Usefulness of measuring electrically evoked auditory brainstem responses in children with inner ear malformations during cochlear implantation.

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.

Appearance of ocular vestibular evoked myogenic potential elicited by bone-conducted vibration in a patient with CHARGE syndrome with aplasia of all semicircular canals.

We report VEMP results in a patient with aplasia of bilateral semicircular canals and a small vestibular cavity. The patient was a 27-year-old male. The computed tomograph showed absolutely no formation of his semicircular canals, together with hypoplasia of his vestibular cavity and cochlea in both ears. His oVEMP was recorded near the extraocular muscles on the left side when elicited by BCV in the Fz. The clinical profile of this patient suggested that oVEMP elicited by BCV recorded near the extraocular muscles originated from otolithic end organs, and not from semicircular canal afferents.

Vestibular myogenic potentials of athletes for the Deaf Olympic Games with congenital profound hearing loss.

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.

Vestibular failure in children with congenital deafness.

Congenitally deaf infants and children commonly suffer vestibular failure in both ears, and impairment of postural control, locomotion, and gait. The development of gross motor functions, such as head control, sitting, and walking is likely to be delayed, but fine motor function is usually preserved unless disorders of the central nervous system are present. These children can eventually catch up with their normal peers in terms of development and growth as a result of central vestibular compensation. The visual and somatosensory systems, pyramidal and extrapyramidal motor system (cerebellum, basal ganglia, cerebrum) and intellectual development, compensate for vestibular failure in infants and children with congenitally hypoactive or absent function of the semicircular canals and otolith organs.

Sex and age differences in the bony nasolacrimal canal: an anatomical study.

OBJECTIVES: To determine the diameters of the bony nasolacrimal canal and to evaluate how they are affected by sex and age. METHODS: Standard axial sinus computed tomographic images of 314 patients were assessed retrospectively; the anteroposterior and transverse diameters, the sectional area of the bony nasolacrimal canal at the level of the infraorbital margin, and the angle between the bony canal and the nasal floor were measured. RESULTS: The mean results were as follows: anteroposterior diameter, 5.6 mm; transverse diameter, 5.0 mm; sectional area of the bony nasolacrimal canal, 22.6 mm(2); and angle between the bony canal and the nasal floor, 78.3 degrees . Females had a significantly smaller anteroposterior diameter (mean 0.6 mm) and a significantly smaller transverse diameter (mean 0.3 mm). The sectional area of the bony nasolacrimal canal was 13% smaller in females (P < .001). The angle between the bony canal and the nasal floor was a mean of 1.1 degrees more obtuse in males (P = .004). With age, the transverse diameter, the sectional area of the bony nasolacrimal canal, and the angle between the bony canal and the nasal floor in males, and the anteroposterior diameter, the sectional area of the bony nasolacrimal canal, and the angle between the bony canal and the nasal floor in females increased significantly. CONCLUSIONS: In females, the narrowness of the bony nasolacrimal canal and the acute angle between the bony canal and the nasal floor predispose to chronic inflammation of the nasolacrimal drainage system. The results of this quantitative anatomical study may explain why primary acquired nasolacrimal duct obstruction is more frequent in younger female patients.

[Effects of administration of high dose steroids for complete idiopathic facial nerve palsy: propriety of a second course of steroid therapy].

OBJECTIVE: The purpose of this study was to investigate the existence of any relationship between the initial or total prednisolone dose and the degree of facial nerve recovery in patients with complete idiopathic facial nerve palsy (Bell's palsy). MATERIALS AND METHODS: This study was carried out on 102 patients with unilateral complete Bell's palsy of no more than 14 days duration. The patients were divided into four study groups: one receiving a single tapering course of steroids after an initial hydrocortisone (HC) dose of 600 mg, one receiving a second tapering course of steroids after an initial HC dose of 600 mg, one receiving a single tapering course of steroids after an initial HC dose of 1200 mg, and one receiving a second tapering course of steroids after an initial HC dose of 1200 mg. The following variables were analyzed among the groups: the cure rate, the average time needed to achieve maximum recovery, and the rate of side effects. RESULTS: The total cure rate of the patients was 77%. No significant differences were detected among the groups in terms of the cure rate, average time to achieve maximum recovery, or the side effects rate (P > 0.05). CONCLUSION: High-dose steroid therapy was considered to be somewhat effective in curing complete Bell's palsy. However, there were no correlations between the initial or total steroid dose and the prognosis if a prednisolone equivalent dose of more than 150 mg initially, or a total dose of more than 880 mg was used. These findings show no significant benefits of treating complete Bell's palsy with a second course of steroids.

