scholarly journals Brain Stem and Inner Ear Abnormalities in Children with Auditory Neuropathy Spectrum Disorder and Cochlear Nerve Deficiency

2010 ◽  
Vol 31 (10) ◽  
pp. 1972-1979 ◽  
Author(s):  
B.Y. Huang ◽  
J.P. Roche ◽  
C.A. Buchman ◽  
M. Castillo
Keyword(s):  
Brain Stem ◽  
Inner Ear ◽  
Auditory Neuropathy ◽  
Cochlear Nerve ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder ◽  
Cochlear Nerve Deficiency

Unilateral auditory neuropathy spectrum disorder: retrocochlear lesion in disguise?

2014 ◽  
Vol 129 (S1) ◽  
pp. S38-S44 ◽  
Author(s):  
A Mohammadi ◽  
P Walker ◽  
K Gardner-Berry
Keyword(s):  
Magnetic Resonance Imaging ◽  
Computed Tomography ◽  
Magnetic Resonance ◽  
Auditory Neuropathy ◽  
Cochlear Nerve ◽  
Spectrum Disorder ◽  
Resonance Imaging ◽  
Auditory Neuropathy Spectrum Disorder ◽  
Cochlear Nerve Aplasia ◽  
Brainstem Response

AbstractObjective:To investigate whether the aetiology for hearing impairment in neonates with unilateral auditory neuropathy spectrum disorder could be explained by structural abnormalities such as cochlear nerve aplasia, a cerebellopontine angle tumour or another identifiable lesion.Methods:In this prospective case series, 17 neonates were diagnosed with unilateral auditory neuropathy spectrum disorder on electrophysiological testing. Diagnostic audiology testing, including auditory brainstem response testing, was supplemented with computed tomography and/or magnetic resonance imaging.Results:Ten of the neonates (59 per cent) showed evidence for cochlear nerve aplasia. Of the remaining seven, four were shown to have another abnormality of the temporal bone on imaging. Only three neonates (18 per cent) were not diagnosed with cochlear nerve aplasia or another lesion. Three computed tomography scans were reported as normal, but subsequent magnetic resonance imaging revealed cochlear nerve aplasia.Conclusion:Auditory neuropathy spectrum disorder as a unilateral condition mandates further investigation for a definitive diagnosis. This series demonstrates that most neonates with unilateral auditory neuropathy spectrum disorder had pathology as visualised on computed tomography and/or magnetic resonance imaging scans. Magnetic resonance imaging is an appropriate first-line imaging modality.

Cochlear Implant Outcomes for Children With Auditory Neuropathy Spectrum Disorder

2009 ◽  
Vol 19 (2) ◽  
pp. 75-84 ◽  
Author(s):  
Jaime Leigh ◽  
Gary Rance ◽  
Shani Dettman ◽  
Richard Dowell
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
Cochlear Implant ◽  
Cochlear Implants ◽  
Auditory Neuropathy ◽  
Cochlear Nerve ◽  
Spectrum Disorder ◽  
Language Outcomes ◽  
Outcomes For Children ◽  
Cochlear Nerve Deficiency

Abstract Cochlear implantation is currently the intervention option of choice for many children with auditory neuropathy spectrum disorder (ANSD) who are unable to obtain benefit from conventional amplification. The aim of this study was to review the speech perception and language outcomes for children with ANSD who had received a cochlear implant and highlight specific clinical considerations for working with this population of children with hearing impairment who are being considered for implantation. Finds for the group of 17 children with ANSD using cochlear implants were compared to previously reported outcomes for children with sensori-neural (SN) type hearing loss using cochlear implants. Two children, identified with cochlear nerve deficiency pre-operatively, received no useful auditory percepts from their cochlear implant and discontinued device use. The remaining children demonstrated speech perception and language outcomes comparable to those observed for SN hearing loss peers using cochlear implants. This paper highlights a number of considerations for clinicians to be aware of and the importance of careful counseling pre-operatively regarding the potential for less-than-optimal outcomes, particularly for those children identified with cochlear nerve deficiency.

