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http://ijdi.sciedupress.com International Journal of Diagnostic Imaging, 2016, Vol. 3, No.

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ORIGINAL ARTICLES

Temporal bone congenital anomalies and variatons
Aysegul Sarsılmaz1, Canan Altay2, Nezahat Erdogan3, Engin Uluc3, Erdem Eren4

1. Yeditepe University Medical School, Department of Radiology, Istanbul, Turkiye. 2. Dokuz Eylul University Medical
School, Department of Radiology, Izmir, Turkiye. 3. Izmir Katip Celebi University, Ataturk Training and Research Hospital,
Department of Radiology, Izmir, Turkiye. 4. Izmir Katip Celebi University, Ataturk Training and Research Hospital,
Department of Ear Nose Throat, Izmir, Turkiye

Correspondence: Aysegul Sarsılmaz. Address: Yeditepe Medical School Hospital, Kozyatagı Istanbul, Turkey.
Email: aysegulsar@hotmail.com

Received: January 6, 2016 Accepted: April 14, 2016 Online Published: April 20, 2016
DOI: 10.5430/ijdi.v3n2p11 URL: http://dx.doi.org/10.5430/ijdi.v3n2p11

Abstract
Background: We aimed to review the imaging findings of the most common congenital anomalies of the temporal bone
and variations of the anatomic landmarks.
Material and methods: We retrospectively reviewed the 1,894 temporal bone computed tomography (CT) and 986
temporal bone magnetic resonance imaging (MRI) with regard to congenital anomaly and accompanying pathologies.
Thin-section CT and thin-section T2-weighted gradient echo or fast spin-echo MRI was performed to identify
malformations. The cases were admitted to our hospital with hearing loss, tinnitus, vertigo and non-specific infection
complaints.
Results: We detected 22 cases with a temporal anomaly among 1,894 temporal CT and 986 temporal MRI images. We
discussed the most common congenital anomalies of the temporal bone with imaging findings of our cases.
Conclusion: This clinical/imaging review illustrates and reviews congenital abnormalities of the temporal bone: external,
middle, and inner ear anomalies, vascular malformations, and variations in high-resolution CT and MR images.

Keywords
Temporal bone, Congenital, Anomaly, Computed tomography, Magnetic resonance imaging

1 Introduction
Anatomic variations and anomalies of the temporal bone include abnormal dimension, contour and abnormal orientation
of the structures. Functional impairment is the crucial distinction between anomaly and variations. In the literature, the
largest temporal bone anomaly series is that reported by Valvassori et al. [1]. In that series, approximately 60% of temporal
congenital anomalies demonstrated deformities of the external auditory canal, middle ear, or both structures [1]. Inner ear
abnormalities accounted for 30% of the congenital defects, and the remaining 10% displayed mixed defects of the external,
middle, and inner ear [1, 2]. We presented temporal anomalies and variations that we determined 22 cases among 2,880
patient’s computed tomography (CT) and magnetic resonance imaging (MRI).

