Organized hematoma of the sphenoid sinus with epistaxis.

Few reports have been made on organized hematoma (OH) originating in the sphenoid sinus. We report the case of a 24-year-old man who presented to the hospital with consistent epistaxis for 1 month. Preoperative computed tomography and magnetic resonance imaging (MRI) detected an expansive heterogeneous mass with minimal bone destruction and hypointense peripheral rim in T2-weighted images of the left sphenoid sinus as a solitary lesion. An OH was suspected first based on the history of repeated epistaxis and characteristic imaging findings, although quite rare. Preoperative vascular embolism was not performed because its effects on the ocular artery should be considered. With endoscopic sinus surgery under hypotensive anesthesia, the mass was carefully and successfully removed without bleeding and diagnosed as OH based on postoperative pathological examination. When solitary lesions are found in the sphenoid sinus with epistaxis, this disease should be differentiated. If it increases, complications, such as visual impairment, frequently occur, and control of intraoperative bleeding is sometimes difficult. Not only missing the characteristic imaging findings, especially MRI with contrast administration but also evaluating the anatomical relationship between OH and other important organs is important for a successful intervention of sphenoid sinus OH.

A manual of Eustachian tube function tests-illustration of representative test results obtained from healthy subjects and typical disorders with suggestion of the appropriate test method of choice.

In the 19th century, Politzer devised a method to measure passage of the Eustachian tube (ET) by pressurizing the nasopharyngeal cavity, which marked the beginning of the ET function test. Since then, various examination methods have been developed. While ET function testing is important, recent advancements in diagnostic imaging and treatments have renewed interest on its importance. In Japan, the main objective methods used for examining ET function include tubotympanoaero-dynamic graphy (TTAG), sonotubometry, and the inflation-deflation test. The Japan Otological Society (JOS) Eustachian Tube Committee has proposed a manual of ET function tests, which presents typical patterns of the normal ear and typical diseases and suggests the ET function test of choice for each disease. However, the diagnosis of each disease should be made based on a comprehensive history and various examination findings, with ET function tests playing a supplemental role in the diagnosis.

Proto-oncogene mutations in middle ear cholesteatoma contribute to its pathogenesis.

BACKGROUND: Chronic inflammation causes bone destruction in middle ear cholesteatomas (MECs). However, the causes of their neoplastic features remain unknown. The present study demonstrated for the first time that neoplastic features of MEC are based on proto-oncogene mutations. RESULTS: DNA was extracted from MEC and blood samples of five patients to detect somatic mutations using depth-depth exome sequencing. Exons with somatic variants were analyzed using an additional 17 MEC/blood test pairs. Variants detected in MECs but not in blood were considered pathogenic variant candidates. We analyzed the correlation between proto-oncogene (NOTCH1 and MYC) variants and the presence of bone destruction and granulation tissue formation. MYC and NOTCH1 variants were detected in two and five of the 22 samples, respectively. Two of the NOTCH1 variants were located in its specific functional domain, one was truncating and the other was a splice donor site variant. Mutations of the two genes in attic cholesteatomas (n = 14) were significantly related with bone destruction (p = 0.0148) but not with granulation tissue formation (p = 0.399). CONCLUSIONS: This is the first study to demonstrate a relationship between neoplastic features of MEC and proto-oncogene mutations.

Long-term speech perception performance in prelingually deafened adult cochlear implant recipients.

To investigate the postoperative long-term outcomes after an average of 9.2 years following cochlear implantation (CI) in prelingually deafened adults, along with preimplantation factors predicting postoperative outcomes.Twenty-six prelingually deafened adults who underwent CI at >18 years were compared with those who had undergone CI in childhood (<9 years) and were >10 years old. Outcome measures includedhearing thresholds, preoperative and postoperative aided hearing level (HL), speech discrimination score (SDS), and Categories of Auditory Performance (CAP) scores. Correlation analyses were performed on the following: SDS results, aided HL, school attendant status, implant manufacturers, and speech processor models.Improvement was achieved in the aided HL and SDS results, although these results were not better than those of the child group. CAP score was also statistically significantly improved after CI. Statistically significant correlation between the preoperative SDS and postoperative HL with CI results was observed. In other words, the better the preoperative SDS results, the better the postoperative SDS results.Prelingually deafened adults achieved considerable improvement through CI. It is important to understand that patients achieving better hearing with a well-fitted hearing aid and good SDS performance before surgery may be good candidates for CI.

