Restudy Of Malformations Of The Internal Auditory Meatus, Cochlear ...

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Abstract

The present study aims to restudy the correlation between the internal auditory meatus (IAM), the cochlear nerve canal (CNC), the cochlear nerve (CN) and inner ear malformations. In this retrospective study design, the abnormal diameter of the IAM, CNC and CN in patients with any kind of inner ear malformations was evaluated using multi-slice spiral computed tomography (MSCT) (37 patients) and magnetic resonance imaging (MRI) (18 patients). Of 37 MSCT-diagnosed patients, 2 had IAM atresia, 11 IAM stenosis, 22 enlarged IAM, and 2 normal IAM with an abnormal CN. MRI diagnoses of 18 patients revealed 8 cases of aplastic CN, 6 hypoplastic CN, and 4 normal CN. CNC stenosis was associated with CN hypoplasia (P < 0.001). Patients with absent or stenotic IAM had less CN development than those with normal or enlarged IAM (P = 0.001). We propose a modification of the existing classification systems with a view to distinguishing malformations of the IAM, CNC and CN.

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Figures

Fig. 1

Fig. 1

Stenosis of bilateral IAMs with…

Fig. 1

Stenosis of bilateral IAMs with an undetected CN. Male, 4 years old. Total…

Fig. 1 Stenosis of bilateral IAMs with an undetected CN. Male, 4 years old. Total hearing loss in the right ear, moderately severe hearing loss in the left ear. a, b, d CT shows bilateral stenosis of IAM, normal development of the cochlea (arrow). The diameters of the CNC are 0.9 and 0.6 mm. c, e Undetected CN with oblique sagittal reconstruction of MRI
Fig. 2

Fig. 2

Enlargement of bilateral IAMs with…

Fig. 2

Enlargement of bilateral IAMs with CN hypoplasia. Male, 6 years old. Bilateral severe…

Fig. 2 Enlargement of bilateral IAMs with CN hypoplasia. Male, 6 years old. Bilateral severe hearing loss. a, b Enlargement of the IAM, binaural enlarged vestibular aqueducts. The CNC on the right side is 1.9 mm (a), whereas 1.8 mm on the left side (b). c, d Oblique sagittal reconstruction of MRI imaging shows normal development of the right CN, FN and vestibular nerve in the IAM (c); the diameter of the left CN is smaller than that of the FN, suggesting CN hypoplasia (d)
Fig. 3

Fig. 3

Enlargement of the IAM, with…

Fig. 3

Enlargement of the IAM, with normal CN. Female, 2 years old. Bilateral severe…

Fig. 3 Enlargement of the IAM, with normal CN. Female, 2 years old. Bilateral severe hearing loss. a, b Axial CT shows an enlarged IAM. The CNC on the right side is 2.1 mm (a); 2.2 mm on the left side (b). c, d Oblique sagittal reconstruction of an MRI image shows normal development of the facial nerve in the IAM, the vestibular nerve, and the CN
Fig. 4

Fig. 4

Normal IAM with an aplastic…

Fig. 4

Normal IAM with an aplastic CN. Female, 2 years old. The right ear…

Fig. 4 Normal IAM with an aplastic CN. Female, 2 years old. The right ear has total hearing loss and the left ear has normal hearing. a Axial CT shows a normal IAM. The diameter of the CNC is 0.8 mm (arrow). b Oblique sagittal reconstruction of an MRI shows the FN and vestibular nerve in the IAM. The CN is absent
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References

    1. Mehl AL, Thomson V. The Colorado newborn hearing screening project, 1992–1999: on the threshold of effective population-based universal newborn hearing screening. Pediatrics. 2002;109:E7. doi: 10.1542/peds.109.1.e7. - DOI - PubMed
    1. Marazita ML, Ploughman LM, Rawlings B, Remington E, Arnos KS, Nance WE. Genetic epidemiological studies of early-onset deafness in the US school-age population. Am J Med Genet. 1993;46:486–491. doi: 10.1002/ajmg.1320460504. - DOI - PubMed
    1. Scheich H, Baumgart F, Gaschler-Markefski B, et al. Functional magnetic resonance imaging of a human auditory cortex area involved in foreground-background decomposition. Eur J Neurosci. 1998;10:803–809. doi: 10.1046/j.1460-9568.1998.00086.x. - DOI - PubMed
    1. Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope. 1987;97:2–14. doi: 10.1002/lary.5540971301. - DOI - PubMed
    1. Sennaroglu L, Saatci I. A new classification for cochleovestibular malformations. Laryngoscope. 2002;112:2230–2241. doi: 10.1097/00005537-200212000-00019. - DOI - PubMed
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