Speech and Language outcomes in Auditory Neuropathy Spectrum Disorder (ANSD) children managed with amplification

Ramanathan, Diya; Mahomva, Chengetai; Goldberg, Donald M.; Liu, Yi‐Chun Carol; Anne, Samantha; Lyle, W. Andrew

doi:10.1016/j.amjoto.2022.103753

Cited by 3 publications

(2 citation statements)

References 13 publications

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“…Similar to previous studies, the worse scores were related to hearing in noise. 22,23 There are some inherent limitations to this study that should be acknowledged. Firstly, this is a retrospective study and carries the characteristic limitations of such a study design.…”

Section: Discussionmentioning

confidence: 98%

“…21 Despite this thought process, over the last decade, successful use of CI in retrocochlear disease processes was reported (such as in auditory nerve hypoplasia, ANSD, and acoustic neuromas with intact cochlear nerve). 3,22,23 In 2015, Modest et al 12 reported the successful results of CI in 7 patients with superficial siderosis-associated SNHL, a disease process with similar retrocochlear pathology as CMT, with a median post-implantation CNC word score of 51% (range 46-64%). Similarly, in 2017, Attias et al 24 demonstrated comparable post-implantation speech performance in 16 pre-lingual children with isolated auditory neuropathy compared to children with SNHL.…”

Section: Discussionmentioning

confidence: 99%

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Cochlear Implantation in Charcot-Marie-Tooth Patients: Speech Perception and Quality of Life

Farber,

Chin,

Mills

et al. 2024

Ann Otol Rhinol Laryngol

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Objectives: There is a limited understanding of the impact of cochlear implantation (CI) in patients with Charcot-Marie-Tooth disease (CMT), given the scarcity of reported cases. We aim to evaluate the audiological outcomes and quality of life (QoL) after CI in CMT. Methods: Multi-institutional, university-affiliated, tertiary-referral centers, retrospective chart review. Our cohort includes 5 patients with CMT. Patients’ charts were reviewed for demographic characteristics, operation notes, and pre- and post-implantation audiology evaluation. Patients completed the Cochlear Implant Quality of Life-10 (CIQOL-10) Global questionnaire. Results: Pre-implantation, the mean pure tone average was 84.1 ± 7.2 dB, and the mean word recognition score was 2.4% in the implanted ear. AzBio sentence test was performed in quiet, revealing a mean of 4 ± 1.4% in the implanted ear. Post-implantation, PTA results were all within the mild hearing loss range (mean 33.0 ± 5.9 dB). Post-CI, AZ-Bio test results were 5%, 65%, and 74% (for 3 patients), and HINT scores were 55% and 58% (for 2 patients). The mean score of the CIQOL-10 questionnaire was 42.7 ± 10.47 (range 1-100). Patients were most satisfied with their ability to listen to the television or radio, have conversations in a quiet environment, and feel comfortable being themselves. Conclusion: To the best of our knowledge, this is the most extensive series of CI in CMT-associated sensorineural hearing loss and auditory neuropathy. Our cohort suggests that CI is a safe and reliable method for hearing rehabilitation that can achieve good speech performance and improve QoL in CMT patients.

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Section: Discussionmentioning

confidence: 98%