Background: Thyroidal hormones influence the inner ear development. Hearing is one of the most important senses in human. Normal hearing in early stage of life, especially in first six months, is essential for speech and education and communication. So diagnosis and treatment of hearing loss in young children is very critical and important.
Therefore, high risk finding and hearing loss screening in these groups is essential. Method and Material: This study was performed upon 50 patients who referred to Imam Reza Hospital with congenital hypothyroidism, The patient was studied according their sex, age at diagnosis, familial history, etiology, clinical presentation, and other ENT abnormalities. Hearing threshold was evaluated by ABR. Result: 38% of patients were male and 62% were female. Mean of age at initial management was 10.96 months. 32% had positive familial history. Etiology was dysgenesy in 64%, dyshormonogenes in 26%, unknown in 24% and transient in 4%. The most common presentation was prolonged jaundice 18% had purulent PND and Rhinorrhea. Prevalence of hearing loss was 22% and speech abnormality 16% There was no relationship between hearing loss and sex, age at the time of diagnosis, and thyroidal hormone level. Conclusion: prevalence of hearing loss was 22% in this study, and it was fairly high. Most of our patients were detected before 6 months age. when it was most important for diagnosis and rehabilitation of hearing loss. We believe that it is essential to screen all congenital hypothyroid patients for hearing loss and treat them as soon as diagnosed.