Published by Sciedu Press 11

7%) pattients were detected with tem mporal bone anoomaly/variationn. vestibular annd semicircular canal aanomalies/variiations are mosst common typee (see Figures 7-9). slice th hickness: 1. 14). 2 2 Mater rial and method d WWe retrospectiively reviewed 2. Axial CT im mage shows thin.com Internatioonal Journal of D Diagnostic Imagiing. had d vestibular and semicircular canal anomalyy or variation.894) patients had h high resoluution temporal CT with 1 mm m thick sectionss. 43-yyear-old patient with w fibrous septta in the right extern nal auditory can nal. And there were 2 patiennts with juguleer vein aanomaly/variattion (see Figurres 13.0/-0 weighted spin eccho ( TR: 600. obliqu ue. We didnn’t consider thee previous diaggnosis of the paatients. oone patient intternal acoustic canal variatio Six patients haad middle ear oossicle on. TE:: 80.5 T system.sciedu upress. Also. sagittal refformated imagees obtained. sim multanously. Vol. 0. S aanomalies alonne or accompan nying with other anomalies (ssee Figures 3-66). matrix: 384 × 320. TTE: 1. Ninee of the patientts had administtered to the hosspital with verttigo complaintt. Nine N thousand aand eighty sixx (986) patientss had temporall MRI. Only one patient’s conggenital ccholesteatoma was accompan nying to other anomalies (seee Figure 2). MR RI sequences are a axial and cooronal (reform mation with 3D D maximum inntensity pprojection [MIIP] reconstructtions from axiaal images) 3D FIESTA F (fast iimaging enablling steady-statte acquisition. TR: 6. slice thickkness: 1). We reviewed anomalies and d clinical complaints.5-year 1 period d. axial T1 w slice thicknesss: 1). 0. The averagee age of tthe patients waas 27 year-old (range: 12-51). EEight patients presented p with h mild hearing loss. Three of the patients p were ppediatric groupp. 33. TE: 20.5. M Most of the paatients (fourteen n patients). Figure 1. 3 Results TTwelve femalee. FFour patients had external acoustic a canal (EAC) or aurricular anomally (see Figure 1). there w were not associaated or aanother congen nital anomaly in i all age group ps. 7 We detectted three patiennts who had.880 patientss who performeed temporal boone imaging (coomputed tomography and maagnetic rresonance imaaging) over a 1. In our groupp. l 5 patients had auricular aand external accoustic canal annomalies and cclinical ddiagnosis had previously p beeen known. No..hhttp://ijdi. Onne patient had middle ear andd also EAC anoomaly. We detectedd 22 cases withh a temporal bbone anomaly and variation among 22. OOne thousand eight hundred ninety four (1. ccoronal.880 patients. Also. NEX:: 6). one patient had facial nerrve variation. innternal aacoustic canall and facial neerve anomaly/v variation (see Figures 10-122). so oft tissue bridg ge at right exterrnal auditory canal localization 12 ISSN 2331--5857 E-ISSN 2331-5865 . coronal T2 T weighted turrbo spin echo (TR: 3000.7 FOV: 16 × 16. aall anomaly caases had been in ncluded. 10 male (totaal 22. 2016. MMRI was perfformed on 1.

(a-d): consecutive coronal. deformed appeereance and hyppoplasia of the m malleus and incuus (b-f. arrows). Left ear strructures are norm mal Published byy Sciedu Press 13 . The T left externaal acoustic canall and auricle is normal. Microtia and atresia of thhe external audiitory canal (EAC) (a-ff. (ee-f): axial CT im mages.sciedupress. Leeft congenital cholesteatooma at prussak space. note. arrows) and small middle earr cavity. accompanying middle eare ossicle anomaalies to EAC atrresia. V Vol. stapes aplasia and incuus and malleus hypoplasiaa (arrows) and small middle ear cavity. 17-year-old malle. Also.http://ijdi. 15-year-olld female with right minor microtia with narrow n and shorrt right external auditory canal (arrows). 2016. s lateral to the ossicular ch hain(arrow). 2 Figure 2. aplassia of the stapes. c: On th he right.com Internnational Journal of Diagnostic Im maging. a: Coronal and b: Axial CT imaages. 3. d: with normaal left ossicular chain(arrow) c butt hypoplasia of the t mastoid air cells Figure 3. dehisscence of the tegm men tympani and d hypoplasia of the t mastoid air cells at rig ght side. No.

(c-d): Congeenital absence of o the oval winddow and the stappes on CT scann. 12-year-old femalee with stapes an nd incus long cru us aplasia. Vol.hhttp://ijdi. Thee lateral semicirccular canal is norrmal. Arrow sh hows cochlear baasal turn and the level of the stap pes is expected to arise 14 ISSN 2331--5857 E-ISSN 2331-5865 . The oval winddow is completely oblliterated with bone (arrows). No.sciedu upress. 2 Figure 4. stapes and d long crus of inccus are undiffereentiated at left sid de (b-c). (a-b)): Incus. Axial images show. in ncudomalleolar joint j is normal (a) ( but.com Internatioonal Journal of D Diagnostic Imagiing. The stapess is agenetic. Axial CT images in a 14-year-old boy with con nductive type heearing loss on right side. malleuus and incudomalleolaar joint is norm mal. 2016. The facial nerve is aberrannt passed in the m middle ear. Note that the nervee is medial in loccation Figure 5. 33.

