Myeloperoxidase-antineutrophil cytoplasmic antibody-associated sensorineural hearing loss.

A 36-year-old female with hyperthyroidism that had been treated with propilthiouracil (PTU) complained of tinnitus and hearing loss in both ears. She was treated with steroid administration by an otolaryngologist; however, hearing continued to fluctuate when the steroids were tapered. Laboratory evaluation revealed a decreased complement level and elevated levels of immunoglobulin M (IgM) and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA). With the withdrawal of PTU and high-dose methylprednisolone, she had excellent return of right-sided hearing. In recent years, there have been many reports about MPO-ANCA-associated small vessel vasculitis. Although any organ may be affected by this disease, there are no reports about MPO-ANCA-associated progressive hearing loss without any other organ involvement. The present case suggests the possibility that inner ear blood flow impairment due to ANCA-associated small vessel vasculitis induces the so-called autoimmune sensorineural hearing loss.

Sensorineural hearing loss induced by radiation as a late effect: five cases followed by audiogram.

OBJECTIVE: We studied the progression of the late radiation-induced sensorineural hearing loss (SNHL) to discuss the pathological mechanism responsible for the progression with referring to past reports. METHODS: Five cases were selected among the SNHL cases diagnosed at the Department of Otolaryngology. Hokkaido University. All the cases were followed up with audiograms. RESULTS: All of the five cases showed gradual progression of SNHL, which developed mostly 1 2 years after irradiation for brain tumors. Two of them showed rapid progression from time to time with sudden onset in their clinical courses. All the patients were resistant to any medication such as steroid, vitamin B12 or cerebral circulation activators. CONCLUSION: Two patterns of progressions of radiation-induced hearing loss were observed. Both of them were considered to be explainable by ischemic changes of the vessels as reported earlier. Since there is no effective treatment for radiation-induced SNHL, it is most important to enlighten clinicians in general on this disease.

Preserved otoacoustic emission in a child with a profound unilateral sensorineural hearing loss.

We report a case of profound unilateral sensorineural hearing loss with good response of otoacoustic emission. The patient was a 5-year-old boy. who was diagnosed to have unilateral hearing impairment on pure tone audiometry at the first visit. The affected ear showed the absence of auditory brainstem response; however, its transiently evoked otoacoutic emission and distortion product otoacoustic emission were considered to be normal. These findings indicated that the outer hair cell of cochlea was not impaired and that the impairment should be localized between inner hair cells, primary afferent fiber or its synapses, spiral ganglion of the cochlea and acoustic fiber, or at a combination of these areas. That is, evaluation of otoacoustic emission was useful in determining the region of impairment in sensorineural hearing loss. Further follow-up will be necessary to differentiate the present case from auditory neuropathy.

Herpes simplex virus type 1 reactivation and antiviral therapy in patients with acute peripheral facial palsy.

OBJECTIVE: Recent studies provide compelling data for the hypothesis that herpes simplex virus type I (HSV-1) is implicated in the pathogenesis of idiopathic peripheral facial palsy (Bell's palsy). The present study analyzed the severity of facial palsy in patients with HSV-1 reactivation and sought to determine the efficacy of acyclovir-prednisone therapy for these patients. MATERIALS AND METHODS: In total, 176 patients, clinically diagnosed with Bell's palsy. were divided into three groups by polymerase chain reaction (PCR) and serological tests--31 patients with HSV-1 reactivation, 45 patients with VZV reactivation (zoster sine herpete) and 100 patients without HSV-1 or VZV reactivation (Bell's palsy). RESULTS: The difference in the worst grade of facial palsy between patients with zoster sine herpete and Bell's palsy was significant (P = 0.01 10, Mann-Whitney U-test). In contrast, no difference in the severity of palsy was observed between patients with HSV-1 reactivation and Bell's palsy. Twelve patients received acyclovir-prednisone treatment within 7 days of onset based on positive PCR results and ten of the 12 (83%) recovered completely. In contrast, 14 patients with HSV-1 reactivation received prednisone treatment because their PCR tests were performed at a later date; ten of these 14 (71%) recovered completely. The difference in the cure rate between the two treatment groups was not significant (P > 0.05, Fisher exact test). CONCLUSIONS: The results indicate that the severity of palsy in patients with HSV-1 reactivation is similar to that in patients with Bell's palsy and suggest that early diagnosis of HSV-1 reactivation by PCR and subsequent acyclovir-prednisone therapy do not improve recovery from facial palsy.

