Cochlear Nerve Hypoplasia: Audiological Characteristics in Children and Adults

2019 ◽  
Vol 24 (3) ◽  
pp. 147-153
Author(s):  
Betul Cicek Cinar ◽  
Emel Tahir ◽  
Merve Ozbal Batuk ◽  
Mehmet Yarali ◽  
Gonca Sennaroglu ◽  
...  
Keyword(s):  
Hearing Loss ◽  
General Term ◽  
Cochlear Nerve ◽  
Type I ◽  
Radiological Findings ◽  
Ear Malformations ◽  
Cochlear Nerve Aplasia ◽  
Incomplete Partition ◽  
Cochlear Malformation ◽  
Cochlear Nerve Hypoplasia

Background: Cochlear nerve deficiency is a general term used to describe both cochlear nerve hypoplasia (CNH) and cochlear nerve aplasia. Although these two conditions can have similar results on audiological evaluation, CNH yields more variation in audiological tests. Objectives: To describe the audiological characteristics of the CNH cases in our series in relation to radiological findings. Methods: We reviewed the medical charts, audiological findings, and radiological findings on cases with CNH. We included cases with CNH in one ear or both ears. Out of 90 subjects with CNH, we included a total of 40 individuals (21 women and 19 men; 49 ears) in the current study. We reviewed and analyzed the participants’ audiological test results according to the radiological findings. Results: Cases with CNH showed variations according to the cochlear structure. There were 13 normal cochleae, 4 with incomplete partition type I, and 32 with cochlear hypoplasia. The accompanying cochlear apertures also showed variation: 17 were normal, 28 stenotic, and 4 aplastic cochlear apertures. The subjects displayed hearing loss ranging from moderate to profound; furthermore, 4 subjects had no response to sound whatsoever. The degree of hearing loss was not statistically significantly different with regard to the presence or absence of cochlear malformation with CNH (p > 0.005). We observed both sensorineural hearing loss and mixed-type hearing loss among the CNH cases. Conclusions: CNH is the presence of a cochlear nerve that is smaller in diameter than the facial nerve. It can be accompanied with other associated inner ear malformations of different degrees of severity. We observed degrees of hearing loss ranging from moderate to profound.

scholarly journals Comparison of ABR and ASSR using narrow-band-chirp-stimuli in children with cochlear malformation and/or cochlear nerve hypoplasia suffering from severe/profound hearing loss

2021 ◽  
Author(s):  
Katharina Eder ◽  
Daniel Polterauer ◽  
Sebastian Semmelbauer ◽  
Maria Schuster ◽  
Tobias Rader ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Narrow Band ◽  
Hearing Threshold ◽  
Cochlear Nerve ◽  
Lower Percentage ◽  
Control Group ◽  
Threshold Estimation ◽  
Severe Hearing Loss ◽  
Cochlear Malformation ◽  
Cochlear Nerve Hypoplasia

Abstract Objectives In pediatric audiology, objective techniques for hearing threshold estimation in infants and children with profound or severe hearing loss play a key role. Auditory brainstem responses (ABR) and auditory steady-state responses (ASSR) are available for frequency-dependent hearing threshold estimations and both techniques show strong correlations but sometimes with considerable differences. The aim of the study was to compare hearing threshold estimations in children with and without cochlear and cochlear nerve malformations. Methods Two groups with profound or severe hearing loss were retrospectively compared. In 20 ears (15 children) with malformation of the inner ear and/or cochlear nerve hypoplasia and a control group of 20 ears (11 children) without malformation, ABR were measured with the Interacoustics Eclipse EP25 ABR system® (Denmark) with narrow-band CE-chirps® at 500, 1000, 2000 and 4000 Hz and compared to ASSR at the same center frequencies under similar conditions. Results ABR and ASSR correlated significantly in both groups (r = 0.413 in malformation group, r = 0.82 in control group). The malformation group showed a significantly lower percentage of “equal” hearing threshold estimations than the control group. In detail, patients with isolated cochlear malformation did not differ significantly from the control group, whereas patients with cochlear nerve hypoplasia showed significantly greater differences. Conclusion ABR and ASSR should be used jointly in the diagnostic approach in children with suspected profound or severe hearing loss. A great difference in hearing threshold estimation between these techniques could hint at the involvement of cochlear nerve or cochlear nerve hypoplasia itself.