Bilateral Rhinogenous Optic Neuropathy Caused by the Regrowth of a Large Sphenoid Sinus Mucocele.

A 59-year-old man, who had a history of left blind at 36 years old, suddenly lost right visual acuity. Magnetic resonance imaging revealed a large left sphenoid sinus cyst, which protruded intracranially. The cyst was fenestrated by endoscopic sinus surgery, but his right vision did not recover. Ten cases of bilateral rhinogenous optic neuropathy caused by mucocele have been reported, and the cause was sphenoid sinus in 9 cases. Postoperative visual acuity in these cases was poor, especially in slow progressive cases, because it was diagnosed as an unknown cause, and surgery was delayed. Rhinogenous optic neuropathy caused by mucocele should be differentiated from bilateral visual impairment of unknown cause. The authors highlight the importance of early diagnosis of sphenoid sinus mucocele and fully informing patients about the future risk of bilateral visual impairment, even if they are asymptomatic or have been treated.

Sphenoid sinus schwannoma with adhesion to the carotid canal.

Few reports have been made on the presentation and treatment of schwannomas originating in the sphenoid sinus due to their rarity. We report the case of a 60-year-old woman who presented with a cyst-like mass in the right sphenoid sinus on magnetic resonance imaging. Computed tomography showed a bone defect in the internal carotid artery (ICA) and enlargement of the round foramen on the affected side. A histopathological diagnosis of schwannoma was made, and endoscopic sinus surgery was performed. The anterior part of the tumor could be easily removed by aspiration; however, its adhesion to the ICA was remarkable, and its boundaries were unclear. Although partial paralysis of the maxillary nerve remained, no recurrence occurred for over 10 years. Careful evaluation should be performed to check for bone defects in the nerve to differentially diagnose schwannoma from cystic masses of the sphenoid sinus. Additionally, excision of masses similar to the presented case may expectedly be difficult because of unclear excisional margins and strong capsule adherence to important organs such as the ICA. Moreover, correct imaging, histopathological diagnosis, and appropriate informed consent are essential before surgery.

CT analysis of predictors for visual acuity in optic neuropathy with mucocele.

OBJECTIVE: To evaluate the causative and risk factors for optic neuropathy with mucocele via imaging studies. METHODS: We included 21 patients with rhinogenous optic neuropathy with mucocele. We collected data on the sinus involved, age, sex, number of days from the onset of visual impairment to surgery, and computed tomography (CT) imaging findings (bone defects in the lamina papyracea, Onodi cell mucocele, exophthalmos, and optic nerve deviation). The results were compared between two groups, the one having nine patients with pre-operative visual acuity of <0.1 (the poor group) and the other having 12 patients with pre-operative visual acuity of ≥0.1 (the fair group). Whether or not there was a difference in pre-operative visual acuity between patients with and without Onodi cell mucocele was determined. RESULTS: After surgery, visual acuity improved in 16/21 (76.2%) patients, and a correlation analysis showed a significant positive correlation between pre-operative and post-operative visual acuity. In imaging, the causative sinuses accounted for 85.7% of both posterior ethmoid and sphenoid sinuses. Bone defects of the lamina papyracea at the optic canal and the vertical downward deviation of the optic nerve at each location, especially in 6/9 patients with Onodi cell mucocele, were characteristic in the poor group. In these conditions, increasing the contact areas of the optic nerve and mucocele can leads to more chances of direct downward compression of the optic nerve and infection occurring, and it may lead to severe pre-operative visual impairment. CONCLUSION: Imaging studies of optic neuropathy with mucocele help to determine the risk factors and perform early and precise diagnostic imaging and decision-making for surgery.

Clinical practice guidelines for the diagnosis and management of otitis media with effusion (OME) in children in Japan - 2022 update.

This is an update of the 2015 Guidelines developed by the Japan Otological Society and Oto-Rhino-Laryngeal Society of Japan defining otitis media with effusion (OME) in children (younger than 12 years old) and describing the disease rate, diagnosis, and method of examination. Recommended therapies that received consensus from the guideline committee were updated in consideration of current therapies used in Japan and based on available evidence. METHOD: Regarding the treatment of OME in children, we developed Clinical Questions (CQs) and retrieved documents on each theme, including the definition, disease state, method of diagnosis, and medical treatment. In the previous guidelines, no retrieval expression was used to designate a period of time for literature retrieval. Conversely, a literature search of publications from March 2014 to May 2019 has been added to the JOS 2015 Guidelines. For publication of the CQs, we developed and assigned strengths to recommendations based on the collected evidence. RESULTS: OME in children was classified into one group lacking the risk of developing chronic or intractable disease and another group at higher risk (e.g., children with Down syndrome, cleft palate), and recommendations for clinical management, including follow-up, is provided. Information regarding management of children with unilateral OME and intractable cases complicated by adhesive otitis media is also provided. CONCLUSION: In clinical management of OME in children, the Japanese Clinical Practice Guidelines recommends management not only of complications of OME itself, such as effusion in the middle ear and pathologic changes in the tympanic membrane, but also pathologic changes in surrounding organs associated with infectious or inflammatory diseases.

