Risk Factors Associated with Delays in Hearing Loss Identification in Pediatric Patients.

OBJECTIVE: To identify sociodemographic factors associated with pediatric late-identified hearing loss (LIHL) and classify novel subgroups within the LIHL population. STUDY DESIGN: Retrospective cohort. SETTING: Tertiary children's hospital. METHODS: Our cohort included children with permanent hearing loss (HL) between 2012 and 2020 (n = 1087). Patients with early-identified HL were compared to patients with LIHL (>6 months of age at diagnosis), and 3 subgroups: (1) late-identified congenital HL: failed NHS but had a diagnostic audiogram >6 months old; (2) late-onset HL: passed NHS and identified with HL after 6 months old; (3) late-identified, unknown-onset: unknown NHS results, identified after 6 months old. Geospatial analysis was performed using ArcGIS Pro. RESULTS: Compared with early-identified children, children with LIHL were more likely to have more comorbidities (odds ratio [OR] = 1.12, [1.01, 1.23]), be an under-represented minority (URM) (OR = 1.92, [1.27, 2.93]) and have a higher social vulnerability index (SVI) (adjusted odds ratio [AOR] = 2.1, [1.14, 3.87]). However, subgroups in the LIHL cohort had variable associations. Children with late-identified unknown onset hearing loss were uniquely associated with a primarily non-English speaking household (AOR = 1.84, [1.04, 3.25]), whereas children with late-onset hearing loss were less likely to have public insurance (AOR = 0.47, [0.27, 0.81]. There were no significant associations for children with late-identified congenital hearing loss. Neighborhood disadvantage, as measured by SVI, had an increased association with late-identified unknown onset HL (AOR = 4.08, [2.01, 8.28]) and a decreased association with late-onset HL (AOR = 0.40, [0.22, 0.72]). CONCLUSION: Sociodemographic factors serve as proxies for health care access, and these factors vary across LIHL pathways. Understanding the risk factors associated with each LIHL subgroup may help address disparities in pediatric HL identification.

Associations Between Age-Related Hearing Loss, Cognitive Impairment, and Multiple Chronic Conditions in a Group Care Setting.

PURPOSE: The purpose of this study was to explore the relationships between hearing loss, cognitive status, and a range of health outcomes over a period of 2 years in a sample of older adults who are enrolled in Program of All-Inclusive Care for the Elderly, which is a Medicare/Medicaid beneficiary program for individuals who are nursing home eligible but living in the community at time of enrollment. METHOD: The sample (N = 144) includes a diverse (47% White/non-Hispanic, 35% Black/African American, and 16% Latin/Hispanic) group of adults ranging from 55 to 93 years old. We used medical chart data to measure respondents' cognitive and health status, including chronic conditions and hospital use. Hearing status was measured once at the beginning of the 2-year review period. We used logistic regression and negative binomial hurdle models for analyses. We used latent class analysis (LCA) to explore the extent to which respondents cluster into a set of "health profiles" characterized by their hearing, cognitive status, and health conditions. RESULTS: We found that hearing loss is weakly associated with heart disease and diabetes and associated with cerebrovascular disease and falls; cognitive impairment is also associated with cerebrovascular disease and the number of falls. LCA indicates that respondents cluster into a variety of health profiles with a consistent pairing of hearing loss and depression. CONCLUSIONS: The results are largely consistent with associations reported in epidemiological studies that include age-related hearing loss. Of particular interest in this study is the LCA that suggested that all of the profiles associated with a high likelihood of hearing loss included a high risk of depression. The co-occurrence of these two factors highlights the need to identify and treat hearing loss in older adults, especially as part of the treatment plan for individuals with depressive symptoms.

Cochlear nerve dysplasia in unilateral severe to profound congenital sensorineural hearing loss - Prevalence in Australian children and the impact of socioeconomic disadvantage on its management.

