This chapter provides detail description of superior semicircular canal dehiscence syndrome evaluation and management. Abstract of the chapter is given in the beginning. Chapter has 9 well illustrated diagrams and pictures. Introduction to the superior semicircircular canal dehiscence syndrome, slow phase nystagmus upon excitation and inhibition of semicircular canal, origin of bony dehiscence, transmastoid approach superior semicircular canal dehiscence plugging are described in detail. 2 tables are added to highlight sign, symptoms and investigations in superior semicircular canal dehiscence syndrome and transmastoid approach for superior semicircular canal plugging. 45 references are included at the end of the chapter to facilitate further reading.