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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 25  |  Issue : 3  |  Page : 162-165

Tympanogenic labyrinthine ossificans as rare sequelae of cholesteatoma


1 Department of Otorhinolaryngology-Head and Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia, Kota Bharu, Kelantan, Malaysia
2 Department of Otorhinolaryngology-Head and Neck Surgery, Hospital Raja Perempuan Zainab II, Kota Bharu, Kelantan, Malaysia

Date of Submission12-Dec-2019
Date of Decision26-Mar-2019
Date of Acceptance19-Jun-2019
Date of Web Publication18-Oct-2019

Correspondence Address:
Dr. Azliana Aziz
of Otorhinolaryngology-Head and Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia, Kota Bharu 16150, Kelantan
Malaysia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/indianjotol.INDIANJOTOL_16_19

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  Abstract 


Labyrinthine ossificans (LO) is the formation of pathological new bone within the membranous labyrinth of the inner ear. Most commonly, it is caused by meningitis, via communication of labyrinth with subarachnoid via the cochlear aqueduct and internal auditory canal. We highlight a case of cholesteatoma with a rare complication of tympanogenic LO that results in unilateral profound mixed hearing loss with concomitant severe vertigo.

Keywords: Cholesteatoma, hearing loss, labyrinthine ossificans, labyrinthitis, vertigo


How to cite this article:
Salehuddin NS, Din NM, Saad MS, Aziz A, Salahuddin Z. Tympanogenic labyrinthine ossificans as rare sequelae of cholesteatoma. Indian J Otol 2019;25:162-5

How to cite this URL:
Salehuddin NS, Din NM, Saad MS, Aziz A, Salahuddin Z. Tympanogenic labyrinthine ossificans as rare sequelae of cholesteatoma. Indian J Otol [serial online] 2019 [cited 2019 Dec 15];25:162-5. Available from: http://www.indianjotol.org/text.asp?2019/25/3/162/269549




  Introduction Top


Cholesteatoma, commonly described as “skin in the wrong place,” is a known benign disease entity for more than 300 years,[1] first described in 1683 by a French anatomist Joseph-Guichard Duverney. The incidence is reported as 3/100,000 in children and 9.2/100,000 in adults with a male predominance.[1] Clinically, cholesteatoma presented with chronic discharging ear, facial nerve paralysis, and conductive or mixed hearing loss. It is very uncommon to have profound hearing loss or dead ear as well as severe vertigo as its presenting complaint.[1]

Labyrinthine ossificans (LO), on the other hand, happened when the membranous labyrinth is replaced by osseous tissue.[2] It followed the three-stage sequelae of labyrinthitis, which are acute inflammation, fibroblastic changes, and ultimately the osseous formation of membranous labyrinth,[2] as described by Lin et al.[3] The most common etiology of LO is from the spread of intracranial infection, which usually involved bilateral ear, and secondly by hematogenous spread.[2] Rarely, the etiology can be attributed from trauma, tumor, prior surgery, vascular, and, in our case, from otologic cause.[2]


  Case Report Top


A 16-year-old male presented with a history of intermittent left ear discharge for 4 years, which worsens for the last 1 year, when it became persistent and foul smelling. He also complained of gradual hearing loss in the left ear, but there was no tinnitus, vertigo, or facial asymmetry. There was a history of polypectomy of an external canal polyp – histopathologically reported as inflammatory polyp. Pure tone audiometry before the polypectomy revealed a left moderate conductive hearing loss, with contralateral normal hearing.

He developed sudden onset of vertigo, tinnitus, and worsening of left ear hearing, which was severe enough to warrant a 2-week duration of ward admission. The vertigo was persistent, associated with nausea and vomiting, and incapacitating that the patient hardly able to ambulate. During the episode, there was no sign suggestive of central vertigo, meningitis, or bacteremia. He was treated as acute labyrinthitis, and fortunately, with the help of steroids, antibiotics, and betahistine, the vertigo resolved completely after 2 weeks. However, the left ear hearing was almost non-existance. Pure tone audiometry noted left profound mixed hearing loss with normal hearing of the right ear [Figure 1].
Figure 1: Pure tone audiometry showing left profound mixed hearing loss with contralateral normal hearing