INTRODUCTION: Congenital unilateral sensorineural hearing loss (cuSNHL) carries potentially significant social, educational, and developmental consequences. Early diagnosis enables investigation, and consideration of options for management and early intervention, helping to mitigate the effects of hearing loss. Cochlear nerve dysplasia (CND) is a prominent cause of cuSNHL and may affect candidacy for cochlear implantation. Socioeconomic disadvantage may impact on a patient's family's capacity to participate in necessary intervention and follow-up. METHODS: Infants with severe-profound cuSNHL referred to a large Australian quaternary pediatric center between October 2004 and December 2020 were retrospectively included. Audiometric and clinical data, and the presence of hearing loss risk factors were obtained from a prospectively collated database. In Australia MRI scans are provided free-of-charge to citizens and residents. MRI scans were reviewed to determine the status of the nerves within the internal acoustic meatus (IAM grade) along with attendance rates. Travel distance to the hospital was also calculated. Reasons for non-attendance at MRI were obtained from patient medical records and correspondence. Socioeconomic, educational, and occupational indices, and travel distances were obtained using patient residential postcodes with reference to Australian Bureau of Statistics data. RESULTS: A total of 98 patients were reviewed, 64.3% (n = 63) of whom underwent MRI. The median age at diagnosis was 40 days (IQR 27). The prevalence of CND was 75% (n = 47). Importantly, there was no significant difference in the degree of hearing loss between IAM grades (F(4,57) = 1.029, p = 0.405). Socioeconomic indices were significantly lower in patients not attending MRI investigations compared with patients who did attend. Travel distance was not significantly different between the two groups. CONCLUSION: CND is a prominent cause of cuSNHL in Australian infants. MRI at a young age allows parent education regarding management options and timely intervention where indicated. Socioeconomic disadvantage significantly impacts on participation in further routine assessment of cuSNHL, potentially limiting management options for these children long term.

[Application of ISO 1999:2013 (E) model in risk assessment of hearing loss caused by industrial noise].

OBJECTIVE: To assess the risk of hearing loss caused by industrial noise exposure of welders and assemblers in a mechanical equipment manufacturing enterprise, and to explore the practical application and possible underestimation of ISO 1999∶2013(E) model. METHODS: A total of 829 noise-exposed male workers from a mechanical equipment manufacturing enterprise were selected as study subjects. The questionnaire survey was administered, and individual noise exposure level(L_(Aeq.8 h)) and hearing loss level were measured. The risk assessment method of ISO 1999∶2013(E) was used to calculate the change of hearing threshold level and the risk of noise-induced hearing loss. By comparing the median of permanent hearing threshold shift caused by actual noise with the median of ISO1999 predicted value, the reason of the difference between the predicted value of ISO 1999∶2013(E) model and the actual value was analyzed. RESULTS: The L_(Aeq.8 h )was 89.5 dB(A), 77.4%(n=62)of the individual noise exposure levels exceeded 85 dB(A), and 24.6% of the participants(n=829) had different degrees of hearing loss. There was significant difference in hearing loss rate between welding and assembly positions(χ~2=10.07, P<0.01). The risk of noise-induced high-frequency hearing loss of 90% welders was 11.2% at the age of 50, and 14.0% at the age of 60. The risk of noise-induced deafness of 90% welders was 4.3% at the age of 60. The risk of high noise-induced frequency hearing loss of 90% assemblers was at the range of 4.0%-9.0% at the age of 40, 20.8% at the age of 50, and 22.5% at the age of 60. The risk of noise-induced deafness of 90% assemblers was at the range of 1.4%-6.4% at the age of 50, and 9.0% at the age of 60. Compared with actual median of permanent hearing threshold shift, ISO1999∶2013(E) predictions underestimated the median of permanent hearing threshold shift at 10.7 dB. CONCLUSION: The noise hazards of welding and assembly positions in mechanical equipment manufacturing enterprises are high relatively. ISO1999∶2013(E) can be used to predict the risk of noise-induced hearing loss in workers, but attention should be paid to the risk underestimation of this model.

Achieving universal reproductive health coverage for deaf women in Ghana: an explanatory study of knowledge of contraceptive methods, pregnancy and safe abortion practices.

