Saba Aftab

Case Western Reserve University, Cleveland, Ohio, United States

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Publications (2)4.76 Total impact

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    ABSTRACT: To compare hearing outcomes in patients with autoimmune inner ear disease (AIED) undergoing cochlear implantation to a group of controls, postlingually deafened by non-immune-mediated causes. Hearing performance in AIED patients who receive unilateral or bilateral cochlear implants is comparable to similarly treated postlingually deafened adults without AIED. Retrospective chart review. Academic neurotologic tertiary referral center. Ten patients with AIED with 12 implanted ears who met the audiological criteria for cochlear implantation were compared with 12 randomly selected controls, postlingually deafened by non-immune-mediated causes. Cochlear implantation using commercially available devices. Preoperative and postoperative hearing thresholds, words, and sentence scores. A note was made regarding the presence or absence of ossification or fibrosis noted within the scala tympani at the time of implant. The mean age was 49.6 ± 14 years in the AIED group and 56.8 ± 17 years in the control group (p = 0.31). The mean duration of deafness was 14 ± 26 months in the AIED group and 6.5 ± 4 months in the control group (p = 0.34). In the AIED group, 42% were men and 58% were women. In the control group, 33% were men and 67% women. Five patients in the AIED group (6 implanted ears) were found to have cochlear fibrosis and variable degrees of ossification. Two patients in that group required drill-out procedures. All patients had full insertion. The mean preoperative pure-tone averages in the AIED and control groups were 102 ± 18 and 90 ± 13 dB, respectively (p = 0.13). In the AIED, the mean short-term (≤12 mo of follow-up) postoperative word and sentence scores were 74.8% ± 15% and 94% ± 6%, respectively. In the control group, the mean short-term postoperative words and sentence scores were 72% ± 12% and 96% ± 4%, respectively. No statistical difference was present in the short-term postimplantation words (p = 0.7) and sentence scores (p = 0.49) between both groups. The mean long-term (after 12 mo of follow-up) postoperative word and sentence scores in the AIED group were 87.2% ± 11% and 96.8% ± 4%, respectively. In the control group, the long-term words and sentence scores were 77.2% ± 14% and 77.2% ± 7%, respectively. No statistical significance was found in the long-term postimplantation words (p = 0.17) and sentence scores (p = 0.7) between both groups. Cochlear implantation is a safe and viable option for hearing rehabilitation in patients deafened by progressive AIED. Hearing outcomes in AIED patients are excellent and support transition to implantation when hearing is lost or long-term steroid therapy becomes undesirable. Cochlear fibrosis or ossification seems to affect nearly 50% of implanted ears (41.6% of patients) in this study and implies that the cochlea is at risk for ossification changes long term. In appropriate candidates, earlier implantation may be indicated before postinflammatory obliterative changes in the cochlea.
    No preview · Article · Oct 2010 · Otology & neurotology: official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
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    ABSTRACT: Endolymphatic hydrops (ELH), hearing loss and neuronal degeneration occur together in a variety of clinically significant disorders, including Meniere's disease (MD). However, the sequence of these pathological changes and their relationship to each other are not well understood. In this regard, an animal model that spontaneously develops these features postnatally would be useful for research purposes. A search for such a model led us to the Phex Hyp-Duk mouse, a mutant allele of the Phex gene causing X-linked hypophosphatemic rickets. The hemizygous male (Phex Hyp-Duk/Y) was previously reported to exhibit various abnormalities during adulthood, including thickening of bone, ELH and hearing loss. The reported inner-ear phenotype was suggestive of progressive pathology and spontaneous development of ELH postnatally, but not conclusive. The main focuses of this report are to further characterize the inner ear phenotype in Phex Hyp-Duk/Y mice and to test the hypotheses that (a) the Phex Hyp-Duk/Y mouse develops ELH and hearing loss postnatally, and (b) the development of ELH in the Phex Hyp-Duk/Y mouse is associated with obstruction of the endolymphatic duct (ED) due to thickening of the surrounding bone. Auditory brainstem response (ABR) recordings at various times points and histological analysis of representative temporal bones reveal that Phex Hyp-Duk/Y mice typically develop adult onset, asymmetric, progressive hearing loss closely followed by the onset of ELH. ABR and histological data show that functional degeneration precedes structural degeneration. The major degenerative correlate of hearing loss and ELH in the mutants is the primary loss of spiral ganglion cells. Further, Phex Hyp-Duk/Y mice develop ELH without evidence of ED obstruction, supporting the idea that ELH can be induced by a mechanism other than the blockade of longitudinal flow of endolymphatic fluid, and occlusion of ED is not a prerequisite for the development of ELH in patients.
    Full-text · Article · Apr 2008 · Hearing Research

Publication Stats

37 Citations
4.76 Total Impact Points


  • 2010
    • Case Western Reserve University
      Cleveland, Ohio, United States
  • 2008
    • Case Western Reserve University School of Medicine
      • Department of Otolaryngology-Head and Neck Surgery
      Cleveland, Ohio, United States