sciedupress.com Internnational Journal of Diagnostic Im maging. Axial CT scan s image in n a 26-year-oldd patient with bilateral b hypoplasia of the laateral semicircu ular canal.http://ijdi. Note leeft inner ear iss normal Published byy Sciedu Press 15 . Also note cochlea c is normaal Figure 9. The T incudomalleeolar joint is absent. 19-year-old giirl with commo on cavity on right side (arrrows). Latteral semicirculaar canals are seeen petite (arrow ws). V Vol. the second d portion of the ffacial nerve show ws that the free passage of the m middle ear (arrow) Figure 7. Axiaal CT scan shows righht external audito ory canal bone aplasia a The volume of thhe middle ear is decreased with hypoplasia of auricuula (arrow). 2 Figure 6. 3. No. b: T (arrows). There arre observed insidentally right mastoiditiss (markings) and left chro onic sphenoid sinusitis (markings) in this imagee Figure 8. a: 12-year-old girl g with profoun nd right sided congenital c conduuctive type heariing loss (Major microtia). Axial 3D FIESTA images i of the right ear show ws cochlea and a the vestibu ule can not be b distinguish hed from each other. Axial A CT images (a-b) of right in nner ear shows saccular s enlargemment of vestibulle and no separattion of the lateraal semicirculaar canal with thee vestibule (arrow ws). 2016.

Vol. c. 33. without any cliinical finding.com Internatioonal Journal of D Diagnostic Imagiing. Also there wwas not inner. Observeed in all the nerrves as normally on thhe left side (F-ffacial nerve.) 16 ISSN 2331--5857 E-ISSN 2331-5865 . a and b. 2016. 19--year-old femalee. d) with norm mal internal acusstic canal and faacial nerve (marrking and arrow: a. Coronal C T2-weig ghted three-dimeensional gradiennt echo sequencee shows abscencce of the right ccohlear nerve. IV--inferior vestibuular nerve. b).hhttp://ijdi. Images shows cohlear hypoplasia (arrows: b. There are a seen normal facial nerve (F) and conjoint vestibuler v nerve (V) on right siide (e). 2 Figure 10. consecutiive axial T2-weiighted FIESTA iimages. C-cohlear nerve: f). (*MR images were ob btained at the lev vel of internal accoustic canal.. SV. a-dd: Sequential axial CT images in a 17-year-old woman with seensorineural hearring loss on righht side. b: Furtheer MR imaging of thee same patient. m middle and externnal ear anomalies Figure 11. No. bilaterall small and short intern nal acoustic canal (arrows). craniaal nerves are normmal.sciedu upress.superior vestib bular nerve. VII and VIII.

sciedupress. The labyrinthic l and ty ympanic segmennts of the normall facial nerve andd cohlea show “eeyes of the snake” apppearance at coronnal CT images. Axial CT imagee. the labyrinthic ssegment of the facial nerve is small according a to norrmal side (arrow w).http://ijdi. a: The labyrinth hic and tympanicc segments of thee facial nerve (bllack arrows) havve normal appearrance at coronal C CT image. No. the jugular div verticulum (blacck arrows) is seen asa smooth outtpouching componentt of the jugular bulb that extends sup periorly and possteriorly Published byy Sciedu Press 17 . Also. V Vol.. b: b The tympanic segment of the fa facial nerve is hyypoplastic.. cohleea is normal (wh hite arrow). 2 Figure 12. Also. Also. therre is hypoplasiia of the mastoid ceellulares and middle m ear anomalies Figure 14. The accomppanying pathologgies of the midddle ear and ossiicles were detected att other images Figure 13. Coronal CT im mage. the high jugular buulb (arrow) is seen that positioned higher from the floor of the tympaanic cavity at left side.com Internnational Journal of Diagnostic Im maging. 2016. 3.

