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Objective: To adapt the LittlEars questionnaire into Polish and to evaluate the psychometric properties of the Polish version of the questionnaire., Methods: A back-translation method was used to translate the LittlEars questionnaire into Polish. The translated version was first evaluated by means of an expert-appraisal method. After having improved the Polish version of LittlEars with the results obtained from that evaluation, various psychometric analyses were conducted. Study participants included 310 parents or other caregivers of children with normal hearing whose ages ranged between 0.5 and 24.0 months. Corrected item-total correlations were calculated to evaluate the extent to which the different questions distinguish levels of auditory development of the assessed children. Cronbach's alpha coefficient--to evaluate internal consistency across items--was also calculated. To obtain estimates of validity, correlations between item/total score and age were calculated. A non-linear regression model was derived to obtain normative data for expected and minimum values of total scores from the questionnaire according to age., Results: Corrected item-total correlations ranged from 0.14 to 0.84. The Cronbach's alpha coefficient was 0.95, indicating that the measurements were highly reliable. The linear correlation between total scores and age was 0.90 (p<.001). The regression analysis conducted to obtain normative data showed that 83% of the variance in the total scores can be explained by age., Conclusion: The results of psychometric analyses support the use of the Polish version of the LittlEars questionnaire as a sensitive and reliable tool to assess the development of auditory behavior in Polish children between 3 and 24 months of age.

The oculoauriculovertebral dysplasia is a rare congenital malformation that is characterized by a hemifacial microsomia with ocular abnormalities and coexisting disorders in the spinal column and other organs. Characteristic features of the disease were first described by Maurice Goldenhar in 1952. A broad spectrum of hearing impairment is a result of complex malformations of the external ear, the middle ear and in some cases-the inner ear. The degree of hearing loss can range from mild to moderate conductive type in cases of chronic otitis media with effusion and severe to profound sensorineural hearing impairment with malformations of the inner ear in various forms of cochlear hypoplasia. This study focuses on surgical methods of hearing improvement in patients with the oculoauriculovertebral dysplasia (OAVD). We present 11 patients diagnosed with OAVD who are under supervision of the Institute of Physiology and Pathology of Hearing in Warsaw. It is, to our knowledge, one of the largest groups of patients reported by a single ORL medical center. The degree of hearing impairment was thoroughly described in all patients and individual treatment was introduced. The aim of this report was to present algorithm for diagnostics and treatment of symptoms of Goldenhar syndrome (GS) based upon methodology used in modern otosurgery.