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Background: There is no report on acute sensorineural hearing loss with congenital cytomegalovirus (cCMV) infection in basic experiments., Aims/objectives: The aim of this study was to evaluate the effect of dexamethasone, an anti-inflammatory steroid, on acute sensorineural hearing loss in the mouse cytomegalovirus (MCMV) infection model mice., Material and Methods: Sensorineural hearing loss model mice were divided into two groups, one with and one without intratympanic dexamethasone. Dexamethasone was injected into the tympanic cavity of only the right ear, and hearing ability was assessed at the ages of three, six, and eight weeks by auditory brainstem response measurement., Results: Among the 23 mice intratympanically injected with dexamethasone (15 μg/mouse) at the age of three weeks, five (21.7%) had a hearing improvement of at least 10 dB and 18 (78.3%) had no improvement at the age of six weeks. Among the 19 mice that did not receive a dexamethasone injection, one (5.3%) showed improvement and 18 (94.7%) showed no improvement ( p  = 0.129)., Conclusions and Significance: In this study, transtympanic infusion of dexamethasone into the tympanic cavity was effective in some mice with sensorineural hearing loss, suggesting that, in addition to angiogenesis, anti-inflammatory activity might be a mechanism of treatment for hearing loss.

Background: In central auditory disorders caused by damage of the cerebral hemispheres, there are cortical deafness and auditory agnosia. Although clinical cases of cortical deafness have been reported, little is known about the hearing problems and localized lesions associated with cortical deafness., Aims/objectives: The aims of our research are to elucidate lesion sites associated with cortical deafness and to clarify why patients with cerebral lesions are not aware of any sound at all., Materials and Methods: Three patients diagnosed as having total loss of hearing participated in this study. We conducted pure-tone audiometry, speech audiometry, distortion product otoacoustic emission (DPOAE), auditory brainstem response (ABR), and brain magnetic resonance imaging (MRI) to diagnose cortical deafness with aphasia tests of these patients., Results: Our studies showed that waves VI and VII as well as waves I to V have normal peak latencies in ABRs in all three patients. In brain MRI, we found complete damage of proximal parts of bilateral auditory radiations in the three patients., Conclusions: We propose 'subcortical deafness' as a subtype of auditory agnosia.

Background: Clinical application of electrically-evoked intracochlear auditory brainstem responses (eABRs) for evaluation of brainstem maturity or aging changes has not been well investigated., Aim/objective: We compare the eV latencies of intraoperative eABR measurements in one-year-olds, infants, children, adults, and the elderly, with the goal of investigating the changes in the brainstem auditory pathway due to development and aging., Materials and Methods: We studied 58 ears of 51 patients who underwent cochlear implantation between 2013 and 2019 using MED-EL's Concerto or Synchrony implants with Flex28 or Flex soft electrodes. EABRs were recorded during cochlear implantation. The stimuli were delivered by the MED-EL Maestro to the apical, middle, and basal turn electrodes at stimulus levels 1000, 800, and 600 cu, with a pulse width of 30 µs., Results: In eABRs recorded from electrodes installed at both the mastoid and nape, there was no difference in latency between age groups within each stimulus level., Conclusion and Significance: ABR latency was not affected by development after age one and aging of the brainstem auditory pathway. Our study will be useful as a control in identifying abnormal eABR wave configurations in patients with cochlear malformations, cochlear nerve deficiencies, or auditory neuropathy, regardless of age.

Background: The electrically evoked auditory brainstem response (eABR) during cochlear implantation in common cavity (CC) deformity has not been clinically well studied. Aim/Objective: To investigate the eABR wave configuration during cochlear implantation, the postoperative development of hearing, and educational settings in infants with CC deformity. Materials and Methods: Nine infants who were congenitally deaf and found to have CC deformity and suspected cochlear nerve deficiency by temporal bone CT and MRI were studied. All of them underwent sequential and unilateral cochlear implantation and their eABRs were recorded during the surgery. The postoperative development was followed up. Results: eABRs of all the nine infants with CC deformity showed that the mean peak of the evoked wave V (eV) was the same as that in controls at latencies but was twofold higher than that in controls at thresholds and their postoperative hearing thresholds improved markedly. Conclusion and Significance: The brainstem auditory pathways of the infants were considered intact on the basis of the eV peak latencies in the eABRs. The postoperative development of their speech and hearing abilities and their educational settings varied, probably because of the twofold higher thresholds of their eABRs and limited number of auditory neural units.

Background： The function of common cavity deformity demonstrated by temporal bone CT and MRI has been unknown., Aim/objective: To investigate the developmental changes of vestibular ocular reflex and acquisition of postural control in infants with common cavity deformity., Material and Methods: Eight infants who were congenitally deaf complicated by common cavity deformity were studied. The damped rotational chair test was carried out to evaluate vestibular ocular reflex. Acquisition of head control and independent walking in these infants was compared with that in normal infant's milestones of gross motor development., Results: All of the eight infants with common cavity deformity did not show per-rotatory nystagmus in the damped rotational chair test around the first year of life. However, a normal number of beats and a longer duration of per-rotatory nystagmus for their age were recorded at around three or four years of age., Conclusions and Significance: In the eight infants with common cavity deformity, vestibular ocular reflex was not present around the first year of life, but appeared after three or four years probably because of some vestibular sensory cells. Head control and independent walking were delayed but eventually acquired by the central vestibular compensation.

Conclusion: Each of eight patients with bilateral microtia and atresia underwent bilateral reconstruction of the auricles and external auditory canals and were fitted bilateral canal-type hearing aids in the operated ears to replace a bone conduction hearing aid. The ability to discriminate inter-aural intensity difference (IID) and even inter-aural time difference (ITD) was retained in all these patients., Objective: This study studied the post-operative sound lateralization ability of patients with bilateral microtia and atresia after total reconstruction of both auricles and external auditory canals, followed by fitting of bilateral canal-type hearing aids., Methods: Eight patients with bilateral microtia and atresia ranging in age from 13-43 years were recruited in this study. Each of them underwent bilateral reconstruction of the auricles and external auditory canals and were fitted canal-type hearing aids in both the operated ears to replace a bone conduction hearing aid. A sound lateralization test was conducted to determine IID and ITD discrimination ability thresholds., Results: In all the patients, the IID discrimination ability thresholds of the patients were more than 3-fold those of the controls, the ITD discrimination ability threshold was more than 5-fold those of controls, and binaural hearing was retained.

