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Human Cochlear Implant Histopathology Xu XH1,2, MD, Cureoglu S2, MD, Maher S1, MD, Pollak N2,MD, Froymovich O2, MD, Paparella MM2, MD 1Department of Otolaryngology, H & N Surgery, Loma Linda University Medical Center, Loma Linda, CA 2Paparella Ear, Head and Neck Institute, Minneapolis, MN 2 International Hearing Foundation, Minneapolis, MN Abstract A C . Objectives: 1) to examine the histopathology of multi-channel cochlear implant temporal bones 2) to evaluate the relationship of residual spiral ganglion cell counts and clinical hearing performance Methods: Eight temporal bones from 4 cochlear implant patients were prospectively examined histologically. Paired comparisons were made between implanted and non-implanted temporal bones. Clinical performance data was obtained from patient charts. Results: There were varying amounts of inflammation (fibrosis and ossification) at the basal turn of the cochlea in all 4 implanted temporal bones. Compared to non-implanted sites, two implanted bones with less than a 10 year duration of implantation had no significant changes of spiral ganglion cell population. One case with prolonged implant duration (15 years) showed a 36% decrease of spiral ganglion cells at the implanted site. The case with the best speech recognition (89% with CID sentence) had the lowest residual spiral ganglion cells (30% of normal spiral ganglion cell population). Two cases with poor clinical performance (<10% with CID sentence) had residual spiral ganglion cells of 11% and 22%. The case with moderate clinical performance (30% with CID sentence) had 14% of normal spiral ganglion cell population. Surviving dendritic cells varied from 5% to 30% among 4 cases with no relationship to clinical performance. Conclusion: Our findings suggest prolonged implantation may affect spiral ganglion cell population. Although our data does show a trend toward a reverse relationship between residual spiral ganglion cells in implanted temporal bones and speech performance (as previously reported), we were unable to obtain statistical significance due to small sample size B Fig. 2. a. This section (case 2, left ear) shows an electrode tract (A) inside the scala tympani at the basal turn of cochlea at the level of round window . There is extensive fibrous reaction inside scala tympani (B). (Hematoxylineosin, magnification x 2). A coincidental finding of cochlear otosclerosis (C) Fig. 2. b. Higher magnification ( x 4 ) of the framed area shows significant inflammatory reaction around the electrode with inflammatory cell infiltration and neovasculization. Organ of Corti and Stria vascularis appear atrophic with a decrease of type II and type III fibrous cells in the spiral ligament. Case 3. This patient had a history of bilateral Meniere’s disease and several endolymphatic sac surgeries. He eventually lost hearing completely in both ears. He received his cochlear implant on the left ear 10 years after deafness at the age of 67. Following implantation, he continued to have difficulty with vertigo and had no improvement of hearing. A Introduction A A Patients with severe to profound sensorineural hearing loss will benefit from cochlear implantation for speech perception. However, this benefit varies significantly from patient to patient. Many studies have been conducted to try to identify possible predictors for improved clinical performance, but at present, no definitive factors have been found for this variation. Studying the histopathology of temporal bones from living donors who have received cochlear implants will help to elucidate this matter further. Methods and Materials A total of eight temporal bones from four patients with unilateral cochlear implants were studied. Clinical information was retrieved from chart review. For each implanted temporal bone, the following data was evaluated: trauma at the cochleostomy site; damages to basal membrane, Organ of Corti, spiral ligaments and stria vascularis; inflammatory reaction including new bone formation and fibrosis; as well as viable spiral ganglion cell counts and dendrites. The implanted bones were compared with the non-implanted bones in each case. Spiral ganglion cells and dendrite cell counts were measured. The relationship between SGC counts and auditory outcomes was also studied. Results TABLE 1 Clinical Information of Patients Case Side No. Gender of CI Etiology