Hearing levels in patients with microtia: correlation with temporal bone malformation.

OBJECTIVE: To evaluate the relationship between hearing level and temporal bone abnormalities in patients with microtia. STUDY DESIGN: Retrospective case series study between 1992 and 2004. SETTING: Academic, tertiary care referral medical center. PATIENTS: We evaluated 115 ears of 89 patients (68 males, 21 females; mean age, 11 yr; range, 5-44 yr) with microtia. MAIN OUTCOME MEASURES: Hearing level was examined in patients with microtia. Developmental abnormalities of the temporal bone were evaluated by Jahrsdoerfer's computed tomography (CT) scoring system using high-resolution CT (HRCT) scans of the temporal bone. Temporal bone malformation scores were divided into four subgroups: ossicular development, windows connected to the cochlea, aeration of the middle ear cavity, and facial nerve aberration. Patients were divided into the stenosis and atresia groups on the basis of the appearance of the external auditory canal (EAC). We also evaluated the relationships between hearing level and four subtotal scores of the HRCT findings in the stenosis and atresia groups. RESULTS: There was no relationship between hearing level and total points of HRCT scoring system or between hearing level and severity of microtia scored by Marx classification. With regard to subtotal points related to ossicles (4 points), the hearing level in ears with low scores (<2) (64.7 +/- 1.6 dB) was significantly different (P = .03) from that in ears with high scores (> or =2) (54.0 +/- 2.8 dB) in the stenosis group. In the atresia group, the hearing level was 64.3 +/- 2.2 dB in ears with low scores and 62.3 +/- 1.1 in ears with high scores (P > .5). As for subtotal points related to the windows connected to cochlea (2 points), the hearing level was 64.8 +/- 2.6 dB in ears with low scores (0) and 55.9 +/- 2.4 dB in ears with high scores (> = 1) in the stenosis group. In the atresia group, the hearing level was 67.7 +/- 2.3 dB in ears with low scores and 61.5 +/- 1.0 in ears with high scores. There was significant difference between ears with low and high scores in the stenosis group (P = .03) and atresia group (P = .009). There was no significant difference between ears with low and high scores with respect to the subtotal points related to aeration of the middle ear cavity and aberration of the facial nerve. CONCLUSION: The hearing level in microtic ears correlated with the formation of oval/round windows and ossicular development but not with the degree of middle ear aeration, facial nerve aberration, or severity of microtia. The hearing level can also serve as an indictor, such as the HRCT findings, to determine whether a subject's hearing will likely improve after reconstructive surgery.

An anatomic study of the horizontal petrous internal carotid artery: sex and age differences.

OBJECTIVE: This study aimed to determine the length and diameter of the horizontal portion of the petrous carotid artery (HPCA) and to evaluate sex and age differences in these variables. METHODS: We examined standard axial sinus computed tomographic images in 345 patients (690 sides), measuring length and diameter of the HPCA and the angle between right and left HPCA using computer software. RESULTS: The HPCA averaged 26.7 mm in length and 5.7 mm in diameter. The angle between the right and left HPCA averaged 101.5 degrees . HPCA length averaged 27.1mm in men and 25.8mm in women; HPCA diameter averaged 5.9 mm in men and 5.3 mm in women. The HPCA of men was approximately 0.8 mm wider and the angle between right and left HPCA was on average 8.7 degrees more obtuse in the 60+ years group than in the

Aplasia and displacement of the horizontal portion of the petrous carotid artery in mandibulofacial dysostosis.

CONCLUSIONS: The horizontal portion of the petrous carotid artery (HPCA) of mandibulofacial dysostosis (MFD) patients had anterior-inferior displacement or was aplasia at birth. Our findings indicate that most MFD patients have aplasia of the internal carotid artery. This information may be very important for safe reconstruction surgery in atresia. OBJECTIVE: MFD rarely has malformations of the cardiovascular system such as those seen in Goldenhar syndrome. This study aimed to compare MFD patients and normal subjects with respect to the length and diameter of the HPCA. SUBJECTS AND METHODS: We radiographically studied 22 MFD patients (44 sides) using high-resolution computed tomography (HRCT), measuring the length and diameter of the HPCA and the angle between the right and left HPCA using computer software. Findings were compared with those in 86 ears with normal auricles (control group) using the nonparametric test. RESULTS: The HPCA in MFD patients averaged 23.2 mm in length and 4.8 mm in diameter. The angle between the right and left HPCA averaged 100.2 degrees. In MFD patients, on average, HPCA were 2.0 mm shorter (p=0.001) and 0.4 mm narrower (p=0.016) than in control subjects. Mean angle between right and left HPCA was 5.4 degrees more acute in MFD patients than in control subjects (p=0.026). Moreover, the genu of the vertical and horizontal petrous ICA in the MFD group was on average 3.2 mm more inferior than in controls (p<0.001).