Conclusions: The majority of hearing loss due to mumps presents as unilateral profound sensorineural hearing loss, which is refractory to treatment. In rare cases of bilateral total deafness, cochlear implants were beneficial for speech perception. Vaccination against mumps is recommended to prevent mumps-associated hearing loss., Objective: The objective of this study is to investigate the clinical characteristics of hearing loss due to mumps and to evaluate hearing outcomes., Subjects and Methods: The clinical parameters were analyzed under a retrospective multi-institutional study design in patients diagnosed with hearing loss due to mumps at the Otolaryngology departments of 19 hospitals between 1987 and 2016., Results: Sixty-seven patients with hearing loss due to mumps were enrolled. The study population consisted of 35 males and 32 females, ranging in age from 1 to 54, with a median age of 9.5 years. Sixty-three patients presented with unilateral, and 4 with bilateral hearing loss. Profound hearing loss was observed in 65 ears. Only one ear with severe hearing loss showed complete recovery. Four patients with bilateral hearing loss received cochlear implant surgery. Most of the patients with hearing loss due to mumps had no history of vaccination.

Objectives: To present the cardiovascular risk factors in idiopathic sudden sensorineural hearing loss (SSNHL) patients enrolled in a nationwide epidemiological survey of hearing disorders in Japan., Materials and Methods: We compiled the cardiovascular risk factors in 3073 idiopathic SSNHL subjects (1621 men and 1452 women) and compared their proportions with controls as part of the National Health and Nutrition Survey in Japan, 2014. The cardiovascular risk factors consisted of drinking and smoking habits, a history of five conditions related to cardiovascular disease and body mass index., Results: The proportion of current smokers was significantly higher among men aged 50-59, 60-69 and 70+ and among women aged 30-39, 40-49 and 60-69. The proportion of patients with a history of diabetes mellitus was significantly higher among men aged 50-59, 60-69 and 70+, but not in women. In addition, male and female SSNHL subjects aged 60-69 showed lower proportions of current drinking; and female SSNHL subjects aged 60-69 showed higher proportions of overweight (BMI ≥25 kg/m 2 )., Conclusions: The present cross-sectional study revealed showed significantly higher proportions of current smokers among both men and women as well as those with a history of diabetes mellitus among men across many age groups in patients with idiopathic SSNHL compared with the controls.

Objectives: Using a large-scale nationwide survey database, we investigated the epidemiological characteristics for idiopathic SSNHL in Japan., Methods: The subjects for this analysis were patients registered in a Japanese multicentre database between April 2014 and March 2016. A total of 3419 idiopathic SSNHL patients were registered in the database, and the clinical characteristics of the idiopathic SSNHL patients were obtained. Several factors associated with the severity of hearing impairment and prognosis were then investigated. Statistical analysis was performed to clarify the factors associated with the severity of hearing impairment and prognosis., Results: There were significant correlations between the severity of hearing loss and diabetes mellitus, kidney disease, past history of brain infarction, heart disease, age (under 16 years/elderly), and symptoms of vertigo/dizziness. We also analyzed the prognostic factors for idiopathic SSNHL, and found that the severity of hearing loss (Grade 3 or 4), heart disease, aged 65 years or over, time from onset to treatment (over 7 days), and symptoms of vertigo/dizziness were all significantly related to poor prognosis., Conclusion: The present large-scale clinical survey revealed current epidemiological trends for idiopathic sudden sensorineural hearing loss (SSNHL) and various factors associated with the severity of hearing impairment and prognosis.

Objective: To investigate the hearing prognosis of idiopathic sudden sensorineural hearing loss (SSNHL) treated with different initial therapies., Methods: Subjects consisted of patients diagnosed with idiopathic SSNHL within 7 days from onset and showing severe hearing loss (≥60 dB), who were registered in a Japanese multicenter database between April 2014 and March 2016. Subjects were divided into four groups according to initial therapy: (1) steroids, (2) steroids + Prostaglandins (PGs), (3) intratympanic steroids (ITS), and (4) no steroids. Hearing outcomes were compared among the groups., Results: In total, 1305 patients were enrolled. The final hearing level and hearing gain of patients treated with steroids + PGs were significantly higher than those of patients treated with steroids alone or no steroids. The ratio of good prognosis (complete recovery or marked improvement) in patients treated with steroids + PGs was higher than that in patients treated with steroids alone or no steroids. There was no difference in the prognosis of patients treated with steroids alone or no steroids., Conclusion: A large number of patients with idiopathic SSNHL were registered in a multicenter database. PG use in combination with steroid administration was associated with a good hearing prognosis in patients with severe hearing loss.

Objectives: The aim of this study was to investigate the differences between idiopathic sudden sensorineural hearing loss (SSNHL), and acute low-tone sensorineural hearing loss (ALHL) using the results of a nationwide survey database in Japan and to analyze the variables associated with their clinical features and the severity of hearing impairment, treatment, and prognosis., Methods: Participants were patients registered between April 2014 and March 2016 in a multicenter epidemiological survey database involving 30 university hospitals and medical centers across Japan. Statistical analysis was performed to clarify the factors associated with their clinical characteristics and the severity of hearing impairment, treatment, and prognosis., Results: Idiopathic SSNHL and ALHL differed significantly in terms of male-to-female ratio, age distribution, and time from onset to start of treatment. The treatment methods and hearing prognosis also differed markedly between the two diseases. A majority (92%) of idiopathic SSNHL patients were administered some type of corticosteroid, while half of the ALHL patients received corticosteroids and a diuretic agent., Conclusion: The results suggested that idiopathic SSNHL and ALHL belonged to different categories of inner ear disease.

Objectives: To evaluate the differences between acoustic trauma (AT) and other types of acute noise-induced hearing loss (ANIHL), we performed a literature search and case reviews., Methods: The literature search based on online databases was completed in September 2016. Articles on ANIHL and steroid treatment for human subjects were reviewed. The source sounds and treatment sequelae of our accumulated cases were also reviewed. Hearing loss caused by gun-shots and explosions was categorized into the AT group, while hearing loss caused by concerts and other noises was categorized into the ANIHL group., Results: Systemic steroid treatment did not appear to be effective, at least in the AT group, based on both the literature and our case reviews. However, effective recovery after treatment including steroids was observed in the ANIHL group. The difference in hearing recovery between the AT and ANIHL groups was statistically significant (p = .030), although differences in age, days from the onset to treatment and pretreatment hearing levels were not significant., Conclusions: Hearing recovery from AT is very poor, whereas, ANIHL is recoverable to some extent. Therefore, it is essential to differentiate between these two groups for accurate prediction of the hearing prognosis and evaluation of treatment effects.

