Problem statement: It is well established that numerous systemic dis¬eases have accompanying otologic manifestations. The diseases include syndromic genetic disorders such as Usher syndrome, branchio-oto-renal syn¬drome, Pendred syndrome, Jervell and Lange-Nielsen syndrome, Treacher Collins syndrome, and many others. In addition, other systemic diseases are asso¬ciated with hearing loss (e.g., osteogenesis imper¬fecta, Paget disease of bone, diabetes mellitus, renal disease, hypothyroidism, and

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... others). Over the past 10 years, however, otologic disease has been newly noted to be associated with several inherited and acquired disorders. This chapter briefly discusses the more important of these.