A rare aneurysmal bone cyst of the maxillary sinus: a case report.

Aneurysmal bone cyst (ABC) is a non-neoplastic expansile bone lesion that is common in the long bones; only 2% occurs in the head and neck. We present a case of ABC in a 23-year-old male and describe the clinical and radiological features, histopathology and treatment. Magnetic resonance imaging (MRI) demonstrated multiple internal septations, cysts with fluid-fluid levels of varying intensity, and an intact rim of low-intensity signal completely surrounding the lesion. The tumor was removed by enucleation with resection of the lateral nasal cavity. Histopathologic diagnosis was ABC and fibrous dysplasia. We suggest that MRI is very useful for the diagnosis of ABC. ABC is thought to follow other lesions, and thus when treating ABC, it is important to determine whether any pre-existing lesion has preceded or not; in particular, if the lesion site is in the head and neck region.

Optimal stimulus level conditions for measurements of distortion product otoacoustic emissions.

OBJECTIVE: Distortion product otoacoustic emission (DPOAE) is a tool for an objective examination of the cochlea function. While the clinical application of DPOEA test is in progress, the measurements vary depending on the stimulus conditions. We aimed to determine the most appropriate stimulus level in the clinical application of DPOAE test. METHODS: Ninety-seven normal hearing ears and 80 hearing-impaired ears (total 177) were subjected to this study. Two levels of stimulations (L1 and L2) were applied as follows: L1 = L2 = 70 dB; L1 = L2 = 60 dB and L1 = 60/L2 = 50 dB, and DP level was measured at each stimulation, and compared with hearing level. RESULTS: DP level was highest at L1 = L2 = 70 dB. Normal hearing ears and hearing-impaired ears were well differentiated at L1 = L2 = 60 dB. CONCLUSION: In clinical application of DPOAE test, DP levels should be measured at the stimulus level most appropriate for the purposes.

Therapy for otitis-prone children in Tenshi hospital.

With the recent emergence and increases of multiple-drugs-resistant Streptococcus pneumoniae, we have been seeing an increasing number of infants with intractable recurrent otitis media which is resistant to the general conservative out-patient treatments such as oral administration of medicines or tympanotomy. In this study, we investigated the inflammation-causing bacteria in the infants with otitis media which were treated in our hospital from January to December in 1997, and in six serious cases among them, we measured IgG subclass and specific IgG2 antibody to S. pneumoniae to examine them. As a result, S. pneumoniae was found to be the cause in 45% of the cases of initial development of otitis media, and in 88% of them the S. pneumoniae was penicillin-resistant. The level of specific IgG2 antibody to S. pneumoniae was low in all the cases, whereas IgG2 subclass was deficient only in one out of the six cases; from these findings, the selectively low level of immune status was thought to be the cause of the recurrences of otitis media. In two cases, clinical condition was markedly improved by immunoglobulin substitute therapy, which demonstrates that immunoglobulin is effective for the intractable recurrent otitis media in infants.

An anti-mumps IgM antibody level in the serum of idiopathic sudden sensorineural hearing loss.

PURPOSE: Measurement of anti-IgM antibody for mumps enables us to diagnose silent mumps infection. From the viewpoint of prophylactic medicine, we examined the incidence of silent mumps infection in idiopathic sudden sensorineural hearing loss (ISSNHL) by measurement of anti-mumps IgM antibody. MATERIALS AND METHODS: Serum level of anti-mumps IgM antibody was evaluated by EIA method in 69 ISSNHL cases. RESULTS: Of the 69 serum samples examined, five samples were positive, two samples were regarded as quasi-positive and other samples were negative for anti-mumps IgM antibody. The positive rate was 7.2% (5/69). The relationship of anti-mumps IgM and IgG antibody was also described. CONCLUSIONS: From the measurement of anti-IgM antibody for mumps, it was possible to diagnose silent mumps infection in ISSNHL. The positive rate of anti-IgM antibody in ISSNHL was 7.2% suggesting that the silent mumps infection could be considered as one of the causative factors of ISSNHL even though its incidence is not so high. Since mumps often occurs without clinical symptoms, it is still considered to be one of the important causes of profound hearing loss. From the viewpoint of prophylaxis of profound hearing loss and deafness, we should understand the situation of virus epidemiology and vaccination.

