Fehlbildungen des Innenohrs – Diagnostik und Einteilung in CT und MRT

Zusammenfassung

In diesem Artikel werden die Fehlbildungen des Innenohrs primär nach dem Vorhandensein der Kochlea und der Art der kochleären Fehlbildung eingeteilt. Diese Einteilung ist für die klinischen Belange sinnvoll, da eine Hauptindikation der CT- und MRT-Untersuchung des Felsenbeins bei Kindern das Cochlear Implant ist und für dieses in erster Linie die Kochlea und die Existenz des N. cochlearis relevant sind. Dem Radiologen gibt dies ein Schema vor, mit dem er systematisch zur Diagnose geführt wird. Unsere Klassifikation verwendet die Begrifflichkeit bereits früher klar definierter Fehlbildungen. Gebräuchliche, aber oft missverständlich verwendete Eponyme werden abschließend ebenfalls erklärt und eingeordnet.

Abstract

Malformations of the inner ear are presented and categorized primarily by the existence and the appearance of the cochlea, including the existence of a modiolus and the cochlear aperture. This classification is useful for clinical purposes as cochlea implantation constitutes as a main indication for CT and MRI examinations of the temporal bone in children. Therefore the cochlea and the existence of a cochlear nerve are of main relevance. The radiologist is given a table that systematically leads to diagnosis. This novel classification uses the terms of earlier defined malformations. Common, but often unclear terms are finally described and clarified.

