Clinical manifestations of clients, who counseled against their genetic problem of hearing impair ment, were investigated. Clients were patients who visited the outpatient clinic of Kyushu Univer sity Hospital from August 2000 to July 2005. There were 37 patients of which 84% were female. Averaged age was 26. First decade of life was the most common generation, followed by third and fourth decades. 35% were progressive type. Mean hearing level was 53dB, whereas nearly 40% was slight loss. Autosomal dominant was the most common hereditary type and autosomal recessive was the next. 22% was syndromic and the other was non-syndromic. For genetic couselling for hearing impairment, audiology, otology, neurotology, speech therapy, supporting social system for hearing impairment are mandatory. It is important that ENT doctor who is familiar to genetics actively con cerns to the genetic counseling for hearing impairment.

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In this study, the evoked responses to 70% CO₂ and the odor of strawberry essence were recorded from 16 electrode positions on the human scalp according to the international 10-20 system. The experiment was carried out in 10 subjects with normal olfaction. The superficial component was calcu lated from these responses using the source derivation method and a deep component was obtained by subtracting the superficial component from the common average referenced waveforms. Three peaks were observed at latencies of 420 msec (Plc), 590 msec (P2c), and 870 msec (P3c) in the superficial component to CO₂ gas. A peak in the deep component, however, was only recognized at the same la tency as P2c. A topographic analysis of the deep component in P2c showed a symmetric pattern. In contrast, a peak to the strawberry essence stimuli was only observed at a latency of 595 msec (P2s) in the superficial component. The topographic characteristics were similar to those of CO₂ gas. The topographic analysis of the deep component to strawberry essence thus showed an asymmetric pattern. These results suggest that a generator of the response to strawberry essence is located in the somatosensory cortex and asymmetrically in part of the deep brain such as the orbit-frontal cortex.

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We herein report 3 cases in 2 families of fluctuating hearing loss associated with an enlarged ves tibular aqueduct caused by head trauma. A hearing impairment was caused by trauma to the right temporal region (case 1), the parietal region (case 2) and the mandibular region (case 3). An im provement in the hearing ability was shown with steroid therapy for acute sensorineural hearing loss, but at times the hearing recovered without steroid therapy. Therefore, whether or not steroid use is necessary remains unclear. In addition, there were times when the hearing threshold was un stable after an improvement in the hearing ability, whereby it seemed that we should carefully ob serve any fluctuation in the hearing ability. Furthermore, it is necessary to inform patients withan enlarged vestibular aqueduct and their families and associates of the importance of preventing head trauma in order to avoid the development of a hearing impairment in such individuals.

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The auditory steady-state responses (ASSRs) have been investigated since the 1990s, which can be examined as the frequency-specific auditory responses. Especially recently, with the introduction of newborn hearing screening, the ASSRs are clinically expected to become one of the objective audio grams for newborn infants. However, so far no report has attempted to determine the ASSRs reli ability or validity regarding the frequency-specific hearing threshold, or the effect on the level of consciousness. We regard it as important to examine the reliability in estimating the hearing level objectively. In this study, we recorded the ASSRs from 36 adults (72 ears) with normal hearing and 17 children and adults with severe sensory neural hearing loss (30 ears), and thus determined any cor relations between the ASSRs and standard audiograms using a multivaliate analysis. In addition, we also performed the bispectral index with a bispectral monitor and ASSRs at the same time from 4 nor mal hearing adults (8 ears), and examined the data both when the subjects were awake and asleep.