Effects of spontaneous otoacoustic emissions on distortion product otoacoustic emission.

OBJECTIVE: It has been reported that spontaneous otoacoustic emission (SOAE) can prolong the responses or increase the echo power of transiently evoked otoacoustic emission (TEOAE), yet the effects of SOAE on distortion product otoacoustic emission (DPOAE) have been studied less thoroughly. As most of the previous studies have not paid attention to the patient's age, sex and hearing level, they have not reflected possible effects of those factors. We studied the effects of SOAE specifically on DPOAE in the following subjects. SUBJECTS AND METHODS: The subjects were all females ranging in age from 19 to 24 (average: 21.4) and the 78 ears had a hearing threshold under 15 dB for 1.2, and 4 kHz on pure-tone hearing test. IL088 (Otodynamics) was used for measurement of SOAE and IL092 (Otodynamics) for DPOAE. SOAEs were measured by time-averaging over 100 of the responses, of which those showing a clear peak 3 dB above the noise floor and being reproducible were considered as SOAE-positive. In all the ears. DPOAE responses were measured at L1 = L2 = 70 dB, and in 42 ears also at L1 = L2 = 60 dB and L1/L2 = 60/50 dB. The subjected ears were grouped into two by the presence or the absence of SOAE, and DPOAE amplitudes of 1, 2, and 4 kHz were compared. respectively. RESULTS: Of the total, 39 ears were SOAE-positive and 39 SOAE-negative. Statistically no significant difference was observed in the average hearing level between the SOAE-positive and SOAE-negative groups. The hearing levels did not significantly differ in the frequencies of 1, 2. and 4 kHz, respectively, indicating that influence of the hearing level on DPOAE could be excludable. DPOAE amplitudes at L1 = L2 = 70 dB in the frequencies of 1, 2, and 4 kHz were higher in the SOAE-positive group than in the SOAE-negative group. And DPOAE amplitudes were also higher in SOAE-positive group at L1 = L2 = 60 dB and L1/L2 = 60/50 dB in the frequency of 1.2, and 4 kHz, but significant differences were observed only in the frequencies of 4 kHz. By grouping the ears by the number of SOAE. we revealed the tendency that the larger the number of SOAE, the higher the DPOAE amplitudes. CONCLUSIONS: We evidenced that SOAE has significant effects on DPOAE responses. In clinical application of DPOAE measurement, therefore, the effects should be seriously taken into account.

A family affected by branchio-oto syndrome with EYA1 mutations.

Branchio-oto (BO) syndrome is complicated with congenital preauricular fistulae, branchial fistulae (cysts), and hearing loss (sensorineural, conductive or mixed). As well as branchio-oto-renal (BOR) syndrome. it is known to be an autosomal dominant hereditary disorder. Since mutations in the EYA1 gene have been identified in both BO and BOR syndromes, mutation screening of this gene has been drawing attention as a genetic test to diagnose BOR/BO syndromes. In this study, we genetically investigated the presence of EYA1 mutations in a BO syndrome family in which we observed congenital preauricular fistulae, branchial fistulae (cysts) and hearing loss in four generations. Whereas there was a variety of phenotype expressions in this family, all subjects tested had a nonsense mutation (R264X) in exon 8 of the EYA1 gene. The present report adds further examples to support the usefulness of molecular genetic testing for the diagnosis of patients with BO syndrome.

[Utility of OAE screener (GSI 70) for the evaluation of distortion product otoacoustic emissions].