scholarly journals Cystic Cochleovestibular Malformation (Incomplete Partition Type 1)

2010 ◽  
Vol 25 (1) ◽  
pp. 41-42 ◽  
Author(s):  
Nathaniel W. Yang
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Temporal Bone ◽  
Cochlear Implantation ◽  
Type I ◽  
Profound Hearing Loss ◽  
Increased Risk ◽  
Ear Malformations ◽  
Temporal Bone Imaging ◽  
Incomplete Partition

A 5-year old female with bilateral profound hearing loss underwent computerized tomographic imaging of the temporal bone as part of the work-up to determine the etiology of her deafness, and to delineate middle and inner ear anatomy prior to cochlear implantation. The examination revealed an inner ear malformation which, based on the newest classification of cochleovestibular malformations by Sennaroglu and Saatci, is called an incomplete partition type I (IP-1) or cystic cochleovestibular malformation. This condition is characterized by (1) a cochlea that is lacking the entire modiolus and cribriform area, resulting in a cystic appearance, and (2) a large cystic vestibule.1   Temporal bone imaging is among the most useful examinations in the etiological investigation of idiopathic sensorineural hearing loss in children, with up to 30%2 of the imaging studies showing an abnormality. The detection of inner ear malformations is important, as some abnormalities are associated with an increased risk of meningitis or progressive hearing loss following head trauma.3 Likewise, the approach to cochlear implantation may be influenced by the type of malformation. In this particular patient, the use of a cochlear implant with a full-band electrode design may be more appropriate, as the location of the neural elements within the cystic cochlea is not definitely known.

Unilateral cochlear nerve hypoplasia in children with mild to moderate hearing loss

2012 ◽  
Vol 132 (11) ◽  
pp. 1160-1167 ◽  
Author(s):  
Hidenobu Taiji ◽  
Noriko Morimoto ◽  
Tatsuo Matsunaga
Keyword(s):  
Hearing Loss ◽  
Cochlear Nerve ◽  
Cochlear Nerve Hypoplasia ◽  
Moderate Hearing Loss

Cochlear nerve anomalies in paediatric single-sided deafness – prevalence and implications for cochlear implantation strategies

2020 ◽  
pp. 1-4
Author(s):  
K Pollaers ◽  
A Thompson ◽  
J Kuthubutheen
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Sensorineural Hearing Loss ◽  
Cochlear Nerve ◽  
Sensorineural Hearing ◽  
Resonance Imaging ◽  
Bilateral Sensorineural Hearing Loss ◽  
Single Sided Deafness ◽  
Cochlear Nerve Hypoplasia

Abstract Objective To determine the prevalence of cochlear nerve anomalies on magnetic resonance imaging in patients with unilateral or bilateral sensorineural hearing loss. Methods A retrospective case series was conducted at a tertiary referral centre. The inclusion criteria were paediatric patients with bilateral or unilateral sensorineural hearing loss, investigated with magnetic resonance imaging. The primary outcome measure was the rate of cochlear nerve hypoplasia or aplasia. Results Of the 72 patients with unilateral sensorineural hearing loss, 39 per cent (28 cases) had absent or hypoplastic cochlear nerves on the affected side. Fifteen per cent (11 cases) had other abnormal findings on magnetic resonance imaging. Eighty-four patients had bilateral sensorineural hearing loss, of which cochlear nerve hypoplasia or aplasia was identified only in 5 per cent (four cases). Other abnormal findings were identified in 14 per cent (12 cases). Conclusion Paediatric patients with unilateral sensorineural hearing loss are more likely to have cochlear nerve anomalies than those patients with bilateral sensorineural hearing loss. This has important implications regarding cochlear implantation for patients with single-sided deafness.

