Establishing the reliability and the validity of the Arabic translated versions of the Effort Assessment Scale and the Fatigue Assessment Scale.

OBJECTIVE: The aim was to establish the reliability and the validity of Arabic translated versions of the Fatigue Assessment Scale (FAS) and the Effort Assessment Scale (EAS). DESIGN: The FAS and the EAS were translated from the original English following a recommended six-step approach for translating hearing-related questionnaires for different languages. The reliability of the scales was investigated using Cronbach's alpha, item-total correlation, and inter-item correlation. Construct validity was investigated using factor analysis and the hypothesis testing method. STUDY SAMPLE: The translated scales were completed by 146 participants from Jordan and Saudi Arabia (age range 19-86 years old, 39% male). Participants' hearing level ranged from normal to profound. RESULTS: Item 3 in the translated FAS was removed to improve the scale's construct validity. The translated version of the EAS was found to be as reliable and valid as the original EAS. CONCLUSIONS: The availability of standardised versions of the FAS and the EAS provides a quick and easy method for improving hearing rehabilitation in Arabic-speaking countries where audiology services can often be costly and not necessarily accessible to all individuals.

SLC26A4 Phenotypic Variability Influences Intra- and Inter-Familial Diagnosis and Management.

SLC26A4 is one of the most common genes causing autosomal recessive non-syndromic sensorineural hearing loss (SNHL). It has been reported to cause Pendred Syndrome (PDS) and DFNB4 which is deafness with enlarged vestibular aqueduct (EVA). However, mutated SLC26A4 is not conclusive for having either DFNB4 or PDS. Three unrelated Jordanian families consisting of eight affected individuals with congenital bilateral hearing loss (HL) participated in this study. Whole-exome and Sanger sequencing were performed to investigate the underlying molecular etiology of HL. Further clinical investigations, including laboratory blood workup for the thyroid gland, CT scan for the temporal bone, and thyroid ultrasound were performed. Three disease-causing variants were identified in SLC26A4 in the three families, two of which were novel. Two families had a novel pathogenic homozygous splice-site accepter variant (c.165-1G>C), while the third family had compound heterozygous pathogenic variants (c.1446G>A; p.Trp482* and c.304G>A; p.Gly102Arg). Our approach helped in redirecting the diagnosis of several affected members of three different families from non-syndromic HL to syndromic HL. Two of the affected individuals had typical PDS, one had DFNB4, while the rest had atypical PDS. Our work emphasized the intra- and inter-familial variability of SLC26A4-related phenotypes. In addition, we highlighted the variable phenotypic impact of SLC26A4 on tailoring a personalized healthcare management.

Development of the Jordanian Arabic early speech perception test.

INTRODUCTION: The Early Speech Perception (ESP) test has been previously used to assess speech perception skills in young hearing-impaired children. As the Arabic language is known to be diglossic, the purpose of this study was to develop a Colloquial Jordanian Arabic version (JAESP) of the ESP test and to check its usefulness. METHODS: The JAESP test encompasses four categories: detection of speech sounds, speech pattern perception, disyllabic word identification, and monosyllabic word identification. Picture boards representing the test materials were created and two equivalent lists for each test category were made. The word identification task was used to test a total of 43 children with reported normal-hearing abilities (15 children aged 3 years, 15 aged 4 years, and 13 aged 5 years). RESULTS: The scores for all age groups were 98-100% for all test materials. Statistical analysis showed that there was no significant difference between age groups and between lists (p ˃ 0.05). CONCLUSION: The findings of this study indicate that the JAESP test materials are appropriate for Jordanian children who are at least 3 years old. Additional studies are needed to verify the validity of the test materials for Jordanian hearing-impaired children.