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High-resolution computed tomography (HRCT) done with the intention for mastoid surgery due to persistent discharging ear, 6 months after the acute labyrinthitis episode, revealed a soft-tissue density lesion within the left middle ear cavity, extending to Prussak's space, epitympanum, and mastoid antrum, with erosion of malleus and incus. There was significant extensive sclerosis of the left cochlear, superior and posterior semicircular canals, and the rest of the mastoid air cells. The lateral semicircular canal was partly ossified, becoming irregular and narrowed. The right ear, both clinically and radiologically, was normal. Bilateral internal auditory meatus appeared symmetrical and normal, measuring 0.6 cm at widest diameter. No leptomeningeal enhancement or cerebral edema was noted [Figure 2]. From the imaging, the diagnosis of tympanogenic LO was made.
Figure 2: High-resolution computed tomography of the temporal bone showing ossification of the cochlea (blue arrow) and a soft-tissue density lesion within the left middle ear cavity

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He underwent left modified radical mastoidectomy. Intraoperatively, the left tympanic membrane was retracted, with retraction pocket at posterosuperior part of tympanic membrane and keratin debris accumulation at the attic [Figure 3]. We also found the presence of myringostapediopexy, sclerotic mastoid, and cholesteatoma sac in the antrum and mastoid cavity [Figure 4]. Fortunately, we were able to completely remove the cholesteatoma, and the patient recovered well postoperatively. For the aim of binaural hearing, choices of bone-anchored hearing aid, CROS hearing aid, and bone conduction hearing aid were offered to the patient.
Figure 3: Retraction pocket of pars tensa (yellow arrow) with accumulation of wax and keratin debris at the attic (red arrow) – pre- and postear toileting

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Figure 4: Intraoperative cholesteatoma sac identification at the mastoid antrum (blue arrow)

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  Discussion Top


The most frequent complication of the middle ear cholesteatoma, accounting for the incidence of 5%–10%, is labyrinthine fistula. It manifests as sensorineural hearing loss, tinnitus, and episodic vertigo, associated with the CT evidence of a dehiscent lateral semicircular canal and, rarely, dehiscent of cochlear promontory.[1] It is agreed that labyrinthine fistula might cause labyrinthitis and later complicated by LO;[1] however, before the episode of severe vertigo, our patient denied any episodic vertigo or tinnitus, and there was no evidence of sensorineural hearing loss element and CT to support prior labyrinthine fistula in our case.

Other common complications associated with cholesteatoma include facial nerve palsy, secondary to direct inflammatory effect, presence on inflammatory neuroma, and effect of compression atrophy.[1] Although, intraoperatively, we noticed the dehiscence of the tympanic part of facial nerve, our patient did not suffer from facial nerve palsy throughout the course of illness. Rare complications of cholesteatoma include sigmoid sinus erosion and thrombosis, as well as erosion of tegmen tympani leading to intracranial invasion, abscess, and recurrent bacterial meningitis.[1]

Tympanogenic LO, a term to describe ossification of labyrinth due to otologic pathology, from middle ear, antrum, or petrous apex,[4] is rare. [3,4] A retrospective study involving 195 patients which was conducted in Taiwan from 2007 to 2011 found that the incidence of tympanogenic LO was 2%, which was positive in four of the patients enrolled in the study.[3]

Acute severe vertigo and worsening of hearing in association with mastoid and middle ear disease, similar to our patient, is the typical history of tympanogenic labyrinthitis.[4] In a study of the association of suppurative labyrinthitis with otitis media and cholesteatoma, almost all labyrinthitisassociated vertigo is completely compensated, leaving the permanent damage of profound or complete hearing loss in all the patients, similar to our case.[5] Regardless of any pathway of spread, i.e., meningogenic, hematogenic, or tympanogenic, it is agreed histologically that LO is the end result of suppurative labyrinthitis,[4],[6] which could occur as early as 2-week postinfection.[4]

The possible spread of infection from the middle ear cholesteatoma to the inner ear may occur either through bony destruction or fistula, directly through round or oval window,[7] along vascular channels[3] or lymphatic communications.[7] Based on these theories, the lateral semicircular canal which is the closest to mastoid cavity and the basal turn of the cochlear which is the closest to middle ear should be the most extensive area of ossification.[7] Unexpectedly, our patient has relatively least ossification at the basal turn of the cochlear and the lateral semicircular canal, as similarly described by Lin et al. in their study of tympanogenic LO, with unknown mechanism for this unique observation.[3] However, the features of marked ossification at the round and oval window in our patient's CT image strongly point toward the otologic cause of LO, rather than other routes of infection.