Objectives: A nationwide epidemiological survey involving 23 hospitals in Japan was conducted and the predictive values of demographic data were examined statistically., Methods: A total of 642 patients from 23 hospitals, including 20 university hospitals, in Japan were enrolled in the study. Age ranged from 8 to 87 years, and all were diagnosed with acute low-tone sensorineural hearing loss (ALHL) between 1994 and 2016. Demographic data for the patients, such as symptoms, gender, mean age, and distribution of ALHL grading, were collected and analyzed in relation to prognosis using Student's t-test, χ 2 test and logistic regression., Results: Female gender (p < .013), younger age (p < .001), low-grade hearing loss (p < .001), and shorter interval between onset and initial visit (p < .004) were significantly predictive of a good prognosis. The prognosis for definite ALHL was significantly better than that for probable ALHL (p < .007)., Conclusions: The severity of initial hearing loss, interval between onset and initial visit and age were important prognostic indicators for ALHL, while female gender was an important prognostic indicator peculiar to ALHL.

Conclusion: In patients with unilateral microtia and atresia after reconstruction of the auricle and external canal and fitting of a canal-type hearing aid for the operated ear, the ability to discriminate the inter-aural intensity difference (IID) was acquired in all of the patients, whereas that to discriminate inter-aural time difference (ITD) was acquired in one-half of the patients., Objective: To study the post-operative sound lateralization ability in patients with unilateral microtia and atresia after reconstruction surgery of the auricle and external canal and fitting of a canal-type hearing aid of the operated ear., Methods: Eighteen patients with unilateral microtia and atresia ranging from 13-24 years of age were recruited in this study. All of them underwent reconstruction of the auricle and external canal and were fitted a canal-type hearing aid for the operated ear. The sound lateralization test was conducted to determine IID and ITD using a self-recording apparatus. The test stimulus was a continuous narrow-band noise at 500 Hz and 50 dBHL presented to the right and left ears through the air conduction receivers., Results: IID could be measured in all of the patients, whereas ITD could be measured in only nine out of the 18 patients. Post-operative binaural hearing could be acquired in all the patients.

Conclusion: A right-handed 38-year-old man's complete loss of hearing could be diagnosed as cortical deafness caused by cerebral vascular lesions in bilateral auditory cortices., Objective: The aim of this case report was to study the pathophysiology of a particular patient who manifested deafness without residual hearing and lost somatosensory sensation, vestibular sensation, and articulation ability after a right internal carotid-posterior communicating artery (IC-PC) aneurysm and subarachnoidal hemorrhage., Methods: MRI, aphasia and neurological tests, subjective and objective audiometry, and vestibular function tests were performed., Results: The neurological test revealed system loss of somatosensory sensation with normal motor function and articulation ability. Brain imaging revealed extensive infarction in the bilateral primary auditory cortices, postcentral gyruses, and the bilateral partial third frontal gyruses. Pure-tone audiometry of both ears revealed off-the-scale results and speech audiometry demonstrated 0% maximum speech discrimination. However, objective audiometry showed normal distortion product otoacoustic emissions (DPOAE) and normal auditory brainstem response (ABR). The patient showed 0% perception of environmental, speech, and music sounds in both ears. He was unable to feel vestibular sensation despite normal caloric nystagmus. He showed no damage to his larynx or articulation organs.

Conclusions: The functional connectivity (FC) between the right and left auditory cortex is weak in tinnitus patients. Transcranial direct current stimulation (tDCS) over the auditory cortex has potential as a tool to modulate auditory-based FC., Objective: This study investigated the effects of applying tDCS in tinnitus patients, and searched for modulation of brain networks in resting-state functional magnetic resonance imaging (rs-fMRI) through an analysis of FC with the stimulated brain region., Subjects and Methods: Nine male patients with chronic tinnitus and 10 male volunteers with normal hearing were enrolled. The subjects were evaluated with rs-fMRI immediately before and after tDCS. The tinnitus patients filled out the self-evaluation questionnaires designed to measure tinnitus conditions before tDCS treatment and 1 week afterwards., Results: The FC between the right and left auditory cortex was significantly weaker in tinnitus patients than in controls. After tDCS treatment, in the tinnitus group, the primary auditory cortex showed a reduction in the amount of statistically significant connectivity with the somatosensory area and motor area, but maintained strong significant connectivity (p < 0.005) with the auditory area and insular cortex. In contrast, in the control group, there remained strong significant connectivity between the primary auditory cortex and the somatosensory area, motor area, insular cortex, and auditory area.

Conclusions: EABR is a reliable and effective way of objectively confirming device function and implant-responsiveness of the peripheral auditory neurons up to the level of the brainstem in cases of inner ear malformation., Objective: To investigate the usefulness of measuring the intra-operative electrically evoked compound action potential (ECAP) and electrically evoked auditory brainstem response (EABR) in patients with and without congenital inner ear anomalies during cochlear implantation., Method: Thirty-eight consecutive children (40 ears) aged 5 or younger with congenital profound hearing loss. Twenty-four (25 ears) lacked congenital inner ear anomalies. The 14 patients (15 ears) with a malformation had common cavities (four ears), incomplete partition type I (three ears), cochlea hypoplasia type III (three ears), enlarged vestibular aqueduct (four ears), and cochlear nerve canal stenosis (one ear). Main outcome measures are ECAP and EABR responses., Results: Of the 25 ears lacking any malformation, 21, three, and one showed 'Good', 'Variable', and 'No' ECAP responses, respectively, and 24 and one showed 'Good' and 'Variable' intra-cochlear responses, respectively. Of the 15 ears with a malformation, two showed 'Good' ECAP responses, nine had 'Variable' ECAP responses, and four showed 'No' ECAP responses. Moreover, five showed 'Good' EABR responses and 10 showed 'Variable' EABR responses.

Conclusions: When vestibular function is reduced in the rotational chair test in children with severe hearing loss, the vestibular function may be acquired later due to maturing vestibular sensory cells and vestibular nerve of the inner ear along with physical growth., Objectives: To examine the relationship between acquisition of motor function and vestibular function in children with bilateral severe hearing loss., Methods: A total of 97 children under 4 years old with hearing loss defined as a hearing threshold of both ears greater than 80 dB were included in this study. For evaluation of vestibular function, a damped-rotational chair test was performed and the horizontal nystagmus was recorded using electronystagmography (ENG)., Results: Head control and independent walking were delayed in 28 of 97 children with severe hearing loss. Reduced response to the rotational chair test was observed in 16 of 97 children (16.5%), with 11 of these children having inner ear anomalies and reduced vestibular function. Of the 10 children who were followed up by the rotational chair test, 2 children with idiopathic congenital hearing loss without inner ear anomalies (100%) and 6 of 8 children with bilateral inner ear anomalies (75%) showed more obvious nystagmus during rotation compared with the initial examination.