[Clinical study on deep neck infection].

Deep neck infection (DNI) remains an emergent and life-threatening otolaryngologic disease. We examined 69 patients, 52 men and 17 women, with DNI, who were treated in our hospital between January 1995 and December 2004. The mean age of the patients with DNI was 50.0 years and the peak incidence was in the sixth decade. Twenty patients suffered from diabetes mellitus (DM). The primary DNI lesion was found in the tonsils in 34 cases, the oral cavity in 16 cases, and the pharynx in 12 cases, respectivelys. Among the 69 patients with DNI, the infection remained in the suprahyoid region in 31 cases, but it extended to the infrahyoid region in 33 cases and to the mediastinum in 5 cases. The titer of C-reacting protein (CRP) and the duration of admission were considered as parameters of the severity of DNI. CRP was significantly higher in elderly patients, in patients with DM, and in patients whose infection extended to the infrahyoid region and to the mediastinum, however, significant difference was not found between men and women, or among the types of primary DNI lesions. Moreover, the duration of admission was significantly longer in elderly patients, in patients with DM, and in patients with infrahyoid and mediastinal DNI, whereas no significant differences were found between men and women or among the types of primary lesions. Therefore, age, DM, and the extension of DNI are considered to be important factors which determining the severity of DNI.

Evaluation of super-high-dose steroid therapy for sudden sensorineural hearing loss.

OBJECTIVE: To determine the efficacy of a high-dose steroid regimen for patients with sudden sensorineural hearing loss (SSNHL) and to evaluate the relationship between outcome and initial steroid dose. STUDY DESIGN AND SETTING: We conducted a retrospective study of 112 patients presenting to Saitama Medical Center. Patients received tapering courses of hydrocortisone (HC) with an initial dose of either 1200 mg or 600 mg. RESULTS: More than 87% of patients had recovery of hearing. When hearing stabilized, there were no significant differences between the 1200 mg and 600 mg groups in terms of hearing outcome (P>0.05). However, at completion of treatment, the 1200 mg group exhibited significantly superior complete recovery rate and improvement rate (P<0.05). CONCLUSION: Application of our treatment protocol of a tapered course of 1200 mg HC significantly and rapidly improves recovery outcomes in patients of SSNHL. EBM RATING: C-4.

[Effect of the external nasal dilator breathe right and nasal cycle influence].

The external nasal dilator Breathe Right is a nonprescription disposable mechanical device worn over the bridge of the nose that supports the outer walls of the nasal vestibule to increase cross-sectional nasal valve area and enhance air flow through the nose. When worn, the device applies a gentle outward pull to the outside walls of the nasal vestibule that helps prevent collapse of the lateral walls of the nasal vestibule. The device appears similar to an adhesive bandage with two polyester springs attached lengthwise to the nonadhesive side of the backing material. The form of the external nose varies significantly between racial groups and some comment is possible about the form of the nasal valve, i.e., Caucasian noses are leptorrhine, Negroid noses platyrrhine, and Oriental noses intermediate. A question exists about whether Breathe Right is as effective in Japanese as in Caucasians against nasal obstruction. We measured the effect by acoustic rhinometry in 35 healthy subjects. We measured 2 cross-sectional areas-the I-notch and C-notch-and 3 nasal volumes-V(0-1), from 0 to 1 cm corresponding to the I-notch, V(1-3), from 1 to 3cm corresponding to the C notch, and V (3-6), from 3 to 6cm. We studied the influence of the nasal cycle on the effect of Breathe Right in the nasal cavity. We evaluated subjects in a stable sitting position before applying Breathe Right, after using 5 minutes, and in a free interval of 25 minutes following. The experiment was repeated 10 times. We found that Breathe Right increases the nasal volume especially in V(1-3) and the cross-sectional area especially in the C-notch. Interestingly, the change in the C-notch decreased gradually with time. One possible mechanism may be the change in compliance between the skin under the Breathe Right and the nasal mucous membrane. The nasal cycle did not significantly influence the effect of Breathe Right.

Facial canal anatomy in patients with mandibulofacial dysostosis: comparison with respect to the severities of microtia and middle ear deformity.