Pharmacotherapy focusing on for the management of otitis media with effusion in children: Systematic review and meta-analysis.

OBJECTIVE: To examine the evidence for treating children with otitis media with effusion with pharmacotherapy. DATA SOURCES: For the systematic review, data were retrieved from PubMed, Cochrane database, and the Japan Medical Abstracts Society Database (1st January 1995 through 31th May 2019). STUDY SELECTION: Articles addressing pharmacotherapy for the management of otitis media with effusion in children were selected in English. DATA EXTRACTION: The database was searched using the keywords "Otitis Media with effusion or secretory otitis media" and the following medical agents: carbocysteine, antihistamines, leukotriene receptor antagonist, and steroid nasal spray. DATA SYNTHESIS: After a critical review of 18 studies, studies addressing steroid nasal spray were eligible for quantitative synthesis. Intranasal steroids for OME showed no benefit with OR 1.155 (95% CI 0.834-1.598) within one month. Conversely, intranasal steroids have effects for OME with OR 1.858 (95% CI 1.240-2.786) for more than one month. CONCLUSIONS: We found evidence of benefit from treatment of OME in children with intranasal steroids and S-carboxymethylcysteine at longer-term follow-up.

Efficacy of clarithromycin in patients with mild COVID-19 pneumonia not receiving oxygen administration: protocol for an exploratory, multicentre, open-label, randomised controlled trial (CAME COVID-19 study).

INTRODUCTION: The COVID-19 pandemic has emerged worldwide. Although several medications have been approved for treating moderate-to-severe COVID-19, very few treatment strategy has been established for patients with mild COVID-19 who do not require oxygen administration. Clarithromycin is a macrolide antimicrobial agent that has been widely used for bacterial respiratory infectious diseases. Clarithromycin also acts an immunomodulating drug and suppresses cytokine storms in viral respiratory diseases, including influenza. In this study, we aim to evaluate the efficacy of clarithromycin in patients with mild COVID-19. METHODS AND ANALYSIS: This is an exploratory, multicentre, open-label, randomised controlled trial. This study was initiated in May 2021 and will end in July 2022. Patients with mild COVID-19 pneumonia who do not require oxygen administration will be enrolled and randomly assigned in a 1:1:1 ratio to group A (administration of clarithromycin 800 mg/day), group B (administration of clarithromycin 400 mg/day) or group C (standard treatment without clarithromycin). The planned number of enrolled patients is 60 (20 patients × three groups). The primary endpoint is the number of days required to improve the clinical symptoms as measured by the severity score. Secondary endpoints include days for recovery of the body temperature, proportion of patients with oxygen administration, inflammatory cytokines, viral load, serum immunoglobulins, peripheral blood lymphocytes, blood biomarkers and pneumonia infiltrations. ETHICS AND DISSEMINATION: The study protocol was approved by the Clinical Research Review Board of Nagasaki University in accordance with the Clinical Trials Act in Japan. The study will be conducted in accordance with the Declaration of Helsinki, the Clinical Trials Act and other current legal regulations in Japan. Written informed consent will be obtained from all the participants. The results of this study will be reported as journal publications. TRIAL REGISTRATION NUMBER: jRCTs071210011.

Anatomy of the Surrounding Tissue of the Eustachian Tube in Patulous Eustachian Tube: 3 Tesla Magnetic Resonance Imaging Approach.