BACKGROUND: The first world conference on sexual and reproductive health (SRH) in 1994 helped create the awareness that reproductive health is a human right. Over the years, attempts have been made to extend services to all persons; however, lapses persist in service provision for all in need. Recently, countries have been encouraged to target minority groups in their reproductive health service provision. However, studies have rarely attempted to develop deeper insights into the experiences of deaf men and women regarding their knowledge of SRH. The purpose of this study was to develop an in-depth understanding of the knowledge of deaf persons regarding services such as knowledge of contraceptive methods, pregnancy and safe abortion practices. METHODS: A sequential explanatory mixed-methods approach was adopted for this study. In the first quantitative phase, 288 deaf persons recruited from three out of the 16 regions in Ghana participated in this study. They completed a 31-item questionnaire on the main issues (knowledge of contraceptive methods, pregnancy and safe abortion practices) addressed in this study. In the second phase, a semi-structured interview guide was used to collect data from 60 participants who took part in the first phase. The key trend emerging in the first phase underpinned the interview guide used for the data collection. While the quantitative data were subjected to the computation of means, t-tests, analyses of variance, correlations and linear regressions to understand the predictors, the in-depth interviews were analysed using the thematic method of analysis. RESULTS: The results showed a convergence between the quantitative and qualitative data. For instance, the interview material supported the initial findings that deaf women had little knowledge of contraceptive methods. The participants offered reasons explaining their inability to access services and the role of religion in their understanding of SRH. CONCLUSION: The study concludes by calling on policymakers to consider the needs of deaf persons in future SRH policies. The study limitations and other implications for future policymaking are discussed.

Assessment of Receptive and Expressive Language Skills Among Young Children With Prelingual Single-Sided Deafness Managed With Early Cochlear Implantation.

Importance: Pediatric single-sided deafness (SSD) can seriously affect development, causing impaired spatial hearing skills, speech-language delays, and academic underachievement. Early cochlear implantation likely improves hearing-related outcomes, but its association with language development remains unclear. Objective: To investigate whether early cochlear implantation is associated with language outcomes for children with prelingual SSD. Design, Setting, and Participants: The Cochlear Implant for Children and One Deaf Ear study was initiated in 2015 and recruited participants at 4 academic hospitals in Flanders, Belgium, through 2019. This cohort study included 3 groups of children aged 2 to 5 years: children with SSD and a cochlear implant, children with SSD without a cochlear implant, and a control group with normal hearing. Language and hearing skills were assessed 1 to 2 times per year until the age of 10 years. Study completion rates were high (82%). Data analysis was performed from October to December 2020. Exposure: Unilateral cochlear implant. Main Outcomes and Measures: Longitudinal vocabulary, grammar, and receptive language scores. The implanted group was hypothesized to outperform the nonimplanted group on all language tests. Results: During the recruitment period, 47 children with prelingual SSD without additional disabilities were identified at the participating hospitals. Fifteen of the 34 children with an intact auditory nerve received a cochlear implant (44%, convenience sample). Sixteen of the remaining children were enrolled in the SSD control group (50%). Data from 61 children (mean [SD] age at the time of enrollment, 2.08 [1.34] years; 26 girls [42%]) were included in the analysis: 15 children with SSD and a cochlear implant, 16 children with SSD without a cochlear implant, and 30 children with normal hearing. Children with SSD and a cochlear implant performed in line with their peers with normal hearing with regard to grammar. In contrast, children with SSD without a cochlear implant had worse grammar scores than the group with implants (-0.76; 95% CI, -0.31 to -1.21; P = .004) and the group with normal hearing (-0.53; 95% CI, -0.91 to -0.15; P = .02). The 3 groups had similar vocabulary and receptive language abilities. Conclusions and Relevance: These findings suggest that early cochlear implantation is associated with normal grammar development in young children with prelingual SSD. Although further follow-up will reveal the long-term outcomes of the cochlear implant for other skills, the current results will help clinicians and policy makers identify the best treatment option for these children.

Applying Kurtosis as an Indirect Metric of Noise Temporal Structure in the Assessment of Hearing Loss Associated With Occupational Complex Noise Exposure.

