Isolated Cochlear Nerve Hypoplasia with Various Internal Auditory Meatus Deformities in Children

Ito, Ken; Ishimoto, Shin-Ich; Karino, Shotaro

doi:10.1177/000348940711600707

Cited by 13 publications

(5 citation statements)

References 6 publications

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“…It is suggested that vestibular dysfunction involves both the superior and inferior vestibular nerve in the majority of CND cases with vestibular dysfunction. Ito et al reported pathological caloric responses in 5 of 10 patients with unilateral CND, although cVEMP showed normal responses in all 9 patients, suggesting that the superior vestibular nerve is more frequently affected than the inferior vestibular nerve [8]. Unlike previous reports, the present study clearly showed that both the superior and inferior vestibular nerves may frequently be impaired.…”

Section: Discussioncontrasting

confidence: 68%

“…In the cases with VND, the IAC diameter was significantly smaller compared with the cases without VND. A previous report has shown that when the diameter of the IAC is smaller, deterioration of vestibular function tends to occur [8]. When the IAC is smaller, it is often difficult to distinguish and ascertain the presence of the nerve in the IAC even with high-resolution MRI, as in Case No.…”

Section: Discussionmentioning

confidence: 97%

“…As the IAC is composed of not only the cochlear nerve but also the vestibular nerves, some patients with CND are thought to have accompanying VND. However, there have been few reports focusing attention on the deficiency of the vestibular nerve and vestibular function in these children [3,7,8]. In this study, we evaluated the frequency of VND and vestibular function in children with unilateral hearing loss caused by CND.…”

Section: Introductionmentioning

confidence: 99%

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Vestibular nerve deficiency and vestibular function in children with unilateral hearing loss caused by cochlear nerve deficiency

Tsukada

Usami

2021

Acta Oto-Laryngologica

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Background: High-resolution MR imaging enables the visualization of individual nerves in the internal auditory canal (IAC). Cochlear nerve deficiency (CND) is recognized as one of the major causes of sensory neural hearing loss (SNHL), especially in cases of unilateral hearing loss in childhood. Some patients with CND are thought to have accompanying vestibular nerve deficiency (VND). However, there have been few reports focusing on VND and vestibular function in these children. Aims: The aim of this study was to evaluate the frequency of VND and vestibular dysfunction in children with unilateral SNHL caused by CND. Material and Methods: Thirty-eight children with unilateral SNHL, who were diagnosed with CND by 3 T-MRI, were evaluated for VND and underwent caloric testing and cervical vestibular evoked potential (cVEMP). Results: Fourteen of 38 patients (37%) had VND, and eleven (29%) of the patients [ten of the patients (71%) with VND] had at least one vestibular dysfunction. The patients with VND had significantly worse hearing and an IAC of smaller diameter than did patients without VND. Conclusions and significance: We should pay attention to VND as well as vestibular dysfunction in hearing loss patients with CND.

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Section: Discussioncontrasting

confidence: 68%

Section: Discussionmentioning

confidence: 97%

Section: Introductionmentioning

confidence: 99%

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Vestibular nerve deficiency and vestibular function in children with unilateral hearing loss caused by cochlear nerve deficiency

Tsukada

Usami

2021

Acta Oto-Laryngologica

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“…This is in large part due to the fact that high-resolution MRI has only recently become widely available. However, even with limited existing data, we and others have been finding that it is far from rare [22,24]. The present study further suggests that CND is a remarkably common cause of unilateral hearing loss, particularly when accompanied by intact outer hair cell function.…”

Section: Cochlear Nerve Deficiencysupporting

confidence: 56%

Etiology of unilateral neural hearing loss in children

Laury

Casey

McKay

et al. 2009

International Journal of Pediatric Otorhinolaryngology

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“…Unilateral hearing loss is more common than bilateral [20], and it was previously reported that more than one out of ten children initially diagnosed with unilateral hearing loss will progress to bilateral hearing loss [21][22][23]. Cochlear nerve deficiency is the most common type of malformation observed in the setting of congenital unilateral hearing loss [24][25][26]. Although there are reports of familial unilateral hearing loss [27][28][29][30], genetic mutations associated specifically with unilateral hearing loss have yet not been identified with certainty [31].…”

Section: Discussionmentioning

confidence: 99%

4q27 deletion and 7q36.1 microduplication in a patient with multiple malformations and hearing loss: a case report

Zheng

Wang

et al. 2020

BMC Med Genomics

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Background: Chromosome deletions of the long arm of chromosome 4 in 4q syndrome are characterized by mild facial and digital dysmorphism, developmental delay, growth retardation, and skeletal and cardiac anomalies, which is regarded as an autism spectrum disorder. Moreover, some scarce reports indicate that patients with 4q interstitial deletion and 7p duplication may present symptoms associated with hearing loss. Case presentation: A boy with a severe developmental delay not only post-natal but also intrauterine and several dysmorphic features including microcephaly, ocular hypertelorism, exophthalmos, low-set ears, single palmar flexion crease, and overlapping toes presented discontinued cyanosis and recurrent respiratory infections. MRI, BAEP, echocardiogram and bronchoscopy revealed that he had persistent falcine sinus with a thin corpus callosum, left auditory pathway disorder, patent foramen ovale (2 mm), and tracheobronchomalacia with the right superior bronchus arising from the lateral posterior wall of the right main bronchus. Finally, the patient died with severe pneumonia at 10 months. Array CGH revealed a 23.62 Mb deletion at chromosome 4q27, arr [hg19] 4q27-q31.21 (121, 148, 089-144, 769, 263) × 1, and a 0.85 Mb duplication at chromosome 7q36.1, arr [hg19] 7q36.1-q36.2 (152, 510, 685-153, 363,5 98) × 3. It is rare for 4q syndrome cases or 7q duplications previously reported to have a hearing disorder, pulmonary dysplasia, and pulmonary arterial hypertension. Conclusions: The phenotype of our patient mainly reflects the effects of haploinsufficiency of FGF2, SPATA5, NAA15, SMAD1, HHIP genes combined with a microduplication of 7q36.1.

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Isolated Cochlear Nerve Hypoplasia with Various Internal Auditory Meatus Deformities in Children

Abstract: The risk of co-involvement of the inner ear and superior vestibular nerve functions is higher in the presence of a narrower bony IAM. Cochlear nerve hypoplasia is proposed as one of the most important causes of juvenile unilateral deafness because of its unexpectedly high incidence.

Cited by 13 publications

References 6 publications

Vestibular nerve deficiency and vestibular function in children with unilateral hearing loss caused by cochlear nerve deficiency

Vestibular nerve deficiency and vestibular function in children with unilateral hearing loss caused by cochlear nerve deficiency

Etiology of unilateral neural hearing loss in children

4q27 deletion and 7q36.1 microduplication in a patient with multiple malformations and hearing loss: a case report

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