Keeping a hearing aid in place is often difficult. Hearing loss is due to inner ear abnormalities such as nerve deafness or middle ear problems such as bone malformations. The hearing loss can range from mild loss to profound deafness. The hearing loss can be difficult to evaluate due to frequent ear infections caused by unusually narrow ear canals, because eustachian tubes may not work well, because of the presence of other medical conditions, and general difficulty of testing. Many children have additional conductive losses due to frequent ear infections.
Many children also have difficulty with balance as a result of middle ear problems.
Because of the difficulty of diagnosing this condition, it is believed that this condition is probably very underdiagnosed. The frequency is the same in males and females. There is no evidence that it is caused by any illness or exposure to drugs, medication or any other substances before or during pregnancy. There are rare reports of several affected individuals in the same family. The risk of an affected individual having an affected child is much higher.