OBJECTIVE: The association of occupational noise-induced hearing loss (NIHL) with noise energy was well documented, but the relationship between occupational noise and noise temporal structure is rarely reported. The objective of this study was to investigate the principal characteristics of the relationship between occupational NIHL and the temporal structure of noise. METHODS: Audiometric and shift-long noise exposure data were collected from 3102 Chinese manufacturing workers from six typical industries through a cross-sectional survey. In data analysis, A-weighted 8-h equivalent SPL (LAeq.8h), peak SPL, and cumulative noise exposure (CNE) were used as noise energy indicators, while kurtosis (ß) was used as the indicator of noise temporal structure. Two NIHL were defined: (1) high-frequency noise-induced hearing loss (HFNIHL) and (2) noise-induced permanent threshold shift at test frequencies of 3, 4, and 6 kHz (noise-induced permanent threshold shift [NIPTS346]). The noise characteristics of different types of work and the relationship between these characteristics and the prevalence of NIHL were analyzed. RESULTS: The noise waveform shape, with a specific noise kurtosis, was unique to each type of work. Approximately 27.92% of manufacturing workers suffered from HFNIHL, with a mean NIPTS346 of 24.16 ± 14.13 dB HL. The Spearman correlation analysis showed that the kurtosis value was significantly correlated with the difference of peak SPL minus its LAeq.8h across different types of work (p < 0.01). For a kurtosis-adjusted CNE, the linear regression equation between HFNIHL% and CNE for complex noise almost overlapped with Gaussian noise. Binary logistic regression analysis showed that LAeq.8h, kurtosis, and exposure duration were the key factors influencing HFNIHL% (p < 0.01). The notching extent in NIPTS at 4 kHz became deeper with the increase in LAeq.8h and kurtosis. HFNIHL% increased most rapidly during the first 10 years of exposure. HFNIHL% with ß ≥ 10 was significantly higher than that with ß < 10 (p < 0.05), and it increased with increasing kurtosis across different CNE or LAeq.8h levels. When LAeq.8h was 80 to 85 dB(A), the HFNIHL% at ß ≥ 100 was significantly higher than that at 10 ≤ ß < 100 or ß < 10 (p < 0.05 and p < 0.01, respectively). CONCLUSIONS: In the evaluation of hearing loss caused by complex noise, not only noise energy but also the temporal structure of noise must be considered. Kurtosis of noise is an indirect metric that is sensitive to the presence of impulsive components in complex noise exposure, and thus, it could be useful for quantifying the risk for NIHL. It is necessary to re-evaluate the safety of permissible exposure limit of 85 dB(A) as noise with a high kurtosis value can aggravate or accelerate early NIHL.

The feasibility, validity and reliability of a child friendly vestibular assessment in infants and children candidates to cochlear implant.

OBJECTIVES: The pediatric vestibular assessment has developed significantly in the past two decades, especially in terms of assessment of cochlear implant (CI) candidates. Different methods and test protocols have been applied, however without a general consensus. We present here the feasibility, validity and reliability of a child friendly vestibular testing in use at our department for the assessment of pediatric CI candidates. METHODS: The test battery consisted of head impulse test (HIT), video head impulse test (vHIT), cervical evoked myogenic potentials (cVEMP) and mini ice water caloric test (mIWC), all adapted from previous methods, mentioned in literature. We defined specific criteria for test feasibility, for test validity and test positivity (i.e. indicating vestibular insufficiency). The reliability of the whole protocol was assessed with test agreement analysis. RESULTS: Complete data from 35 children, all CI candidates, age ranging 4-79 months (67% under 2 years) and recruited over two years, were obtained. All but one child could complete at least one test with valid responses bilaterally, with the best compliance for HIT (97,1%) and least for cVEMP (68,6%). Feasibility did not appear to be affected by age or hearing loss etiology. Among the valid responses there was a substantial test agreement between HIT and vHIT, moderate agreement between vHIT/HIT and mIWC and no apparent agreement between the canal tests and cVEMP. Simple clinical guidelines were introduced to solve the tests' disagreement and to improve the protocol reliability: a) a pathological response had to be confirmed in at least two different canal tests and in at least three cVEMP trials; b) a canal/otolith disagreement was interpreted as a partial vestibular loss to be opposed to a complete vestibular insufficiency. CONCLUSIONS: The search for vestibular insufficiency in infants and young children can be attained with an opportunely adapted vestibular assessment, such the test battery proposed here. That assessment resulted easy to conduct and to interpret in a representative sample of CI candidates in preschool age, the most of whom were younger than 2 years. This method appears to particularly suit the demands of a vestibular assessment in young children CI candidates.