Inner-ear malformations as a cause of single-sided deafness

2020 ◽  
Vol 134 (6) ◽  
pp. 509-518
Author(s):  
E Tahir ◽  
M D Bajin ◽  
S Jafarov ◽  
M Ö Yıldırım ◽  
B Ç Çınar ◽  
...  
Keyword(s):  
Inner Ear ◽  
Internal Auditory Canal ◽  
Cochlear Nerve ◽  
Resonance Imaging ◽  
Radiological Findings ◽  
Inner Ear Malformations ◽  
Disease Prognosis ◽  
Inner Ear Malformation ◽  
Ear Malformations ◽  
Single Sided Deafness

AbstractObjectiveTo determine the prevalence and distribution of inner-ear malformations in congenital single-sided deafness cases, as details of malformation type are crucial for disease prognosis and management.MethodsA retrospective study was conducted of 90 patients aged under 16 years with congenital single-sided deafness. Radiological findings were evaluated using computed tomography and magnetic resonance imaging. Inner-ear malformations were identified and cochlear nerve status was determined in affected ears.ResultsOut of 90 ears, 42 (46.7 per cent) were found to have inner-ear malformation. Isolated cochlear aperture stenosis was the most common anomaly (n = 18, 20 per cent), followed by isolated cochlear aperture atresia (n = 11, 12.2 per cent) and cochlear hypoplasia (n = 7, 7.8 per cent). Cochlear nerve deficiency was encountered in 41 ears (45.6 per cent). The internal auditory canal was also stenotic in 49 ears (54.4 per cent).ConclusionInner-ear malformations, especially cochlear aperture anomalies, are involved in the aetiology of single-sided deafness more than expected. The cause of single-sided deafness differs greatly between congenital and adult-onset cases. All children with single-sided deafness should undergo radiological evaluation, as the prognosis and management, as well as the aetiology, may be significantly influenced by inner-ear malformation type.

scholarly journals Revision Stapedectomy in a Female Patient with Inner Ear Malformation

2016 ◽  
Vol 2016 ◽  
pp. 1-3
Author(s):  
Tirth R. Patel ◽  
Aaron C. Moberly
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Female Patient ◽  
Stapes Surgery ◽  
Mixed Hearing Loss ◽  
Inner Ear Malformation ◽  
Ear Malformations ◽  
Surgical History ◽  
History Of ◽  
Cochlear Malformation

Objectives. We describe an unusual case of surgical management of congenital mixed hearing loss in a female patient with inner ear malformation. This report outlines the role of temporal bone imaging and previous surgical history in evaluating a patient’s risk of perilymph gusher during stapes surgery.Methods. A 68-year-old female patient with a history of profound bilateral mixed hearing loss due to ossicular and cochlear malformation presented to our otology clinic. She had undergone multiple unsuccessful previous ear surgeries. Computed tomography revealed bilateral inner ear malformations. She elected to proceed with revision stapedectomy.Results. The patient received modest benefit to hearing, and no operative complications occurred.Conclusions. Although stapedectomy has been shown to improve hearing in patients with stapes fixation, there is risk of perilymph gusher in patients with inner ear abnormalities. Evaluation and counseling of the risk of gusher during stapes surgery should be done on a case-by-case basis.

scholarly journals Sensorineural Hearing Loss in a Patient Affected by Congenital Cytomegalovirus Infection: Is It Useful to Identify Comorbid Pathologies?

2017 ◽  
Vol 06 (03) ◽  
pp. 181-185 ◽  
Author(s):  
D. Melis ◽  
A. D'Amico ◽  
G. Cappuccio ◽  
G. Auletta ◽  
P. Vassallo ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cytomegalovirus Infection ◽  
Sensorineural Hearing ◽  
Congenital Cytomegalovirus Infection ◽  
Congenital Cytomegalovirus ◽  
Ear Malformations ◽  
Incomplete Partition ◽  
Prompt Diagnosis

AbstractSensorineural hearing loss (SNHL) is a common defect with a multifactorial etiology. Congenital cytomegalovirus infection (cCMV) is the most common infectious cause, and its early detection allows a prompt pharmacological treatment that can improve hearing prognosis. In a consistent percentage of profound SNHL, genetic causes and/or inner ear malformations are involved; their prompt diagnosis might change therapeutic options. This study reports a case of a 3- year-old female patient with symptomatic cCMV infection who also exhibits developmental delay, dysmorphic facial features, bilateral hearing loss, and cochlear incomplete partition, type 2, in 7q21.3 deletion. This deletion includes the genes DLX5 and DLX6, which could be the candidate genes for the ear malformation named incomplete partition, type 2.