Pelizaeus-Merzbacher disease (PMD) is a white matter dystrophy of the brain. Most children with PMD require comprehensive nursing care. Their speech and language abilities are poor or absent. Therefore, evaluating hearing ability is difficult in children with PMD. We have followed up two patients with PMD since early childhood. Patient 1 is an 11-year-old boy, and patient 2 is a 15-year-old adolescent boy in whom horizontal nystagmus was recognized after birth. Magnetic resonance imaging showed diffuse dysmyelination of the cerebral white matter. Auditory brainstem response recordings showed only waves I and II and the absence of all subsequent components. However, conditioned orientation reflex audiometry showed a threshold of 20-30 dB. Both patients can converse orally and have auditory perception and speech abilities better than those of most patients with PMD in the literature. We report on the development of their hearing and speech abilities.

The case of an 8-year-old girl who manifested cortical blindness and whose color drawings of faces and objects were without outlines is reported. Her birth was uneventful. When she was 10 months old, she fell down to the floor from a chair, resulting in a subarachnoidal hemorrhage. A repeat brain MRI revealed localized lesions in the visual cortices in the right and left hemispheres. As she grew older she was found to have visual imperceptions. She was found to have difficulties in learning visually the names of objects with form and letters, and in recognizing faces of her family. However, she was able to discriminate well the colors of faces and objects and learn easily the names of the objects with form by touching. She seemed to utilize subcortical vision for seeing colors of faces and objects.

A 27-year-old female showed auditory agnosia after long-term severe hydrocephalus due to congenital spina bifida. After years of hydrocephalus, she gradually suffered from hearing loss in her right ear at 19 years of age, followed by her left ear. During the time when she retained some ability to hear, she experienced severe difficulty in distinguishing verbal, environmental, and musical instrumental sounds. However, her auditory brainstem response and distortion product otoacoustic emissions were largely intact in the left ear. Her bilateral auditory cortices were preserved, as shown by neuroimaging, whereas her auditory radiations were severely damaged owing to progressive hydrocephalus. Although she had a complete bilateral hearing loss, she felt great pleasure when exposed to music. After years of self-training to read lips, she regained fluent ability to communicate. Clinical manifestations of this patient indicate that auditory agnosia can occur after long-term hydrocephalus due to spina bifida; the secondary auditory pathway may play a role in both auditory perception and hearing rehabilitation.

The influence of aging on hearing and vestibular function in patients with auditory neuropathy has not been investigated. The purpose of this study was to investigate how hearing and vestibular function in this disease change with aging. The subjects were three female patients with auditory neuropathy. We checked their hearing and vestibular function by speech discrimination tests, ABR, ECochG, DPOAE, caloric test, damped-rotational chair test, and VEMPs. In all three patients, speech discrimination ability and vestibular function markedly declined with aging. However, speech language understanding and higher brain function were less affected by aging.

In 1999, three workers received high doses of radiation in a small Japanese plant while they were preparing fuel for an experimental reactor. This criticality accident at melting point was caused by the addition of too much uranium enriched to a relatively high level, causing a 'criticality' (a limited uncontrolled nuclear chain reaction), which continued intermittently for 20 h. The three workers concerned were hospitalized, two in a critical condition. The first worker died 12 weeks later, and the second worker 7 months later. The third worker is in a healthy condition. We report on the temporal bone histopathological features of the second worker. Our temporal bone study revealed: 1) the large loss of bone marrow tissue with a small number of myelocytes remaining in the mastoid bone and the abundance of fatty tissue in the mastoid bone, 2) inflammation of the mucosal layer of the middle ear and the mastoid air cells, 3) mild degeneration of the spiral ganglions and the sensory hair cells of the cochlea, 4) mild degenerative changes of sensory hair cells of the semicircular canals and otolith organs, and 5) vestibular ganglions and geniculate ganglions were well preserved.

Conclusion: GJB2 mutations are responsible not only for deafness but also for the occurrence of vestibular dysfunction. However, vestibular dysfunction tends to be unilateral and less severe in comparison with that of bilateral deafness., Objectives: The correlation between the cochlear and vestibular end-organs suggests that some children with congenital deafness may have vestibular impairments. On the other hand, GJB2 gene mutations are the most common cause of nonsyndromic deafness. The vestibular function of patients with congenital deafness (CD), which is related to GJB2 gene mutation, remains to be elucidated. The purpose of this study was to analyze the relationship between GJB2 gene mutation and vestibular dysfunction in adults with CD., Methods: A total of 31 subjects, including 10 healthy volunteers and 21 patients with CD, were enrolled in the study. A hearing test and genetic analysis were performed. The vestibular evoked myogenic potentials (VEMPs) were measured and a caloric test was performed to assess the vestibular function. The percentage of vestibular dysfunction was then statistically analyzed., Results: The hearing level of all CD patients demonstrated a severe to profound impairment. In seven CD patients, their hearing impairment was related to GJB2 mutation. Five of the seven patients with CD related to GJB2 mutation demonstrated abnormalities in one or both of the two tests. The percentage of vestibular dysfunction of the patients with CD related to GJB2 mutation was statistically higher than in patients with CD unrelated to GJB2 mutation and in healthy controls.

Conclusions: Our study revealed that VEMPs in responses to clicks and 500 Hz STB were abnormal in 25% of athletes with congenital profound sensorineural hearing loss for the Deaf Olympic Games. These results confirm that a part of deaf athletes with congenital vestibular failure is well compensated for higher level sport activities., Objectives: The aim of this study was to investigate the appearance rate of VEMP, vestibular failure and its compensation in Japanese deaf athletes by the VEMP test, caloric test and balance test., Methods: Sixteen subjects among the Deaf Olympic Games athletes of Japan volunteered to participate in this study. All the subjects have congenital profound sensorineural hearing loss., Results: In the VEMP test, 24 ears (75%) showed normal click-evoked responses, 5 ears (16%) showed decreased click-evoked responses, 3 ears (9%) showed no click-evoked responses, 28 ears (87.5%) showed normal 500 Hz short tone burst (STB)-evoked responses and 4 ears (12.5%) showed decreased responses. In the ice-water caloric test, 11 subjects (69%) showed normal responses bilaterally, 3 subjects showed bilateral responses with asymmetrical responses and 2 subjects (12%) showed no response bilaterally. All the subjects showed normal balance test results.

