Validation of the Czech Version of the Voice Handicap Index.

OBJECTIVE(S): The present study aims to evaluate the reliability and construct validity of the Czech version of the Voice Handicap Index (VHI-CZ) and determine the cut-off value to distinguish dysphonic patients from nondysphonic individuals. STUDY DESIGN: Prospective study, Parallel group design. METHODS: The study investigated 100 adult patients with dysphonia, divided into three groups based on the etiology of the voice problem (neurogenic, functional, and structural). Out of these, 25 patients were used for test-retest analysis, and 45 patients to determine the responsiveness to change. The control group consisted of 51 healthy subjects. All 151 individuals completed the VHI-CZ and were examined with the videolaryngostroboscopy. The internal consistency (Cronbach's alpha), the test-retest reliability (Intra-class Correlation Coefficient, ICC), and the construct validity were analyzed and the normative cut-off value was determined. RESULTS: The internal consistency of the VHI-CZ was excellent (Cronbach α = 0.984), and test-retest reliability was also excellent (ICC = 0.95, P < 0.001). The correlation between the self-assessed severity of the voice disorder and the VHI-CZ score was strong (Spearman's ρ = 0.877, P < 0.001). The VHI scores differences between dysphonic and nondysphonic patients were statistically significant (Mann-Whitney U test, P < 0.001). The differences among the three etiological subgroups (neurogenic, functional, and structural) were also statistically significant (Kruskal-Wallis test, P < 0.001). Moreover, the differences in the VHI-CZ total scores between pretreatment and posttreatment were statistically significant (Wilcoxon test, P < 0.001). The cut-off score of 13 points was found, by the analysis of the Receiver Operating Characteristic (ROC, Youden Index), to be most suitable for preselecting dysphonic individuals. CONCLUSION: The existing VHI-CZ showed excellent reliability and construct validity. The Czech VHI is a useful and valid monitoring tool for clinicians.

Comparison of Short and Long-Term Results after Injection Laryngoplasty with Radiesse® Voice and Thyroplasty Type I in Unilateral Vocal Fold Palsy.

OBJECTIVES: Unilateral vocal fold palsy independently of etiology results in glottic insufficiency leading to unfavorable short or long-term impact on voice quality. Our aim was to evaluate the effect of injection laryngoplasty using Radiesse® Voice and thyroplasty type I on glottic closure, voice quality and aerodynamics by comparing preoperative, short- and long-term results. MATERIALS AND METHODS: Data of 32 consent patients were reviewed between 2012 and 2023. All patients underwent either injection laryngoplasty (14 patients) or thyroplasty type I (18 patients) under local anesthesia. Maximum phonation time, glottic closure based on videolaryngostroboscopy, VHI-30 values and GRBAS scale were recorded prior, short-term (3 month) and long-term (12 months) after procedures for statistical comparison. Friedman test, Mann-Whitney test and Wilcoxon signed rank tests were used for statistical analysis. RESULTS: In injection laryngoplasty group, we found significant improvement in maximum phonation time (p = 0.002), grade of hoarseness (p = 0.002) and breathiness (p = 0.000) when comparing results before and short-term after procedure. In thyroplasty type I group we saw significant improvement of maximum phonation time (p = 0.000), glottic insufficiency (p = 0.000), all three VHI-30 components (p = 0.000), as well as grade of hoarseness, breathiness (both p = 0.000) and roughness (p = 0.011) of GRBAS scale when comparing voice outcome before and short-term after procedure. There was no significant difference in voice outcome results neither between short and long-term results nor between the two groups in any parameter. CONCLUSION: These results demonstrate both short and long-term efficiency of injection laryngoplasty and thyroplasty type I in the improvement of voice quality and glottic closure.

Effectiveness of Neonatal Hearing Screening System: A 12-Year Single Centre Study in the Czech Republic.

PURPOSE: The study aims to evaluate the number of examined newborns and the results of screening for twelve years (2008-2019) and to assess the effectiveness of the established system of neonatal hearing screening. DESIGN AND METHODS: The study was designed as a retrospective longitudinal data analysis. The data included all the children (19,043) born in the hospital and also children (74) transferred from other healthcare facilities. A total of 19,117 children were included in the research group. RESULTS: In the first three years, a higher number of children did not pass the hearing screening, which was followed by a declining trend in the following years. After the first year of screening (2008), there was an improvement in diagnosis linked with a decrease in false-positive screening results (from 9.4% to 6.4%; p = 0.002). From 2008 to 2015, the ratio of children with positive screening to those with negative screening had a steady or declining trend. CONCLUSIONS: The results showed a reduction in false-positive results after the first year of the screening program, probably due to improved care management and a gradual increase in the skills of the nurses performing the screening. PRACTICE IMPLICATIONS: The cornerstones of neonatal hearing screening are a sufficient number of trained neonatology nurses, their mutual substitutability and the availability of a hearing screening device in the newborn ward every day. The results imply the importance of periodic evaluation of the obtained data, enabling early detection of possible deficiencies in the hearing screening system.

