Cochlear Implantation in Patients with Keratitis-Ichthyosis-Deafness Syndrome: A Report of Two Cases

Background. Keratitis-ichthyosis-deafness (KID) syndrome is a syndrome which presents with hearing loss and visual and keratinization disorders. In such patients, hearing aids cannot be effectively used in the rehabilitation of hearing loss because of the frequent blockage of the external ear canal with epithelial debris and due to dry and tense skin of the external ear canal. Moreover, severe or profound hearing loss also limits the benefits gained from the conventional hearing aids. On the other hand, cochlear implantation is a method that has been used in limited cases in the literature. Case Report. This study presents the results of cochlear implantation applied in our clinic to two children who had been diagnosed with KID. Audiological assessments before and after the cochlear implant operation were performed using pure-tone audiometry, immittance audiometry, and auditory brainstem response (ABR), and the postoperative follow-up was conducted using pure-tone audiometry. Conclusion. Skin problems, visual disturbances, and other additional problems complicate the short-term and long-term rehabilitation after implantation in individuals with KID syndrome. Close monitoring should be exercised due to possible skin complications that may develop during the postoperative period. The families and rehabilitation teams should be warned about the possible visual disturbances and skin complications

Evaluation of hearing with pure-tone audiometry in alopecia areata patients

WOS: 000463183800004Background and Design: We aimed to evaluate whether alopecia areata (AA) patients had hearing loss with pure-tone audiometry. Materials and Methods: In the study, 48 patients with AA and 39 healthy volunteers were induded. All the patients were examined by the otorhinolaryngologist and audiometric examinations were performed using a pure-tone audiometer. Pure-tone thresholds for both ears were evaluated at frequencies of 250-8000 Hz for air conduction. Results: Sensorineural hypoacusis was found in 18.8% of AA patients and 10.3% of control subjects. There was no statistically significant difference between the two groups (p=0.422). The pure-tone average for the right ear and the left ear in AA patients was 10.89 +/- 5.45 and 10.50 +/- 5.98, respectively. In control subjects, it was 10.25 +/- 3.43 for the right ear and 10.23 +/- 5.46 for the left ear. Pure-tone averages for both ears in AA patients were higher than controls; but the differences were not statistically significant (p=0.829 and p=0.526, respectively). When we compared hearing thresholds for both ear for frequencies, there were not any significant differences between AA patients and controls (p>0.05). Conclusion: There was no difference in terms of hearing loss between AA patients and healthy controls