The purpose of this study was to investigate the ability of the OAE screener GSI 70 to evaluate of cochlea function in neonates, infants and adults. Distortion product otoacoustic emissions (DPOAEs) were measured using the GSI 70 DPOAE Analyzer and evaluated in 123 human ears between December 1999 and June 2000. We performed the following four general clinical tests to determine the reliability of the GSI 70: 1) comparison of DPOAE levels measured using the ILO 92 and the GSI 70 in 55 adult ears, 2) comparison of DPOAE levels measured using the GSI 70 and hearing levels in 55 adult ears, 3) comparison of DPOAE levels measured using the GSI 70 and ABR levels in 45 neonate and infant ears, and 4) evaluation of the utility of DPOAE measurements obtained using the GSI 70 in functional deafness cases. The following results were obtained: 1) DPOAE levels measured with the ILO 92 and the GSI 70 were closely correlated (correlation coefficient, 0.773 at 2 kHz and 0.813 at 4 kHz). 2) The sensitivity of the GSI 70 in adult ears confirmed to have normal hearing was 80% at 2 kHz and 100% at 4 kHz; the specificity of hearing-impaired ears was 94% at 2 kHz and 94% at 4 kHz. 3) The sensitivity of the GSI 70 in normal neonate and infant ears with normal hearing was 100% at 2 kHz and 100% at 4 kHz; the specificity of hearing-impaired neonate and infant ears was 97% at 2 kHz and 94% at 4 kHz. 4) All of the functionally deaf ears showed excellent DPOAE responses. Examinations using the GSI 70 DPOAE analyzer were very easy and fast. Also, the results were highly reliable, with the exception of one adult who was classified as having normal hearing in an out-of-scale hearing level at 2 kHz. We suggest that specific criteria be established for the clinical usage of the GSI 70 in performing objective hearing evaluations.

[Influence of spontaneous otoacoustic emission (SOAE) on transiently evoked otoacoustic emission (TEOAE)].

The literature has reports on the influence of spontaneous otoacoustic emission (SOAE) on transiently evoked otoacoustic emission (TEOAE), but most do not take factors such as age, gender, and hearing level into consideration. We focused on these conditions. Subjects were 78 women with normal hearing aged 19 to 24 years (mean = 21.4). All had pure tone thresholds of 15 dB HL or better at 1 kHz, 2 kHz, and 4 kHz. ILO88 was used to record TEOAE and SOAE. Echo power (EP) and reproducibility (Repro) were compared between groups with and without SOAE. No significant audiometric difference was seen between groups. Total echo power (TEP) and whole reproducibility (WR) were significantly greater in the group having SOAE, consistent with previous reports (p < 0.01). EP and Repro classified by frequency bands were also significantly greater in the group having SOAE at 1 kHz to 4 kHz. Subjects were divided based on the number of SOAE and the above parameters compared. We found that as the SOAE number increased, EP and Repro increased. In conclusion, the existence of SOAE influences TEOAE parameters and must be taken into account during clinical testing.

[Transient evoked otoacoustic emission measurement in infants].

Transient evoked otoacoustic emissions (TEOAEs) were measured and evaluated in 194 ears of 101 subjects under 4 years old who were suspected of hearing loss, using a ILO88 Otoacoustic Emission Analyzer, to study the basic characteristics of this measure and its utility for hearing screening. The mean time necessary to record TEOAEs in both ears was short, about 3 minutes. In 58 ears judged as normal hearing within 30 dB in ABR, TEOAE levels in infants aged less than 2 months were significantly higher than in those aged more than 1 year, especially in the high frequency bands (4-kHz and 5-kHz bands). One case which had been judged as bilateral moderate-to-profound hearing impairment in initial ABR testing showed good responses in TEOAEs, indicating normal cochlear function, and obvious wave Vs in follow-up ABRs recorded at 30 dB nHL confirmed normal auditory function. Therefore, in a case like this one, suspected of retardation in brain stem maturation, TEOAE is more useful than ABR as a hearing screening technique. Since external and middle-ear factors caused poor TEOAE responses in some cases which had been judged as normal hearing by ABR. TEOAE seems to be more sensitive in detecting external and middle ear problems than ABR. Moreover, TEOAE measurement of infants was easy and noninvasive. We conclude that TEOAE is an excellent tool for screening auditory function in infants.

[Distortion product otoacoustic emissions for the assessment of auditory sensitivity].

