![]() Generally, the sooner a child obtains the right treatment, the better its outcome. ![]() These children with hearing loss and other ear issues are referred to our cochlear implant centre by audiovestibular physicians, ENT surgeons and audiology departments. ![]() A small number of children whose hearing is severely affected by ANSD will need cochlear implantation. I see children who have hearing loss for a variety of reasons. Now, in all referred babies including those from NICU, we request gene panels to see if there is an underlying genetic cause.Ĭould you describe your role as an otologist within the team treating children with ANSD? However, we have recently been able to build a much better understanding of the genetic causes of ANSD. In NICU babies with abnormal ABRs but present OAEs, ANSD was previously assumed to be acquired due to prematurity, hypoxia or jaundice. All children with ANSD will have an MRI scan of the inner ears and brain.īased on the current hearing screening pathway, I mainly see referrals from neonatal intensive care (NICU) who routinely have an ABR. ![]() Since ANSD is a complex diagnosis, I rely on clinical history and examination before requesting further investigations. My role is to investigate the causes of ANSD, manage the child holistically and monitor how ANSD impacts a child’s development. What is your role as an audiovestibular physician for patients with ANSD? Current UK NHSP pathway for well babies and neonatal ICU (NICU) babies. NHSP (newborn hearing screening programme) ĪOAE (automated otoacoustic emissions test) ĪABR (automated auditory brainstem response) įigure 1. However, they still may have ANSD, and this can only be picked up via an ABR test. The UK NHSP pathway is well established (Figure 1), but by using automated otoacoustic emissions (AOAE), well babies with clear responses will not proceed to have an auditory brainstem response (ABR) test. Their outcomes of treatment are also hard to predict based on audiological assessments.Ĭould you describe the current UK newborn hearing screening programme (NHSP) and how it may fail to detect ANSD? Their behavioural hearing assessments vary from mild to profound hearing loss, and their comprehension of speech is typically worse than predicted. The term ‘auditory neuropathy spectrum disorder’ (ANSD) was introduced for patients with hearing problems who have a combination of recordable otoacoustic emissions or cochlear microphonics and absent or atypical auditory brain stem responses. They have initiated a project to raise awareness and educate clinicians and families about the clinical characteristics of ANSD, the importance of genetic testing, and the opportunities that inner ear gene therapies may offer in the future.Ĭhrysa Spyridakou, Consultant Audiovestibular Physician We hear from a team of clinicians who care for families with children with ANSD. Some forms of ANSD have a genetic cause and, in this field, rapid progress is being made in developing gene therapies to correct gene mutations. It is difficult to detect ANSD via national hearing screening programmes, and outcomes of current treatments vary. It accounts for 10% of children with permanent hearing loss. Jaclyn Tan interviews members of the London-based multidisciplinary hearing team, about their insights to auditory neuropathy spectrum disorder, and their vision for the future management of this challenging range of conditions.Īuditory neuropathy spectrum disorder (ANSD) describes a wide range of conditions causing varying severities of hearing loss. Auditory neuropathy spectrum disorder - new treatments on the horizon
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