Cochlear Nerve Aplasia with Detectable Olivocochlear Efferent Function: A Distinct Presentation of Auditory Neuropathy Spectrum Disorder

2018 ◽  
Vol 23 (1) ◽  
pp. 39-47 ◽  
Author(s):  
Adrian L. James ◽  
Peter R. Dixon ◽  
Robert V. Harrison
Keyword(s):  
Broadband Noise ◽  
Auditory Neuropathy ◽  
Cochlear Nerve ◽  
Spectrum Disorder ◽  
Nerve Function ◽  
Simultaneous Application ◽  
Auditory Neuropathy Spectrum Disorder ◽  
Distortion Product ◽  
Cochlear Nerve Aplasia ◽  
Brainstem Response

Background: Cochlear nerve aplasia (CNA) may present with features of auditory neuropathy spectrum disorder (ANSD), having detectable otoacoustic emissions (OAE) but profound hearing loss. We propose that some children with CNA have a distinct form of afferent ANSD in which efferent cochlear nerve function can be detected using contralateral suppression of OAE. Methods: Children were prospectively enrolled with MRI and auditory brainstem response evidence of unilateral CNA, a normal contralateral ear, and detectable OAE bilaterally. Distortion product OAE (DPOAE) levels were recorded in real time with default primary tone settings: frequency (f)2 = 4.5 kHz and f2/f1 = 1.22 kHz, with level (L)1 = 65 dB SPL and L2 = 55 dB SPL. Recordings were made over 2 min with simultaneous application of an intermittent contralateral broadband noise (CBBN) stimulus at 60 dB SPL. Results: Three girls, aged 4.5, 7, and 8 years, participated. Suppression of DPOAE of 0.15–1.3 dB was detected in all 3 ears with CNA in response to CBBN stimulation. No response was detected in the normal ears. Conclusions: Children with unilateral ANSD can have normal efferent cochlear nerve function despite MRI evidence of ipsilateral CNA. The importance of these findings for newborn hearing screening and cochlear implantation is discussed.

Auditory testing outcomes with hearing aids in patients with auditory neuropathy spectrum disorder

2021 ◽  
Vol 42 (5) ◽  
pp. 103057
Author(s):  
Firas Sbeih ◽  
Donald M. Goldberg ◽  
Sara Liu ◽  
Maxwell Y. Lee ◽  
Gina Stillitano ◽  
...  
Keyword(s):  
Hearing Aids ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder

Outcomes of audiometric testing in children with auditory neuropathy spectrum disorder

2020 ◽  
Vol 129 ◽  
pp. 109757 ◽  
Author(s):  
Joseph B. Meleca ◽  
Gina Stillitano ◽  
Maxwell Y. Lee ◽  
Whitney Lyle ◽  
Yi-Chun Carol Liu ◽  
...  
Keyword(s):  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder

Gender Differences in Audiological Findings and Hearing Aid Benefit in 255 Individuals with Auditory Neuropathy Spectrum Disorder: A Retrospective Study

2016 ◽  
Vol 27 (10) ◽  
pp. 839-845 ◽  
Author(s):  
Vijaya Kumar Narne ◽  
Prashanth Prabhu ◽  
Hunsur S. Chandan ◽  
Mahadeva Deepthi
Keyword(s):  
Gender Differences ◽  
Retrospective Study ◽  
Hearing Aids ◽  
Otoacoustic Emissions ◽  
Hearing Aid ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder ◽  
Acoustic Reflexes ◽  
Hearing Aid Benefit

Background: There are many studies reported in the literature that have summarized audiological findings and possible rehabilitation in individuals with auditory neuropathy spectrum disorder (ANSD). However, there are very few studies that have attempted to delineate the gender differences in audiological characteristics and hearing aid benefit in individuals with ANSD. Purpose: The study aimed to explore the differences between males and females in terms of demographic details, audiogram, speech identification scores, otoacoustic emissions, acoustic reflexes, long latency responses, and hearing aid benefit. Research Design: A retrospective study. Study Sample: A total of 255 individuals diagnosed with ANSD were selected for the study. The study included 137 females and 88 males. Data Collection and Analysis: The demographic details, results of diagnostic audiological testing, and hearing aid benefit were analyzed retrospectively. The differences in findings across gender were compared. Results: The study shows that females have a relatively higher degree of hearing loss and that the majority of females show a rising type of audiometric configuration. The study shows that females have poorer speech perception abilities and experience limited benefits from hearing aids compared to males. Conclusions: The results of the study show that there are gender differences in audiological findings and hearing aid benefits in individuals with ANSD. However, well-controlled prospective studies are essential to confirm the results obtained and to identify the possible mechanisms underlying the gender differences.

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