Distortion product otoacoustic emissions (DPOAEs) were evaluated in 494 normal and 506 cochlear-impaired human ears, to determine whether DPOAEs depend on factors such as background noise, the shape of the pure tone audiogram, sex and aging, and whether a DPOAE test can perform well in distinguishing normal-hearing from hearing-impaired ears. The amplitudes of DPOAEs were measured at the frequency of 2f1-f2 (f1 < f2, f2/f1 = 1.22, f2 at 1, 2 and 4 kHz) using as stimuli two pure tones at level of 70 dB from an ILO92 Otoacoustic Emission Analyzer. The correlation coefficients between the DPOAE level and the auditory threshold decreased as the background noise levels at 1 kHz and 2 kHz increased. Therefore, it appeared that ears with large background noise levels would be inadequate for the study of DPOAEs predicting the hearing state. The sensitivity (normal-hearing ears identified as normal hearing) and the specificity (hearing-impaired ears identified as hearing impaired) at the equal-sensitivity-specificity condition were 80.7-86.7% at 1, 2 and 4 kHz, and the areas under the receiver operating characteristic (ROC) curves, which were used to estimate the test performance, were 0.88 for 1 kHz, 0.91 for 2 kHz and 0.92 for 4 kHz. Since these results suggest that a DPOAE can be used as a reliable technique for objective auditory tests, it is thought that actual values (DPOAE level: 4.3 dB at 1 kHz, 5.0 dB at 2 kHz and 2.9 dB at 4 kHz) of false-positive (hearing-impaired ears identified as normal hearing) rates corresponding to 5% can be used in clinical evaluation to separate normal hearing from hearing-impaired ears. There was, however, a significant age effect at 4 kHz on DPOAEs in the ears with the same pure tone hearing thresholds, and the areas of the ROC curves in subjects ranging from 10 to 29 years old were larger than in subjects over 50 years (1 kHz: 0.88 to 0.94 versus 0.83 to 0.84, 2 kHz: 0.95 versus 0.89, 4 kHz: 0.95 to 0.96 versus 0.88 to 0.89). Therefore, it is thought that age-adjusted norms may be necessary for the accurate interpretation of DPOAE results.

Reactivation of herpes simplex virus type 1 in patients with Bell's palsy.

Reactivation of herpes simplex virus type 1 (HSV-1) has been implicated in the pathogenesis of idiopathic peripheral facial palsy (Bell's palsy). The present study used the polymerase chain reaction (PCR) to analyze the saliva of patients with Bell's palsy for the presence of shed HSV-1. The study involved 47 patients with Bell's palsy, 24 patients with Ramsay Hunt syndrome, and 16 healthy HSV-seropositive volunteers. HSV-1 DNA was not detected in the saliva samples from HSV-seronegative patients. The prevalence of shed HSV-1 in patients with Bell's palsy (50%) was significantly higher than that in healthy volunteers (19%, p<0.05). When saliva samples were tested within 7 days after the onset of palsy, the prevalence of shed HSV-1 in patients with Bell's palsy (40%) was significantly higher than that in patients with Ramsay Hunt syndrome (7%, p<0.05). Furthermore, HSV-1 usually became undetectable by the second week after the onset of Bell's palsy when HSV-1 was detected during the acute phase of the disease. These findings strongly suggest that reactivation of HSV-1 is involved in the pathogenesis Bell's palsy, and indicate that PCR is a useful tool for early diagnosis of HSV-1 reactivation in patients with Bell's palsy.

[Relationship between distortion product otoacoustic emissions and pure tone thresholds in normal and hearing-impaired ears].

To establish clinical criteria for distortion product otoacoustic emissions (DPOAEs) in order to distinguish normal-hearing from hearing-impaired ears, DPOAEs were measured and evaluated in 325 normal and 291 cochlear-impaired human ears using an ILO92 Otoacoustic Emission Analyzer. The background noise level was too variational in each individual ear to ignore in DPOAE measurements. Therefore, the value of the difference between the DPOAE amplitude and the background noise level (DPOAE noise) was calculated and compared with the pure tone threshold at 1 kHz, 2 kHz and 4 kHz. It was difficult, however, to precisely separate normal-hearing and hearing impaired ears with DPOAE noise values because many normal ears presented small amplitudes of DPOAEs and some impaired ears showed high amplitudes. Therefore, cumulative distributions of sensitivity (normal hearing ears identified as normal hearing) and specificity (hearing impaired ears identified as hearing impaired) were constructed and used to calculate a cut off point for DPOAE-noise in order to separate normal hearing from hearing impaired ears. It is considered that the cut-off points (DPOAE noise values of 7.3 dB at 1 kHz, 10.3 dB at 2 kHz and 16.1 dB at 4 kHz) at which the false alarm rates (1 specificity: the rate of hearing impaired ears incorrectly identified as normal hearing) correspond to 5% can be useful for clinical evaluation of DPOAEs.

