Joel Guss and Michael J Ruckenstein

Department of Otorhinolaryngology: Head and Neck Surgery, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, U.S.A.

Introduction 386

Definition 386

Pathogenesis 386

Clinical Manifestations 387

■ Genetic Diseases 387

■ Pendred Syndrome 387

■ Jervell and Lange-Nielsen Syndrome 387

■ Alport Syndrome 387

■ Branchio-Oto-Renal Syndrome 387

■ Neurofibromatosis Type 2 388

■ Mitochondrial Syndromes 388

■ Infectious Diseases 388

■ Cytomegalovirus 388

■ Rubella 389

■ Varicella-Zoster Virus 389

■ Mumps and Measles Viruses 389

■ Human Immunodeficiency Virus 389

■ Meningitis 389

■ Otitis Media 390

■ Syphilis 390

■ Lyme Disease 390

■ Autoimmune Diseases 391

■ Ototoxicity 391

Diagnosis 392

Treatment 392

■ Medications 392

■ Ototoxicity Prophylaxis 393

■ Prosthetics 393

Summary 394

References 394

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