Background: The effect of unilateral sensor ineural hearing loss (UHL) on the quality of life is well documented. Studies found a direct link between UHL and educational and social delays. Radiological studies aimed to detect the associated anomalies of the earen countered a wide variation in the rates of incidence. Aim of the work: Our study aims to detect and give an estimate about the rate of incidence of different anomalies. Patients and methods: A review of the medical records of Audiology department in Eldemerdash hospital between 2014 to 2017 was done. Only cases with severe to profound USNHL diagnosed before age of 12 years were included. Clinical examination, full audiological assessment, MRI and CT temporal bone were done for all cases and results were reviewed for each patient. Results: 50 cases were included (8 cases with severe USNHL and 42 cases with profound USNHL). The mean age of diagnosis was 7.6 years.Only16 cases (32%) had risk factors known to contribute to SNHL. The most common abnormality found was cochlear nerve (CN)
deficiency in 22 cases (44%).Bilateral findings were present in 4 cases (8%). Conclusion: Radiological evaluation is not only for detection of the cause but also allows proper counselling and exploring the possible options of rehabilitation.