Auditory neuropathy: Endocochlear lesion or temporal processing impairment? Implications for diagnosis and management

Vlastarakos, Petros V.; Nikolopoulos, Thomas P.; Tavoulari, Evangelia; Papacharalambous, George; Korres, Stavros

doi:10.1016/j.ijporl.2008.04.004

Cited by 58 publications

(52 citation statements)

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“…AN is a hearing disorder characterized by absent or severely impaired ABR in the presence of normal outer hair cell function and is evaluated by otoacoustic emissions and/or cochlear microphonics [3] . Some possible sites of lesions that can produce the audiometric and electrophysiological profile of AN include: the inner hair cells, the synaptic junction between the inner hair cells and type I afferent nerve fibers, the SGNs and the specific damage or demyelinization of type I auditory nerve fibers [3] .…”

Section: Discussionmentioning

confidence: 99%

“…Some possible sites of lesions that can produce the audiometric and electrophysiological profile of AN include: the inner hair cells, the synaptic junction between the inner hair cells and type I afferent nerve fibers, the SGNs and the specific damage or demyelinization of type I auditory nerve fibers [3] . The clinical features of AN can vary considerably with respect to the age of onset, etiology, severity of hearing loss and site of lesions [3] . Although the underlying mechanisms of AN are key to understanding and treating the disease, these mechanisms are still largely unknown.…”

Section: Discussionmentioning

confidence: 99%

“…It was recently identified to define a specific type of hearing deficit resulting from the impairment of auditory nerve function [2] . Two hallmark features of AN are normal outer hair cell function and the presence of absent/abnormal auditory brainstem responses (ABR) [3] . Unfortunately, there is no effective treatment for AN mainly because its mechanism is unclear [3] .…”

Section: Introductionmentioning

confidence: 99%

“…Two hallmark features of AN are normal outer hair cell function and the presence of absent/abnormal auditory brainstem responses (ABR) [3] . Unfortunately, there is no effective treatment for AN mainly because its mechanism is unclear [3] .…”

Section: Introductionmentioning

confidence: 99%

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Inhalation of hydrogen gas attenuates ouabain-induced auditory neuropathy in gerbils

Xie

et al. 2012

Acta Pharmacol Sin

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Aim: Auditory neuropathy (AN) is a hearing disorder characterized by abnormal auditory nerve function with preservation of normal cochlear hair cells. This study was designed to investigate whether treatment with molecular hydrogen (H 2 ), which can remedy damage in various organs via reducing oxidative stress, inflammation and apoptosis, is beneficial to ouabain-induced AN in gerbils. Methods: AN model was made by local application of ouabain (1 mmol/L, 20 mL) to the round window membrane in male Mongolian gerbils. H 2 treatment was given twice by exposing the animals to H 2 (1%, 2%, and 4%) for 60 min at 1 h and 6 h after ouabain application. Before and 7 d after ouabain application, the hearing status of the animals was evaluated using the auditory brainstem response (ABR) approach, the hear cell function was evaluated with distortion product otoacoustic emissions (DPOAE). Seven days after ouabain application, the changes in the cochleae, especially the spiral ganglion neurons (SGNs), were morphologically studied. TUNEL staining and immunofluorescent staining for activated caspase-3 were used to assess the apoptosis of SGNs. Results: Treatment with H 2 (2% and 4%) markedly attenuated the click and tone burst-evoked ABR threshold shift at 4, 8, and 16 kHz in ouabain-exposed animals. Neither local ouabain application, nor H 2 treatment changed the amplitude of DPOAE at 4, 8, and 16 kHz. Morphological study showed that treatment with H 2 (2%) significantly alleviated SGN damage and attenuated the loss of SGN density for each turn of cochlea in ouabain-exposed animals. Furthermore, ouabain caused significantly higher numbers of apoptotic SGNs in the cochlea, which was significantly attenuated by the H 2 treatment. However, ouabain did not change the morphology of cochlear hair cells. Conclusion:The results demonstrate that H 2 treatment is beneficial to ouabain-induced AN via reducing apoptosis. Thus, H 2 might be a potential agent for treating hearing impairment in AN patients.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Inhalation of hydrogen gas attenuates ouabain-induced auditory neuropathy in gerbils

Xie

et al. 2012

Acta Pharmacol Sin

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“…Existen algunos autores que señalan la posible disminución o desaparición de EOAs debido a una "sobreamplificación" en presencia de actividad coclear, hecho que aún no está del todo claro. También se ha observado la ausencia de EOAs en individuos con DENA no usuarios de audífonos 2,5 .…”

