A review of the type and degree of Hearing loss in patient with Alport syndrome.

A review of the type and degree of Hearing loss in patient with Alport syndrome.
Sahar Neemati,^1,*
1. Departmam of Rehabilitation,Student of Audiology,School of Allied Medical Science,Student Research Committee,Ahvaz Jundishapur University of Medical Sceince,Ahvaz,Iran.
Introduction: Alport syndrome (AS) includes a group of hereditary diseases caused by mutations in the COL4A3, COL4A4 or COL4A5 genes. These genes are responsible for the biosynthesis of α3, α4 and α5 collagen IV chains, which are located in the glomerular basement membrane of the kidney, the inner ear and the eye. Alport syndrome is caused by mutations (changes) to specific genes before a child is born. In this article we synthesise the main features of deafness associated with Alport syndrome.
Methods: This study is a review of the Hearing loss caused by Alport syndrome, by analyzing articles from dependable scientific databases,includingGoogleScholar, ScienceDirect,PubMed, and Scopus.
Results: Hearing loss is one of the first signs of AS. It becomes evident at the end of childhood or beginning of adolescence in boys with X-linked disease and the progression suggests poor prognosis of renal disease.Hearing loss caused by alport syndrom is sensori neural ,bilatral,symetrical and progressive. It was observed in 55% of men and45% of women.configuration of the Hearing loss in Alport syndrome in audiometric studies.
Conclusion: The concept of hearing loss severity must be redefined, as there is a clear need for more active hearing management in Alport's syndrome patients with severe and profound hearing loss. Sensorineural hearing loss (SNHL) caused by Alport's syndrome generally does not exceed 60-70 dB, because a cochlear lesion is responsible for this hearing loss. Careful management of renal function improves the prognosis and the longevity of Alport's syndrome patients; it is useful to reassess SNHL caused by Alport's syndrome. The level of hearing is positively correlated with disease duration. The otologist should follow up these patients. Ophthalmologic and genetic examinations should be ordered to provide counseling to the patients and the family members.
Keywords: alport syndrome,hearingloss,mutation,sensorineural hearing loss.