We report a 60-year-old case diagnosed as the amyotrophic lateral sclerosis with patulous eustachian tube (ET). To our knowledge, this is the first case report of the amyotrophic lateral sclerosis complicating patulous ET in the literature, demonstrating the movement of the tympanic membrane, the pharyngeal orifice of the ET and abnormal movement of the uvula because of paralysis of the soft palate on video.