12. hypoplasia/aplasia of the internal carotid artery. or a duplication anomaly (cyst. 17-19]. No. fusion of the short process to the lateral semicircular canal. In microtia. We didn't determine Mondini dysplasia among our patients. hypoplasia or fetal form. Three types of vestibulocochlear nerve hypoplasia and aplasia can be distinguished. dilatation of the vestibular aqueduct may be present. Vol. 2 4 Discussion Microtia is characterized by a small.http://ijdi. 6]. and footplate fixation [2. This malformation was first described by Mondini [2. or fistula). as follows: type 1 displays stenotic IAC and VCN aplasia. The malformed canals may be short.com International Journal of Diagnostic Imaging. 12. which is usually bilateral. facial nerve hypoplasia may be seen with middle ear anomalies. Hypoplasia or aplasia of the vestibulocochlear nerves and facial nerve can be identified by MRI. Incus anomalies include aplasia. absence of the head and crura. The internal acoustic canal (IAC) may also be atretic or may have a bony septum. The bony margins of the stenotic/atretic canal can be demonstrated by CT. Additionally. 6-8]. a condition known as lateral semicircular duct-vestibule dysplasia or common utriculosaccular-lateral semicircular duct cavity [2. 11-13]. Common cavity anomaly is seen as a large cystic cavity with no inner ear structure. and isolated fixation of the incus to th scutum [2. and an anomalous course of the facial nerve may be observed [2. abnormally shaped auricle. a dilated vestibule may form a common lumen. type 2A involves a common VCN with aplasia/hypoplasia of the cochlear branch and labyrinthine malformation. Middle ear ossicular deformities can be associated with EAC malformations. isolated congenital cochlear nerve hypoplasia has been reported [2. Also. Stapes footplate fixation is the most common isolated congenital ossicular anomaly. often a small tissue tag and a small pit are present in place of the normal auricle and external canal [2-5]. mandibular condyle dysplasia. 12. there may be a soft-tissue plug or a bony plate localized at the tympanic membrane (TM). 7-12]. Incomplete partition and dilatational defects (including Mondini’s dysplasia) of the inner ear. The semicircular canals may be normal or malformed [2]. The incidence of primary and secondary cholesteatomas or epidermoids increases in EAC malformations [2. 18 ISSN 2331-5857 E-ISSN 2331-5865 . also referred to as a bifid facial nerve. Stapes anomalies include aplasia. is another anomaly. absent or fibrous union of the incudostapedial joint. In severe malformations. Additionally. Additionally. EAC malformations may be observed as atresia. aplasia of the oval and round windows. shortening or malformation of the long process. or narrow [2]. sinus. 3]. 3. The most common inner ear anomaly is malformation of the lateral semicircular canal. stenosis.sciedupress. blocked or absent external ear canal. 19]. It is usually accompanied by a narrow. 12-17]. Aplasia or hypoplasia of the tympanic part or mastoid process of the temporal bone. 18]. fusion of the head to the promontory. rotation or elevation of the carotid canal. the air content of the middle ear may be reduced [2. The bony deformity may compress the vestibulocochlear nerves (VCNs) [2. type 2B shows normal labyrinthine structures [2. There is a small cochlea with incomplete partitioning of the interscalar septum. The interscalar septal defects and absence of the osseous spiral lamina can be visualized on MRI. 2016. 6 . wide. 18. Additionally. Duplication of the facial nerve. The vestibule and semicircular canals may be normal or malformed. 6]. Stapes and incus are the most frequently malformed or absent ossicles.