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Recurrent or persistent acute otitis media (rAOM) and acute otitis media treatment failure are commonly encountered in the pediatric population. The is mounting evidence to suggest that rAOM is associated with multidrug antibiotic resistance. However, there has yet to be any study on mul tidrug antibiotic resistance in rAOM patients in Saga prefecture in Japan. This retrospective study was undertaken to elucidate the inhibitory activity with 70 rAOM pediatric patients from whom bac terial cultures were obtained from middle ear effusion, otorrhea, or the nasopharynx from January 2002 to September 2004. All bacterial cultures studies were performed for rAOM pediatric patients after the initial failure of the first-line antibacterial therapy. All children presented with acute oti tis media (AOM) associated with recurrent episodes after oral medicen therapy. We also investi gated the effect of tympanostomy tube placement in six rAOM patients. The pathogens in rAOM are Haemophilus influenzae (n=26, 37.1%), Streptococcus pneumoniae (n=25, 35.7%), Staphylococcus aureus (n=11, including 10 MRSA, 15.7%), and others. These pathogens were also not susceptible to penicillin. In children with H influenzae (n=26), three of 26 cases were highly resistant to penicillin (β-lactamase non-procucing ABPC resistant Haemophilus influemzae, BLNAR), and 12 cases showed in termediate resistance (low-BLNAR), while one case was BLPAR (β-lactamase positive ampicillin resistant). In children with S peneumoniae (n=25), 10 of 25 were highly resistant to penicillin (penicillin-resistant Streptococcus pneumoniae, PRSP) while 13 cases showed intermediate resistance (PISP). All 70 children received either AMPC (40m-80mg/kg per day), CFPN-I (9-15mg/kg per day) or CFTM-PI (15mg/kg per day) after the initial failure of first-line antibacterial therapy. All rAOM patients received nasal therapies and antibiotic treatments based on the results of suscep tibility testing. Myringotomies were performed in all severe cases. The patients caused by mul tidrug antibiotic resistance microbes such as MRSA or DRSP (drug-resistant S. pneumoniae), treated with myringotomies, syringing and suctioning to clear the discharge without using antibiotics. Sixty-four of 70 (91%) patients could thus be controlled by these medical therapies. The remaining unsuccessful six children (9%) underwent tympanostomy tube placement. The ages of all six children were younger than 2 years. The use of such tubes reduced the frequency of recurrent acute oti tis media in all of the patients. Thereafter all of them could be successfully controlled by surgery. The data suggest that the placement of the tympanostomy tubes therefore appears to be a highly use ful option for rAOM pediatric patients.

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The local findings of MEO and SCC are similar to each other, therefore it is sometimes difficult to make a differential diagnosis. The former is basically inflammation while the later is carcinoma. It is necessary to accurately diagnose MEO and SCC as soon as possible in order to begin the appropri ate treatment in a timely manner. We compared 2 cases of MEO with 9 cases of SCC that we experi enced in our clinic over the last 7 years with the following results: It is almost impossible to differentiate them based on the symptoms or general findings, however, we did notice that vagus pa ralysis tends to occur in cases of MEO, while facial paralysis tends to develop in cases of SCC. In ad dition, such factors as any associated illness or past history, bacteriological examination or SCC antigen in the blood do not play a significant role in making a correct daignosis. CRP in the blood is comparatively useful because it is apt to increase in the case of MEO. A pathological examination is extremely useful and exfoliative cytology, in particular, is important when a biopsy is difficult to perform under some conditions. Exfoliative cytology is also useful for following up these cases. We have thus come to the conclusion that it is difficult to make a differential diagnosis based on the find ings of CT or Ga scintigraphy, except in highly typical cases.

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We investigated the clinopathological and immunohistochemical features of 22 cases of malig nant melanoma of the head and neck who were treated at our hospital from 1993 to 2003. Thirteen patients were males and 9 were females. Their ages ranged from 42 to 81 years old, with an average age of 65 years. The primary tumor sites were the nasal cavities in 12, the paranasal sinuses in 5, the head and neck skins in 3, the oral cavity in one and the nasopharynx in one. Seventeen patients re ceived radical treatment while palliative treatment was performed in 5 patients. The disease-specific survival rate was 25.7% at 3 years and 5 years. The treatment group consisted of a local resection and adjuvant chemotherapy showed a better progmosis than the others. Immunohistologically, 13 patients expressed HMB45, 4 expressed VEGF, 6 expressed RCAS-1 and 8 expressed MMP9 in a total of 18 patients. These results indicate that a local resection of the tumor provides the best efficacy in managing head and neck malignant melanoma. Adjuvant chemotherapy as well as radiotherapy therefore appears to have a beneficial effect in controlling the disease.