Cochlear malformation and sensorineural hearing loss in the Silver-Russell Syndrome

2018 ◽  
Vol 70 (6) ◽  
Author(s):  
Stefania Bigoni ◽  
Antonio Mauro ◽  
Alessandra Ferlini ◽  
Virginia Corazzi ◽  
Andrea Ciorba ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Cochlear Malformation ◽  
Silver Russell Syndrome

An Evaluation of the Relationship between Retinal Nerve Fiber Thickness, Cochlear Nerve Thickness, the Level of Tinnitus, and Hearing Loss in Unilateral Tinnitus Patients

2021 ◽  
pp. 1-8
Author(s):  
Mustafa Avcu ◽  
Mehmet Metin ◽  
Raşit Kılıç ◽  
Muhammed Alpaslan
Keyword(s):  
Hearing Loss ◽  
Nerve Fiber ◽  
Normal Hearing ◽  
Cochlear Nerve ◽  
Significant Difference ◽  
The Relationship ◽  
Fiber Thickness ◽  
Moderate Hearing Loss ◽  
Group 1 ◽  
Retinal Nerve Fiber

Background: In this study, optic coherence tomography (OCT) examination was performed to check whether there was any interaction between ophthalmic axonal structures in unilateral tinnitus patients, and the relationship between optic nerve thickness and cochlear nerve thickness was evaluated. Objective: The aim of the study was to evaluate the relatioship between hearing loss, tinnitus, and nerve thicknesses. Study Design: Prospective study. Setting: Tertiary referral university hospital. Patients: The study included 88 patients with unilateral tinnitus, for which no organic cause could be found in physical examination, psychiatric evaluation, or with imaging methods. Study groups were formed of the tinnitus side and control groups were formed of the healthy side as follows: Group 1 (Non-tinnitus side normal hearing values – n = 30), Group 2 (non-tinnitus side minimal hearing loss – n = 27), Group 3 (non-tinnitus side moderate hearing loss – n = 31), Group 4 (tinnitus side normal hearing values – n = 25), Group 5 (tinnitus side minimal hearing loss – n = 25), and Group 6 (tinnitus side moderate hearing loss – n = 38). Intervention: Retinal nerve fiber layer (RNFL) thickness was evaluated with OCT, and the cochlear nerve cross-sectional area was evaluated with MRI. Main Outcome Measures: RNFL measurements were taken with OCT from the subfoveal area (RNFL-SF) and 1.5 mm temporal to the fovea (RNFL-T µm) and nasal (RNFL-N µm) sectors. On MRI, 3 measurements were taken along the nerve from the cerebellopontine angle as far as the internal auditory canal, and the mean value of these 3 measurements was calculated. Results: When the groups were evaluated in respect of cochlear nerve thickness, a significant difference was seen between Group 1 and both the groups with hearing loss and the tinnitus groups. In the subgroup analysis, a statistically significant difference was determined between Group 1 and Groups 3, 4, 5, and 6 (p = 0.013, p = 0.003, p < 0.001, and p < 0.001, respectively). When the groups were evaluated in respect of the RNFL-SF (µm), RNFL-T (µm), and RNFL-N (µm) values, the differences were determined to be statistically significant (p < 0.001 for all). In the correlation analysis, a negative correlation was determined between hearing loss and cochlear nerve diameter (r: −0.184, p = 0.014), and RNFL-N (r: −0.272, p < 0.001) and between tinnitus and cochlear nerve diameter (r: −0.536, p < 0.001), and RNFL-T (r: −0.222, p < 0.009). Conclusion: The study results clearly showed a relationship between cochlear nerve fiber thickness and hearing loss and the severity of tinnitus in cases with unilateral tinnitus and that there could be neurodegenerative factors in the disease etiology. A similar relationship seen with the RNFL supports the study hypothesis.

scholarly journals Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes

2021 ◽  
pp. 014556132199683
Author(s):  
Wenqi Liang ◽  
Line Wang ◽  
Xinyu Song ◽  
Fenqi Gao ◽  
Pan Liu ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Next Generation Sequencing ◽  
Inner Ear ◽  
Internal Auditory Canal ◽  
Cochlear Nerve ◽  
Transverse Diameter ◽  
Congenital Hearing Loss ◽  
Canal Stenosis ◽  
Ear Anomalies ◽  
Generation Sequencing

The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyndromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in MYH9 and MYH14, encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient’s hearing was rehabilitated with bilateral cochlear implantation.

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