Keywords

Malformations of the inner ear - cochlea - cochlea implantation - cochlear nerve

• Literatur

• 1 Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: a classification based on embryogenesis. The Laryngoscope 1987; 97: 2-14
• 2 Sennaroglu L, Saatci I. A new classification for cochleovestibular malformations. The Laryngoscope 2002; 112: 2230-2241
• 3 Joshi VM, Navlekar SK, Kishore GR et al. CT and MR imaging of the inner ear and brain in children with congenital sensorineural hearing loss. Radiographics 2012; 32: 683-698
• 4 Papsin BC. Cochlear implantation in children with anomalous cochleovestibular anatomy. The Laryngoscope 2005; 115: 1-26 [pii]
• 5 Marsot-Dupuch K, Dominguez-Brito A, Ghasli K et al. CT and MR findings of Michel anomaly: inner ear aplasia. AJNR 1999; 20: 281-284
• 6 Giesemann AM, Goetz F, Neuburger J et al. From labyrinthine aplasia to otocyst deformity. Neuroradiology 2010; 52: 147-154
• 7 Michel P. Memoire sur les anomalies congenitales de poreille intern. Gazette Med de Strasbourg 1863; 23: 55-58
• 8 Giesemann AM, Kontorinis G, Jan Z et al. The vestibulocochlear nerve: aplasia and hypoplasia in combination with inner ear malformations. Eur Radiol 2012; 22: 519-524
• 9 Purcell D, Johnson J, Fischbein N et al. Establishment of normative cochlear and vestibular measurements to aid in the diagnosis of inner ear malformations. Otolaryngol Head Neck Surg 2003; 128: 78-87
• 10 Giesemann AM, Goetz F, Neuburger J et al. Appearance of hypoplastic cochleae in CT and MRI: a new subclassification. Neuroradiology 2011; 53: 49-61
• 11 Morimoto AK, Wiggins 3rd RH, Hudgins PA et al. Absent semicircular canals in CHARGE syndrome: radiologic spectrum of findings. AJNR 2006; 27: 1663-1671
• 12 Satar B, Mukherji SK, Telian SA. Congenital aplasia of the semicircular canals. Otol Neurotol 2003; 24: 437-446
• 13 Giesemann AM, Goetz GF, Neuburger J et al. Persistent Petrosquamosal Sinus: High Incidence in Cases of Complete Aplasia of the Semicircular Canals. Radiology 2011; 259: 825-833
• 14 Mondini C. Minor works of Carlo Mondini: the anatomical section of a boy born deaf. Am J Otol 1997; 18: 288-293
• 15 Sennaroglu L, Saatci I. Unpartitioned versus incompletely partitioned cochleae: radiologic differentiation. Otol Neurotol 2004; 25: 520-529 ; discussion 529
• 16 Cock E. A contribution to the pathology of congenital deafness. Guys Hosp Rep 1838; 7: 289-307
• 17 Khan AM, Levine SR, Nadol Jr JB . The widely patent cochleovestibular communication of Edward Cock is a distinct inner ear malformation: implications for cochlear implantation. Ann Otol Rhinol Laryngol 2006; 115: 595-606
• 18 Phelps PD, Michaels L. The common cavity congenital deformity of the inner ear. An important precursor of meningitis described in 1838. ORL J Otorhinolaryngol Relat Spec 1995; 57: 228-231
• 19 Kontorinis G, Goetz F, Giourgas A et al. Radiological diagnosis of incomplete partition type I versus type II: significance for cochlear implantation. Eur Radiol 2012; 22: 525-532
• 20 de Kok YJ, van der Maarel SM, Bitner-Glindzicz M et al. Association between X-linked mixed deafness and mutations in the POU domain gene POU3F4. Science 1995; 267: 685-688
• 21 Phelps PD, Reardon W, Pembrey M et al. X-linked deafness, stapes gushers and a distinctive defect of the inner ear. Neuroradiol 1991; 33: 326-330
• 22 Casselman JW, Offeciers EF, De Foer B et al. CT and MR imaging of congential abnormalities of the inner ear and internal auditory canal. Eur J Radiol 2001; 40: 94-104
• 23 Giesemann AM, Neuburger J, Lanfermann H et al. Aberrant course of the intracranial facial nerve in cases of atresia of the internal auditory canal (IAC). Neuroradiology 2011; 53: 681-687
• 24 Lee SY, Cha SH, Jeon MH et al. Narrow duplicated or triplicated internal auditory canal (3 cases and review of literatur): can we regard the separated narrow internal auditory canal as the presence of vestibulocochlear nerve fibers?. J Comput Assist Tomogr 2009; 33: 565-570
• 25 Koesling S, Bootz F. Bildgebung HNO-Heilkunde. Berlin, Heidelberg: Springer; 2010
• 26 Lai CC, Shiao AS. Chronological changes of hearing in pediatric patients with large vestibular aqueduct syndrome. Laryngoscope 2004; 114: 832-838
• 27 Jackler RK, De La Cruz A. The large vestibular aqueduct syndrome. Laryngoscope 1989; 99: 1238-1242 ; discussion 1242-1233
• 28 Koesling S, Rasinski C, Amaya B. Imaging and clinical findings in large endolymphatic duct and sac syndrome. Eur J Radiol 2006; 57: 54-62
• 29 Casselman JW, Offeciers FE, Govaerts PJ et al. Aplasia and hypoplasia of the vestibulocochlear nerve: diagnosis with MR imaging. Radiology 1997; 202: 773-781
• 30 Adunka OF, Roush PA, Teagle HF et al. Internal auditory canal morphology in children with cochlear nerve deficiency. Otol Neurotol 2006; 27: 793-801
• 31 Adunka OF, Jewells V, Buchman CA. Value of computed tomography in the evaluation of children with cochlear nerve deficiency. Otol Neurotol 2007; 28: 597-604
• 32 Lo WW. What is a ‘Mondini’ and what difference does a name make?. AJNR 1999; 20: 1442-1444
• 33 Phelps PD. Mondini and ‘pseudo Mondini’. Clin Otolaryngol Allied Sci 1990; 15: 99-101
• 34 Huang BY, Zdanski C, Castillo M. Pediatric sensorineural hearing loss, part 1: Practical aspects for neuroradiologists. AJNR 2012; 33: 211-217
• 35 Verloes A. Updated diagnostic criteria for CHARGE syndrome: a proposal. Am J Med Genet A 2005; 133A: 306-308
• 36 Propst EJ, Blaser S, Gordon KA et al. Temporal bone findings on computed tomography imaging in branchio-oto-renal syndrome. Laryngoscope 2005; 115: 1855-1862
• 37 Phelps PD, Coffey RA, Trembath RC et al. Radiological malformations of the ear in Pendred syndrome. Clinical radiology 1998; 53: 268-273
• 38 Buchman CA, Copeland BJ, Yu KK et al. Cochlear implantation in children with congenital inner ear malformations. Laryngoscope 2004; 114: 309-316
• 39 Graham JM, Phelps PD, Michaels L. Congenital malformations of the ear and cochlear implantation in children: review and temporal bone report of common cavity. J Laryngol Otol Suppl 2000; 25: 1-14
• 40 Lanson BG, Green JE, Roland Jr JT et al. Cochlear implantation in Children with CHARGE syndrome: therapeutic decisions and outcomes. Laryngoscope 2007; 117: 1260-1266