAS) at increased perioperative risk should be placed in nonsupine positions throughout the recovery process; however, not all patients with OSAS show positional dependence. The authors hypothesized that morphological differences exist in three-dimensional (3D) soft tissue and craniofacial structures of the pharyngeal airway between positional and nonpositional OSAS. METHODS: The subjects of the study were body mass index-matched, age-matched, and apnea hypopnea index-matched positional (n = 10) and nonpositional (n = 10) Japanese OSAS patients and body mass index-matched Japanese control subjects (n = 10). Pharyngeal magnetic resonance imaging and cephalometric radiography were performed during wakefulness. RESULTS: The patients with positional OSAS had a smaller volume of the pharyngeal lateral wall soft tissues, larger maxilla-nasion-mandible angle, and smaller lower facial height than the nonpositional OSAS and the control subjects. The patients with positional OSAS showed a significantly steeper sella-nasion-mandible angle and smaller craniofacial volume than the control subjects. There were no significant differences in tongue volume and 3D pharyngeal anatomical balance between positional and nonpositional OSAS. Multivariate stepwise regression for positional dependence showed that the dominant determinant was the volume of the lateral pharyngeal wall, followed by lower facial height and maxilla-nasion-mandible angle. CONCLUSIONS: Patients with positional OSAS have wider airways in the lateral parts, lower facial height, and more backward position of the lower jaw, which may explain differences in the maintenance of pharyngeal airway patency in the lateral sleep position.

[Stapes surgery performed for congenital stapes foot plate fixation--comparison of prosthesis fixation to the malleus vs. the incus].

We report the surgical results of stapes surgery using the Schukneht-type wire piston prosthesis performed on 30 ears with fixation of the stapes footplate and absence of the long process of the incus. The prosthesis was reformed to avoid dislocation and fixed to the handle of the malleus. The surgical results in another 49 ears, the comparator group, in which the prosthesis was fixed to the long process of the incus because the anomaly was restricted to fixation of the footplate without other associated anomalies, were also analyzed for comparison. The mean postoperative air conduction hearing levels were 28.6dB in the subjects and 21.6dB in the comparator group, and the mean hearing improvements were 35.7dB and 29.7dB, respectively. The success rate of the operation, based on the criteria established by the Japan Otological Society, was 90% in the subjects and 98% in the comparator group. The mean postoperative air-bone gap in the subjects was 15.8dB, which was 4.3dB higher than that in the comparator group. The difference between the two groups was considered to be due to the difference in the effectiveness of the conduction mechanism after the surgery. We therefore concluded that the surgical procedure using the Schukneht-type wire piston prosthesis to fix the malleus handle is a useful surgical method that yields satisfactory results.

[Immunohistochemical and clinical features of patients with benign middle ear tumors].

The middle ear is a rare site for benign tumors. We report six patients with benign middle ear tumors surgicaly removed: three with carcinoid, one with adenoma, and two with paraganglioma. These tumors showed similar histological features in trabecular and glandular architecture, so further immunohistochemical study was needed to make a definitive histopathological diagnosis. When we analyzed clinical features of patients diagnosed immunohistochemically, the differentiation of two patients with paraganglioma from the remaining four with adenomatous tumors such as adenoma and carcinoid was relatively easy from otomicroscopic findings and enhancement in temporal bone MRI. It was however difficult to differentiate patients with adenomatous tumors from each other because they induced similar clinical symptoms and showed similar findings in images. For a definitive diagnosis, it is necessary to conduct exploratory tympanotomy or removal of the tumors to obtain enough specimen for histological and immunohistochemical studies. Tumors induced no bone destruction or complications. We recommend staged operations for total removal of tumors and for good postoperative hearing.

[Eosinophil chemoattractants and related factors in nasal polyps].

