Dataset Information

Vestibular Imaging and Function in Patients With Inner Ear Malformation Presenting With Profound Hearing Loss.

ABSTRACT:

Objective

Vestibular impairment has been observed in patients with congenital hearing loss, but little is known about the vestibular anatomy and function of those in this group with inner ear malformations. This study aims to investigate the association between vestibulocochlear anatomy and vestibular function test results in children with inner ear malformations.

Study design

Case series with chart review.

Setting

Pediatric patients with inner ear malformations presenting with bilateral profound hearing loss at a tertiary hospital from 1999 to 2017.

Methods

Ears were classified into subgroups based on anatomic abnormalities seen on computed tomography imaging. Cervical vestibular evoked myogenic potential (cVEMP), rotatory chair, and caloric test results were obtained and collated. Descriptive and inferential statistics were calculated.

Results

Of 82 ears, 29.3% had incomplete partition type II malformation, the most common type. The second-most common type was isolated vestibular organ anomaly (20.7%), which is not included in currently accepted categories. Most ears exhibited abnormal vestibular function. Abnormal vestibule volume was associated with a nonreactive cVEMP (P < .001). Radiologically abnormal lateral semicircular canals were associated with abnormal caloric and rotatory chair results (P < .001).

Conclusion

With a relatively large number of cases of isolated vestibular organ anomaly not only in our study but also in previous publications, we suggest that this category be added to the subsets of inner ear malformations. Abnormal vestibule volume was significantly associated with a nonreactive cVEMP finding. The majority of patients with hearing loss secondary to inner ear malformations have abnormal vestibular function test results.

SUBMITTER: Dy AES

PROVIDER: S-EPMC9516417 | biostudies-literature | 2022 Jul-Sep

REPOSITORIES: biostudies-literature

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Publications

Vestibular Imaging and Function in Patients With Inner Ear Malformation Presenting With Profound Hearing Loss.

Dy Alexander Edward S AES Kashio Akinori A Fujimoto Chisato C Kinoshita Makoto M Kikkawa Yayoi S YS Hoshi Yujiro Y Igarashi Kazunori K Uranaka Tsukasa T Iwasaki Shinichi S Yamasoba Tatsuya T

OTO open 20220701 3

<h4>Objective</h4>Vestibular impairment has been observed in patients with congenital hearing loss, but little is known about the vestibular anatomy and function of those in this group with inner ear malformations. This study aims to investigate the association between vestibulocochlear anatomy and vestibular function test results in children with inner ear malformations.<h4>Study design</h4>Case series with chart review.<h4>Setting</h4>Pediatric patients with inner ear malformations presenting ...[more]

PMID: 36187437

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Project description:Although recent advances in magnetic resonance imaging (MRI) techniques have contributed to the detection of tiny lesions in the internal auditory canal (IAC) that may be responsible for sudden sensorineural hearing loss (SSNHL), there have been relatively few studies on the clinical characteristics of intra-labyrinthine hemorrhage (ILH) and labyrinthitis versus those regarding IAC tumors. Our purpose was to investigate the frequency of those IAC lesions on MRI and their clinical characteristics. Initial MRIs of 200 patients with SSNHL (93 men, 107 women; mean age = 48.61 years, range: 18-84 years), as well as detailed clinical histories, audiological examinations, and thyroid function, lipid battery, and serological tests (for viral agents and autoimmune disease), were performed. All patients were hospitalized at the time of diagnosis of SSNHL and were administered the same treatment protocol. Patients were divided into idiopathic and secondary groups according to their MRI results. After discharge, they underwent follow-up audiometry and clinical examination at predetermined intervals (2 weeks, 1, 2, 4, and 6 months, and 1 and 2 years). Propensity score-matching and receiver operating characteristics curves of the initial parameters were used for estimating clinical characteristics. Of the 200 patients, 25 (12.55%) who had abnormal findings suggesting inner ear lesions on MRI were assigned to the secondary SSNHL group; within this group, 10 patients (10/200, 5%) had a tumor invading the IAC, 7 (7/200, 3.5%) had ILH, 6 (6/200, 3%) had labyrinthitis, and 2 (1%) had a structural deformity of the IAC. The secondary group showed significantly poor recovery of hearing function compared with that in the idiopathic group. Patients with ILH or labyrinthitis showed prognoses that were equally poor as those of patients with tumors in the secondary group. Additionally, patients with such lesions showed significant canal paresis on the lesion side at an early stage and a high prevalence of benign paroxysmal positional vertigo (BPPV). In conclusion, the prevalence of non-tumorous lesions on MRI represents common findings and showed a poorer treatment response than that of vestibular Schwannoma in patients with SSNHL. Abnormal canal paresis (cut-off value of 35% on the lesioned side, sensitivity 65.2% and specificity 67%), spontaneous nystagmus directed to the contralesional side, and positional vertigo would be the clinical presentation of SSNHL with IAC lesions, in which the presence of acute prolonged vertigo or positional vertigo compatible with BPPV suggests the possibility of a non-tumorous lesion, such as ILH or a labyrinthitis rather than an IAC tumor.

Dataset Information

Vestibular Imaging and Function in Patients With Inner Ear Malformation Presenting With Profound Hearing Loss.

Objective

Study design

Setting

Methods

Results

Conclusion

Publications

Vestibular Imaging and Function in Patients With Inner Ear Malformation Presenting With Profound Hearing Loss.

Similar Datasets

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