doi. 2005. 1109-1172. et al. Nicholas BD. PMid:25132855 http://dx. Radiological assessment of the Indian children with congenital sensorineural hearing loss. Parmar HA.2005. Neuroradiology. A high lateral jugular bulb may obstruct the round window.1016/j. is called a high jugular bulb [2]. 2009.doi.org/10. Mosby. 3. Kirtane MV. as well as vascular and nerve anomalies.org/10. PMid:19775757 http://dx. [2] Som PM.2010. 2011. Pictorial review of MRI/CT scan in congenital temporal bone anomalies. 84: 859-863. 2016. Eyibilen A.1016/j. The British Journal of Radiology. Int J Pediatr Otorhinolaryngol. 20]. 2009. Tang PH.4103/0971-3026.12.org/10. Unilateral isolated incus agenesis: detection by virtual endoscopy. 30: 774-80.A1426 Published by Sciedu Press 19 . 53: 49-61. 499-504. Cochlear abnormalities associated with enlarged vestibular aqueduct anomaly.org/10. Neuburger J. Int J Pediatr Otorhinolaryngol. References [1] Mafee MF. Mirror ear: a reconstructive technique for substantial tragal anomalies or polyotia. J Larynol Otol. PMid:21849370 http://dx. Hedlund G. et al. et al. Karmody CS.doi.0b013e318149dda7 [9] Clary MS. Oguz KK. J Plast Reconstr Aesthet Surg.doi. 2003.http://ijdi. 19: 99-106. No. New York.nic. 19].1001/archoto. Walker R. 41-68.org/10.1258/002221503321046612 [4] Rankin JS. 2011.1016/j. Becker M.08.doi. Head and neck imaging.026 [6] Mukerji SS. Valvassori GE. 2 Anterior migration of the facial nerve has also been described with cochlear malformations. Indian J Radiol Imaging. 18. 4 Conclusion Recognizing congenital abnormalities of the temporal bone guides management of these conditions by clinicians.ijporl. Appearance of hypoplastic cochleae in CT and MRI: a new subclassification. Bvahatti SV. middle. Imaging of the head and neck.doi.bjps.org/10. PMid:16749196 http://dx. Curtin HD.doi. Ghuman SS. PMid:20837365 http://dx. Complete labyrinthine aplasia. Am J Neuroradiol. Arch Otolaryngol Head Neck Surg. 73: 1682-5.ijporl. Myers SR. External auditory canal duplication anomalies associated with congenital aural atresia. PMid:20959971 http://dx.org/10.org/10. http://dx.2009. Cutis. 2010. Atas A. Tan TY.1016/j. 74: 1338-9. PMid:21916271 [3] Blevins NH. clinical and radiologic findings with review of the literature. We aimed to review and illustrate anomalies of the temporal bone and outer.doi. Gunasekaran S. in patients for cochlear implant. Maheshwari SS. Identification of the position of the facial nerve is a crucial part of preoperative planning [2.1007/s00234-010-0777-3 [15] Ozgen B. Conflict of interest disclosure The authors declare no conflicts of interest. A relationship exists between pneumatization of the mastoid air cells with a high jugular bulb [2. PMid:21502480 http://dx. 2014: 808759. 137: 398-400. Review of congenital inner ear abnormalities on CT temporal bone. Goetz F.028 [14] Giesemann AM. Schwartz RA.org/10. Gault D. Accessory tragus in the middle ear: A rare congenital anomaly. 2009. J Comput Assist Tomogr. 2011.org/10.09.com International Journal of Diagnostic Imaging. [5] Gore SM. Neuroimag Clin N Am. 20].008 [11] Yiin RSZ. 2008. when positioned higher from the floor of the tympanic cavity. 2 nd ed.sciedupress. Thieme. 32: 660-1.2011. Ibrahim M.org/10. et al. et al.3174/ajnr.005 [7] Agarwal SK.05. A jugular diverticulum (JD) is a very rare anomaly characterized by smooth outpouching of the jugular bulb that extends superiorly. 88: 62-4. et al. 2011. PMid:18664858 http://dx.1097/RCT. PMid:21807314 http://dx.org/10. Singh S. 4 th ed. Vol.08. et al.doi. Accessory tragus: a possible sign of Goldenhar syndrome. medially.1155/2014/808759 [8] Sarikaya B.50825 [13] Atkin JS. 2003. 21: 603-619. and inner ear structures. 117: 32-8. Int J Otolaryngol. Arachnoid cyst: Middle ear mass diagnosis to consider. St Louis. 2014. or posteriorly [2. PMid:19881062 http://dx. Congenital malformations of the temporal bone.doi. 2006: 59. Grimmer JF.doi.2011. Frewer J. PMid:19147720 http://dx. Aladag I. with or without hearing loss on high-resolution CT and MR images.31 [10] Chintalapati K. et al.1259/bjr/18998800 [12] Gupta SS. A high medial jugular bulb may compress the distal portion of the vestibular aqueduct.doi. The dome of the jugular bulb. 2011.

Nystagmus in enlarged vestibular aqueduct: A Case Series. 24: 1334-7. et al. et al. Hoesli RC. 2015. The relationship between juguler bulb-vestibular aquaduct dehiscence and hearing loss in pediatric patients. 2016. Am J Neuroradiol. 2 [16] White J. Anteverted internal auditory canal as an inner ear anomaly in patients with craniofacial microsomia.ijporl. 3. PMid:25389321 http://dx. Fischbein NJ.org/10.org/10.1177/0194599814555837 [19] L'Heureux-Lebeau B.1177/0194599811430045 20 ISSN 2331-5857 E-ISSN 2331-5865 .doi.004 [20] Kupfer RA. Congenital bifurcation of the intratemporal facial nerve.org/10. Saliba I. Green GE.1016/j. Systemic review.doi. Diagnostic yield of MRI for pediatric hearing loss. 78:1551-3.120 [17] Glastonbury CM.sciedupress. PMid:26557362 http://dx. Otolaryngol Head Neck Surg. Int J Pediatr Otorhinolaryngol. 2003. No.doi.06. 152: 5-22. Chen JX. Audiol Res.2015. 2012. PMid:22114311 http://dx.4081/audiores.org/10. Otolaryngol Head Neck Surg. Gilani S. 146: 473-7.http://ijdi. Harnsberger HR. et al.doi.2014.com International Journal of Diagnostic Imaging. PMid:12917123 [18] Kachniarz B. PMid:25002156 http://dx. Krakovitz P. 2014. Vol. 2015. 5: 120.