OBJECTIVE: In vitro studies and animal experiments have shown that cytokines and chemokines are closely related to eosinophil migration, activation, and survival. It remains controversial, however, whether some chemokines or cytokines are actually responsible for the accumulation of eosinophils in nasal polyp tissues. We studied cytokines and chemokines in nasal polyp tissues taken from patients with chronic rhinosinusitis to clarify the pathogenesis of eosinophil accumulation. MATERIALS AND METHODS: Nasal polyp tissues obtained from 20 patients with chronic rhinosinusitis were studied. Concentrations of interleukin (IL-) 5, IL-13, eotaxin, regulated upon activation in normal T cell expressed and secreted (RANTES), and thymus and activation-regulated chemokine (TARC) in homogenates of polyp tissues were measured by ELISA. Nasal polyp tissues were stained by hematoxillin and eosin and were immunostained by an antibody against EG2. The numbers of eosinophils and immunopositive cells for EG2 in the submucosal layer were counted using a microscope. RESULTS: No significant differences were seen in the numbers of eosinophils and EG2-positive cells, or in the concentration of IL-5, eotaxin, TARC, RANTES in nasal polyp tissues between patients with and without atopic predisposition. Significant positive correlations existed, however, between the number of eosinophils and IL-5, eotaxin, and TARC concentration. IL-13 concentration was below detection in all patients. CONCLUSION: We hound that IL-5, eotaxin, and TARC may play an important role in the accumulation of eosinophils in nasal polyps regardless of the presence of atopic predisposition.

Eustachian tube function in patients with eosinophilic otitis media associated with bronchial asthma evaluated by sonotubometry.

OBJECTIVE: To determine eustachian tube function in patients with asthma and with or without eosinophilic otitis media (EOM), a new middle ear disease entity with a highly viscous middle ear effusion containing many eosinophils and usually associated with bronchial asthma. One of the most important causes of otitis media (OM) is eustachian tube dysfunction. DESIGN: Retrospective case review. SETTING: A referral center. PATIENTS: Twenty patients with EOM and patients with asthma but without OM. MAIN OUTCOME MEASURES: We studied eustachian tube function using sonotubometry and a questionnaire. Sonotubometry was also performed on 13 control patients with chronic otitis media (COM) and 7 normal controls. RESULTS: The tubal opening duration was significantly longer in patients with EOM than in patients with asthma but without OM, controls with COM, and normal controls, indicating the presence of patulous eustachian tubes in patients with EOM. Responses to the questionnaire also supported the presence of patulous eustachian tubes in the patients with EOM. CONCLUSIONS: The presence of a patulous eustachian tube may be a major cause of EOM in patients with bronchial asthma. In patients with asthma who have a helper T-cell 2-dominant predisposition, a patulous eustachian tube easily allows the entry of antigenic materials into the middle ear, causing eosinophil-dominant inflammation.

Effectiveness of instillation of triamcinolone acetonide into the middle ear for eosinophilic otitis media associated with bronchial asthma.

BACKGROUND: Eosinophilic otitis media (EOM) is a newly recognized middle ear disease found in asthmatic patients. EOM is characterized by a highly viscous middle ear effusion that contains many eosinophils and is extremely unresponsive to conventional treatments for common otitis media. To our knowledge, no systemic study regarding the efficacy of treatments for EOM has been performed. OBJECTIVE: To determine the effectiveness of instillation of triamcinolone acetonide, which is a suspension of steroids, into the mesotympanum and eustachian tube as a treatment for patients with EOM. METHODS: We studied the efficacy of the instillation of triamcinolone acetonide in 43 ears of 24 patients with EOM. Efficacy was evaluated according to the length of the period without middle ear effusion or otorrhea. We also determined the otomicroscopic findings and the hearing levels before and after therapy. For controls, 27 ears of 14 patients treated by topical administration of betamethasone were similarly evaluated. RESULTS: The middle ear effusion or otorrhea was controlled for more than 3 weeks after 1 instillation of triamcinolone acetonide in 35 ears, and the efficacy rate (81%) was significantly higher than that in the controls (7 ears, 26%). In the triamcinolone acetonide group, the average air conduction hearing level at the speech frequency range was significantly improved, and deterioration of the bone conduction hearing threshold was rarely found during therapy. CONCLUSION: The instillation of triamcinolone acetonide into the mesotympanum and eustachian tube is an effective treatment for patients with EOM.

