It has been revealed that the pure-tone audiometry demonstrates large air-bone gaps at low pitches due to the presence of inner ear fistulae. When a third mobile window resulting from an inner ear fistula is present, in addition to the 2 normally present windows consisting of the oval window and the round window, a portion of the air-conducted waves escape from the scala vestibuli through the inner ear fistula. On the other hand, bone-conducted waves traveling to the scala vestibuli are reduced by an inner ear fistula; however, bone-conducted waves traveling to the scala tympani are not affected by an inner ear fistula. This results in a larger gap than usual in compliance between both perilymphatic spaces and leads to a decrease in the bone conduction threshold. This phenomenon, so-called the third mobile window effects, sometimes may lead otology/neuro-otology surgeons to misunderstand the reason why large air-bone gaps still exist after ossicular reconstruction in tympanoplasty. This review article gives good examples regarding the third mobile window effects in otology/neuro-otology diseases and surgeries.