Balance Impairment in Patients with Rheumatoid Arthritis and Ankylosing Spondylitis.

BACKGROUND: Balance weaknesses related to mobility and fall risk in patients with rheumatic diseases are well-known. Vestibular dysfunction could negatively contribute to the balance ability of this patient population. This study aims to investigate the effects of Rheumatoid Arthritis (RA) and Ankylosing Spondylitis (AS), among the most common rheumatic diseases, on postural balance related to vestibular function. METHODS: Seventy-eight participants were grouped as RA (n=34, 43%), AS (n=24, 30.7%), and the control group consisted of healthy individuals (n=20, 25.6%). Cervical Vestibular Evoked Myogenic Potentials (cVEMP) test, which assesses the vestibular function objectively, Dizziness Handicap Inventory (DHI), which evaluates vertigo subjectively, and Berg Balance Scale (BBS) were performed. RESULTS: Different degrees of VEMP latency prolongations were found in the AS and RA groups. Right, and left ear N1 latencies were significantly longer in the AS group than in RA and control. Right ear P1 latency prolongation was statistically significant in the RA group. Amplitude asymmetry ratio (AAR) was found to be considerably higher in the RA and AS groups than in the control group (p<0.05). The mean BBS score in the AS group was below the fall risk score of 45. A negative statistically significant effect was observed between latency prolongation and BBS in AS groups. CONCLUSION: The abnormal VEMP findings in individuals with RA and AS shows inner ear vestibular system dysfunction. This vestibular impairment strictly contributes to their postural imbalance and requires a focused vestibular rehabilitation program for balance treatment.

Regional differences of Turkey in risk factors of newborn hearing loss.

OBJECTIVE: The aim of this study was to discover Turkish regional differences in the risk factors of newborn hearing loss. METHOD: A multi-centered retrospective design was used. A total of 443 children, registered to the national newborn hearing screening programme, with bilateral hearing loss, from five different regions of Turkey, were evaluated in terms of the types of hearing loss, the degree of hearing loss, the types of risk factors, parental consanguinity, age at diagnosis and age of auditory intervention, respectively. RESULTS: There was no significant difference in the prevalence of hearing loss between regions (χ2 = 3.210, P = 0.523). Symmetric Sensorineural Hearing Loss (SSHL) was the most common type of HL in all regions (91.8%). Profound HL was the most common degree of HL in all regions (46.2%). There were statistically significant differences between regions in terms of types of HL (χ2 = 14.151, P = 0.000). As a total, 323 (72.9%) of subjects did not have any risk factors. There were statistically significant differences between regions in terms of the types of risk factors (pre, peri and post-natal) for SSNHL (χ2 = 16.095, P = 0.000). For all regions, the age of diagnosis was convenient with the JCIH criteria. However the age of hearing aid application was prolonged in some regions. There were statistically significant differences between regions in terms of the age of diagnosis (χ2 = 93.570, P = 0.000) and the age of auditory intervention (χ2 = 47.323, P = 0.000). The confounding effects of gender, age of diagnosis, age of hearing aids applications, HL in the family, types of risk factors for HL on SSNHL were detected. CONCLUSION: To reach the goal of a high quality newborn hearing screening, there is a need to develop an evidence-based standard for follow up guideline. In addition, risk factors should be re-evaluated according to regional differences and all regions should take their own precautions according to their evidence based data.

Genotypic-phenotypic features and enzyme replacement therapy outcome in patients with mucopolysaccharidosis VI from Turkey.