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We herein report a case of ectopic parathyroid adenoma in the thyroid gland. Double parathy roid adenomas are a rare cause of primary hyperparathyroidism. A 66-year-old man presented with hypercalcemia and an elevated intact parathyroid hormone level. Ultrasonography and CT scan ex aminations showed an enlarged superior right side of the parathyroid gland. ^99mTc methoxyibutyli sonitrile scintigraphy showed a hot-spot in the superior right side of parathyroid gland. From these data, he was diagnosed to have primary hyperparathyroidism. As a result, he underwent his first op eration in December 2003. However, he had persistent hypercalcemia for five months. ^99mTc methoxyibutylisonitrile scintigraphy was performed and the findings showed a hot-spot on the right side of the thyroid gland in April 2004. He therefore underwent a second operation consisting of a hemithyroidectomy and a total parathyroidectomy, which thus resulted in normocalcemia. Histo logical examinations of both operations revealed parathyroid adenomas. We also reviewed 50 pa tients with hyperparathyroidism treated at the Department of Otolaryngology, Head and Neck Surgery, Kurume University Hospital between 1996 and 2004, consisting of 24 primary and 26 secon dry hyperparathyroidisms. Among the primary cases, 2 cases were ectopic (8.3%). Histopathogi cal examinations revealed 18 parathyroid adenomas and 6 hyperplasia. Ultrasonography was thus found to be the most effective tool for accurately diagnosing of hyperparathyroid gland tumors.

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The efficacy of combination therapy using an antiallergic agent, fexofenadine hydrochloride, and a macrolide, clarithromycin, in allergic rhinitis patients exhibiting a shadow in their paranasal sinus X-ray findings was evaluated by comparing the results with treatment using an antiallergic alone. The total clinical efficacy rates were 17.4% (4/23) in the single-agent group and 57.1% (16/28) in the combination group, and the rate was significantly higher in the combination group. For subjec tive symptoms, the combination therapy was shown to be significantly superior in improving postna sal discharge, cough and discomfort in the throat, while for objective findings, it was also shown to be significantly more effective in reducing the amount of pituita than an antiallergic agent alone. How ever, for the other findings, there was no significant difference between the two treatments. An X ray evaluation revealed an improvement in 5.0% of the patients (1/20) in the single-agent group and an improvement in 45.5% of the patients (10/22) in the combination group, and this improvement rate was significantly high in the patients receiving the combination therapy. From these results, for allergic rhinitis the patients exhibiting shadows in the paranasal sinus X-ray findings, the com bination therapy with an antiallergic agent and a macrolide was therefore considered to be more effec tive than the treatment with an antiallergic agent alone.

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The combination treatment of cetirizine hydrochloride, which is one of the second generation an tihistamines, plus fluticasone propionate nasal spray was administered for 4 weeks in cases of peren nial allergic rhinitis. In the patients who showed improved nasal symptoms, we evaluated whether the improvement could be maintained when the fluticasone propionate nasal spray was stopped. As a result, all nasal symptom scores including sneezing, rhinorrhea and nasal obstruction and severity were found to have significantly improved after 4 weeks in comparison to before the administration of both drugs. In addition, the improving effect could be maintained for 4 weeks even after the flu ticasone propionate nasal spray had been discontinued. In conclusion, the combination therapy of cetirizine hydrochloride plus fluticasone propionate nasal spray was thus suggested to be very effec tive for all nasal symptoms such as sneezing, rhinorrhea and nasal obstruction in cases with peren nial allergic rhinitis. In addition, the improvement by the single administration of cetirizine hydrochloride was found to be maintained even after the fluticasone propionate nasal spray had been discontinued.

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