Background: Fanconi anemia (FA) is an autosomal recessive genetic disorder wich characterized by progressive pancytopenia, multiple congenital anomalies,  increased susceptibility to acute myelogenous leukemia and epithelial cancers specially in head and neck and GUT. Characteristic feature of FA include short stature, café-au-laitspots, small eyes, mental retardation, skeletal and ear anomalies.
Case: A 23-year-old man who was a known case of FA since he was ten reffered to audiology clinic because of severe hearing loss. His initial diagnosis was pneumonia. Audilogic evaluation revealed  bilateral profound hearing loss.
Conclusion: One of the anomalies in FA are  ear anomalies. These included conductive haering loss, external auditory canal stenosis and auricular malformation and progressive sensoryneural hearing loss. In this report external auditory canal and tympanic membrane were normal. The result of tympanometry were type An and audiometry were bilateral profound hearing loss that is a rare finding  in FA patient.

Background and Aim: Turner syndrome is the most common sex chromosome abnormality in females, affecting an estimated 3% of all conceiving females. Otologic disease is a common problem in Turner syndrome patients that is due to a combination of small dysfunction Eustachian tube, palatal dysfunction and cochlear malformation.
Methods: This study assessed the otologic and audiologic characteristics of a group of Turner syndrome patients. We studied 40 Turner patients aged 10 to 20 years (mean age: 15.84 years, SD=2.67). Pure tone audiometry was carried out for all of them.
Results: Forty percent of the patients reported a history of middle ear disease. Analysis of audiometric data in 40 patients tested reveals normal hearing in 47.5%, pure sensorineural hearing loss in 32.5%, pure conductive hearing loss in 17.5% and mixed hearing loss in 2.5% of patients.
Conclusion: Careful follow up during early childhood of children with Turner syndrome is necessary to detect middle ear disease and prevent the probable sequel. However, long term periodic follow up is mandatory even after resolution of middle ear disease to detect sensorineural hearing loss.