Mucopolysaccharidosis type VI (MPS VI) is a lysosomal storage disorder (LSD) characterized by a chronic, progressive course with multiorgan involvement. In our study, clinical, biochemical, molecular findings, and response to enzyme replacement therapy (ERT) for at least 6 months were evaluated in 20 patients with MPS VI. Treatment effects on clinical findings such as liver and spleen sizes, cardiac and respiratory parameters, visual and auditory changes, joints' range of motions, endurance tests and changes in urinary glycosaminoglycan excretions, before and after ERT were analyzed. ERT caused increased physical endurance and decreased urinary dermatan sulfate/chondroitin sulfate ratios. Changes in growth parameters, cardiac, respiratory, visual, auditory findings, and joint mobility were not significant. All patients and parents reported out an increased quality of life, which were not correlated with clinical results. The most prevalent mutation was p.L321P, accounting for 58.8% of the mutant alleles and two novel mutations (p.G79E and p.E390 K) were found. ERT was a safe but expensive treatment for MPS VI, with mild benefits in severely affected patients. Early treatment with ERT is mandatory before many organs and systems are involved.

Auditory evoked potentials in a newborn Wistar rat model of hyperbilirubinemia / Potenciais evocados auditivos em um modelo de rato Wistar neonato com hiperbilirrubinemia

ABSTRACT INTRODUCTION: Hyperbilirubinemia is a common health problem in newborns. Its effects can be different according to the level and duration of the hyperbilirubinemia. The toxic effect of bilirubin on the auditory system can be seen as a sensory neural hearing loss or auditory neuropathy spectrum disorder (ANSD). OBJECTIVE: The purpose of our study was to determine the effects of toxic bilirubin level on the auditory system by using Auditory Brainstem Response audiometry. METHODS: Rats are used as animal models due to their low cost and easy attainability. Auditory Brainstem Response was used for auditory assessment. In this study, three groups were established: experimental, control and placebo groups. RESULTS: In the experimental group, which consists of rats with hyperbilirubinemia, sensory neural hearing loss was found bilaterally in 4 rats (66.67%) and unilaterally in 2 rats (16.67%) and auditory neuropathy spectrum disorder was found unilaterally in 1 rat (8.33%). Auditory Brainstem Response thresholds were significantly elevated compared to control and placebo groups (p < 0.05). CONCLUSION: Hyperbilirubinemia of newborn rats may result both in sensory neural hearing loss and auditory neuropathy spectrum disorder.

RESUMO INTRODUÇÃO: A hiperbilirrubinemia é um problema de saúde comum em neonatos. Seus efeitos podem variar, dependendo do nível e da duração da hiperbilirrubinemia. O efeito tóxico da bilirrubina no sistema auditivo pode ser observado na forma de deficiência auditiva sensorioneural ou de distúrbio do espectro da neuropatia auditiva. OBJETIVO: A finalidade de nosso estudo foi determinar os efeitos de nível tóxico de bilirrubina no sistema auditivo, com o uso da audiometria da resposta auditiva evocada de tronco cerebral. MÉTODO: Os ratos são empregados como modelos animais graças a seu baixo custo e fácil obtenção. Utilizamos a resposta auditiva evocada de tronco cerebral para avaliação da audição. No estudo, foram estabelecidos três grupos: experimental, controle e placebo. RESULTADOS: No grupo experimental, constituído de ratos com hiperbilirrubinemia, disacusia auditiva neurosensorial foi diagnosticada bilateralmente em quatro ratos (66,67%), e unilateralmente em dois (16,67%); e distúrbio do espectro da neuropatia auditiva foi observado unilateralmente em um rato (8,33%). Os limiares da resposta evocada de tronco cerebral estavam significantemente elevados, em comparação com os grupos controle e placebo (p < 0,05). CONCLUSÃO: A hiperbilirrubinemia de ratos neonatos pode resultar tanto em disacusia auditiva neurosensorial como em distúrbio do espectro da neuropatia auditiva.

Auditory evoked potentials in a newborn Wistar rat model of hyperbilirubinemia.