Pathological findings in the temporal bone of newborn infants with neonatal asphyxia.

Hypoxia in adults frequently causes damage to the brain, but not the inner ear. However, hypoxia in neonates with inadequate blood-inner ear barrier function causes damage to the inner ear, leading to hearing loss and equilibration disorder. Clinically, asphyxiated neonates sometimes have the complication of sensorineural hearing impairment at high frequencies. Many reports suggest that this problem is caused by hypoxic encephalopathy. However, it is not clear whether inner ear disorders are present. In this report we examined the inner ear pathology of four asphyxiated neonates using serial sections of temporal bone. The subjects (n=4) were 1-13-day-old neonates with pregnancy periods of 24-36 weeks. Their fetal growth curves were within the normal range. There was one case each of twin pregnancy, placental abruption, placenta previa and meconium aspiration syndrome. For the case of severe neonatal asphyxia, damage to the inner ear included the degeneration and disappearance of outer hair cells of the organ of Corti and edematous changes in the stria vascularis. Degeneration of spiral ganglion and vestibular ganglion cells was observed in two of the other cases.

Discrepancy in polysomnography scoring for a patient with obstructive sleep apnea hypopnea syndrome.

Overnight polysomnography (PSG) is indispensable for diagnosis of obstructive sleep apnea hypopnea syndrome. However, studies on interscorer agreement on PSG scoring between laboratories are few. The purpose of this study was to examine the reliability of interscorer agreement on PSG scoring among 16 sleep laboratories in Japan. We found a relatively moderate interscorer reliability of the index of oxygen desaturation and arousal during sleep, but a relatively low reliability of the index of transient reduction in and complete cessation of breathing (apnea hypopnea index). The median rate of interscorer coincidence of sleep staging was the lowest for slow wave (deep) sleep (23.5%), followed by those for Stage 1 (59.8%), Wake (73.2%) and Stage 2 (74.2%) in this order, and rapid eye movement was the most reliably identified stage (91.3%). The median rate of interscorer coincidence for all stages was 71.8%. The present study demonstrates that scorers tend to analyze PSG data according to a relatively empirical decision as opposed to a rule-dependent decision. Further detailed scoring manuals are required to decrease the interscorer discrepancy in PSG scoring.

[Temporal bone histopathology in a patient suspected of inner ear extension of otitis media].

We report the histopathological findings in the temporal bone of a 30-year-old female who died of cervical esophageal carcinoma. The temporal bone sections revealed severe bilateral suppurative labyrinthitis and otitis media that presumably occurred immediately before her death. Many inflammatory cells were present in the middle ear, particularly around the stapes and the round window niche. They had also infiltrated the inner ear via the annular ligament of the stapes and the round window membrane. Inflammatory cell accumulation was also observed in the peri- and endolymphatic spaces, and it was most severe in the basal turn. Most of the inner and outer hair cells were preserved, but some had degenerated or were missing. Numerous round cells were observed in the modiolus, and some of the spiral ganglion cells had degenerated. On the basis of these findings, we concluded that bacterial otitis media had extended in to the inner ear via the oval window and round window membrane and had resulted in suppurative labyrinthitis. These findings are consistent with those of stage II suppurative labyrinthitis according to the classification of Schuknecht.

[Temporal bone histopathology of noise-induced hearing loss].