OBJECTIVE: To study the difference in the facial canal anatomy in terms of the severity of microtia and deformity of the middle ear in patients with mandibulofacial dysostosis using high-resolution computed tomography. STUDY DESIGN: Retrospective analyses. SETTING: The study was carried out at the Department of Otorhinolaryngology, University of Tokyo, Tokyo, Japan. PATIENTS: Thirty-six ears of 18 patients with mandibulofacial dysostosis were examined by high-resolution computed tomography. These ears were graded based on the Marx classification and Jahrsdoerfer scoring systems. MAIN OUTCOME MEASURES: The high-resolution computed tomography findings and age distribution of each group were compared with those of other groups by multiple comparison using Tukey's honestly significant difference test. RESULTS: The course of the facial nerve was not significantly different in terms of the severity of microtia and deformity of the middle ear. The bony cochlea in the patients with mandibulofacial dysostosis was displaced by a mean value of 2 mm more anteriorly and a mean value of 0.7 mm shallower than that in the cases with normal auricles. CONCLUSION: The facial nerve of patients with mandibulofacial dysostosis is displaced more anterolaterally than that of the cases with normal auricles; however, the auricle anomaly is not severe.

Correlation between microtia and temporal bone malformation evaluated using grading systems.

OBJECTIVE: To evaluate the relationships between temporal bone abnormalities and the severity of microtia in Japanese patients using objective grading systems. DESIGN: Retrospective case series study conducted between 1992 and 2003. SETTING: Academic, tertiary care, referral medical center. PATIENTS: One hundred forty-two ears of 109 Japanese patients (85 male and 24 female patients; mean age, 12.8 years [range, 2-36 years]) with microtia. MAIN OUTCOME MEASURES: The severity of microtia was classified according to Marx classification. Developmental abnormalities of the temporal bone were evaluated by a computed tomographic (CT) scoring system modified after the system used by Jahrsdoerfer and colleagues, using high-resolution CT scans of the temporal bone. Correlations between the scores obtained from these 2 grading systems were evaluated using a nonparametric statistical method. RESULTS: Male preponderance and incidence of bilateral cases of approximately 30% were observed in our Japanese patients with microtia. There was no significant difference in the severity of microtia between unilateral and bilateral cases. The mean +/- SEM total points in the CT scoring system (full marks, 10) was 7.9 +/- 0.4 for grade I microtia, 6.6 +/- 0.6 for grade II, and 6.4 +/- 0.3 for grade III; the total points correlated inversely with the microtia grade. Development of the auricle correlated significantly with aeration in the middle ear spaces but not with ossicular development or formation of the oval/round windows. Proportion of acceptable surgical candidates according to the CT scoring system (>5 points) was 79% for grade I microtia, 52% for grade II microtia, and 65% for grade III microtia. CONCLUSION: The principle "the better developed the auricle, the better developed middle ear" was confirmed in Japanese patients with microtia; however, even with grade II/III microtia, more than half of the patients were considered suitable for atresia surgery.

Sound-evoked neurogenic responses with short latency of vestibular origin.

OBJECTIVE: In ABR recording, a large negative deflection with a latency of 3 ms (N3) has been recorded in patients with peripheral profound deafness. It has been suggested that N3 might be of vestibular origin. So far, N3 has been recorded only in patients with peripheral profound deafness. If we can record N3 potentials in subjects with preserved hearing, recording N3 potentials might be a new clinical test of the vestibular system. To record neurogenic potentials (N3) of vestibular origin in healthy volunteers and patients with vestibular disorders. METHODS: Twelve healthy volunteers (10 men and two women, aged 23-37 years) and 12 patients with vestibular disorders (6 men and 6 women, aged 29-71 years) were enrolled in this study. To record responses, surface electrodes were placed on the ipsilateral mastoid and the vertex. An electrode on the nasion served as the ground. Recording was performed using an auditory evoked potential recording system with a mini-mixer and a stereo-amplifier. Signals at the vertex to the ispilateral mastoid were amplified and bandpass filtered (100-3000 Hz). One thousand-hertz short tone bursts (1 kHz STB; rise/fall time=0.5 ms, plateau time=1 ms) were presented to either ear through a headphone with or without white noise (WN) ipsilateral to the stimulated ear. The stimulation rate was 10 Hz, and the analysis time was 10 ms. The responses to 500 stimuli were averaged twice. RESULTS: When 1 kHz STB (95 dBnHL, equivalent to 130 dBSPL) were presented with 100 dBSPL WN (ipsilateral to the stimulated ear), a negative peak with 3-4 ms latency (N3) was observed in 23 of the 24 ears (95.8%) with reproducibility in healthy subjects. Without WN, N3 was observed in 17 of the 24 ears (70.8%). The threshold of N3 was 90.2 dBnHL on the average. The presence of N3 in the patients was in agreement with the presence of the VEMP, which were also recorded. CONCLUSIONS: Using techniques of WN exposure ipsilateral to the stimulated ear, we recorded N3 in healthy subjects and in vestibular disorder patients with preserved hearing. This negative peak is likely to be of vestibular origin. SIGNIFICANCE: N3 may be measured from subjects who cannot contract neck muscles due to their ages, mental states, or consciousness disorders. In other words, N3 may be measured from subjects from whom VEMP cannot be recorded. In combination with VEMP, N3 may be useful for the detection of lesion sites.

