Letter to the Editor

Abstract

To the Editor We compliment Drs. Kang, Shim, and Lee on their report about gratifying results of cochlear implantation for 3 of 4 patients with X-linked deafness (1). Prior authors had found “limited auditory perception and language acquisition” in patients with this disorder (2). Our long-term (38 months postoperative) experience with 2 patients with genetically confirmed X-linked mixed deafness echoes the experience of Dr. Kang and colleagues. Our patients, each age 28 months at implantation, progressed from awareness of environmental sounds (categories of auditory performance [CAP] Level 1) to understanding common phrases without lip-reading (CAP Level 5) at 36 months after surgery. For each child, both family and educators are requesting implantation into the contralateral ear in hopes of improving auditory perception and language development. Our thinking, however, is that the prior uncomplicated courses may not be replicated, and that the risks may outweigh the potential benefits for second-ear implantation for these patients. Emboldened by our meager experience, we would like to do the following: 1) offer video imaging of an implantation (Supplemental Digital Content, Video 1 https://links.lww.com/MAO/A173). The wax-suture plug technique, which we used for transient control of gushing [third segment of the video], is after Marks (3); 2) endorse the usefulness of the cork-plug of the electrode array in stopping gushing [fourth segment of video] (4); 3) present a “sucking claw” instrument to facilitate insertion of the electrode into the stream of gushing (Fig. 1); and 4) call attention to the abnormal anatomy of the promontory in each of our patients, which can be seen in the video. The promontory did not have a smooth contour or obvious round window niche but rather a trabecular pattern reminiscent of a forme fruste of air cells. Clinical CT images of our 2 patients do not exhibit such roughness of the promontory. To our knowledge, mention of such trabecular pattern has not been made in cases of X-linked hearing loss.FIG. 1: “Sucking claw” instrument (claw swaged onto a 3 Fr suction) enables 1-instrument suctioning of liquid plus insertion of electrode array.Kara K. Prickett M.D. Emory University Department of Otolaryngology–Head and Neck Surgery Atlanta, Georgia, U.S.A. N. Wendell Todd M.D., M.P.H. Department of Otolaryngology Emory Children’s Center Atlanta, Georgia, U.S.A. [email protected]