What is Hearing

Neurodevelopmental disorder is an umbrella term comprising many muscular, skeletal, metabolic, endocrinal, systemic, and immune-related diseases, which are caused due to the improper/inaccurate development of the central nervous system. Most of these disorders are highly prevalent, but some express rarely in human beings. Such disorders with least prevalence rates are known as rare neurodevelopmental disorders. The sensory system is affected in all individuals with these rare neurodevelopmental disorders, although to a varying extent. Sensory processing in terms of hearing loss is reported by many researchers in many rare neurodevelopmental disorders, but the pathophysiology of audiological findings are seldom investigated. In this chapter, the authors highlight the possible relationship between underlying cause and the resultant audiological symptoms in some of the rare neurodevelopmental disorders. Further, the research studies on the audiological profiling in such disorders are discussed.