We have developed a special purpose adaptor making it possible to use a commercially available high-speed camera to observe vocal fold vibrations during phonation. The camera can capture dynamic digital images at speeds of 600 or 1200 frames per second. The adaptor is equipped with a universal-type attachment and can be used with most endoscopes sold by various manufacturers. Satisfactory images can be obtained with a rigid laryngoscope even with the standard light source. The total weight of the adaptor and camera (including battery) is only 1010 g. The new system comprising the high-speed camera and the new adaptor can be purchased for about $3000 (US), while the least expensive stroboscope costs about 10 times that price, and a high-performance high-speed imaging system may cost 100 times as much. Therefore the system is both cost-effective and useful in the outpatient clinic or casualty setting, on house calls, and for the purpose of student or patient education.

Conclusions: Our results show that among the patients with inner ear malformations, there were two patients with sensory cells of sacculus and inferior vestibular neurons and at least five patients with inferior vestibular neurons only, but no patients without sensory cells and vestibular neurons, as determined on the basis of vestibular evoked myogenic potentials (VEMPs)., Objective: The aim of this study was to investigate whether sensory cells of sacculus or inferior vestibular neurons or both are present in cochlear implant patients with inner ear malformations, particularly common cavity (CC) deformity, using VEMPs., Patients and Methods: Seven children with inner ear malformation who underwent cochlear implantation (CI) participated in this study. The patients had CC deformity (n=2), incomplete partition type I (n=2), incomplete partition type II (n=1), or a narrow internal auditory canal (IAC) (n=2). It was possible to record VEMPs before and after CI in three patients and not before but after CI in four patients. After surgery, VEMPs were recorded with the cochlear implant device switched both off and on., Results: Before the CI, two patients showed VEMPs and one patient showed no VEMPs, whereas in four patients no VEMPs could be recorded. All the patients showed VEMPs with the cochlear implant on.

Conclusion: We regard spontaneous otoacoustic emission (SOAE) as a unique phenomenon due to spontaneous outer hair cell hyperactivity during the infantile period in these two infants., Objectives: We report the cases of SOAEs in two infants, which were audible to their parents. We were able to hear continuous sounds from these two infants' ears. We studied their hearing and the emitted sounds objectively., Patients and Methods: Patient 1 was a 5-month-old girl and patient 2 was an 11-month-old girl. Their parents heard continuous sounds emanating from their infants' ears. Audiological examinations and frequency analysis were conducted., Results: The distortion-product otoacoustic emissions (DPOAEs) and auditory brainstem responses (ABRs) of the two patients were normal. Analysis of the continuous sounds demonstrated frequencies of 4.2 and 6.5 kHz for patient 1 and 6.7 kHz for patient 2. These audible sounds were diagnosed as SOAEs. The SOAEs in these infants did not disappear with their development in the last 2 years.

Conclusions: This study showed that delayed auditory pathway myelination is common in Pelizaeus-Merzbacher disease (PMD), but this delay does not necessarily indicate poor hearing function., Objective: PMD is a rare recessively inherited X-linked leukodystrophy characterized by defective central nervous system myelination owing to a mutation in the proteolipid protein gene (PLP). The aims of this study were to evaluate the hearing function and auditory brain response (ABR) findings of patients with PMD and relate these findings to MRI-assessed myelination in the central auditory pathway., Patients and Methods: We retrospectively studied eight male pediatric patients with PMD. Serial auditory examinations included audiometry, behavior audiometry, distortion product otoacoustic emission (DPOAE), and ABR. MRI-assessed myelination in the auditory pathway was evaluated in the PMD patients and in 23 normal young children as a control group., Results: Audiometry showed normal to moderate hearing impairment and the hearing threshold improved with age and became almost normal over time. DPOAEs positivity and only ABR wave I or waves I and II were found in all the patients. MRI showed delayed myelination in all the patients and the auditory pathway was myelinated up to the inferior colliculus in four cases and up to the medial geniculate body in four cases. Serial MRIs showed no progression in myelination. No clear relation was found between hearing threshold and MRI-assessed myelination in the auditory pathway.

Conclusions: This study showed that vestibular evoked myogenic potentials (VEMPs) evoked by cochlear implant (CI), could be related to the comfortable level (C level), particularly in the channels that are closer to the apical turn of the cochlea., Objective: The purpose of this study was to investigate the correlation between VEMPs and C level of each channel., Subjects and Methods: We investigated 24 children who underwent cochlear implantation. VEMPs were recorded from the operated ears with the CI switched 'off' or 'on'. To investigate the correlation between VEMPs and C level, we selected 13 patients with Nucleus 24 (SPrint), and divided them into group A (normal VEMPs) and B (absence of VEMPs). In these children, all the 22 electrodes were active, and were mapped in the same frequency range for each channel., Results: Twenty children (83%) showed no VEMPs with the CI 'off'. Among them, 10 elicited VEMPs with the CI 'on', but the other 10 did not. In all channels, the mean C levels of CI were higher in group A than in group B. The p values in channels 1-12 were >0.10, in channels 13-16 were 0.06-0.09, and in channels 17-22 were 0.05-0.06, which were lower but not statistically significant.

We report a case in which metastasis occurred from a left-side maxillary carcinoma to bilateral temporal bones through different routes, manifested by rapidly progressing left-side mixed hearing loss, left-side vestibular dysfunction, and serous otitis media. Later the left-side hearing threshold became severely elevated, suggesting profound sensory hearing loss. Histopathology of the temporal bones revealed that the side with the lesion was severely damaged by tumor through direct and hematogenous metastasis. On the contralateral side, it showed four findings: (i) sparse and separate tumor invasion of the petrous bone, the mastoid cavity, and the facial canal (hematogenous spread); (ii) tumor involvement in the lower part of the cochlear aqueduct without invasion of the internal acoustic canal or cochlea, implying early meningeal carcinomatosis; (iii) vascular stria atrophy, spiral ganglion diminution, and well preserved hair cells; and (iv) diffuse effusion in the middle ear and mastoid cavity. Our observations revealed that tumor cells dispersed to the same side through different routes, whereas early metastasis to the contralateral side was mainly through hematogenous and subarachnoid spread.