[Otoacoustic emissions of full-term and preterm neonates].

Transiently evoked otoacoustic emission (TEOAE), distortion product otoacoustic emission (DPOAE), and spontaneous otoacoustic emission (SOAE) were measured in 45 full-term neonates (68 ears) and 12 preterm neonates (20 ears) with ILO88 & 92. Measurements were performed in the nursery of the obstetrics ward or NICU (not sound proof room) under natural sleeping condition after nursing. No sedating agent was used. TEOAEs were rated "good response" in 61 (89.7%) of 68 full-term neonate ears. DPOAEs were rated "good response" in 40 (71.4%) of 56 full-term neonate ears. SOAEs were detectable in 25 (62.5%) of 40 full-term neonate ears. Considering the high positive rate of TEOAE in full-term neonates and the easy and noninvasive method of measurement, we concluded that TEOAE is useful for auditory screening in neonates. There was failure to detect TEOAEs in 7 ears and the measurements were all performed within 6 days after birth. Some reports claim that residual amnion in the external auditory canal or the middle ear in the first few days after birth causes slight hearing loss. Thus, we expected that making the measurements more than 7 days after birth might yield higher "good response" rates. We sometimes found that the Total Echo Powers of TEOAEs were reduced by the poor condition of the ear probe. Thus, we must be very careful in regard to this technical problem in order to perform accurate examinations. Because of its lower "good response" rate, DPOAE was not as useful for screening as TEOAE. Because of the movements or respiratory noises of the newborn infants, it was hard to detect reliable DPOAEs, particularly in the low frequency range. On the other hand, because of its frequency specificity, particurally at high frequencies, DPOAE will be useful for detecting the partial hearing impairment such as congenital high-tone hearing impairment. It would be difficult to use SOAE as a clinical test. Because it is not an evoked response, its mechanism of generation is not well understood. We expect that following longitudinal changes in SOAE in neonates may yield some information about it. We measured mainly TEOAE in preterm neonates because we had to complete the measurements as soon as possible. High Total Echo Powers of TEOAEs were recorded in most infants over 38 weeks of PCA (post conceptional age). The earliest case showed reliable TEOAE at 35 weeks PCA. In most cases that could be measured twice on different days, the Total Echo Powers of TEOAE, were higher in the second time. We therefore concluded that TEOAE might serve as an examination for monitoring the maturation of preterm neonate hearing.

Muscular sarcoidosis.

A 57-year-old woman with sarcoid nodules in muscles of the lower leg was reported. A star-shaped low signal intensity in the lesion on MR imaging, supposedly corresponding to fibrous scar, appears characteristic of this condition, although its specificity is still uncertain.

[Three cases of hypersensitivity pneumonitis caused by inhalation of spores of Pholiota nameko and the background of the disease].

Here we report a new type of hypersensitivity pneumonitis caused by inhalation of spores of Pholiota nameko. P. nameko is a kind of mushroom which is of Japanese delicacy. Among people who are working in nameko cultivation, we found three patients suffering from hypersensitivity pneumonitis, and identified that it was caused by inhalation of spores of P. nameko. Every patient was engaged in indoor nameko cultivation inside a building equipped with a air and moisture conditioning system for two to ten years. In the indoor environment, it was thought that they were exposed to and inhaled a high density of nameko spores. Among 15 healthy indoor nameko growers, we found 6 who had precipitating antibody against extracts from nameko spores; however, there were no antibody positive people in 17 outdoor nameko growers. As seen in dairy farmers, it was clarified that there is a percentage of precipitin positive nameko growers who are asymptomatic. This is concrete evidence that changes of mushroom cultivation style can induce a new type of hypersensitivity pneumonitis. These findings were considered to be an important experience from the view points of environmental pulmonary diseases and industrial medicine.