[Exploration of Service Needs of Persons who are Deaf, Communicate in LSQ and who Present Mental Health Issues]. / Exploration des besoins de services des personnes sourdes locutrices LSQ présentant des troubles de santé mentale.

Introduction Lack of access to mental health services for persons who are deaf and communicate in LSQ and who present mental health issues has been noted by service providers. However, very few studies have examined the needs of this population in Quebec. Objective The purpose of this study was to explore the needs with regards to services of persons who are deaf and communicate in LSQ and who have mental health issues. Methods A qualitative single case study design was used. The case was the territory affiliated to a specialized rehabilitation center in deafness and communication in Montreal. The sample was composed of clinicians and administrators of this center and community organizations (n = 12), persons who are deaf and communicate in LSQ and had mental health issues (n = 4), as well as family members (n = 1). Each participant took part in a semi-structured individual interview. A verification of the information provided was done in person or by phone one month later. A thematic content analysis was done for all the transcriptions, following the recommendations of Miles and Huberman (2003). Results Certain services that were seen as needed did not exist, such as support groups and networks for the deaf who have mental health issues or leisure activities in community services. Certain services were available but several obstacles limited their use. For example, a lack of knowledge about deafness and the deaf culture in the health care system that lead to consequences such as incorrect diagnoses or difficulty in establishing a therapeutic alliance. Conclusion What seemed to be consensual among these participants was the importance to have access to clinicians in the various health care establishments with a very good knowledge of mental illness, deafness as well as linguistic and cultural aspects that characterize the deaf population.

Understanding the Needs of Families of Children Who Are Deaf/Hard of Hearing with an Autism Spectrum Disorder.

BACKGROUND: There is a significant lack of evidence guiding our understanding of the needs of families of children who are deaf/hard of hearing (Deaf/HH) with an autism spectrum disorder (ASD). Much of our current knowledge is founded in case report studies with very small numbers of children with the dual diagnosis. PURPOSE: The purpose of this study was to gain an understanding of the factors relating to caregiver stress and needs (i.e., supports and interventions) in families of children who are Deaf/HH with ASD. RESEARCH DESIGN: Comparison groups of families of children who were Deaf/HH, families with a hearing child with ASD, and families of children who were Deaf/HH with ASD were administered standardized questionnaires of stress with brief qualitative questionnaires focusing on family-identified needs. STUDY SAMPLE: Six families of children with the dual diagnosis, four families of children who were Deaf/HH, and three families of children with ASD. DATA COLLECTION AND ANALYSIS: Surveys included demographic and support questionnaires, the Parenting Stress Index (PSI), the Pediatric Hearing Impairment Caregiver Experience, and a qualitative questionnaire. RESULTS: Families of children who were Deaf/HH with ASD had a higher median total stress score on the PSI as compared to families of children who were Deaf/HH only (58.5 versus 41.5, respectively; p = 0.02) and higher Child Domain scores (60 versus 43, respectively; p = 0.02), indicating higher levels of stress in families of children with the dual diagnosis. The families of children who were Deaf/HH with ASD reported similar levels of stress as families of children with ASD. CONCLUSIONS: Families of children who are Deaf/HH with an ASD experience stress and describe similar needs and priorities as families of hearing children with ASD. This suggests the needs related to having an autism spectrum disorder are of high priority in families of children with the dual diagnosis.

Assessment of cancers' diagnostic stage in a Deaf community - survey about 4363 Deaf patients recorded in French units.

BACKGROUND: Deaf people represent 0.1% of the French population and their access to public health campaigns is limited due to their frequent illiteracy and the infrequent use of sign language in campaigns. There is also a lack of general health knowledge in spite of the existence of French Deaf Care Units (UASS). The aim of this study is to assess the average diagnostic stage of cancer in the Deaf Community and discuss deafness as a contributing factor. METHODS: Four thousand three hundred sixty-three Deaf patients recorded in five UASS, 80 diagnosed between 2005/01/01 and 2014/12/31 were selected from medical records and/or ICD-10 coding. Data regarding cancers were extracted, grouped by stage and compared to literature. Statistical significance was tested with Fisher's Exact Test. RESULTS: Eighty patients were selected. Most cancers were diagnosed at advanced stages: of 11 prostate cancers, 46% were locally advanced and 18% were metastatic. (In the general population, this was respectively 3% and 10.4% (p < 0.01)). Of six colorectal cancers, 67% were diagnosed at stage III and 33% at stage IV. (Respectively 20.6% and 26.6% (p = 0.03) in the general population). In contrast, of the 15 breast cancers, 93% were diagnosed at stages T1-T3 that was earlier than in the general population (p = 0.43). CONCLUSION: In this study, we observed a delay cancer diagnosis among Deaf people. Complicated and/or non-systematic screening procedures for cancers would be involved. Which is most likely the result of many factors (communication, medical knowledge). Increasing UASS coverage and health information campaigns in sign language could assist in earlier cancer diagnosis.