OBJECTIVES: To clarify the possible pathological conditions of cartilaginous portion of the Eustachian tube (ET) and its surrounding tissues. STUDY DESIGN: Retrospective review. PATIENTS: Twenty-five normal subjects (control group) and 25 patients with patulous ET (patulous group). METHODS: We reconstructed cross-sectional 3 Tesla magnetic resonance (MR) images using the multiplanar reconstruction (MPR) technique. Cross-section area widths that were measured included the following: 1) inferior head of lateral pterygoid muscle (PM); 2) pterygoid venous plexus (PVP); 3) medial soft tissues of ET, including ET cartilage; and 4) maximum cross-sectional area of high intensity surrounding the medial ET lumen. RESULTS: A statistically significant difference was found in the thickness of the inferior head of the lateral PM between male and female; it was thicker in the male participants. Ostmann's fatty tissue (OF) was significantly smaller in the patulous ET group. CONCLUSION: Smaller size of the surrounding tissues of the ET, viz., the OF, may contribute to the pathogenesis of patulous ET.

Cochlear implantation in patients with bilateral deafness caused by otitis media with ANCA-associated　vasculitis (OMAAV): A report of four cases.

OBJECTIVE: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) without systemic symptoms but with initial symptoms related to the ear, such as hearing loss, otalgia, and dizziness, has recently been reported. We have categorized this condition as otitis media with AAV (OMAAV), and have recently proposed its diagnostic criteria. METHODS: To determine the effectiveness of cochlear implantation (CI) in patients with profound hearing loss due to OMAAV. We examined the language understanding ability of four patients with bilateral profound or total deafness due to OMAAV, who underwent CI. RESULTS: In three of the four patients, the language understanding ability with CI was poor. These three patients with poor performance had characteristic features, including a short interval from the onset of ear symptoms to total deafness and clear enhancement of the cochlea on magnetic resonance imaging (MRI). CONCLUSION: The poor results observed in patients with a rapidly progressive history of hearing loss were attributed to possible severe and profuse intracochlear bleeding and/or destruction of structures, including the spiral ganglion. All the three patients showed contrast enhancement in the inner ear on MRI. We believe that preoperative evaluation of the history of hearing loss as well as the findings of contrast-enhanced MRI is important for predicting the prognosis after CI.

Long-term Outcomes of Cochlear Implantation in Children With Congenital Cytomegalovirus Infection.

OBJECTIVE: To investigate the role of the developmental delay often observed in children with congenital cytomegalovirus (CMV) infection on the improvement of language understanding after cochlear implantation (CI). STUDY DESIGN: Retrospective chart review. PATIENTS: Sixteen children with severe and/or profound hearing loss due to congenital CMV infection (CMV group) and 107 congenitally deaf children (168 ears) without CMV infection as the cause of deafness (non-CMV group). Mean age at which patients underwent CI was 2.9 years in both groups. The mean follow-up period was 7.8 versus 8.2 years, respectively. INTERVENTIONS/MAIN OUTCOME MEASURES: The Enjoji Scale of Infant Analytical Development was used to evaluate/compare pre- and postoperative hearing level, word recognition score, speech discrimination score, and language production and perception skills. The Picture Vocabulary Test-Revised was used to assess vocabulary understanding skill. Correlation between the final vocabulary understanding skill assessment and several factors was also examined. RESULTS: Improvement in hearing thresholds (mean: 106.0 dB) was greater after the first CI, (27-45 dB; mean: 33.8 dB) compared with hearing aid (48-74 dB; mean: 63.1 dB). Similarly, language perception and production were better in the CMV group. However, in the long term, differences between good and poor cases became prominent, especially in children with motor or cognitive delay and brain abnormalities who performed poorly in the CMV group. CONCLUSION: Long-term language perception and production after CI were overall satisfactory in congenital CMV-deafened children. CI was effective, particularly in the absence of CMV-induced disorders. However, this effectiveness was limited in those with motor or cognitive delay.

Clinical practice guidelines for the diagnosis and management of otitis media with effusion (OME) in children in Japan, 2015.

OBJECTIVE: To (1) indicate the definition, the disease state, methods of diagnosis, and testing for otitis media with effusion (OME) in childhood (<12 years); and (2) recommend methods of treatment in accordance with the evidence-based consensus reached by the Subcommittee of Clinical Practice Guideline for Diagnosis and Management of OME in Children. METHODS: We produced Clinical Questions (CQs) concerning the treatment of OME and searched the literature published until April 2014 according to each theme including CQ, the definition, the disease state, the method of diagnosis, and examination. The recommendations are based on the results of the literature review and the expert opinion of the Subcommittee. RESULTS: Because children with Down's syndrome and cleft palate are susceptible to OME, we categorized OME into low-risk and high-risk groups (e.g., Down's syndrome and cleft palate), and recommended the appropriate treatment for each group. CONCLUSION: In the clinical management of OME in children, Japanese Clinical Practice Guidelines recommend management not only of OME itself, such as effusion in the middle ear and pathological changes in the tympanic membrane, but also pathological abnormality in surrounding organs, such as infectious or inflammatory diseases.

