Sudhira Ratnayake
The Royal Wolverhampton NHS Trust, UK
Title: “Why does my child have a hearing loss?†– An overview of medical causes of permanent hearing disorders in children
Biography
Biography: Sudhira Ratnayake
Abstract
Deafness is the commonest congenital sensory impairment in humans. About 1-2 babies per 1000 births have severe to profound deafness and the prevalence rises with age. Causes of permanent deafness are varied. Approximately 50% is genetic in origin and the rest are environmental reasons. Of the genetic reasons, about 15% is syndromic and 85% is non-syndromic. Non-syndromic causes can be autosomal dominant, autosomal recessive, x-linked or mitochondrial mutations. Most of the syndromes can cause disorders in other body systems. e.g. vision and vestibular disorders in Usher Type 1, renal disease in Alport Syndrome. There are numerous environmental causes. Congenital cytomegalovirus (CMV) is the commonest congenital infection and can present with late-onset or progressive deafness. Symptomatic congenital CMV, if diagnosed early, can be treated with anti-viral medication to prevent the progression. Infections such as measles, mumps and rubella are becoming rare due to immunization. Meningitis needs urgent auditory and vestibular assessments to establish amplification and cochlear implantation. Ototoxic and vestibulotoxic medication can cause sudden or progressive auditory and balance difficulties. Head injuries can cause sudden deafness. There are several reasons why it is important to establish the cause of deafness. It can be the initial presentation of a much wider health problem. The aetiology can indicate the prognosis of deafness to help plan future care. Parents and young people may wish for genetic counseling. Cochlear implant outcomes may relate with the aetiology of deafness as some causes have associated learning and neurological disabilities. Aetiology is also important for epidemiological studies.