ROBINSON KOJI TSUJI
Projetos de Pesquisa
Unidades Organizacionais
Instituto Central, Hospital das Clínicas, Faculdade de Medicina
LIM/32 - Laboratório de Otorrinolaringologia, Hospital das Clínicas, Faculdade de Medicina
LIM/32 - Laboratório de Otorrinolaringologia, Hospital das Clínicas, Faculdade de Medicina
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- Molecular and genetic characterization of a large Brazilian cohort presenting hearing loss(2022) BATISSOCO, Ana Carla; PEDROSO-CAMPOS, Vinicius; PARDONO, Eliete; SAMPAIO-SILVA, Juliana; SONODA, Cindy Yukimi; VIEIRA-SILVA, Gleiciele Alice; LONGATI, Estefany Uchoa da Silva de Oliveira; MARIANO, Diego; HOSHINO, Ana Cristina Hiromi; TSUJI, Robinson Koji; JESUS-SANTOS, Rafaela; ABATH-NETO, Osorio; BENTO, Ricardo Ferreira; OITICICA, Jeanne; LEZIROVITZ, KarinaHearing loss is one of the most common sensory defects, affecting 5.5% of the worldwide population and significantly impacting health and social life. It is mainly attributed to genetic causes, but their relative contribution reflects the geographical region's socio-economic development. Extreme genetic heterogeneity with hundreds of deafness genes involved poses challenges for molecular diagnosis. Here we report the investigation of 542 hearing-impaired subjects from all Brazilian regions to search for genetic causes. Biallelic GJB2/GJB6 causative variants were identified in 12.9% (the lowest frequency was found in the Northern region, 7.7%), 0.4% carried GJB2 dominant variants, and 0.6% had the m.1555A > G variant (one aminoglycoside-related). In addition, other genetic screenings, employed in selected probands according to clinical presentation and presumptive inheritance patterns, identified causative variants in 2.4%. Ear malformations and auditory neuropathy were diagnosed in 10.8% and 3.5% of probands, respectively. In 3.8% of prelingual/perilingual cases, Waardenburg syndrome was clinically diagnosed, and in 71.4%, these diagnoses were confirmed with pathogenic variants revealed; seven out of them were novel, including one CNV. All these genetic screening strategies revealed causative variants in 16.2% of the cases. Based on causative variants in the molecular diagnosis and genealogy analyses, a probable genetic etiology was found in similar to 50% of the cases. The present study highlights the relevance of GJB2/GJB6 as a cause of hearing loss in all Brazilian regions and the importance of screening unselected samples for estimating frequencies. Moreover, when a comprehensive screening is not available, molecular diagnosis can be enhanced by selecting probands for specific screenings.
- Can the use of the CROS system provide head shadow effect contribution to unilateral Cochlear Implant Users?(2022) HOSHINO, Ana Cristina Hiromi; GOFFI-GOMEZ, Maria Valeria Schmidt; SIERRA, Paola Angelica Samuel; AGRAWAL, Smita; RODRIGUEZ, Carina; CARVALHO, Ana Claudia Martinho de; TSUJI, Robinson KojiPurpose: The aim of this study was to evaluate the contribution of the CROS system on the head shadow effect in unilateral implant users. Methods: Prospective cross-sectional study, approved by the ethics committee under protocol 2.128.869. Eleven adults with post-lingual deafness users of unilateral Advanced Bionics CI were selected. Speech recognition was evaluated with recorded words presented at 65dBA at 0o azimuth and at 90o on the side contralateral to the CI, with noise at 55dBA, using CI alone and CI + CROS system. The results were analyzed using paired t-test with a 0.05 alpha. Results: The mean speech recognition scores were significantly better with CI + CROS in relation to the condition of CI alone (p <0.05, p <0.005 and p <0.005 respectively). In the presentation at 0o azimuth, no significant differences were found. Conclusion: Users of unilateral CI without useful residual hearing for the use of hearing aids or unable to undergo bilateral surgery can benefit from the CROS device for speech recognition, especially when the speech is presented on the side contralateral to the CI.