[A statistical analysis of epistaxis between outpatients and inpatients].

Although we found several statistical studies on epistaxis, we found no reports discussing clinical differences in nasal bleeding between outpatients and inpatients. We therefore examined 103 inpatients (1998-2001) and 337 outpatients (2001) with epistaxis for gender, age, season, bleeding points, and blood pressure. We then compared these parameters statistically in both groups using Mann-Whitney's U test. The incidence of epistaxis in inpatients was statistically higher in winter than in any other season, and most had uncontrolled borderline hypertension. We concluded that borderline hypertension worsens in winter and that patients with borderline hypertension should be medicated to prevent severe epistaxis.

Aplasia of zygomatic arch and dislocation of temporomandibular joint capsule in Treacher-Collins syndrome: three-dimensional reconstruction of computed tomographic scans.

Seven patients with Treacher-Collins syndrome were studied. All of patients were children or teenagers. Helical CT scanner (Toshiba) was used to reconstruct zygomatic arch and temporomandibular joint capsule on lateral aspect of temporal bone in five patients of microtia and atresia of both ears and two patients of narrow ear canals of both ears without microtia.Three-dimensional reconstructions of computed tomography on lateral aspect of temporal bone demonstrated various congenital abnormality including aplasia of zygomatic arch in seven patients and dislocation of temporomandibular joint capsule in seven patients.

Difference in facial canal anatomy in terms of severity of microtia and deformity of middle ear in patients with microtia.

OBJECTIVE: To study the difference in the facial canal anatomy in terms of the severity of microtia and deformity of the middle ear in patients with microtia using high-resolution computed tomography (HRCT). STUDY DESIGN: Forty-six ears with microtia were examined by HRCT. These ears were graded based on two systems of classification and scoring, respectively. The HRCT findings of each group were compared with those of other groups by multiple comparison using the Tukey honestly significant difference test. METHODS: Forty-six ears with microtia (unilateral microtia, n = 12; bilateral microtia, n = 34) were examined by HRCT. These ears were graded based on two systems of classification and scoring, respectively. The HRCT findings and age distribution of each group were compared with those of other groups by multiple comparison using the Tukey honestly significant difference test. RESULTS: The mastoid portion of the facial nerve was 3 mm more anteriorly displaced in patients with grades II and III microtia than in those with grade I microtia (P <.01). From the scoring system that was used, the course of the facial nerve was not significantly different between patients with a score of 5 points or less and those with a score of 6 points or more. CONCLUSION: The mastoid portion of the facial nerve in patients with grades II and III microtia would be more anteriorly displaced because of hypoplasia of the second genu.

Effect of white noise on vestibular evoked myogenic potentials.

OBJECTIVES: To clarify if p13-n23 of vestibular evoked myogenic potentials (VEMPs) is independent of cochlear afferents. METHODS: Twenty normal volunteers and 10 patients with hemifacial palsy enrolled into this study. VEMP and auditory brainstem responses (ABRs) were recorded with or without white noise (WN, 75 dBnHL or 95 dBnHL) ipsilaterally or contralaterally to the stimulated ear. Short tone bursts (STBs) of 0.5 kHz (95 dBnHL, rise/fall time=1 ms, plateau time=2 ms) were presented though headphones. For recording VEMPs surface electromyographic activity was recorded in the supine subjects form symmetrical sites over the upper half of each SCM with a reference electrode over the upper sternum. RESULTS: The amplitude of p13-n23 significantly decreased during exposure of the contralateral ear as well as the ipsilateral ear to 95 dBnHL WN in normal volunteers (41% reduction by the ipsilateral exposure and 38% reduction by the contralateral exposure). Exposure to 75 dBnHL WN caused only minimal reduction of the amplitudes of p13-n23 (5% reduction) although it caused remarkable reduction of the amplitudes of V-SN(10) (ABRs) (23% reduction). However, 95 dBnHL WN exposure showed no effect on the amplitudes of p13-n23 in the ipsilateral side to hemifacial palsy. CONCLUSION: Overall the data are in favor of the hypothesis that cochlear afferents could affect the amplitude of p13-n23 only through the stapedial reflex, although our data might not be strict proof of an absence of influence of cochlear afferents' activity.