Audiological and Vestibular Functions in Patients With Lateral Semicircular Canal Dysplasia and Aplasia

Objectives. The aim of the present study was to evaluate audiologic and vestibular functions in patients with lateral semicircular canal (LSCC) dysplasia/aplasia.Methods. We conducted a retrospective study of a patients with LSCC dysplasia and aplasia at tertiary referral center. The subjects included 15 patients with LSCC dysplasia or aplasia, with or without combined inner ear anomalies. Medical history, temporal bone computed tomography scans, pure-tone audiograms, and vestibular function test results were analyzed.Results. LSCC anomaly was identified in 15 patients (20 ears). Nine patients had unilateral LSCC dysplasia only and showed a mean pure-tone average of 45.5±28.7 dB, while three patients (33.3%) among them had normal hearing. Six patients had bilateral LSCC dysplasia/aplasia combined with other inner ear anomalies and profound bilateral hearing loss. Notably, only four out of 15 patients (26.7%) had dizziness symptoms. On caloric test, patients with isolated LSCC dysplasia showed a 51.8%±29.3% level of canal paresis (eight out of nine patients showed anomalies), whereas patients with bilateral LSCC dysplasia/aplasia presented bilateral vestibular loss. One patient with isolated LSCC underwent video-head impulse test; horizontal canal gain decreased to 0.62 (17% asymmetry) and anterior canal gain was 0.45 (52.6% asymmetry), whereas posterior canal gain was normal.Conclusion. Bilateral LSCC dysplasia/aplasia is comorbid with other inner ear anomalies and presents as profound bilateral hearing loss and vestibulopathy. In contrast, isolated unilateral LSCC dysplasia presents as ipsilateral horizontal canal paresis. Hearing function in isolated LSCC dysplasia is usually, but not always, impaired with varying severity.

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Unilateral Horizontal Semicircular Canal Malformation Causing Recurrent Vertigo

Philippine Journal of Otolaryngology Head and Neck Surgery ◽

10.32412/pjohns.v32i2.87 ◽

2018 ◽

Vol 32 (2) ◽

pp. 58-59

Author(s):

Nathaniel W. Yang

Keyword(s):

Hearing Loss ◽

Inner Ear ◽

Temporal Bone ◽

Semicircular Canal ◽

Lateral Semicircular Canal ◽

Vestibular Loss ◽

Vestibular Neurectomy ◽

Horizontal Semicircular Canal ◽

Ear Malformations ◽

Vestibular Symptoms

A 62-year-old man consulted for recurrent episodes of vertigo lasting from seconds to several minutes. The vertigo was variably described as spinning, lateral swaying, and a feeling of being “unsure of his position in space.” These episodes were noted to have begun when the patient was still in his 20’s. Standard pure tone audiometry revealed a mild-to-moderate downsloping mixed hearing loss in the left ear. Bithermal caloric testing indicated the presence of a significant left-sided peripheral vestibular loss. Due to the fact that the vertigo episodes presented relatively early in life, the possibility of a congenital inner ear malformation was considered as a cause for his symptoms. Computerized tomographic (CT) imaging of the temporal bone was performed. This clearly showed the left horizontal semicircular canal lacking a central bony island. (Figure 1 and 2) The cochlea, superior and posterior semicircular canals, vestibular and cochlear aqueducts, and ossicular chain were grossly normal. A malformation of the horizontal or lateral semicircular canal is one of the most common inner ear malformations, as it is the last vestibular structure to be formed during inner ear embryogenesis. As such, it may occur in isolation or may be associated with other vestibular, cochlear, or middle ear malformations.1,2 Although vertigo and dizziness are symptoms to be expected in such a condition, existing data indicates that it may be totally asymptomatic, or it may also present as a sensorineural, conductive, or mixed type of hearing loss.1,3 Radiologic imaging is of prime importance in diagnosing such conditions, especially when auditory and/or vestibular symptoms manifest early in life. This case perfectly illustrates the need for such studies, as the patient went undiagnosed for more than forty years! No definitive statements can be gleaned from existing medical literature with respect to treatment. However, in patients with debilitating vestibular symptoms, management with modalities that selectively target the vestibular system, but spare the auditory system, such as vestibular neurectomy and trans-tympanic aminoglycoside therapy appear to be reasonable options. References Johnson J, Lalwani AK. Sensorineural and conductive hearing loss associated with lateral semicircular canal malformation. Laryngoscope 2000 Oct;110(10):1673–1679. DOI:10.1097/00005537-200010000-00019 PMID: 11037823 Casselman JW, Delanote J, Kuhweide R, van Dinther J, De Foer B, Offeciers EF. Congenital malformations of the temporal bone. In: Lemmerling M, De Foer B, editors. Temporal bone imaging. Berlin Heidelberg: Springer-Verlag; 2015, pp. 120-154. Kim CH, Shin JE, Lee YJ, Park HJ. Clinical characteristics of 7 patients with lateral semicircular canal dysplasia. Res Vestib Sci 2012;11(2):64-68.