This report shows the changes that occurred in consecutive audiograms of a patient who underwent chemotherapy and radiotherapy for nasopharyngeal cancer and the histopathological examination of the temporal bones. Both conductive and sensorineural hearing loss developed, but followed different modes of progression. In the left ear, an air-bone gap appeared and deepened, while in the right ear, severe conductive hearing loss was present upon admission and improved after treatment. Sensorineural hearing loss was worse in the right ear, but deteriorated gradually in both ears. Histology revealed tumor invasion in the right temporal bone. Both middle ears showed effusion, but no radiation-induced changes that may be responsible for the conductive loss. The main changes in the cochlea were vascular stria degeneration, spiral ligament atrophy, and spiral ganglion cell depletion, while the hair cells were only occasionally missing. Apart from confirming what has been established previously by other authors, some interesting findings were observed: (1) in addition to the high frequency hearing loss typically caused by cis-platinum and by radiation, there was also low frequency hearing loss, and (2) the cochlear damage was most severe in the vascular stria and spiral ganglions, sparing the hair cells.

Conclusions: While infants under the age of 1 year exhibited a high rate of abnormal intensities in the middle ear and mastoid cavities, older infants showed no abnormal intensities in these regions. From the signal intensity on T1- and T2-weighted MRI, the abnormal intensities in the middle ear cavity were considered to represent liquid effusion. Taken together with the findings of temporal bone CT, the abnormal intensities in the mastoid cavity were considered to represent bone marrow., Objective: Histopathological studies of the temporal bone and tympanometry investigations have reported the presence of mesenchyme and liquid effusion in the middle ear cavity of infants. However, very few CT or MRI middle ear cavity findings of newborns and infants have been published, and none have included the mastoid cavity. We therefore performed an MRI study of the middle ear and mastoid cavities of infants under 2 years old (83 cases, 88 imaging series)., Subjects and Methods: MRI (1.5 T) was originally performed on suspicion of brain disorders in infants aged under 2 years. All MRI slices were studied and classified on the basis of the distribution of abnormal intensities in the middle ear and mastoid cavities., Results: All the abnormal imaging appeared in infants under 1 year old, in particular, 74.24% (n=49) of abnormal imaging appeared in the first 20 weeks after birth.

Conclusions: The vestibular function can be assessed by ice-water caloric test, rotational chair test and VEMP recording in severely hearing impaired infants and young children, and 85% of these patients showed abnormal responses in these tests., Objectives: To evaluate the vestibular function of infants and young children with congenital and acquired deafness, we examined the semicircular canal and otolith function in their early childhood., Materials and Methods: Our subjects were 20 children (11 boys, 9 girls; age range 31-97 months, mean age 54.2 months) with severe hearing impairment. Their vestibular functions were assessed by the ice-water caloric test, rotational chair test and vestibular-evoked myogenic potential (VEMP) recording., Results: Among these 20 severely hearing impaired children, only 3 (15%) showed normal responses in the caloric test, rotational chair test and VEMP recording bilaterally. Seven (35%) showed responses asymmetrically in the caloric test despite normal responses in the rotational chair test and VEMP recording bilaterally. Five (25%) showed hyporeflexia or areflexia in the caloric test bilaterally, but showed normal responses in the rotational chair test and normal reproducible or decreased VEMPs. Five (25%) showed no responses at all in the caloric test, rotational chair test and VEMP recording.

The relationship between meningitis and sensorineural hearing loss (SNHL) has long been studied. Many histopathological studies of animal models and human temporal bones with respect to bacterial meningitis have been carried out. However, the relationship between SNHL and tuberculous meningitis was seldom addressed and the pathophysiology remains unclear. We carried out temporal bone studies on material from a 22-year-old patient who developed a right unilateral SNHL before dying from tuberculous meningitis. The histopathological findings for the right temporal bone were as follows: (1) inflammation mainly appeared in the internal auditory canal, modiolus and Rosenthal's canal and extended to the osseous spiral ligament, whereas the perilymphatic spaces were less involved; (2) the organ of Corti, cochlear nerve fibres and spiral ganglion cells were severely degenerated, particularly in the basal and middle turns; (3) the contralateral side (for which the patient had no complaints) showed an inner space free from inflammation, but some granulomatous formations were observed in the middle ear cavity. We conclude that the modiolus and cochlear aqueduct are the main routes for the spread of infection from the meninges to the inner ear. The progression of hearing loss resembles that of bacterial meningitis and shares attributes of retrocochlear SNHL.

Conclusions: Patients treated by mastoidectomy in the past often present with hearing loss and cavity problems such as pus discharge. Total middle ear reconstruction (TMER) improves the hearing of these patients by correcting cavity problems and resolution of ear discharge, which facilitates ossicular chain reconstruction such as type III or type IV tympanoplasty., Objective: To evaluate the effectiveness of TMER in improving hearing., Patients and Methods: We reviewed the audiograms of 56 ears of 48 patients who underwent TMER in combination with either type III or type IV tympanoplasty. Audiometric pure tone thresholds averaged over three frequencies (500, 1000, and 2000, pure tone average) were measured and compared before and after surgery. Successful outcome was defined as improvement of 15 dB or more. The mean follow-up was 5.7 years (range 1.1-12.6). We also analyzed the relations between hearing improvement and factors such as type of tympanoplasty (types III and IV), choice between one-stage and two-stage operation, and the interval between original mastoidectomy and final operation., Results: The mean hearing gain was 13.6 (+/-11.9) dB. Twenty-seven procedures (48.2%) were considered successful, with improvement of 15 dB or more. The results of type III tympanoplasty group were significantly superior to those of type IV (p<0.05, Student's t test). One- and two-stage surgery did not significantly influence outcome. The interval between the initial operation correlated weakly and negatively (r = - 0.266, p<0.05) with hearing gain.

Conclusion: Our bedside interviews with patients with head neck cancer are unique and valuable educational experiences for undergraduate medical students., Objective: How can we help medical students to understand patients' thoughts and feelings? We believe that students can learn and practice empathy at the patient's bedside., Subjects and Methods: Sixty medical students in their final year at the University of Tokyo and 10 patients with head and neck cancer were supervised by a professor and senior head and neck surgeons. Questionnaires were delivered to each student before and after bedside teaching, to investigate their impressions on thoughts and feelings of patients with head and neck cancer. Questions to patients were composed of: 1) backgrounds: birthplace, education, occupation and hobby; 2) cancer: the time of the first visit to clinic and the time of referral to the University of Tokyo Hospital; 3) preoperative feelings; 4) impression of surgical treatment and postoperative care; and 5) messages to medical students from the patient. Medical students also asked additional questions., Results: Before the bedside teaching our medical students believed that it must be important to listen to patients (100%); and that it must be possible to encourage patients (50%). After the bedside teaching they noticed that a medical interview could have a positive influence on the patient (93%), probably because of general conversation (45%), the patient's personality (50%), listening to information about the patient's birthplace, education, occupation and hobby (48%), and the instructor's life experiences (25%). Most patients asked medical students to be good doctors who can understand their thoughts and feelings.

