Auditory neuropathy: clinical characteristics and therapeutic approach

Raveh, Eyal; Buller, Nora; Badrana, Ola; Attias, Joseph

doi:10.1016/j.amjoto.2006.09.006

Cited by 71 publications

(69 citation statements)

References 26 publications

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“…A similar pattern was previously reported in patients with severe infantile hyperbilirubinemia, ischemic/hypoxic encephalopathy, and central nervous system immaturity Raveh et al, 2007]. Transient deafness associated with an increase in core temperature was first reported by Starr et al [1998] who attributed it to a conduction block of the neural tissue induced by a demyelinating disorder of the auditory nerve.…”

Section: Discussionsupporting

confidence: 52%

Auditory System Dysfunction due to Infantile Thiamine Deficiency: Long-Term Auditory Sequelae

Attias

Raveh²,

Aizer-Dannon

et al. 2012

Audiol Neurotol

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Eleven infants who were fed a thiamine-deficient formula for a mean of 3 months were evaluated for immediate and long-term auditory abnormalities. At presentation, 8 infants had auditory neuropathy spectrum disorder (ANSD), which resolved with supplementary thiamine in 5 children, was permanent in 2 children, and deteriorated in 1 patient who died at the age of 7 years. An additional patient had an auditory pattern corresponding to that of auditory neuropathy of brain stem origin. The 2 remaining patients had unilateral cochlear hearing loss. Six to 8 years later, all patients with transient ANSD had normal audiograms, 2 patients had unilateral cochlear hearing loss, and the rest had neural hearing loss. All survivors had a language developmental delay and impaired speech intelligibility of varying degrees, especially in the presence of background noise. Thiamine is crucial for normal auditory development and function, and its deficiency may be considered an acquired metabolic cause of ANSD in infants.

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Section: Discussionsupporting

confidence: 52%

Auditory System Dysfunction due to Infantile Thiamine Deficiency: Long-Term Auditory Sequelae

Attias

Raveh²,

Aizer-Dannon

et al. 2012

Audiol Neurotol

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“…: The child recognized more than 50% of the words in the Word Recognition task but was not able to carry out the Sentence Comprehension task ANSD, the exons of this gene must be researched in order to identify the greatest number of possible pathogenic mutations. The main complaint found among cases of ANSD is speech perception difficulty, especially in the presence of noise (8) . Since measuring the influence of noise on the speech perception of these children was not a purpose of this study, we opted to conduct the assessment of this ability only in silence, within a soundproof booth.…”

Section: Discussionmentioning

confidence: 99%

Auditory and communicative abilities in the auditory neuropathy spectrum disorder and mutation in the Otoferlin gene: clinical cases study

Costa

Martinho-Carvalho

Cunha

et al. 2012

J. Soc. Bras. Fonoaudiol.

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This study had the aim to investigate the auditory and communicative abilities of children diagnosed with Auditory Neuropathy Spectrum Disorder due to mutation in the Otoferlin gene. It is a descriptive and qualitative study in which two siblings with this diagnosis were assessed. The procedures conducted were: speech perception tests for children with profound hearing loss, and assessment of communication abilities using the Behavioral Observation Protocol. Because they were siblings, the subjects in the study shared family and communicative context. However, they developed different communication abilities, especially regarding the use of oral language. The study showed that the Auditory Neuropathy Spectrum Disorder is a heterogeneous condition in all its aspects, and it is not possible to make generalizations or assume that cases with similar clinical features will develop similar auditory and communicative abilities, even when they are siblings. It is concluded that the acquisition of communicative abilities involves subjective factors, which should be investigated based on the uniqueness of each case.

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“…As such determination of patient who will benefit from hearing aids or cochlear implantation is difficult (Raveh, Buller, Badrana, & Attias, 2007). The development of improved testing techniques that can be used to diagnose, characterize, and differentiate between the numerous diseases that make up this spectrum may allow patients to be treated earlier.…”

Section: Auditory Neuropathy Spectrum Disorder (Ansd)mentioning

confidence: 99%

Contralateral Suppression of Otoacoustic Emissions: Working Towards a Simple Objective Frequency Specific Test for Hearing Screening

Wolter¹,

Harrison²,

James³

2012

Hearing Loss

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Auditory neuropathy: clinical characteristics and therapeutic approach

Cited by 71 publications

References 26 publications

Auditory System Dysfunction due to Infantile Thiamine Deficiency: Long-Term Auditory Sequelae

Auditory System Dysfunction due to Infantile Thiamine Deficiency: Long-Term Auditory Sequelae

Auditory and communicative abilities in the auditory neuropathy spectrum disorder and mutation in the Otoferlin gene: clinical cases study

Contralateral Suppression of Otoacoustic Emissions: Working Towards a Simple Objective Frequency Specific Test for Hearing Screening

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