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Partial Duplication of the Lateral Semicircular Canal—A Novel Anatomical Malformation in a Child with Barakat Syndrome

Journal of Pediatric Neurology ◽

10.1055/s-0041-1740366 ◽

2021 ◽

Author(s):

Varun Kannan ◽

Brandon Tran

Keyword(s):

Hearing Loss ◽

Inner Ear ◽

Semicircular Canal ◽

Sensorineural Deafness ◽

Anatomical Variations ◽

Lateral Semicircular Canal ◽

Partial Duplication ◽

Posterior Limb ◽

Ear Malformations ◽

Ear Anomalies

AbstractSeveral known genetic causes of sensorineural deafness are associated with dysplasia of inner ear structures, including the cochlea and labyrinth. Here, we present a child with Barakat syndrome and sensorineural hearing loss, found to have multiple inner ear anomalies including partial duplication of the posterior limb of the left lateral semicircular canal. To our knowledge, duplication of the semicircular canal has not previously been reported. This finding expands our understanding of the range of anatomical variations observed in congenital inner ear malformations, and further characterizes the phenotypic manifestations of Barakat syndrome.

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Which Is More Important for the Prognosis of Sudden Sensorineural Hearing Loss with Vertigo, Canal Paresis or Benign Paroxysmal Positional Vertigo?

Research in Vestibular Science ◽

10.21790/rvs.2021.20.3.101 ◽

2021 ◽

Vol 20 (3) ◽

pp. 101-107

Author(s):

Yong-Hwi An ◽

Hyun Joon Shim

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Pure Tone ◽

Benign Paroxysmal Positional Vertigo ◽

Sudden Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Horizontal Canal ◽

Canal Paresis ◽

Positional Vertigo ◽

Paroxysmal Positional Vertigo

Objectives: This study was performed to determine characteristics and the prognostic values in idiopathic sudden sensorineural hearing loss (SSNHL) with comorbid ipsilateral canal paresis (CP) and/or benign paroxysmal positional vertigo (BPPV). Methods: Of the 338 patients with a diagnosis of idiopathic SSNHL, 29 patients (8.6%) with CP and 24 patients (7.1%) with BPPV were recruited and compared to 23 patients with SSNHL and vertigo but without CP or BPPV. The patients were evaluated for their initial hearing threshold, type of canal involved, response to repositioning maneuvers, and hearing outcome for 6 months. Results: Patients with CP (+) BPPV (‒) showed lower pure-tone averages than those with CP (‒) BPPV (+) on initial and follow-up audiograms. The improvement in pure-tone averages was less in the CP (+) BPPV (‒) group than in the CP (‒) BPPV (+) group. The improvement in speech discrimination scores was less in the CP (+) BPPV (‒) group than in the CP (‒) BPPV (‒) group. BPPV most commonly involved the posterior canal (15 of 24, 62.5%), followed by the horizontal canal (13 of 24, 54.2%). Three of 24 patients (12.5%) had recurrences of BPPV. Conclusions: CP is a more serious sign for hearing recovery than BPPV, although both CP and BPPV are negative prognostic indicators of auditory function in SSNHL. Concurrent CP and/or BPPV in SSNHL suggest combined damage to the vestibule and may indicate severe and widespread labyrinthine damage, leading to a poor prognosis.