Dificuldades de profissionais na atenção à saúde da pessoa com surdez severa / Professional difficulties in health care of patients with severe deafness / Dificultades profesionales en la atención en salud a la persona con sordera severa

Objetivo: Investigar as dificuldades de profissionais da saúde para a realização da consulta com a pessoa com surdez severa. Materiais e métodos: Estudo transversal, descritivo e qualitativo, realizado em março de 2011, na rede de atenção primária em saúde de Campina Grande - Paraíba. Utilizou-se um questionário estruturado, e 89 profissionais da saúde da Estratégia de Saúde da Família responderam sobre dificuldades para a consulta ao surdo. As respostas foram categorizadas por meio de Análise de Conteúdo na Modalidade Temática. Resultados: Dentre as dificuldades, destacaram-se: comunicação prejudicada, déficit na formação de recursos humanos para a consulta e reconhecimento das necessidades de saúde, infraestrutura inadequada para acolhimento e atendimento ao surdo, incerteza com relação aos cuidados em saúde prescritos na consulta e prejuízo da au tonomia do paciente. Conclusão: Percebeu-se que a comunicação prejudicada constitui uma barreira para a promoção de saúde e que profissionais e unidades de saúde não estão capacitados para acolher e atender às necessidades de saúde da pessoa com surdez severa.

Objective: To investigate the difficulties for health care professionals when dealing with patients with severe deafness in a medical appointment. Method: This is a cross-sectional, descriptive and qualitative study conducted in March 2011 in the Primary Health Care Network of Campina Grande - Paraíba. We used a structured questionnaire completed by 89 health professionals working in the Health Family Strategy. They were asked about the difficulties when dealing with deaf patients in a medical appointment. Their answers were categorized by means of a Thematic Content Analysis. Results: Among the main difficulties we find: impaired communication, a lack of trained human resources for these appointments and an insufficient recognition of these health needs, inappropriate infrastructure for deaf patient reception and care provision, uncertainty regarding the quality of the care provided at the medical appointment and the patient's lack of autonomy. Conclusions: We found that an impaired communication is a barrier to health promotion and that both health professionals and health units are not able to receive and meet the health needs of a patient with severe deafness.

Objetivo: Investigar las dificultades de los profesionales de salud para la realización de la consulta con la persona con sordera severa. Materiales y métodos: Estudio transversal, descriptivo y cualitativo, realizado en marzo de 2011, en la red de atención primaria de salud de Campina Grande - Paraíba. Se utilizó un cuestionario estructu rado, y 89 profesionales de salud de la Estrategia de Salud de la Familia respondieron sobre las dificultades para la consulta con el sordo. Las respuestas fueron categorizadas a través del Análisis de Contenido en la Modalidad Temática. Resultados: Entre las dificultades, se destacaron: comunicación perjudicada, déficit en la formación de recursos humanos para la consulta y reconocimiento de las necesidades de salud, infraestructura inadecuada para acoger y atender al sordo, incertidumbre con respecto a la atención sanitaria prescrita en la consulta y per juicio de la autonomía del paciente. Conclusiones: Se observó que el déficit en la comunicación constituye una barrera para la promoción de la salud y que profesionales y unidades de salud no están cualificados para acoger y atender a las necesidades de salud de la persona con sordera severa.

Maternally inherited diabetes and deafness (MIDD): diagnosis and management.

