Auditory nerve disease and auditory neuropathy spectrum disorders

Kaga, Kimitaka

doi:10.1016/j.anl.2015.06.008

Cited by 44 publications

(33 citation statements)

References 29 publications

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“…Several factors are involved in its physiopathology such as OTOF or OPA1 gene mutation. 17 In our study 1 post-lingual and 3 pre-lingual cases were linked to auditory neuropathy.…”

Section: Discussionsupporting

confidence: 44%

Hearing Loss Etiology in Patients Submitted to Cochlear Implant

Pontes

Loures

Cruz

2019

International Bulletin of Otorhinolaryngology

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Introduction: Hearing impairment is the most common sensorial disorder. Deafness has several etiologies. It is important to investigate them, not only because some may lead to lower performance with a cochlear implant, but also to define preventive strategies. Objective: To determine deafness etiology among patients submitted to cochlear implantation. Methods: Retrospective sampling of patients submitted to cochlear implantation between 2006 and 2017 in a Brazilian referral hearing impairment center. Patients were grouped into post-lingual or pre-lingual deafness and an etiological profile was outlined for each group. Results: 159 patients were evaluated, 74 post-lingual and 85 prelingual. In post-lingual group, the most common cause of hearing impairment was meningitis (n = 16/21,62%). Other etiologies were: non-syndromic genetic hearing loss (n = 12/16,22%), otosclerosis (n = 7/9,46%), autoimmune (n = 5/6,76%), viral infections-mumps and measles (n = 3/4,05%), Alport syndrome (n = 2/2,7%), Meniиreґs disease (n = 2/2,7%), ototoxicity (n = 2/2,7%), enlarged vestibular aqueduct (n = 2/2,7%), otitis media complications (n = 2/2,7%), trauma (n = 2/2,7%), lues (n = 1/1,35%), vestibular schwannoma (n = 1/1,35%), stroke (n = 1/1,35%), auditory neuropathy (n = 1/1,35%) and 15 patients (20,27%) had undefined etiology. In pre-lingual group, non-syndromic genetic hearing loss was the most prevalent cause (n = 22/25,88%), followed by perinatal complications (n = 20/23,53%), congenital infections-cytomegalovirus, rubella and mumps (n = 8/9,41%), genetic syndromes such as Waardenburg (n = 5/5,88%), meningitis (n = 5/5,88%), malformation-mostly incomplete partition type II (n = 3/3,53%), auditory neuropathy (n = 3/3,53%), ototoxicity (n = 2/2,35%) and 17 patients had undefined causes (20%). Conclusion: In our population, the most frequent etiology for postlingual deafness was meningitis and for pre-lingual deafness was non-syndromic genetic causes.

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“…Several factors are involved in its physiopathology such as OTOF or OPA1 gene mutation. 17 In our study 1 post-lingual and 3 pre-lingual cases were linked to auditory neuropathy.…”

Section: Discussionsupporting

confidence: 44%

Hearing Loss Etiology in Patients Submitted to Cochlear Implant

Pontes

Loures

Cruz

2019

International Bulletin of Otorhinolaryngology

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“…In Japanese patients with AN, OTOF mutations accounted approximately 60% of the cases . Within at least the first one or two years after birth they show preserved OAE or CM without an ABR response, so they are diagnosed with AN. The trans‐membrane protein OTOFERLIN, encoded by the OTOF gene, is expressed in the inner and outer hair cells of the rodent cochlea .…”

Section: Introductionmentioning

confidence: 99%

Elongated EABR wave latencies observed in patients with auditory neuropathy caused by OTOF mutation

Minami

Enomoto

Matsunaga

et al. 2018

Laryngoscope Investig Oto

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ObjectivesWe sought to determine how the pathology altered electrically evoked auditory brainstem responses (EABRs) in patients with hearing loss by evaluating EABRs in auditory neuropathy patients with OTOF mutations comparing with various types of congenital deafness.MethodsWe included 15 patients with congenital hearing loss, grouped according to pathology: OTOF mutations (n = 4), GJB2 mutations (n = 4), SLC26A4 mutations (n = 4), or cytomegalovirus infections (n = 3). EABRs were recorded when patients underwent cochlear implantation surgery. We evaluated the latencies and amplitudes of the recorded EABRs and compared them statistically between four groups.ResultsThe EABR latencies of Wave III and Wave V, and of the interval between them, were significantly longer in the OTOF mutation group than in the GJB2 and SLC26A4 mutation groups (Wave III) and in all three other groups (Wave V and Wave III‐V latency); amplitudes were not significantly different between groups.ConclusionsOur results suggest OTOF mutations cause delayed (or slowed) postsynaptic neurotransmission, although the presumed mechanism involved reduced presynaptic transmission between hair cells and spiral ganglion neurons.Level of EvidenceMainly a case report

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“…Regarding the second case, it is worth emphasizing that the data indicating retro-cochlear impairment is atypical, as in general, patients with neuronal alterations have hearing loss, as well as poor performance on speech comprehension in silence (11) , which did not occur. However, it is noteworthy that in adult patients suffering from retro-cochlear pathology, a variety of clinical findings has been discussed in the literature, where it is claimed that the audiological findings vary from normal hearing thresholds to profound hearing loss, which can be of the fluctuating type, and the acoustic reflexes can be either absent or increased (11,12) . As ZIKV is now recognized as a factor that can lead to neurological complications in adults following infection, such as Guillaine-Barré syndrome, facial paralysis, myelitis, and others that are still being analyzed, the importance of studying the damage caused by ZIKV action is further emphasized (13,14) .…”

Section: Discussionmentioning

confidence: 99%

Achados otológicos em pacientes pós-infecção pelo zika vírus: estudos de caso

Martins

Rodrigues²,

Santos

et al. 2017

Audiol., Commun. Res.

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The Zika virus is transmitted between humans by the Aedes aegypti mosquito and has become a public health emergency, as declared by the World Health Organization. Studies have reported the virus to be associated with hearing alterations in patients, following infection. This study was conceived in view of the importance of hearing for communication, associated with the limited number of reports on the impact of Zika virus on the hearing system of adults. The aim of the study was to characterize the otological findings in two adult patients, following infection with the Zika virus. The otological findings observed in two outpatients of an otorhinolaryngology service were described, both of whom reported hearing complaints immediately following the clinical symptoms of Zika virus infection. Both adult patients reported the following symptoms: discomfort, skin rash, joint pain, auricular fullness, and hearing loss. Serologic testing (IgG) for Zika virus was performed. The audiological findings were unilateral peripheral hearing loss in one of the patients, alterations in contralateral acoustic reflexes in both patients, and alteration in the brainstem auditory-evoked potential in one patient. The audiological findings demonstrate possible neuronal involvement in the manifested complaints, whether or not associated with the peripheral component, in patients infected with Zika virus. Keywords

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Auditory nerve disease and auditory neuropathy spectrum disorders

Cited by 44 publications

References 29 publications

Hearing Loss Etiology in Patients Submitted to Cochlear Implant

Hearing Loss Etiology in Patients Submitted to Cochlear Implant

Elongated EABR wave latencies observed in patients with auditory neuropathy caused by OTOF mutation

Achados otológicos em pacientes pós-infecção pelo zika vírus: estudos de caso

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