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Bilateral, isolated, lateral semicircular canal malformation without hearing loss

The Journal of Laryngology & Otology ◽

10.1017/s0022215108002740 ◽

2008 ◽

Vol 122 (8) ◽

pp. 858-860 ◽

Cited By ~ 8

Author(s):

I Dallan ◽

S Berrettini ◽

E Neri ◽

A P Casani

Keyword(s):

Hearing Loss ◽

Inner Ear ◽

Semicircular Canal ◽

Imaging Techniques ◽

Three Dimensional ◽

Lateral Semicircular Canal ◽

Normal Limits ◽

Dimensional Reconstruction ◽

Ear Malformations ◽

Molecular Aspects

AbstractHypothesis:Inner-ear malformations are frequently found in patients with sensorineural hearing loss. However, isolated anomalies of the vestibular part of the inner ear are seldom described, and for this reason their impact on balance is poorly understood.Care report:We present the case of a 38-year-old Caucasian man with recurrent vestibular complaints, with a sensation of linear tilting, but no hearing impairment. Clinical and neuro-otological examinations showed peripheral involvement of the vestibular system, while audiological investigation was within normal limits. High-resolution magnetic resonance imaging of the inner ear, with three-dimensional reconstruction, demonstrated isolated vestibular anomalies involving both the lateral semicircular canal and the utricle.Conclusions:Bearing in mind this case, we speculate that isolated vestibular malformation may not be as rare as previously thought, and should be investigated with the aid of sophisticated imaging techniques. A review of the relative literature, focussing attention on the molecular aspects, is also reported.

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Congenital Anomalies of the Inner Ear

Annals of Otology Rhinology & Laryngology ◽

10.1177/00034894840930s419 ◽

1984 ◽

Vol 93 (4_suppl) ◽

pp. 110-118 ◽

Cited By ~ 38

Author(s):

Isamu Sando ◽

Tetsuo Takahara ◽

Akira Ogawa

Keyword(s):

Inner Ear ◽

Semicircular Canal ◽

Congenital Anomalies ◽

Vertical Section ◽

Lateral Semicircular Canal ◽

Horizontal Section ◽

Cochlear Aqueduct ◽

Hematoxylin And Eosin ◽

Ear Anomalies ◽

Temporal Bones

This study investigated congenital anomalies occurring in the inner ear, with particular attention to their features, localizations, and frequencies. Seventy-seven human temporal bones obtained from 53 individuals, aged one day to 39 years, each of whom had anomalies of the external ear, middle ear, and/or inner ear, were used for this study. The temporal bones had been removed at autopsy, fixed, dehydrated, embedded in celloidin, and sectioned horizontally or vertically at 20 μm. Every tenth horizontal section or every 20th vertical section was stained with hematoxylin and eosin, mounted, and studied under a light microscope. There were 206 inner ear anomalies (117 in the vestibular system, 79 in the cochlea, 10 in the internal auditory meatus) in the 51 bones studied. Fifty-four different features of anomalies were present at 33 different locations in the inner ear. The most frequent anomalies observed were shortened cochlea (23 bones), enlarged vestibule (17 bones), wide cochlear aqueduct (16 bones), absence of lateral semicircular canal (14 bones), and large osseous lateral semicircular canal (10 bones). The most common site of inner ear anomalies was thus the lateral semicircular canal, which was involved in 46 of the 206 anomalies observed; the anomalies in this organ were variable in type. The implications of inner ear anomalies are discussed as they relate to fetal development, inner ear dysfunction, and clinical interpretation of polytomographic studies.

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Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes

Ear Nose & Throat Journal ◽

10.1177/0145561321996839 ◽

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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Role of Computed Tomography and Magnetic Resonance Imaging in detecting the Prevalence of inner ear anomalies among cochlear implant candidates

QJM ◽

10.1093/qjmed/hcab106.007 ◽

2021 ◽

Vol 114 (Supplement_1) ◽

Author(s):

Alaa Nasser Hussain Zaher ◽

Tougan Taha Abd El Aziz ◽

Ahmed Samy Abdelrahman

Keyword(s):

