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Cochlear Implants and Auditory Neuropathy (Without Confounding Factors)

Cochlear Implants and Auditory Neuropathy (Without Confounding Factors)

May 22, 2013 In the News

Budenz et al (2013) reported on 26 pediatric patients (less than 18 years of age) who received cochlear implants (CI). Seventeen of the 26 children had a diagnosis of auditory neuropathy spectrum disorder (ANSD) alone (without confounding cognitive or developmental disorders); the other 9 had sensorineural hearing loss (SNHL) without ANSD. The mean pre-op pure tone averages for the two groups were statistically different and were reported as 89 dB (+/- 21 dB for the ANSD group) and 104 dB (+/-13dB for the SNHL group).  However, age-appropriate speech perception tests (including the IT-MAIS, MAIS, ESP,HINT, PBK, GASP and more) were used to determine the "Preoperative Speech Category Score." With regard to speech category scores, the two groups were similar at their pre-op analysis and were similar at 6 months, 12 months, and 24 months post-op. The authors caution that children with ANSD—who have concomitant cognitive or developmental disabilities—are likely to perform significantly less well than children who have ANSD alone. Budenz et al conclude that "children with isolated ANSD are likely to perform at a level comparable to their…peers, whereas the coexistence of a cognitive or developmental disorder significantly adversely impacts CI outcomes within the ANSD population."  

For More Information, References and Recommendations

Breneman AI, Gifford RJ, DeJong MD. (2012) Cochlear Implantation in Children with Auditory Neuropathy Spectrum Disorder – Long Term Outcomes. Journal of the American Academy of Audiology (JAAA) 23(5):5–17.

Budenx CL, Telian SA, Arnedt C, Starr K, Arts HA, El-Kashlan HK, Zwolan TA. (2013) Outcomes of Cochlear Implantation in Children with Isolated Auditory Neuropathy Versus Cochlear Hearing Loss. Otology & Neurotology 34:477-483.

Roche JP, Huang BY, Castillo M, Bassim MK, Adunka OF, Buchman CA. (2010) Imaging Characteristics of Children with Auditory Neuropathy Spectrum Disorder. Otology & Neurotology 31(5):780–788.

Roush PA. (2011) Children with Auditory Neuropathy Spectrum Disorder. In the "Comprehensive Handbook of Pediatric Audiology."  Eds Richard Seewald and Anne Marie Tharpe. Plural Publishing, San Diego, CA,734–750.

Roush P, Frymark T, Venediktov R, Wang B. (2011) Audiologic Management of Auditory Neuropathy Spectrum Disorder in Children – A systematic review of the Literature.  American Journal of Audiology 20(December):159–170.

Spirakis SE. (2011) Auditory Neuropathy Spectrum Disorder and Hearing Aids: Re-Thinking Fitting Strategies. Hearing Review. October.

Teagle HFB, Roush PA, Woodard JS, Hatch DR, Zdanski CJ, Buss E, Buchman CA. (2010) Cochlear Implantation in Children with Auditory Neuropathy Spectrum Disorder. Ear & Hearing 31(3):325–335

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