- Surdez pré-lingual: benefícios do implante coclear versus prótese auditiva convencional(2012) BITTENCOURT, Aline Gomes; TORRE, Ana Adelina Giantomassi Della; BENTO, Ricardo Ferreira; TSUJI, Robinson Koji; BRITO, Rubens deINTRODUCTION: The majority of patients with hearing loss, including those with severe hearing loss, benefits from the use of hearing aids. The cochlear implant is believed to achieve better results in a child with hearing loss in cases where the severity of disability renders hearing aids incapable of providing adequate sound information, as they require sufficient cochlear reserve so that acoustic detention occurs. OBJECTIVE: To assess if cochlear implants provide more benefit than conventional hearing aids in prelingually deaf patients. SUMMARY OF THE FINDINGS : The study was a systematic review of scientific papers selected by a search of the SciELO, Cochrane, MEDLINE, and LILACS-BIREME databases. Among the 2169 articles found, 12 studies proved relevant to the issue and presented an evidence strength rating of B. No publications rated evidence strength A. Seven of the studies analyzed were prospective cohorts and 5 were cross-sectional studies. CONCLUSION: Based on several studies, cochlear implants were demonstrated to be the best current alternative for bilateral severe or profound hearing loss, achieving better results in speech perception and development in prelingual children when compared to conventional hearing aids.
- Speech perception in adolescents with pre-lingual hearing impairment with cochlear implants(2011) SOUZA, Izi Patricia Souza de; BRITO, Rubens de; BENTO, Ricardo Ferreira; GOMEZ, Maria Valeria S. Goffi; TSUJI, Robinson Koji; HAUSEN-PINNA, MarianaProfound hearing loss is a disability that affects personality and when it involves teenagers before language acquisition, these bio-psychosocial conflicts can be exacerbated, requiring careful evaluation and choice of them for cochlear implant. Aim: To evaluate speech perception by adolescents with profound hearing loss, users of cochlear Implants. Study Design: Prospective. Materials and Methods: Twenty-five individuals with severe or profound pre-lingual hearing loss who underwent cochlear implantation during adolescence, between 10 to 17 years and 11 months, who went through speech perception tests before the implant and 2 years after device activation. For comparison and analysis we used the results from tests of four choice, recognition of vowels and recognition of sentences in a closed setting and the open environment. Results: The average percentage of correct answers in the four choice test before the implant was 46.9% and after 24 months of device use, this value went up to 86.1% in the vowels recognition test, the average difference was 45.13% to 83.13% and the sentences recognition test together in closed and open settings was 19.3% to 60.6% and 1.08% to 20.47% respectively. Conclusion: All patients, although with mixed results, achieved statistical improvement in all speech tests that were employed.
- Surgical complications in 550 consecutive cochlear implantation(2012) BRITO, Rubens; MONTEIRO, Tatiana Alves; LEAL, Aquiles Figueiredo; TSUJI, Robinson Koji; PINNA, Mariana Hausen; BENTO, Ricardo FerreiraCochlear implantation is a safe and reliable method for auditory restoration in patients with severe to profound hearing loss. Objective: To describe the surgical complications of cochlear implantation. Materials and Methods: Information from 591 consecutive multichannel cochlear implant surgeries were retrospectively analyzed. All patients were followed-up for at least one year. Forty-one patients were excluded because of missing data, follow-up loss or middle fossa approach. Results: Of 550 cochlear implantation analyzed, 341 were performed in children or adolescents, and 209 in adults. The mean hearing loss time was 6.3 +/- 6.7 years for prelingual loss and 12.1 +/- 11.6 years for postlingual. Mean follow-up was 3.9 +/- 2.8 years. Major complications occurred in 8.9% and minor in 7.8%. Problems during electrode insertion (3.8%) were the most frequent major complication followed by flap dehiscence (1.4%). Temporary facial palsy (2.2%), canal-wall lesion (2.2%) and tympanic membrane lesion (1.8%) were the more frequent minor complications. No death occurred. Conclusion: There was a low rate of surgical complications, most of them been successfully managed. These results confirm that cochlear implant is a safe surgery and most surgical complications can be managed with conservative measures or minimal intervention.