Conclusions: A new giant magnetostrictive bone conduction transducer (GMT) measuring 8 mm (length) by 2 mm (diameter) is capable of outputting sounds over a wider range of frequencies than conventional electromagnetic transducers (EMTs). The GMT can vibrate the rat skull about 3-100 times faster than the EMT, especially above 2 kHz., Objectives: Since the GMT can generate high sound quality and output sounds above 10 kHz, we investigated the width of the frequency response of the GMT and evaluated its mechanical performance., Materials and Methods: We chose Wistar rats to estimate the GMT performance by measuring the bone-conducted ABR and the skull vibratory velocity because their hearing frequency range extends from 0.15 kHz to 67 kHz. We also measured the bone-conducted auditory brainstem responses (ABRs) and the skull vibratory velocity of rats with the EMT. Skull vibratory velocity was measured with a laser Doppler vibrometer (LDV)., Results: The skull vibratory velocity at the ABR threshold obtained with each transducer was within the -35 to -50 dB range (0 dB re 1 mm/s). When 1 Vpp was applied to each transducer, the GMT had a higher velocity at frequencies of 2-30 kHz, and the EMT at frequencies of 0.5-1 kHz.

Conclusions: Our results suggest that the sacculi of most children with cochlear implants can easily be damaged, as shown by the absence of vestibular-evoked myogenic potentials (VEMPs) in response to click stimuli. Also, in most of the children, the vestibular nerve was seemingly not stimulated by the cochlear implant. These results suggest that electrical stimulation at the C level can stimulate the cochlear nerve; however, this stimulation did not spread to the vestibular nerve in our children. In some children with Mondini dysplasia or vestibulocochlear nerve abnormality, the vestibular nerve was stimulated when the cochlear implant device was on, because of a VEMP response to electrical stimulation., Objective: To clarify the diagnostic value of VEMPs in cochlear implant patients., Material and Methods: The click-evoked myogenic potentials of 12 children who underwent cochlear implantation surgery were investigated. The latency and amplitude of the VEMP responses were measured., Results: Before surgery, 6 of the 12 children showed normal VEMPs, 1 showed a decrease in the amplitude of VEMPs and five showed no VEMP response. After surgery, with the cochlear implant device off, 1 child showed a decreased VEMP and 11 showed no VEMPs. With the cochlear implant device on, four children showed VEMPs and eight did not.

The aim of this study was to investigate auditory cortex function in the context of auditory stimuli in a patient with auditory agnosia due to bilateral lesions confined to the auditory radiations. A male patient experienced mild left temporal hemiplegia because of right putaminal hemorrhage at the age of 43 years. Thereafter he recovered completely but hypertension persisted. When he was 53 years old, he suffered left putaminal hemorrhage and went into a coma. After recovering from the coma and right hemiplegia he could hear but could not discriminate speech sounds. Brain CT and MRI demonstrated small bilateral lesions confined to the auditory radiations. Magnetoencephalography demonstrated the disappearance of middle latency responses and auditory-evoked potential studies showed a very small Pa peak. In contrast, a positron emission tomography study demonstrated a marked increase in blood flow in the bilateral auditory cortex in response to both click and monosyllable stimuli. It is speculated that the auditory cortex receives functional projections from the cochlea via non-specific pathways in the cerebral hemispheres.

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.

Three young adult males with Pelizaeus-Merzbacher disease have been followed up since childhood. This disease is thought to be a dysmyelinating disorder of the brain during the prenatal period caused by gene mutations. The patients manifested horizontal nystagmus and severe rigidity of the extremities. Although the patients showed only waves I and II in auditory brainstem responses, they had relatively good hearing ability at approximately equal to dB. They could not speak words at all but could hear well and enjoy listening to conversation and music. One of them had a normal hearing threshold in pure-tone audiometry and a normal speech discrimination rate in speech audiometry. This can be explained by a nerve conduction blockade through dysmyelinated axons or the desynchronization of neurons and nerves responsible for the waves following waves I and II. At present, all three patients are living with their families. We report their present hearing, speech and language abilities.

Conclusions: In a patient with bilateral auditory neuropathy (AN), the vestibular-evoked myogenic potential (VEMP) was probably absent because of a neuropathy involving the inferior vestibular nerve and/or its end organ, the saccule. Our result can therefore be interpreted as a concomitant unilateral sacculo-collic neuropathy. We suggest the use of more precise terms to characterize AN patients with involvement of different parts of the inner ear and its innervations. We encourage detailed vestibular assessment in patients with AN in order to assess the co-existence of any symptomatic or asymptomatic vestibular disorder. Information such as that provided in this report will be valuable for clinicians caring for this group of patients., Objective: AN is a disorder characterized by the absence or severe impairment of auditory brainstem responses in the presence of normal cochlear outer hair cell function as revealed by otoacoustic emissions (OAEs) and/or electrocochleography (ECoG). A variety of processes and etiologies are thought to be involved in its pathophysiology. In most literature reports the auditory profile of patients with AN is discussed. However, the extent of vestibular involvement, especially that involving the saccule, is not known. We performed vestibular tests to assess the status of the saccule in a patient with AN., Material and Methods: One patient with AN was studied. The patient was a right-handed 21-year-old female with chief complaints of hearing loss and speech perception difficulty., Results: The auditory test results were consistent with the diagnosis of AN, i.e. absent auditory brainstem responses, moderate hearing loss, an inappropriately profound speech discrimination score and the presence of OAEs and measurable cochlear microphonics on ECoG. On neurological examination, gait and balance tests were normal. Ice-water caloric testing induced a sensation of dizziness in both ears. Short tone-burst VEMPs showed no response on left-ear stimulation and a biphasic response with normal latency and amplitude on right-ear stimulation.