Maternally inherited diabetes with deafness is rare diabetes caused by a mitochondrial DNA defect. 85% of cases are associated with m.3243A>G mutation. It is important to diagnose this form of diabetes because of the unique management issues and associated comorbidities. A very strong family history of diabetes, deafness and presence of retinal dystrophy should prompt an investigation for MIDD. Microvascular complications out of keeping with duration of diabetes are another clue to the diagnosis. Retinal and renal manifestations of mitochondrial disease may be confused for diabetic complications. Glutamic acid decarboxylase (GAD) autoantibody negativity in a nonobese diabetic is another clue. Cardiac conduction defects and GDM may also raise suspicion as to the diagnosis. Recognizing this etiology of DM should promote family screening, genetic counseling, screening of associated comorbidities, avoidance of metformin, and cautious use of statins. We report a 77 years old lady with MIDD who was being followed up as insulin requiring type 2 diabetes. We then identified 5 more patients with MIDD in the same clinic. They all had A3243 mutation with characteristic clinical presentation. The pharmacological approaches discussed in the paper are unlikely to work in these patients as they were diagnosed late.

Review of intellectual assessment measures for children who are deaf or hard of hearing.

OVERVIEW: Intellectual assessment of children who are deaf or hard of hearing presents unique challenges to the clinician charged with attempting to obtain an accurate representation of the child's skills. Selection of appropriate intellectual assessment instruments requires a working knowledge of the strengths and weaknesses of the measure and what changes in standardized administration might be necessary to accommodate for the needs of children who are deaf or hard of hearing. In the case of some available instruments, there is limited guidance and objective research available examining the performance of children who are deaf or hard of hearing. This review summarizes available information on widely used and most recent editions of intellectual assessment measures with special attention to guidance on accommodations, score interpretation, subtest selection and other test-specific considerations when assessing children who are deaf or hard of hearing. SUMMARY: There is much opportunity for further inquiry in the field of intellectual assessment as it applies to children who are deaf or hard of hearing, as many measures have not been closely scrutinized for their appropriate use with this population. Clinicians must recognize inherent difficulties with intellectual assessment measures with children who are deaf or hard of hearing and issues in providing for an accessible and accurate administration of test items.

Children with Usher syndrome: mental and behavioral disorders.

BACKGROUND: Mental and behavioral disorders among adults with Usher syndrome have been discussed and reported in some case studies but no research has been reported on children with Usher syndrome. METHODS: This article investigates the prevalence and characteristics of mental and behavioral disorders among 26 children, 3-17 years of age, with Usher syndrome. RESULTS: Six of the 26 children were diagnosed with a mental or behavioral disorder (1 with schizophrenia and mild mental retardation, 1 with atypical autism and severe mental retardation, 1 with atypical autism and mild mental retardation, 1 with mild mental retardation, and 2 with conduct disorder). Another 3 children had had a mental or behavioral disorder previously in their childhood. CONCLUSION: Even though vision impairment first manifests in late childhood, some children with Usher syndrome seem to develop mental and behavioral disorders during childhood. The aetiology and treatment of mental and behavioral disorders among children with Usher syndrome are discussed. Children with Usher syndrome and their parents may need clinical support during early childhood to prevent development of mental and behavioral disorders.

Cervical cancer control: deaf and hearing women's response to an educational video.

Deaf people encounter barriers to accessing cancer information. In this study, a graphically enriched educational video about cervical cancer was created in American Sign Language, with English open captioning and voice overlay. Deaf (n = 127) and hearing (n = 106) women completed cancer knowledge surveys before and after viewing the video. Hearing women yielded higher scores before the intervention. Both groups demonstrated a significant increase in general and cervical cancer knowledge after viewing the video, rendering posttest knowledge scores nearly equal between the groups. These findings indicate that this video is an effective strategy for increasing cervical cancer knowledge among deaf women.

[Assessment of speech and linguistic disorders in patients with cochlear implant--preliminary results]. / Ocena mowy i zaburzen jezykowych u chorych z implantem slimakowym--doniesienie wstepne.