INTRODUCTION: Hyperbilirubinemia is a common health problem in newborns. Its effects can be different according to the level and duration of the hyperbilirubinemia. The toxic effect of bilirubin on the auditory system can be seen as a sensory neural hearing loss or auditory neuropathy spectrum disorder (ANSD). OBJECTIVE: The purpose of our study was to determine the effects of toxic bilirubin level on the auditory system by using Auditory Brainstem Response audiometry. METHODS: Rats are used as animal models due to their low cost and easy attainability. Auditory Brainstem Response was used for auditory assessment. In this study, three groups were established: experimental, control and placebo groups. RESULTS: In the experimental group, which consists of rats with hyperbilirubinemia, sensory neural hearing loss was found bilaterally in 4 rats (66.67%) and unilaterally in 2 rats (16.67%) and auditory neuropathy spectrum disorder was found unilaterally in 1 rat (8.33%). Auditory Brainstem Response thresholds were significantly elevated compared to control and placebo groups (p<0.05). CONCLUSION: Hyperbilirubinemia of newborn rats may result both in sensory neural hearing loss and auditory neuropathy spectrum disorder.

Postnatal risk factors associated with hearing loss among high-risk preterm infants: tertiary center results from Turkey.

The aim of this study was to determine the postnatal risk factors associated with hearing loss as well as the prevalence of hearing loss among high-risk preterm infants in newborn hearing screening (NHS). We performed a retrospective study of high-risk preterm infants born with a gestational age ≤32 weeks and/or a birth weight ≤1,500 g. A NHS procedure was performed by automated auditory brainstem response (AABR) and automated evoked otoacoustic emission (TEOAE). Infants who failed TEOAE or AABR or both tests were referred to a tertiary audiology center for diagnosis confirmation and management. Postnatal risk factors associated with hearing loss were evaluated and compared for preterm infants with and without hearing loss. 1,360 high-risk preterm infants were assessed. Permanent hearing loss was found in 19 (1.4%) infants. Multivariate analysis revealed that proven sepsis (p = 0.019), mechanical ventilation ≥5 days (p = 0.024), loop diuretics (p = 0.001), patent ductus arteriosus ligation (p = 0.018) and operation for retinopathy of prematurity (ROP) (p = 0.034) were significant related factors for the hearing loss. This study showed a low prevalence of hearing loss and an association between operation for ROP and hearing loss in preterm infants, which has not been defined previously. Our results suggest that every neonatal intensive care unit should determine their own risk factors and take precautions to prevent hearing loss for these high-risk preterm infants.

Is it possible to predict diffuse obliterative otosclerosis preoperatively by audiologic examination.

Patients with diffuse obliterative otosclerosis have more extensive footplate pathology than annular cases. As a result of this more skill is required for diffuse otosclerosis cases, and postoperative hearing results are usually worse than annular cases. In this retrospective study we compared the preoperative audiological features of annular and diffuse otosclerosis patients. The subjects were 60 patients with conductive hearing loss who had undergone stapedectomy. Annular and diffuse groups were comprised of 30 patients each. Annular otosclerosis was defined as the footplate pathology involving the annular ligament only, where the footplate of the stapes is very thin and retains its bluish color. On the other hand diffuse, obliterative otosclerosis was defined as the pathology involving the whole footplate and also in some cases extending beyond the confines of the annular ligament. In each group preoperative air- and bone-conduction levels at 125-6000 Hz and 500-4000 Hz were noted respectively. Average air-bone gap for the obliterative otosclerosis group was 37.5 dB; the same value for the annular group was 23.8 dB (p<0.05). The gap characteristics of the audiogram were different for the two groups. The annular group had an air-bone gap which was nearly constant for all the frequencies. In the diffuse otosclerosis group, the air-bone gap was more prominent in the low frequencies and it decreased at higher frequencies. No difference was noted in bone-conduction thresholds, and Carhart notch between the two groups. This study demonstrated that a large air-bone gap in patients with conductive hearing loss may be a sign of diffuse obliterative otosclerosis. This may warn the surgeon that a more challenging surgery is possible, and the patient may have a less favorable hearing result. Therefore, in the presence of a large air-bone gap, it may be appropriate to inform the patient of the strong possibility of diffuse otosclerosis.