Section: Discussionunclassified

Estudio electrofisiológico y electroacústico de un desorden del espectro de neuropatía auditiva: Reporte de un caso en recién nacido de alto riesgo

Cañete

L²

2011

Rev. Otorrinolaringol. Cir. Cabeza Cuello

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El desorden del espectro de neuropatía auditiva (DENA) describe una condición en la cual se observa o se ha observado emisiones otoacústicas (EOA) junto con potenciales evocados auditivos de tronco (PEAT) anormales o ausentes. Su identificación se basa en la presencia de potencial microfónico coclear (PMIC) junto con un PEAT anormal o ausente y EOA presentes o antecedentes de su presencia. Los umbrales tonales pueden encontrarse entre los rangos de normalidad hasta pérdidas auditivas profundas. El reconocimiento del habla generalmente se encuentra afectado particularmente en presencia de ruido de fondo, sin embargo en algunos individuos se mantiene conservado (sin ruido).Se presenta caso clínico de un recién nacido de sexo femenino de pretérmino de 29 semanas y 810 grs (GIII), apgar 7/8, presenta displasia broncopulmonar, sospecha de infección connatal tratada, hiperbilirrubinemia (max 11 mg/dl) tratada con fototerapia. Es derivada al Servicio de Otorrinolaringología para su evaluación auditiva. La evaluación auditiva realizada corresponde a potenciales evocados auditivos de tronco (PEAT), impedanciometria, audiometría de campo de respuesta reflejas y emisiones otoacústicas. Los resultados obtenidos concuerdan con lo descrito en la literatura para un DENA.Palabras claves: Neuropatía auditiva, Desorden del espectro de neuropatía auditiva, Hipoacusia neural, Hipoacusia en neonatos de riesgo, Potenciales evocados auditivos de tronco cerebral. ABSTRACTAuditory neuropathy spectrum disorder (ANSD) describes a condition in which it is possible to find otoacoustic emissions (OAE) in presence of abnormal or absent auditory brainstem evoked potentials (ABR). The pure tone thresholds may show a

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Bilirubin induces auditory neuropathy in neonatal guinea pigs via auditory nerve fiber damage

Shi

Wang

et al. 2012

J of Neuroscience Research

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Bilirubin can cause temporary or permanent sensorineural deafness in newborn babies with hyperbilirubinemia. However, the underlying targets and physiological effects of bilirubin-induced damage in the peripheral auditory system are unclear. Using cochlear functional assays and electron microscopy imaging of the inner ear in neonatal guinea pigs, we show here that bilirubin exposure resulted in threshold elevation in both compound action potential (CAP) and auditory brainstem response (ABR), which was apparent at 1 hr and peaked 8 hr after drug administration. The threshold elevation was associated with delayed wave latencies and elongated interwave intervals in ABR and CAP. At 72 hr postinjection, these measures returned to control levels, except for the CAP amplitude. Cochlear microphonics remained unchanged during the experiment. Morphological abnormalities were consistent with the electrophysiological dysfunction, revealing fewer auditory nerve fibers (ANFs) in the basal turn, myelin sheath lesions of spiral ganglion neurons (SGNs) and ANFs, and loss of type 1 afferent endings beneath inner hair cells (IHCs) without loss of hair cells at 8 hr posttreatment. Similar to the electrophysiological findings, morphological changes were mostly reversed 10 days after treatment, except for the ANF reduction in the basal turn. These results suggest that hyperbilirubinemia in neonatal guinea pigs impaired auditory peripheral neuromechanisms that targeted mainly the IHC synapses and the myelin sheath of SGNs and their fibers. Our observations indicate a potential connection between hyperbilirubinemia and auditory neuropathy.

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Auditory neuropathy: Endocochlear lesion or temporal processing impairment? Implications for diagnosis and management

Cited by 58 publications

References 100 publications

Inhalation of hydrogen gas attenuates ouabain-induced auditory neuropathy in gerbils

Inhalation of hydrogen gas attenuates ouabain-induced auditory neuropathy in gerbils

Estudio electrofisiológico y electroacústico de un desorden del espectro de neuropatía auditiva: Reporte de un caso en recién nacido de alto riesgo

Bilirubin induces auditory neuropathy in neonatal guinea pigs via auditory nerve fiber damage

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