of Deafness Deaf Duration (yr) Age at Duration Age at CI of CI (yr) Death 1 M L Unknown 5 70 2 72 2 3 4 F M M L L R Ototoxicity Meniere’s Cochlear Otosclerosis 50 10 3 46 58 51 7 9 15 53 67 66 Cause of Death Unknown S/p bypass surgery Heart attack Stroke B A Fig. 3. (case 3, left ear) In this implanted temporal bone section at the level of the round window, the electrode path (A) is seen in scala tympani at the basal turn of the cochlea. Severe new bone formation (B) and a fibrous sheath around the electrode occupy the entire scala tympani. (Hemtoxylineosin, magnification x 2) Fig. 4. This section of a temporal bone (case 3, left side) shows evidence of endolymphatic hydrops with distortion of Reissner’s membrane (A). Case 4. This patient had a history of progressive hearing loss secondary to noise exposure according to clinic history. However the temporal bone study shows the true cause of deafness to be bilateral severe cochlear otosclerosis. He underwent an attempted right cochlear implant at age of 51, 3 years after bilateral deafness. At the time of surgery, entrance into the cochlea was hindered by bony obstruction at the level of the round window. Three months later, right cochlear implantation was completed with direct drilling into the promontory. The patient benefited from cochlear implantation with improvement of his CID sentences scores from 5% to 50% post-implantation . D E A B C Fig. 5. This is a section of an implanted temporal bone (case 4, right ear) at the level of the cochleostomy site (A) shows surgical trauma at the promontory following cochleostomy. Initial attempt at electrode insertion at the routine cochleostomy site failed. The scala tympani is completely occluded with bony structures (B), severe and extensive cochlear otosclerosis is shown in otic capsule (C). The electrode tract (D) appears within the scala vestibuli with significant new bone formation (E) around the electrode which extends onto the cochleostomy site. TABLE 2. Spiral Ganglion Cell (SGC) Counts of implanted bones and non-implanted bones CI = cochlear implant. Case 1. This is a patient who is status post right acoustic neuroma resection via a translabyrinthine approach at the age of 63. He had progressive hearing loss of his left ear within 5 years prior to cochlear implantation due to unknown etiology. His pure tone average (PTA) of the left ear was 100dB with 28% SDS prior to implantation. The patient received a Clarion AB 5000 cochlear implant at the age of 70. He was doing very well with his cochlear implant. CID Sentences scores improved from 18 to 68 after implantation. Basal turn Middle turn Apex case No. implant non-imp implant non-imp implant 1 2826 N/A 2556 N/A 1098 2 7614(1%) * 7722 4968 4221 288 3 5346(24%) * 7074 3726 4068 693 4 3537(36%)* 5553 1233 5022 0 Total non-imp implant non-imp N/A 6480 N/A 549 12492 891 9765 12033 360 4770 12870 * (%): SGC counts decrease in percentage compared with non-implant bone Case 2. This is a young woman who lost her hearing at 3 years of age secondary to drug induced ototoxicity. She received her cochlear implant at the age of 46. The patient showed significant benefit from the cochlear implant: Her post implant CID everyday sentence test after 12 months improved from 6% to 89%. Case #4 20 80 00 70 00 60 00 0 50 00 Fig. 1. b. Higher magnification (x10) of the area C shows an empty appearance of Rosenthal canal at ascending limb of basal turn of cochlear. This is the case with the lowest spiral ganglion cells in our series. Case #3 40 40 00 Fig 1a. Midmodiolar histologic section of temporal bone (case 1, left ear) showing distortion of basal membrane (A) and fracture of osseous spiral lamina (B) at basal turn of cochlear caused by electrode insertion. Spiral ligament, stria vascularis and organ of Corti appear atrophic. (Hematoxylineosin, magnification x 2). Case #2 30 00 C Case #1 60 20 00 B 80 10 00 A 100 0 Post-implant Best CID sentence score (%) Relationship Between Speech Perception Score and Basal Turn SGC Counts SGC counts at basil turn of cochlear Conclusions 1. Cochlear implants caused varied degrees of inflammation and trauma to cochlea 2. Prolonged implantation may affect spiral ganglion cell population 3. There is no reverse relationship between residual spiral ganglion cells in implanted temporal bones to clinical speech performance observed from our limited cases. Studies of a larger number of cases are needed.