Endolymphatic sac surgery in congenital malformations of the inner ear.

A retrospective analysis of 40 patients (49 ears) with congenital progressive sensorineural hearing loss who underwent endolymphatic sac surgery was performed. The inner ears were radiographically abnormal in 57% of operated ears. In the remaining cases, subtle malformations beyond the resolving power of radiographic studies were suspected. Early postsurgical hearing loss (defined as a loss greater than 10 dB in three-tone average or greater than 15% in speech discrimination score) was found in 29% of operated ears (14/49). However, only two of these patients lost all of their residual hearing postoperatively (2/49 or 4%). An enlarged endolymphatic sac was noted at surgery in 50% of those with significant postoperative hearing losses. Longer-term stability of hearing was assessed in 22 patients with bilateral inner-ear pathology who underwent surgery on one side only. A comparison of the hearing fate of the operated and nonoperated ears suggested no benefit from the surgical intervention when compared to the natural history of the disease. Based upon this experience, endolymphatic sac surgery for the purpose of hearing stabilization in patients with congenital malformations of the inner ear is no longer recommended.