Novel variants in EDNRB gene in Waardenburg syndrome type II and SOX10 gene in PCWH syndrome.

Waardenburg syndrome (WS) is a clinically and genetically heterogeneous group of inherited disorders manifesting with sensorineural hearing loss and pigmentary anomalies. Here we present two Caucasian families with novel variants in EDNRB and SOX10 representing both sides of phenotype spectrum in WS. The c.521G>A variant in EDNRB identified in Family 1 leads to disruption of the cysteine disulfide bridge between extracellular segments of endothelin receptor type B and causes relatively mild phenotype of WS type II with low penetrance. The novel nonsense variant c.900C>A in SOX10 detected in Family 2 leads to PCWH syndrome and was found to be lethal.

Hearing screenings for preschool children: A comparison between whispered voice and pure tone audiogram tests.

INTRODUCTION: This prospective study compares the efficiency of two hearing screening tests performed on preschool children. These tests are known as whispered voice test and pure tone audiometry. METHODS: Standard hearing screenings were performed on five-year old children using a whispered voice test followed by ENT examination with pure tone audiometry. RESULTS: A total of 827 children were included in the study. Hearing loss (>25 dB) was observed in 5.8% of the evaluated children (n = 48), being bilateral in only 1.6% (n = 13) of these cases. Slight hearing impairment (hearing loss of 16-25 dB) was observed in 25.4% (n = 210) of the children, with 14.5% bilateral cases (n = 120). Interestingly, 62 children (7.5%) were under suspicion of hearing loss by their parents; however, an audiogram revealed the poor consistency of this diagnosis (sensitivity 20.8%, Cohen's kappa coefficient of 0.048). The whispered voice test (6 m distance) throwed a hearing impairment diagnosis in 807 (48.8%) of examined ears; however, its sensitivity was of only 56.5%, with a specificity of 51.6% and Cohen's kappa coefficient of 0.0254 (poor). CONCLUSION: The hearing loss incidence in preschool children coupled with the low efficacy of whispered voice tests and the parents' unreliability during the hearing impairment survey advocate for a more efficient audiometric hearing screening before beginning school attendance.

Updating the nationwide methodology for hearing screening of newborns in the Czech Republic.

Screening programs examining neonatal hearing serve to detect hearing defects, as a prerequisite for hearing rehabilitation, communication skills, and the enhancement of speech development. There are two methods through which neonatal hearing screening is carried out - the transiently evoked otoacoustic emissions (TEOAE) or the automatic BERA (AABR, automated auditory brainstem response). Positive screening means the discovery of a hearing defect (permanent hearing loss), and negative screening (normal TEOAE or the AABR results) means the absence of a hearing defect. The procedural aim is to update and adjust the neonatal hearing screening, which is determined by the Bulletin of the Ministry of Health of the Czech Republic No. 7/2012. Neonatal screening is performed at three levels: at neonatological site, at the ENT (phoniatric) rescreening site and at the ENT regional centre. The activities at each level are accurately and concretely identified including the issue of billing the performance to health insurance companies and informed consent to personal data protection (GDPR). The correct functioning of screening for hearing loss is based on the simple organization of the screening, patient examination comfort, medical recovery from it, and its economic viability. The schedule for neonatal hearing screening and rehabilitation recommends the following steps: 1. screening of a newborns hearing on the second or third day after delivery by a neonatological nurse using otoacoustic emissions, alternatively AABR for newborns at risk; 2. hearing rescreening in the third to sixth week of child`s age at the ENT rescreening site; 3. completion of hearing impairment diagnostics within three to sixth months of age at the ENT regional centre. The failure to follow the procedure above is a threat to the hearing and speech development of the child with severe permanent hearing impairment. The collaboration of ENT doctors with neonatologists and paediatricians allows for creating conditions under which the functional nationwide hearing screening of newborns can be established throughout the Czech Republic.

[Newborn hearing screening - importance, current state in the Czech Republic]. / Novorozenecký screening sluchu - význam, soucasný stav v CR.