PET-CT observations of cortical activity in pre-lingually deaf adolescent and adult patients with cochlear implantation.

CONCLUSIONS: The present study yielded useful information concerning pre-lingually deaf adolescents and adults who try or manage to understand languages. PET-CT can provide insights into brain plasticity and elucidate which mode of communication is the most effective for education of such patients. OBJECTIVES: To study the cortical activity in pre-lingually deaf adolescent and adult cochlear implants (CI) users who have been trained in auditory-verbal/oral communication since childhood. METHODS: Using positron emission tomography (PET) and 18F-fluorodeoxyglucose (FDG), brain activities in six pre-lingually and two post-lingually deaf CI users (mean age at CI surgery = , 20.3 years; three males, five females) were compared with those of 10 normal age-matched controls (mean age = 27.1 years). Regional cerebral blood flow changes were measured during an acoustic presentation of a story. RESULTS: In compliant CI users, the number of hypermetabolic auditory-related areas was greater in those who had a CI in their 20s than in those who did so in their adolescence. In poor and non-compliant users, hypermetabolism was not seen in the auditory association area, but in the primary auditory areas (BA41) and the Broca's area (BA45). In post-lingually deaf CI users, no increase in the number of hypermetabolic areas was found in auditory-related regions.

Relationship between CT findings and sensorineural hearing loss in chronic otitis media.

OBJECTIVE: To investigate the relationships between the temporal bone CT findings and sensorineural hearing loss in ears with non-cholesteatomatous chronic otitis media (COM). METHODS: Preoperative bone conduction (BC) hearing thresholds of 266 patients (304 ears) with COM were compared with those of 342 normal individuals (440 ears) by audiometry. The incidence of abnormal BC threshold at lower frequencies (250-1000 Hz) and at higher frequencies (2000-4000 Hz) were examined and the differences between control and COM groups were compared by using χ2 test. In the COM group, the cross-sectional area of the mastoid air cells based on the axial CT image (n=255) were correlated with the results of BC threshold. RESULTS: The percentage in the COM group exceeds 15% in their 50s at lower frequencies while in their 40s at higher frequencies. The BC thresholds were significantly better in the group with normal mastoid area than in those with smaller mastoid area at each decadal age group. The BC impairment from COM becomes worse as the course of the disease progresses and deteriorated from 40s rapidly, especially at higher frequencies and in the group with smaller mastoid area. CONCLUSION: These results recommend that early treatment, including surgery, should be considered as early as possible before BC impairment occurs, especially for COM cases with smaller mastoid area, which may indicate the existence of more severe otitis media since earlier childhood.

GJB2-associated hearing loss undetected by hearing screening of newborns.

The hearing loss caused by GJB2 mutations is usually congenital in onset, moderate to profound in degree, and non-progressive. The objective of this study was to study genotype/phenotype correlations and to document 14 children with biallelic GJB2 mutations who passed newborn hearing screening (NHS). Genetic testing for GJB2 mutations by direct sequencing was performed on 924 individuals (810 families) with hearing loss, and 204 patients (175 families) were found to carry biallelic GJB2 mutations. NHS results were obtained through medical records. A total of 18 pathological mutations were identified, which were subclassified as eight inactivating and 10 non-inactivating mutations. p.I128M and p.H73Y were identified as novel missense GJB2 mutations. Of the 14 children with biallelic GJB2 mutations who passed NHS, eight were compound heterozygotes and 3 were homozygous for the c.235delC mutation in GJB2, and the other three combinations of non-c.235delC mutations identified were p.Y136X-p.G45E/p.V37I heterozygous, c.512ins4/p.R143W heterozygous, and p.V37I/p.R143W heterozygous. These 14 cases demonstrate that the current NHS does not identify all infants with biallelic GJB2 mutations. They suggest that the frequency of non-penetrance at birth is approximately 6.9% or higher in DFNB1 patients and provide further evidence that GJB2 hearing loss may not always be congenital in onset.

Long term speech perception after cochlear implant in pediatric patients with GJB2 mutations.

