Cochlear Nerve Deficiency and Associated Clinical Features in Patients With Bilateral and Unilateral Hearing Loss

Nakano, Atsuko; Arimoto, Yukiko; Matsunaga, Tatsuo

doi:10.1097/mao.0b013e3182804b31

Cited by 58 publications

(47 citation statements)

References 10 publications

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“…Similar to the fin-dings of the current study, the cochlear nerve surface area of the patients with idiopathic hearing loss and loss associated with connexin26 gene mutation was determined to be smaller than that of the ears with normal hearing. Nakano et al evaluated the computed tomography (CT) findings of children with congenital hearing loss, and again similar to the findings of the current study, the cochlear nerve canal was found to be smaller in children with hearing loss 9. …”

supporting

confidence: 86%

İdiyopatik Tek Taraflı Sensörinöral İşitme Kayıplı Hastalarda Kohlear Sinir Çapının Manyetik Rezonans Görüntüleme ile Değerlendirilmesi

Taydaş¹,

İnan²

2019

Sakarya Medical Journal

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Objective The aim of this study was to evaluate the cochlear nerve diameter in patients with unilateral sensorineural hearing loss and to compare the diameter of the normal side with the hearing loss side. Materials and Methods A total of 36 patients with idiopathic unilateral sensorineural hearing loss were included in the study. Magnetic resonance imaging (MRI) and pure sound audiogram were performed on all the patients. Both cochlear nerve diameters were measured on axial thin-section constructive interference in steady state (CISS) sequences. Results The patients were comprised of 16 males and 20 females with a mean age of 52.3±13.7 years. Hearing loss was in the right ear in 11 patients, and in the left ear in 25. The average cochlear nerve diameter on the side with hearing loss was 0.13 cm while it was 0.19 cm on the normal side. There was a statistically significant difference between the two sides (p < 0.001). Conclusion In patients with unilateral sensorineural hearing loss, MRI allows the assessment of the cochlear nerve with anatomic detail, and enables the exclusion of possible organic pathologies. Demonstration of decreased cochlear nerve diameter in idiopathic sensorineural hearing loss will contribute to elucidating the etiology of this disease in the future. Keywords cochlear nerve; sensorineural hearing loss; magnetic resonance imaging Öz Amaç Bu çalışmanın amacı, tek taraflı idiyopatik sensörinöral işitme kaybı bulunan hastalarda kohlear sinir çapını değerlendirmek ve normal taraf ile işitme kaybı bulunan tarafın çaplarını karşılaştırmaktır. Gereç ve Yöntemler Tek taraflı idiyopatik sensörinöral işitme kaybı bulunan toplam 36 hasta çalışmaya dahil edildi. Tüm hastalara manyetik rezonans görüntüleme (MRG) ve saf ses odyometrisi uygulandı. Her iki taraftaki kohlear sinir çapları ince kesit aksiyal "constructive interference in steady state (CISS)" sekansından sekansından elde edilmiş sagittal reformat görüntüler üzerinden ölçüldü. Bulgular Hastaların 16'sı erkek 20'si kadındı ve yaş ortalamaları 52.3±13.7 idi. 11 hastada sağ tarafta, 25 hastada ise sol tarafta işitme kaybı vardı. Ortalama kohlear sinir çapı işitme kaybı olan tarafta 0,13 cm iken normal tarafta 0,19 cm idi. İki taraf arasında anlamlı farklılık mevcuttu (p < 0.001). Sonuç Tek taraflı sensörinöral işitme kaybı bulunan hastalarda MRG kohlear sinirin anatomik açıdan detaylı incelenmesine olanak sağlar ve olası organik patolojilerin dışlanmasına yardımcı olur. Tek taraflı idiyopatik sensörinöral işitme kaybında kohlear sinir çapının daha az olduğunun gösterilmesi gelecekte hastalığın etiyolojisinin aydınlatılmasına katkı sağlayacaktır.

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supporting

confidence: 86%

İdiyopatik Tek Taraflı Sensörinöral İşitme Kayıplı Hastalarda Kohlear Sinir Çapının Manyetik Rezonans Görüntüleme ile Değerlendirilmesi

Taydaş¹,

İnan²

2019

Sakarya Medical Journal

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“…As UNHS now allows for better identification of children with UHL, the most common time of onset is now understood to be congenital, accounting for 45% of infants and children with sensorineural UHL (Ghogomu et al, 2014). Of congenital causes, it appears that cochlear nerve deficiency may be the most common, accounting for up to 50% of children with congenital severe-to-profound UHL (Nakano, Arimoto, & Matsunaga, 2013). For the purposes of prevention, acquired causes of HL including infections such as congenital cytomegalovirus (CMV) (Kumar et al, 1984) and meningitis (Fortnum & Davis, 1993), as well as temporal bone trauma (Morgan, Coker, & Jenkins, 1994) should be recognized (See Table 1).…”

Section: Causes Of Unilateral Hearing Lossmentioning

confidence: 99%

Asymmetric and unilateral hearing loss in children

Vila

Lieu

2015

Cell Tissue Res

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Asymmetric and unilateral hearing losses in children have traditionally been underappreciated, but health care practitioners are now beginning to understand their effect on development and the underlying pathophysiologic mechanisms. The common wisdom among medical and educational professionals has been that at least one normal hearing or near-normal hearing ear was sufficient for typical speech and language development in children. The objective of this review is to illustrate to the non-otolaryngologist the consequences of asymmetric and unilateral hearing loss in children on developmental and educational outcomes. In the process, etiology, detection, and management are discussed. Lastly, implications for further research are considered.

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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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Cochlear Nerve Deficiency and Associated Clinical Features in Patients With Bilateral and Unilateral Hearing Loss

Abstract: Our results suggest differences in the causes and mechanisms of CND in children with bilateral versus unilateral hearing loss.

Cited by 58 publications

References 10 publications

İdiyopatik Tek Taraflı Sensörinöral İşitme Kayıplı Hastalarda Kohlear Sinir Çapının Manyetik Rezonans Görüntüleme ile Değerlendirilmesi

İdiyopatik Tek Taraflı Sensörinöral İşitme Kayıplı Hastalarda Kohlear Sinir Çapının Manyetik Rezonans Görüntüleme ile Değerlendirilmesi

Asymmetric and unilateral 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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