Conclusions: Hearing loss and equilibrium dysfunction have different etiologies in patients with large vestibular aqueduct syndrome. We suggest that all children with distal renal tubular acidosis (dRTA) should be subjected to an equilibrium study and audiological evaluation, as well as to a CT or MRI scan., Objective: dRTA has been described in association with sensorineural hearing loss, but there are no reported cases that have been examined in detail using audiological and equilibrium studies. We report here a case of progressive sensorineural hearing loss with a large vestibular aqueduct and dRTA, and the results of audiological and equilibrium studies., Material and Methods: A 31-year-old female presented with hearing loss, tinnitus and vertigo. She had been treated with oral sodium citrate, potassium citrate and potassium chloride supplementation because of dRTA since the age of 1 month., Results: The pure-tone audiogram of the patient was off the scale for the right ear and showed progressive sensorineural hearing loss for the left ear. Ice-water caloric testing showed canal paresis on the left side. Temporal bone CT and inner ear MRI revealed a large vestibular aqueduct and a large endolymphatic sac on both sides.

Conclusion: These results suggest that COX-2 and bcl-2 protein were overexpressed and that apoptosis was reduced in MEC compared to PMA, and that COX-2 may regulate the degree of apoptosis by modulating bcl-2 protein in PMA and MEC., Objective: Cyclooxygenase (COX)-2 plays a crucial role in tumorigenesis and overexpression of COX-2 in vitro accompanied by overexpression of bcl-2 protein has been shown to reduce apoptosis. The purpose of this study was to verify that COX-2 regulates the degree of apoptosis by modulating bcl-2 protein in benign and malignant parotid gland tumors. : We examined archival formalin-fixed, paraffin-embedded tissue sections of 10 pleomorphic adenomas (PMAs) and 10 mucoepidermoid carcinomas (MECs) by immunostaining with anti-COX-2, anti-bcl-2 and anti-single-stranded DNA (ssDNA) antibodies. Labeling indices of the three antibodies were calculated using computer-assisted image analysis., Results: Labeling indices (mean+/-SD) of anti-COX-2 antibody in PMA and MEC were 2.05+/-1.30 and 11.2+/-2.95, respectively (p < 0.001), those of anti-bcl-2 antibody were 2.00+/-1.28 and 9.68+/-4.05, respectively (p < 0.001) and those of anti-ssDNA antibody were 8.06+/-2.54 and 2.08+/-1.47; respectively (p <0.001). Correlation coefficients between the labeling indices of anti-COX-2 antibody and anti-bcl-2 antibody, anti-bcl-2 antibody and anti-ssDNA antibody and anti-COX-2 antibody and anti-ssDNA antibody were 0.88, -0.75 and -0.76, respectively (p <0.001).

Objective: To demonstrate the influence of auditory cortex lesions on auditory middle-latency responses (AMLRs) and middle-latency auditory-evoked magnetic fields (MLAEFs) in humans., Material and Methods: A total of 15 normal subjects, 9 patients with left auditory cortex lesions and 1 patient with a right auditory cortex lesion were studied. MLAEFs were recorded from each hemisphere of the brain in a magnetically shielded room using a 37-channel SQUID gradiometer. Simultaneously, AMLRs were recorded from the scalp at the vertex, C3 and C4. Tone bursts were used as auditory stimuli., Results: Pam responses of the MLAEF, which are typically evoked in the latency range of the Pa of the AMLR, and are localized at the auditory cortex as dipoles, were impaired or abolished over the left auditory cortex lesion in the patients with left-hemisphere lesions, but the Pa of the AMLR persisted., Conclusion: The main generator of the Pam in MLAEF was demonstrated to be the auditory cortex. The results also show that the Pa of the AMLR is evoked only partly from the auditory cortex.

Generally, cortical deafness is not complicated by anarthria and cortical anarthria does not affect auditory perception. We report a case of simultaneous progressive cortical deafness and anarthria. At the age of 70 years, the patient, a woman, noticed hearing problems when using the telephone, which worsened rapidly over the next 2 years. She was then referred to our hospital for further examinations of her hearing problems. Auditory tests revealed threshold elevation in the low and middle frequencies on pure-tone audiometry, a maximum speech discrimination of 25% and normal otoacoustic emissions and auditory brainstem, middle- and long-latency responses. An articulation test revealed abnormal pronunciation. Because of these problems only written and not verbal communication was possible; her ability to read and write was unimpaired. She showed no other neurological problems. Brain MRI demonstrated atrophic changes of the auditory cortex and Wernicke's language center and PET suggested low uptake of (18F) 2-fluoro-2-deoxy-d-glucose around the Sylvian fissures in both hemispheres. Neurologically, the patient was suspected of having progressive aphasia or frontotemporal dementia. Her cortical deafness and anarthria are believed to be early signs of this entity.

Four pediatric patients whose bilateral auditory cortices were damaged by herpes encephalitis at an early age were studied. Their brain CT and MRI scans demonstrated common bilateral lesions of the auditory cortices. Their auditory perception was investigated by means of behavioral and objective hearing tests and auditory perception tests. All four patients showed mild or moderate hearing loss in the behavioral hearing test and normal auditory brainstem responses but did not manifest total deafness. Moreover, perception tests involving speech, environmental sounds and music demonstrated that most auditory perception ability had been lost in all patients. On reaching school age, the patients were enrolled in schools for the deaf or special schools for handicapped children.

Peptide growth factors and cytokines modulate both normal and impaired wound healing. Topical application of growth factors in the form of ear drops may counteract impairment of wound healing in the tympanic membrane (TM). We applied keratinocyte growth factor (KGF), transforming growth factor (TGF)-alpha or basic fibroblast growth factor (bFGF) to the perforated TMs of rats in which healing impairment had been caused by systemic administration of a glucocorticoid. Histologic studies of the injured TM, including anti-5-bromo-2'-deoxyuridine immunohistochemistry, were performed on the third day after wounding. In the control ear, epidermal migration was markedly inhibited by glucocorticoid treatment and no hyperplasia was observed in any layer at the perforation edge. TMs treated with KGF showed marked hyperplasia in the epithelial layer at the perforation edge. In the bFGF- and TGF-alpha-treated groups, hyperplasia was observed in the epithelial and intermediate layers of the TM near the malleus handle and annulus, while no hyperplasia was seen in any layer at the perforation edge. Only KGF, therefore, improved epidermal migration in the TM, while all the growth factors tested induced hyperplasia in the TM.

Three infants with an almost complete absence of the cerebral hemispheres as a result of brain anomalies were studied both audiologically and neurologically. The brain anomalies were diagnosed by means of MRI and CT scans. Behavioral audiometry revealed reactions only to loud sound stimulations but auditory brainstem responses showed wave configurations and thresholds compatible with the ages of the infants. There were significant differences in the thresholds obtained by behavioral audiometry and auditory brainstem responses. It can be considered that these auditorily stimulated behavioral responses are evoked by auditory motor reflexes originating in the brainstem, but not by auditory perception.