Magnetic Resonance Imaging ◽

Computed Tomography ◽

Hearing Loss ◽

Cochlear Implant ◽

Inner Ear ◽

Resonance Imaging ◽

High Resolution Computed Tomography ◽

Vestibular Aqueduct ◽

Incomplete Partition ◽

Ear Anomalies

Abstract Background Hearing loss management using cochlear implants in patients with inner ear anomalies has long been discussed in the otology community. Magnetic resonances imaging (B,/IRI) and Computed tomography (CT) play important roles in the preoperative assessment of inner ear abnormalities such as cochlear nerve deficiency and variant anatomy as these abnormalities may not only affect the decision of the implantation procedure or the patient's prognosis regarding auditory improvement, but also the risk of complications. Objective To examine the prevalence of inner ear anomalies among cochlear implant recipients in patients with congenital sensorineural hearing loss among the pediatric age group in the Demerdash hospital, Ain Shams university using High resolution computed tomography (HRCT) and MRI imaging. Methods A retrospective descriptive study over the course of 9 months that included all patients that are candidates for cochlear implant referred to the Radiology department, Ain Shams University Hospitals for a preoperative imaging in the form of CT and VIRI scans. Results CT and MRI scans of 33 patients who had congenital hearing loss and were candidates for cochlear implantation with total 66 ears were reviewed. Inner ear anomalies were identified in 8 patients representing a prevalence (24.2%) with 14 ear diseased. Anomalies were seen bilaterally in 6 patients and unilaterally in 2 patients. Among the 14 diseased ear, 9 ears (64.3%) were seen with incomplete partition Il, 7 ears (50%) were seen with enlarged vestibular aqueduct, 4 ears (28.6%) were seen with cochlear hypoplasia, 3 ears (21.4%) were seen with semicircular canal aplasia, 2 ears (14.3%) were seen with incomplete partition type I, 2 ears (14.3%) were seen with cochlear nerve aplasia, 2 ears with cochlear aplasia (14.3%), I ear (7.1%) was seen with common cavity ear (7.1%) with complete labyrinthine aplasia. Conclusion Prevalence of inner ear anomalies among cochlear implant candidates was 24.2%. This result is consistent with results worldwide and the most common anomalies were Incomplete partition Il and large vestibular aqueduct. Abbreviations Computed tomography (CT), Magnetic resonance imaging (MRI), High resolution computed tomography (HRCT), Internal auditory canal (IAC), Cerebellopontine angle (CPA).

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Prognosis of Inner Ear Periphery and Central Vestibular Plasticity in Sudden Deafness with Vertigo

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348940511401008 ◽

2005 ◽

Vol 114 (10) ◽

pp. 786-791 ◽

Cited By ~ 12

Author(s):

Tadashi Kitahara ◽

Noriaki Takeda ◽

Suetaka Nishiike ◽

Shin-Ichi Okumura ◽

Takeshi Kubo

Keyword(s):

Everyday Life ◽

Inner Ear ◽

Pure Tone ◽

Vestibular Function ◽

The Other ◽

Sudden Deafness ◽

Vestibular Neurectomy ◽

Canal Paresis ◽

Clinical Problems ◽

Caloric Tests

Objectives: We sought to elucidate the clinical problems and otopathology of patients with sudden deafness with vertigo (SDV). Methods: In 24 patients with SDV who had significant canal paresis (CP) at their first visit to our hospital between 1997 and 2001, we examined pure tone audiograms, caloric tests, and several questionnaires twice, at the first visit within 5 days after the onset and around 2 years after steroid therapy. Results: These examinations revealed that improvements of auditory and vestibular function in patients with SDV tended to be correlated with one another. Sixteen of the 24 patients (66.7%) still had CP. This rate in SDV was significantly worse than that reported previously for vestibular neuritis (VN). On the other hand, patients with SDV with long-lasting CP had a faster reduction of head-shaking afternystagmus and of handicaps in their everyday life due to dizziness than did patients with VN and CP. Conclusions: These findings suggest that SDV may deteriorate the inner ear function more severely but accelerate the central vestibular compensation more effectively than VN after the lesion. It is well known that vestibular neurectomy causes much more severe motion-induced dizziness after surgery than does labyrinthectomy. Taken together, these findings suggest different regions of damage in SDV (mainly the labyrinth, as in labyrinthectomy) and VN (mainly the ganglion, as in vestibular neurectomy).

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