The importance of early detection of hearing impairment in newborns and children and the early rehabilitation of hearing disorder with hearing aid or cochlear implant was demonstrated in a number of papers. As a result, newborn hearing screening was introduced in many countries around the world. The incidence of congenital hearing impairment has been underestimated for a long time, empirically determined incidence was 1:1000 neonates. Thanks to newborn hearing screening was revealed that incidence of congenital hearing impairment is 3 times higher. One out of 300 newborns has profound hearing loss (deafness) and 1 in 300 newborns has mild to moderate hearing loss. Moreover, in 1 out of 300 children hearing impairment would develop before the age of 18. In the Czech Republic, the screening of all newborns is still systematical and multilevel conducted in only three regions (Moravskoslezský, Královéhradecký, Pardubický). In these regions, statistics as well as assessments of individual stages of screening are carried out. In other regions, records of the number of screened children, number of rescreened children and detailed statistics of hearing impairment is missing. The authors summarize the basic information about the importance of screening, the history of screening and its organization. Provided information is based on experience with the gradual introduction of screening over recent years in the regions in which they work. Newborn hearing screening should be organized in several stages (1 - screening at maternity hospitals, 2 - rescreening on collaborating otolaryngology/phoniatric workplaces and 3 - detailed hearing examination in centers in children whose screening was negative). The authors focus on problems related to the organization of screening and offer practical advice (e.g. implementation of screening coordinators). They consider it is essential to have statistical evidence of examination at all levels. Introducing of newborn hearing screening is not a short-term task but a long-term (many years) challenge.

Rheopheresis in treatment of idiopathic sensorineural sudden hearing loss.

BACKROUND: Only few therapeutic options exist for patients with refractory sudden idiopathic sensorineural hearing loss (SISHL). Little is known about the efficacy of second-line therapies. Rheopheresis seems to be an effective therapeutic possibility. METHODS: Between 2012 and 2015, 106 patients with SISHL were enrolled in the study, of whom 52 were refractory to initial treatment. As salvage therapy, these patients were offered either 3 sessions of rheopheresis (33 pts) or intratympanic steroid treatment through MicroWick application (19 pts). Pure tone audiometry was performed at diagnosis, at the 1st month and the 1st year during the follow-up. RESULTS: Patients in the rheopheretic arm had higher hearing loss than in the MicroWick arm (81% vs. 52%, p = 0.04). In spite of this, there was a significant improvement for patients in the rheopheretic arm (27% of hearing loss reduction, p < 0.001) after the 1st month and this remained unchanged during the 1st year, while no improvement was seen in the MicroWick arm (0% of hearing loss reduction, p = 0.424). We found no predictive factor for steroid-failure in first-line therapy. Older age (p = 0.003), presence of vertigo (p = 0.006) and more profound initial hearing loss (p < 0.001) were identified as negative prognostic markers. CONCLUSION: Rheopheresis can be used as a potentially effective and safe salvage therapy for patients with cortico-refractory SISHL.

The importance of rheological parameters in the therapy of the dry form of age-related macular degeneration with rheohaemapheresis.

To date, rheological treatment is the only chance to control the advanced dry form of age-related macular degeneration and arrest its progression to legal blindness. Rheohaemapheresis can change the main rheological parameters, blood and plasma viscosity, as well as change erythrocyte aggregability, improve erythrocyte flexibility and lead to substantial improvement when other methods of therapy fail. In this study, we describe changes in the levels of rheological efficacy indicators after rheohaemapheresis and their clinical significance in the dry form of age-related macular degeneration (AMD). Seventy-two patients with AMD were randomised; 34 controls, and 38 patients were treated with rheohaemapheresis (separator Cobe Spectra + Evaflux filter). After the procedures, α2-macroglobulin levels decreased by approximately 58%, fibrinogen by approximately 65%, IgM by approximately 67%, LDL cholesterol by approximately 71%, apolipoprotein B by approximately 65%, and lipoprotein (a) by approximately 42%. These decreases correspond with a decrease in blood and plasma viscosity (14/12%), clinical improvement (arrest of disease progression, even visual improvement in some cases), and heretofore-unreported improvement (even reattachment) of drusen retinal pigment epithelium detachment. Our modification of rheohaemapheresis is safe (5.4% of patients experienced clinically insignificant side effects).

Neurofibromatosis 2: two case reports.

Our study presents two cases of neurofibromatosis 2 (NF2) that have been diagnosed at the Ear, Nose, and Throat Department of Hradec Králové (Czech Republic). The first case involved a young man with a history of sudden hearing loss accompanied by tinnitus on the left side. The diagnosis of NF2 was made, and an operation for left acoustic neuroma was performed. Looking toward the future, the acoustic neuroma on the right side should be resolved as well. The second case concerned a woman (the mother of our patient 1) examined at the same Ear, Nose, and Throat Department in 1980, after 4 years of gait instability and progressive loss of hearing and tinnitus on the right side. Computed tomography scan detected a bilateral expansion in the pontocerebellar angles, and a large tumor on the right side was removed. The patient is deaf and has facial palsy without progression of symptomatology during long-term follow-up. These two cases document the rare but serious hereditary disease of NF2. Its most frequent first presentation is acoustic neuroma; further, benign tumors of the nervous system and juvenile cortical cataract also are often detected. The variability of number, location, and biological behavior of tumors associated with NF2 require an individual patient treatment approach, long-term follow-up, and insertion of appropriate hearing aids. Important also is a genetic examination to exclude pathological NF2 genes in the first-degree relatives of the affected individuals.