To examine the relationship between hearing and changes in the inner ear, we investigated human temporal bone specimens from 2 patients with noise-induced hearing loss and prepared audio-cytocochleograms as described by Schuknecht et al. Patient 1 was a 50-year-old male who died of thyroid cancer and had worked at a printing house for 38 years. Patient 2 was a 58-year old male who died of maxillary sinus cancer and had worked in construction for 22 years. A pure-tone audiogram showed high-tone sensorineural hearing loss with c5-dip-type hearing disorder in both ears in Patient 1, and a high-tone abrupt form of sensorineural hearing loss in Patient 2. Pathological examination of the temporal bone revealed degeneration and disappearance of the organ of Corti at the basal turn and disappearance of cochlear neurons in both patients. Audio-cytocochleograms revealed hearing disorder consistent with the changes in the inner ear in both patients. Marked degeneration and disappearance of the organ of Corti and stria vascularis were present in patient 1. It is generally known that disorders of the organ of Corti for a long period is involved in the etiology of noise-induced hearing loss. This degeneration of the organ of Corti is produced at a basilar membrane with the maximum amplitude related to exposure to noise according to a physical and mechanical factors. Moreover, animal experiments have shown that exposure to noise decrease cochlear blood flow. In Patient 1 both the organ of Corti and the stria vascularis exhibited degeneration, suggesting that not only physical and mechanical factors but a cochlear circulatory disorder related to exposure to noise was involved in the etiology of the pathological changes in the temporal bone related to noise-induced hearing loss.

[Case of recurring middle ear adenoma].

We report a case of recurring middle ear adenoma with bone formation that was histologically difficult to differentiate from adenocarcinoma. The patient was a 31-year-old female. Preoperative clinical and CT findings in the 31-years-old woman examined suggested benign tumor of the middle ear. Although intraoperative pathological examination indicated adenocarcinoma, tympanoplasty in canal reconstruction was done. Malignancy was not considered because the tumor had not increased in size for 1 year and no bone erosion had occurred in the ossicles or middle ear cleft. The definitive pathological diagnosis was middle ear adenoma. The tumor repeatedly recurred thereafter, closely resembling normal middle ear mucosa and making it difficult to remove completely. When middle ear adenoma is suspected, surgical procedures should be determined carefully based on radiological and intraoperative findings in tumor expansion.

[Temporal bone histopathology exhibiting cochleosaccular degeneration in a patient with profound deafness].

We herein report the histopathological findings of the temporal bone taken from a patient with unilateral profound deafness since early childhood. The patient was a 72-year-old male who died of lung cancer and extensive metastases including the tongue. The patient had a history of profound hearing loss in his left ear since childhood. The histopathological finding of the left temporal bone revealed a severe atrophy of the organ of Corti, a detached and rolled-up tectorial membrane, a moderate loss of the stria vascularis, and a severe loss of spiral ganglion cells. In addition, the macula of the saccule was severely degenerated. The marked degeneration in the inner ear indicated a cochleosaccular disorder, which is a typical temporal bone finding in cases of viral labyrinthitis and hereditary hearing impairment. The present patient was suspected to have suffered cochleosaccular degeneration as a result of an inner ear viral infection during childhood because the number of spiral ganglion cells was significantly reduced because of secondary neural degeneration.

Nasopharyngeal flora and drug susceptibility in children with macrolide therapy.

OBJECTIVES/HYPOTHESIS: Low-dose, long-term administration of macrolides (macrolide therapy) has been used as an effective treatment for chronic respiratory tract diseases. The authors reported on the nasopharyngeal flora in children treated with macrolide therapy. STUDY DESIGN: Prospective study. METHODS: Nasopharyngeal cultures were obtained from 73 children with chronic rhinosinusitis and/or otitis media with effusion at the end of the low-dose administration of clarithromycin (macrolide group). As control subjects, 98 children with chronic rhinosinusitis and/or otitis media with effusion who were not given macrolides were also included in the study. The culture results were evaluated with respect to antimicrobial susceptibility patterns, risk factors for carriage of erythromycin-resistant Streptococcus pneumoniae, and the clinical efficacy of the therapy. RESULTS: The macrolide therapy did not have a significant effect on the incidence or the susceptibility patterns of potential pathogens except for Moraxella catarrhalis. Most of children in the macrolide group possessed a normal flora compared with the control children. The risk factors for carriage of erythromycin-resistant S pneumoniae were male gender in the macrolide group and age under 6 years and use of antimicrobial drugs other than macrolides in the control group. The clinical efficacy of the therapy was independent of carriage of erythromycin-resistant S pneumoniae. CONCLUSION: Macrolide therapy has little effect on carriage of drug-resistant pathogens, and the efficacy of the therapy depends on the anti-inflammatory effect of the drugs, which is independent of their antimicrobial effect.