UNLABELLED: Cochlear Implant (CI) is the most advanced technical solution in the treatment of profound hearing loss and deafness in patients of all age groups. The aim of the study was to analyse the speech and linguistic disorders in adults with pre- and postlingual deafness rehabilitated with Cochlear implant (CI). MATERIAL AND METHODS: 21 adults with pre- (I group) and postlingual (II group) deafness deriving no benefit from hearing aids participated in this study. Phonatory organ was examined by endoscopy (VLS) and stroboscopy (VLSS). Test of Auditory Perception (TSS) was conducted directly after the activation of speech processor and after rehabilitation. The logopedic assessment before and after CI was based on the examination of motoric activity of articulatory organs, type of respiration, loudness of speech and the assessment of articulation. The linguistic assessment of grammatical and lexical aspect was carried out. The prosodic elements of speech were also analysed. RESULTS: Stroboscopic examination pointed to hypofunction of larynx in patients with prelingual deafness and hyperfunction--in postlingual deafness. The values of TSS were improved after CI, especially in patients with postlingual deafness. The most severe disorders of the efficiency of articulatory organs were observed in patients with prelingual deafness before CI. In the group of prelingually deaf patients disorders of the articulation of vowels and consonants as well as the lexical and grammatical aspect of speech were improved after CI. The correctness of syntax and inflection, development of active vocabulary, improvement of prosodic elements of speech were noticed in patients with postlingual deafness after CI. CONCLUSIONS. Patients with postlingual deafness achieved better results of rehabilitation in auditory perception than prelingually deaf patients. The linguistic improvement of grammatical and lexical aspect was noticed especially in prelingually deaf patients after implantation. This group of patients achieved also the significant progress in prosodic elements of speech in comparison with the results before implantation.

Mental health referral and services for maltreated children and child protection evaluations of children with special needs: a national survey of hospital- and community-based medically oriented teams.

OBJECTIVE: To survey the self-perceived capability of medically oriented child maltreatment teams in the US to provide mental health referrals and services when needed and to evaluate children with special health care needs (CSHCN). METHODS: Mailed questionnaire with 5 items related to mental health, 12 items on services for CSHCN, and 28 items on financial issues previously reported. RESULTS: Responses were received from 320 of 472 organizations (67.8%); 153 respondents had at least 1 physician or nurse practitioner and were included in the analysis; 91 were hospital-based teams (HBTs); and 62 were community-based teams (CBTs). CBTs were significantly more likely to offer mental health treatment (65.0% vs 35.6%). When mental health services were needed in another language, only half (50.7%) indicated that services were available in Spanish, less than a third (29.3%) could find services in sign language, and only 20.3% expected to find services for patients who used other languages. Of all children evaluated, 31.2% had special health care needs. CBTs reported seeing significantly more CSHCN than HBTs (38.3% vs 26.5%). Over two-thirds (67.7%) did not have a special program or specialized staff to serve CSHCN. Overall, teams had some training and experience with CSHCN. Children who were deaf were evaluated by 84.5% of teams, while only 50.5% reported using professionally trained sign language interpreters. Most teams (82.2%) indicated that more time was needed to evaluate CSHCN, and 69.1% found arranging for mental health treatment for CSHCN more difficult than children without special needs. CONCLUSIONS: Medically oriented child maltreatment teams are generally able to arrange for mental health services for the children served, and most feel capable of serving CSHCN. Significantly more mental health service providers are needed for children and families who communicate in languages other than English (e.g., Spanish, American Sign Language [ASL]). PRACTICE IMPLICATIONS: Our results suggest that medically oriented child maltreatment teams and mental health services for maltreated children would improve gaps in services by: (1) recruiting and training bilingual professionals, (2) ensuring that children or family members who are deaf receive professional ASL services, and (3) ensuring that training is provided related to the needs of CSHCN.

Deafness and autistic spectrum disorders.

An orientation to autistic spectrum disorders (ASD), also known as autism, is provided, and the specific syndrome of autism and deafness is addressed. The two conditions have in common a major problem: communication. Case histories are provided, the development of treatment for autism is discussed, and the separate disorders that make up ASD are defined. Important medical conditions often present in ASD are named, and their roles in treatment and diagnosis are described. Because autism is generally regarded as increasing in prevalence, some say to epidemic proportions, there is an increase in children who are both deaf and autistic. The resulting pressure on day and residential school programs for the Deaf to accept and educate these difficult, multiply disabled children is increasing. The parents of autistic children are a sophisticated, politically active group who are demanding services through legal and legislative means, among others.