Systematic Literature Review and Early Benefit of Cochlear Implantation in Two Pediatric Auditory Neuropathy Cases

Abstract

Approximately 1 in 10 children with hearing loss is affected by auditory neuropathy spectrum disorder (ANSD). People who have ANSD usually have great difficulty understanding speech or communicating. However, it is possible for these patients to have audiograms that may indicate profound hearing loss up to normal hearing. This disorder is prognosed with positive, intact or present otoacoustic emissions (OAE) and/or cochlear microphonics (CM) as well as abnormal or absent auditory brainstem responses (ABR). Treatment methods include conventional hearing aids as well as cochlear implants. Cochlear implants (CI) usually promise better speech understanding for ANSD patients. We performed a systematic literature review aiming to show what improvements can effectively be achieved with cochlear implants in children with ANSD and compare this with our experience with two cases of ANSD implanted at our clinic. The retrospective review of two young CI patients diagnosed with ANSD during infancy demonstrated improvements over time in speech development communicated by their parents.

Keywords: auditory neuropathy spectrum disorder; daily wearing time; speech development; speech understanding.

Figure 2

Results for all included publications. AZBio = AZBio sentence test; BKB = Bamford–Kowal–Bench sentence test; CAEP = cortical auditory evoked potential, CAP = Categories of Auditory Performance; CID = Central Institute of Deafness test; CNC = Consonant–Nucleus–Consonant words; CVC = Consonant–Vowel–Consonant words; ESP = Early Speech perception test; HA = Hearing aid; HSM = Hochmair–Schulz–Moser sentence test; IT-MAIS = Infant–Toddler Meaningful Auditory integration scale; LNT = Lexical Neighbourhood Test; MAPTB = Mandarin Auditory Perception Test Battery; MLNT = Multisyllabic Lexical Neighbourhood Test; MSP = Melbourne Speech Perception; MUSS = Meaningful Use of Speech Scale; MWT = Monosyllabic Word Test; NU-CHIPS = Northwestern University—Children’s Perception of Speech; PBK = Phonetically Balanced Kindergarten word list; SADL = Satisfaction with Amplification in Daily Life; SIR = Speech Intelligibility Rating, SRT = Speech Recognition Threshold [4,5,6,9,12,15,16,17,18,19,20,22,23,24,25,29,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72].

Figure 2

Results for all included publications. AZBio = AZBio sentence test; BKB = Bamford–Kowal–Bench sentence test; CAEP = cortical auditory evoked potential, CAP = Categories of Auditory Performance; CID = Central Institute of Deafness test; CNC = Consonant–Nucleus–Consonant words; CVC = Consonant–Vowel–Consonant words; ESP = Early Speech perception test; HA = Hearing aid; HSM = Hochmair–Schulz–Moser sentence test; IT-MAIS = Infant–Toddler Meaningful Auditory integration scale; LNT = Lexical Neighbourhood Test; MAPTB = Mandarin Auditory Perception Test Battery; MLNT = Multisyllabic Lexical Neighbourhood Test; MSP = Melbourne Speech Perception; MUSS = Meaningful Use of Speech Scale; MWT = Monosyllabic Word Test; NU-CHIPS = Northwestern University—Children’s Perception of Speech; PBK = Phonetically Balanced Kindergarten word list; SADL = Satisfaction with Amplification in Daily Life; SIR = Speech Intelligibility Rating, SRT = Speech Recognition Threshold [4,5,6,9,12,15,16,17,18,19,20,22,23,24,25,29,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72].

Figure 5

Daily wearing time (x-axis) combined with the parents’ and speech pathologist’s subjective impression of the child’s post-operative development. y-axis (centre) denotes the follow-up in months. (right) Case one; (left) Case two.

Figure 1

Flow diagram of study selection according to the PRISMA guidelines [43].

Figure 3

Report conflicts of interest, financial support and Oxford level of confidence of included publications.

Figure 4

ASSR and pure tone results. Case 1: the follow-up time (months, mo) is denoted on the left. Post-op left/right hearing levels were identical.

Figure 6

ASSR and pure tone results. Case 2; the respective follow-up time (months, mo) is denoted in the middle of the graph for the left and right side.

Figure 7

At home play training as part of the rehabilitation process with the mother.