- Benefit of Cochlear Implantation in Children with Multiple-handicaps: Parent's Perspective(2018) NASRALLA, Heloisa Romeiro; MONTEFUSCO, Adilson Marcos; HOSHINO, Ana Cristina H.; SAMUEL, Paola Angelica; MAGALHAES, Ana Tereza de Matos; GOFFI-GOMEZ, Maria Valeria Schmidt; TSUJI, Robinson Koji; BENTO, Ricardo FerreiraIntroduction The cochlear implants centers in the world are studying the cochlear implantation in children with multiple handicaps. Objective To develop a questionnaire to analyze the subjective benefits of the cochlear implantation in multiple handicapped children according to their parent's perspective. Methods A questionnaire was applied to 14 families of multiple handicapped children, aging from 2-12 years old and having from 11 months to 11 years of implant use. Results The social-emotional abilities were improved because of many factors, such as: auditory exposure, which happened in 84% of the children; recognition of their own names, which increased in 56%; and development of eye contact, in 28% of the subjects. Other benefits appeared to be: music appreciation and more attention and adherence to other therapies and school activities. Besides, some children became interested in objects, playing with other children, and more adapted to daily routines. Thirty-five percent of the children acquired oral language, mainly the bilaterally implanted, while 14% of them were engaged in sign language. Although all of the children showed a significant improvement in communication, the emotional issues of some families and the severity of the handicaps negatively impacted the outcomes. In spite of the families' acknowledgement of some benefits, the diagnosis of autism spectrum disorder caused frustration, requiring a readjustment of the expectations. Conclusion The questionnaire turned out to be an adequate tool to reveal the social-emotional benefits of cochlear implantation. Although oral language was not the major outcome in these cases, the cochlear implant benefits involved the whole family. All of the families recommended the implant to other children in a similar situation.
- Cochlear implantation trough the middle cranial fossa: a novel approach to access the basal turn of the cochlea(2013) BITTENCOURT, Aline Gomes; TSUJI, Robinson Koji; TEMPESTINI, Joao Paulo Ratto; JACOMO, Alfredo Luiz; BENTO, Ricardo Ferreira; BRITO, Rubens deThe classic approach for cochlear implant surgery includes mastoidectomy and posterior tympanotomy. The middle cranial fossa approach is a proven alternative, but it has been used only sporadically and inconsistently in cochlear implantation. Objective: To describe a new approach to expose the basal turn of the cochlea in cochlear implant surgery through the middle cranial fossa. Method: Fifty temporal bones were dissected in this anatomic study of the temporal bone. Cochleostomies were performed through the middle cranial fossa approach in the most superficial portion of the basal turn of the cochlea, using the meatal plane and the superior petrous sinus as landmarks. The lateral wall of the internal acoustic canal was dissected after the petrous apex had been drilled and stripped. The dissected wall of the inner acoustic canal was followed longitudinally to the cochleostomy. Results: Only the superficial portion of the basal turn of the cochlea was opened in the fifty temporal bones included in this study. The exposure of the basal turn of the cochlea allowed the visualization of the scala tympani and the scala vestibuli, which enabled the array to be easily inserted through the scala tympani. Conclusion: The proposed approach is simple to use and provides sufficient exposure of the basal turn of the cochlea.