It is also worth noted that meningogenic etiology of LO usually involves bilateral inner ear,[8] secondary to spread from cochlear aqueduct, modiolus,[7] or internal auditory canal (IAC).[8] According to Aralasmak et al., spread of infection from subarachnoid space through the cochlear aqueduct causes more intense ossification at the basal turn of the cochlear, whereas more pronounced disease in the first and second turn of cochlea would suggest spread through the IAC.[8] Severe LO that causes total ossification of the membranous labyrinth is to be differentiated with cochlear aplasia or hypoplasia.[8] The convexity of the otic capsule will be reduced in this condition, which is also known as Michel deformity. On the other hands, the otic capsule convexity will be preserved in LO.[8]

There are reported rare cases of direct cholesteatoma invasion into IAC causing facial paralysis and complete hearing loss.[9] In these cases, the cholesteatoma mostly extends to the IAC via supralabyrinthine route,[9] and there was no ossification of labyrinth noted. It is possible in cases of extensive acquired cholesteatoma with aggressive erosion of labyrinth; or in patient with congenital cholesteatoma which primarily developed in IAC or extend from primary petrous apex cholesteatoma.[9],[10]

Complete eradication of the cholesteatoma which invades IAC would require difficult option of subtotal petrosectomy, translabyrinthine, or middle cranial fossa approach combined with radical mastoidectomy.[9] In our patient, the intact facial nerve, normal diameter and configuration of IAC, normal appearance of petrous apex, and the ability to completely remove cholesteatoma with modified radical mastoidectomy make it unlikely for the patient left almost dead ear to be attributed to direct disease invasion into IAC.

HRCT of the temporal bone is usually unable to detect labyrinthine changes during the first acute early inflammatory response and the second stage of fibroproliferation of suppurative labyrinthitis.[3],[11] Another drawback of HRCT is its suboptimal assessment of the cochlear compartment.[8],[11] In our case, CT scan was performed a few months after acute labyrinthitis episode settled, where all the ossification changes were established. Magnetic resonance imaging (MRI) demonstrated low T2 signal during the fibrous phase of LO,[11] allowing earlier diagnosis, before it progresses to the last stage of ossification of the membranous labyrinth. Therefore, in center with ample resources, we suggest the utilization of MRI during acute presentation of labyrinthitis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Baráth K, Huber AM, Stämpfli P, Varga Z, Kollias S. Neuroradiology of cholesteatomas. AJNR Am J Neuroradiol 2011;32:221-9.  Back to cited text no. 1
    
2.
Larson C, Jorgensen SA, Towbin AJ, Towbin R. Labyrinthine ossificans. Appl Radiol 2016;45:40-1.  Back to cited text no. 2
    
3.
Lin HY, Fan YK, Wu KC, Shu MT, Yang CC, Lin HC. The incidence of tympanogenic labyrinthitis ossificans. J Laryngol Otol 2014;128:618-20.  Back to cited text no. 3
    
4.
Hoffman RA, Brookler KH, Bergeron RT. Radiologic diagnosis of labyrinthitis ossificans. Ann Otol Rhinol Laryngol 1979;88:253-7.  Back to cited text no. 4
    
5.
Maranhão AS, Godofredo VR, Penido Nde O. Suppurative labyrinthitis associated with otitis media: 26 years' experience. Braz J Otorhinolaryngol 2016;82:82-7.  Back to cited text no. 5
    
6.
Swartz JD, Mandell DM, Faerber EN, Popky GL, Ardito JM, Steinberg SB, et al. Labyrinthine ossification: Etiologies and CT findings. Radiology 1985;157:395-8.  Back to cited text no. 6
    
7.
Cureoglu S, Schachern PA, Rinaldo A, Tsuprun V, Ferlito A, Paparella MM. Round window membrane and labyrinthine pathological changes: An overview. Acta Otolaryngol 2005;125:9-15.  Back to cited text no. 7
    
8.
Aralaşmak A, Dinçer E, Arslan G, Cevikol C, Karaali K. Posttraumatic labyrinthitis ossificans with perilymphatic fistulization. Diagn Interv Radiol 2009;15:239-41.  Back to cited text no. 8
    
9.
Migirov L, Bendet E, Kronenberg J. Cholesteatoma invasion into the internal auditory canal. Eur Arch Otorhinolaryngol 2009;266:657-62.  Back to cited text no. 9
    
10.
Silver AJ, Janecka I, Wazen J, Hilal SK, Rutledge JN. Complicated cholesteatomas: CT findings in inner ear complications of middle ear cholesteatomas. Radiology 1987;164:47-51.  Back to cited text no. 10
    
11.
Vyas S, Bhatia V, Panda NK, Singh P, Khandelwal N. Labyrinthitis ossificans after meningitis: Superiority of high-resolution magnetic resonance imaging in demonstration of disease extent compared to high-resolution computed tomography. J Neurosci Rural Pract 2016;7:327-9.  Back to cited text no. 11
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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