OBJECTIVES: To determine the long term effect of cochlear implant (CI) in children with GJB2-related deafness in Japan. METHODS: Genetic testing was performed on 29 children with CI. The speech perception in 9 children with GJB2 gene-related deafness fitted with CI was compared with those in matched 10 children who were diagnosed as having no genetic loci. The average follow-up period after CI was 55.9 months and 54.6 months, respectively. RESULTS: A definitive inherited hearing impairment could be confirmed in 12 (41.4%) of the 29 CI children, including 10 with GJB2-related hearing impairment and 2 with SLC26A4-related hearing impairment. The results of IT-MAIS, word or speech perception testing under the noise, and development of speech perception and production testing using the Enjoji scale were slightly better for the GJB2 group after CI than for the control group without statistical significant difference. CONCLUSION: The long-term results of this study show that CI is also effective in the development of speech performance after CI in Japanese children with GJB2-related hearing impairments as HL due to other etiologies.

What factors are associated with good performance in children with cochlear implants? From the outcome of various language development tests, research on sensory and communicative disorders project in Japan: nagasaki experience.

OBJECTIVES: We conducted multi-directional language development tests as a part of the Research on Sensory and Communicative Disorders (RSVD) in Japan. This report discusses findings as well as factors that led to better results in children with severe-profound hearing loss. METHODS: We evaluated multiple language development tests in 33 Japanese children with cochlear implants (32 patients) and hearing aid (1 patient), including 1) Test for question and answer interaction development, 2) Word fluency test, 3) Japanese version of the Peabody picture vocabulary test-revised, 4) The standardized comprehension test of abstract words, 5) The screening test of reading and writing for Japanese primary school children, 6) The syntactic processing test of aphasia, 7) Criterion-referenced testing (CRT) for Japanese language and mathematics, 8) Pervasive development disorders ASJ rating scales, and 9) Raven's colored progressive matrices. Furthermore, we investigated the factors believed to account for the better performances in these tests. The first group, group A, consisted of 14 children with higher scores in all tests than the national average for children with hearing difficulty. The second group, group B, included 19 children that scored below the national average in any of the tests. RESULTS: Overall, the results show that 76.2% of the scores obtained by the children in these tests exceeded the national average scores of children with hearing difficulty. The children who finished above average on all tests had undergone a longer period of regular habilitation in our rehabilitation center, had their implants earlier in life, were exposed to more auditory verbal/oral communication in their education at affiliated institutions, and were more likely to have been integrated in a regular kindergarten before moving on to elementary school. CONCLUSION: In this study, we suggest that taking the above four factors into consideration will have an affect on the language development of children with severe-profound hearing loss.

Bilateral cochlear implantation for children in nagasaki, Japan.

OBJECTIVES: The number of patients with bilateral cochlear implant (CI) has gradually increased as patients and/or parents recognize its effectiveness. The purpose of this report is to evaluate the efficacy of 29 bilateral CI out of 169 pediatric CI users, who received auditory-verbal/oral habilitation at our hearing center. METHODS: We evaluated the audiological abilities 29 Japanese children with bilateral CIs including wearing threshold, word recognition score, speech discrimination score at 1 m from front speaker (SP), 1 m from second CI side SP, speech discrimination score under the noise (S/N ratio=80 dB sound pressure level [SPL]/70 dB SPL, 10 dB) at 1 m from front SP, word recognition score under the noise (S/N ratio=80 dB SPL/70 dB SPL, 10 dB) at 1 m from front SP. RESULTS: Binaural hearing using bilateral CI is better than first CI in all speech understanding tests. Especially, there were significant differences between the results of first CI and bilateral CI on SDS at 70 dB SPL (P=0.02), SDS at 1 m from second CI side SP at 60 dB SPL (P=0.02), word recognition score (WRS) at 1 m from second CI side SP at 60 dB SPL (P=0.02), speech discrimination score (SDS) at 1 m from front SP under the noise (S/N=80/70; P=0.01) and WRS at 1 m from front SP under the noise (S/N=80/70; P=0.002). At every age, a second CI is very effective. However, the results of under 9 years old were better than of over 9 years old on the mean SDS under the noise (S/N=80/70) on second CI (P=0.04). About use of a hearing aid (HA) in their opposite side of first CI, on the WRS and SDS under the noise, there were significant differences between the group of over 3 years and the group of under 10 months of HA non user before second CI. CONCLUSION: These results may show important binaural effectiveness such as binaural summation and head shadow effect. Bilateral CI is very useful medical intervention for many children with severe-to-profound hearing loss in Japan as well as elsewhere.