- Balancing the Loudness in Speech Processors and Contralateral Hearing Aids in Users of Unilateral Cochlear Implants(2021) MAGALHAES, Ana Tereza Matos; CARVALHO, Amanda; TSUJI, Robinson Koji; BENTO, Ricardo Ferreira; GOFFI-GOMEZ, Maria Valeria SchmidtIntroduction The use of cochlear implants and hearing aids (bimodal) has been growing with the expansion of the indication for them, and it is important to ensure protocols so that there is a balance of the loudness regarding the two devices. Objective To evaluate if the limited complex sounds present in the frequency bands of the current devices enable the balance of the loudness in adult users of bimodal stimulation, and to analyze if speech recognition improves after balancing. Methods A prospective cross-sectional study with convenience sampling. The sample was composed of 25 adults who had used either a cochlear implant for at least 6 months or a contralateral hearing aid, with a mean age of 46 years. The balancing of the loudness was performed in an acoustic room with the computer's sound box (0 degrees azimuth at 70dB SPL). The instrumental sounds were filtered through eight different frequency bands. The patients used both hearing devices and were asked if the sound was perceived to be louder in one of the ears or centrally. The speech test was evaluated with sentence silence (65dB SPL) and/or noise signal ratio of 0dB/+10dB in free field at 0 degrees azimuth, before and after balancing. Results : Out of the 25 patients, 5 failed to achieve balance at every tested frequency, and 3 achieved balance at almost every frequency, except 8kHz. There was a significant difference between the speech recognition test only in silence before and after balancing. Conclusion : Most patients achieved sound equalization at all evaluated frequencies under the complex-sound protocol. Additionally, most patients experienced improved speech recognition after balancing.
- Neurofibromatosis 2: hearing restoration options(2012) MONTEIRO, Tatiana Alves; GOFFI-GOMEZ, Maria Valeria Schmidt; TSUJI, Robinson Koji; GOMES, Marcos Queiroz Telas; BRITO NETO, Rubens Vuono; BENTO, Ricardo FerreiraNeurofibromatosis 2 (NF2) is an autosomal dominant disease in which hearing loss is predominant. Auditory restoration is possible using cochlear implants (CI) or auditory brainstem implant (ABI). Objective: To assess the auditory results of CI and ABI in NF2 patients and review the literature. Methods: Four NF2 patients were prospectively evaluated. They were submitted to tumor resection followed by ipsilateral CI or ABI depending on cochlear nerve preservation. Long term auditory results were described for CI (12 months) and ABI (48 months). Results: All patients achieved auditory perception improvements in their hearing thresholds. The CI patient does not recognize vowels or sentences. The 3 ABI patients discriminate 70% of vowels and 86% in the 4-choice test. One of them does not recognize sentences. The other two recognize 100% of closed sentences and 10% and 20% of open sentences. Conclusion: The choice of implant type to restore hearing to NF2 patients will relay on anatomical and functional cochlear nerve preservation during tumor resection surgery. Although our experience was different, the literature shows that if this condition is achieved, CI will offer better auditory results. If not, ABI is recommended.
- Auditory brainstem implant in postmeningitis totally ossified cochleae(2018) MALERBI, Andrea Felice dos Santos; GOFFI-GOMEZ, Maria Valeria Schmidt; TSUJI, Robinson Koji; GOMES, Marcos de Queiroz Teles; NETO, Rubens de Brito; BENTO, Ricardo FerreiraIntroduction: An auditory brainstem implant (ABI) is an option for auditory rehabilitation in patients with totally ossified cochleae who cannot receive a conventional cochlear implant. Objective: To evaluate the outcomes in audiometry and speech perception tests after the implantation of an ABI via the extended retrolabyrinthine approach in patients with postmeningitis hearing loss. Materials and methods: Ten patients, including children and adults, with postmeningitis hearing loss and bilateral totally ossified cochleae received an ABI in a tertiary center from 2009 to 2015. The extended retrolabyrinthine approach was performed in all the patients by the same surgeons. A statistical analysis compared pure tonal averages and speech perception tests before and at least 12 months after the ABI activation. Results: Eight patients (80%) showed improvements in tonal audiometry and the word and vowel perception tests after an average follow-up of 3.3 years. Two patients recognized up to 40% of the closed-set sentences without lip-reading. Two patients had no auditory response. Conclusions: The ABI improved hearing performance in audiometry and speech perception tests in cases of postmeningitis hearing loss. The extended retrolabyrinthine approach is a safe surgical option for patients with postmeningitis hearing loss and bilateral totally ossified cochleae.