Beatriz de Castro Andrade Mendes

Fatores determinantes no desenvolvimento de habilidades comunicativas em crianças com deficiência auditiva

PURPOSE To establish relationships between age at onset of individual hearing aid use, functional hearing, communication skills, family involvement and family expectations regarding language development of children diagnosed with hearing loss during the first three years of life. METHODS Thirty-five babies diagnosed with moderate to severe hearing loss who were receiving treatment at the Childrens Hearing Center/Derdic (CeAC) were evaluated during a period of 24 months. Assessments were carried out every six months and included: VRA--Visual reinforcement audiometry (with and without amplification); IT-MAIS; MUSS; and satisfaction of family regarding child development. RESULTS Cluster analysis was performed among the subjects. Consistent use of hearing aids was the only variable that exhibited a strong relationship with hearing and language skills. Children whose parents were not satisfied exhibited severe hearing loss and limited auditory capacity even with the use of hearing aid, and, consequently, poor auditory skills and speech production. CONCLUSION Datalogging monitoring can guide the knowledge of speech-language pathologists and audiologists and it can also be used on strategic planning. Family involvement, quality of parental participation in the intervention program as well as expectations about the future are also important aspects to consider as these can aid therapists and researchers on the assessment of deaf babies intervention effectiveness.

Pronto atendimento a usuários de dispositivos de amplificação sonora

PURPOSE: To establish the demographic and audiological characteristics of subjects users of hearing aids (HA) that requested a return at a Hearing Health Service, and to identify and analyze the reasons for the return and conducts generated during the Emergency Care (EC). METHODS: Participants were 440 subjects older than three years, with diagnosis and provision of hearing aids at the service where the research was conducted. Demographic and audiological characteristics and the reason for the use of hearing aids were collected from medical records and specific form used at the EC. Data were analyzed using descriptive and inferential statistics. RESULTS: The studied group was characterized mostly by female individuals, elderly, with incomplete elementary school education, moderate hearing loss, and bilateral sensorineural hearing loss. Most subjects used hearing aids with binaural BTE. As for the time of use, more than 70% used the hearing aid for the first time, 38% did so for a period inferior or equal to 12 months, and more than 65% for more than eight hours a day. The conducts hearing aid adjustment and orientation were more frequent for subjects with shorter use of the device, while the conducts technical assistance and replacement were more common for subjects with longer use. CONCLUSION: To identify the characteristics of patients users of HA, as well as the reasons of the return and conducts generated by the demand, contributes to better organization and quality of the service provided.

Characterization of a novel MYO3A missense mutation associated with a dominant form of late onset hearing loss

Whole-exome sequencing of samples from affected members of two unrelated families with late-onset non-syndromic hearing loss revealed a novel mutation (c.2090 T > G; NM_017433) in MYO3A. The mutation was confirmed in 36 affected individuals, showing autosomal dominant inheritance. The mutation alters a single residue (L697W or p.Leu697Trp) in the motor domain of the stereocilia protein MYO3A, leading to a reduction in ATPase activity, motility, and an increase in actin affinity. MYO3A-L697W showed reduced filopodial actin protrusion initiation in COS7 cells, and a predominant tipward accumulation at filopodia and stereocilia when coexpressed with wild-type MYO3A and espin-1, an actin-regulatory MYO3A cargo. The combined higher actin affinity and duty ratio of the mutant myosin cause increased retention time at stereocilia tips, resulting in the displacement of the wild-type MYO3A protein, which may impact cargo transport, stereocilia length, and mechanotransduction. The dominant negative effect of the altered myosin function explains the dominant inheritance of deafness.

Hearing loss classification by degree, configuration and relationship with amplified Speech Intelligibility Index (SII)

Purpose To establish the relationship between speech intelligibility index (SII) values generated at the verification of hearing aids programmed according to DSLm [i/o] v5 prescription rule and a proposed individual classification that considers the combination of hearing loss degree and configuration. Methods Forty-one children aged between 4 and 80 months were selected, totaling 78 ears for analysis. We considered hearing thresholds at the frequencies of 250, 500, 1000, 2000 and 4000 Hz; and analyzed values of the Speech Intelligibility Index (SII) for the input signal of 65 dB SPL obtained during the verification of hearing aids using the equipment Verifit®Audioscan. Results Hearing losses were classified into five homogeneous groups regarding audiometric degree and configuration. The groups were heterogeneous when compared to each other. From the groups, three ranges of SII values were determined. Equations were developed for classification of hearing loss according to groups and for determination of the adjusted SII values. Conclusion The SII value is a useful indicator of audibility for speech sounds in different characteristics of hearing losses, and can guide observations of auditory skills. The SII has stronger relationship with the association of the audiometric degree and configuration when compared with degree of hearing loss alone.

LittlEars® – Questionário auditivo: adaptação semântica e cultural da versão em Português Brasileiro em pais de crianças com deficiência auditiva

Purpose Semantic and cultural adaptation of a preliminary Portuguese version of the questionnaire LittlEars® in families of children with hearing loss. Methods Instrument administered as an interview or questionnaire, using a validated translation of LittlEars® Questionnaire in Portuguese with parents of children with hearing loss up to two years of hearing age. Parent’s comments, questions, suggestions, examples, comments or difficulties were used for development of the adapted version. A brainstorming session with a group of experts was held to validate the final version. Results Participants were 37 individuals who answered the questionnaire, parents of 32 children. Only four parents preferred to answer the questionnaire format, while 28 preferred to answer it as an interview. In the interview format, parents had difficulty understanding different items. Upon review and discussion of the comments of the families, a modified version was proposed. Conclusion Changes in the questionnaire items were related to vocabulary, idiomatic expressions and unknown examples. The results of the questionnaire LittlEars® varied depending on hearing age and Speech Intelligibility Index - SII, suggesting that the adapted translation has sensitivity for measuring the hearing abilities. Other studies should apply the new version and final validation.