International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 172–174

Case report

Unilateral conductive hearing loss secondary to an ipsilateral high jugular bulbwith contralateral agenesis of the lateral dural sinuses in a pediatric patient

Ariel Katz a,*, Daniel M. Kaplan a, Benzion Joshua a, Ilan Shelef b, Marc Puterman a

a Department of Otolaryngology Head and Neck Surgery, Soroka University Medical Center and the Ben Gurion University, Beer Sheva, Israelb Radiology Institute, Soroka University Medical Center and the Ben Gurion University, Beer Sheva, Israel

A R T I C L E I N F O

Article history:

Received 20 March 2010

Received in revised form 30 June 2010

Accepted 3 July 2010

Available online 27 July 2010

Keywords:

Otology

Audiology

Serous otitis media

Pressure equalizing tubes

High jugular bulb

A B S T R A C T

We present a case of a 7-year-old boy with a left sided conductive hearing loss accompanied by pulsating

tinnitus. Otomicroscopic examination of the left ear revealed a bluish, non-pulsating mass behind the

posterior inferior quadrant of the tympanic membrane. The audiogram demonstrated a left conductive

hearing loss and a type B tympanogram. A myringotomy with insertion of a pressure equalizing (PE) tube

was performed with no change in symptoms and hearing. CT demonstrated a high jugular bulb (HJB) on

the left side, intruding the middle ear space, in contact with the ossicular chain. Venous stage of magnetic

resonance demonstrated a complete dominance of the dural sinuses on the left side, with a huge internal

jugular vein diverticulum. In addition, a complete agenesis of the lateral dural sinuses was demonstrated

on the contralateral side. The combination of pulsatile tinnitus and unilateral conductive hearing loss

with a red or blue to purple mass behind the tympanic membrane should alert the physician. Assessment

of the anatomy and blood flow by CT scan and MRI of the ear and head are advised to delineate the

finding, before considering any type of intervention.

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International Journal of Pediatric OtorhinolaryngologyExtra

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1. Case presentation

A 7-year-old boy with no prior otologic history, presented to theotolaryngology service with left side hearing loss lasting for severalmonths, accompanied by pulsating tinnitus exacerbated by effort.Otomicroscopic examination of the left ear revealed a bluish, non-pulsating mass behind the posterior inferior quadrant of thetympanic membrane.

The audiogram demonstrated a left conductive hearing losswith an air-bone gap of 25 dB (Fig. 1) and a type B tympanogram.There was no change on consequent audiograms. Assuming thediagnosis of serous otitis media, a myringotomy with insertion of apressure equalizing (PE) tube was performed.

Postsurgical follow up has not shown any change in symptomsor audiogram. High resolution computed tomography of thetemporal bone (HRTBCT) (Fig. 2) demonstrated a high jugular bulb(HJB) on the left side, intruding the middle ear space andcontacting the ossicular chain. Magnetic resonance venography(MRV) demonstrated complete dominance of the dural sinuses onthe left side, with a huge internal jugular vein diverticulum.

* Corresponding author at: Department of Otolaryngology-Soroka Medical

Center, P.O.B. 151, Beer Sheva, Israel. Tel.: +972 86400635/545901073;

fax: +972 86403037.

E-mail addresses: arielka@clalit.org.il, katzgal@bezeqint.net (A. Katz).

1871-4048/$ – see front matter � 2010 Elsevier Ireland Ltd. All rights reserved.

doi:10.1016/j.pedex.2010.07.002

Additionally, the contralateral side showed complete agenesis ofthe lateral dural sinuses (Fig. 3).

2. Discussion

A HJB is usually an incidental finding on HRTBCT and onmagnetic resonance (MR) of the temporal bone. The finding is morecommon on the right side, since dural sinuses and the internaljugular vein are larger on the right side in the majority of thepatients [1,2]. Occasionally, significant bleeding may result frominadvertent puncture of a HJB during myringotomy, placement of aventilation tube (VT) or during elevation of the inferior portion of atympanomeatal flap. A fatal case of a myringotomy, whichprobably injured a high jugular bulb was first reported in 1914 [3].

When symptoms are present, conductive hearing loss (CHL) andhumming tinnitus is commonly reported [2]. The CHL in thepresence of a HJB may result from one or more of the followingmechanisms: (1) Contact with the tympanic membrane. (2)Obstruction of the round window niche. (3) Interference withthe ossicular chain [1,2]. Weiss et al. [1] reported five patients witha reddish blue mass behind an intact tympanic membrane onotoscopic examination with unilateral conductive hearing loss,without pulsatile tinnitus. All patients underwent HRTBCTexamination, confirming HJB. The authors stated that in none oftheir patients the hearing loss could be attributed to interferencewith the ossicular chain, but rather to contact of the jugular bulb

Fig. 1. Audiogram of the child, at presentation. A conductive hearing loss with a 20–

30 dB air-bone gap is exhibited on the left side. This was accompanied by a type B

tympanogram.

A. Katz et al. / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 172–174 173

with the tympanic membrane or round window niche obliteration.Haupert et al. [2] reported a patient with a HJB totally covering theround window, an intact ossicular chain, with a persistentconductive hearing loss of 40 dB. In the current patient, the HJBwas not in contact with the tympanic membrane, thus, obstructionof the round window and interference with the ossicular chain arethe most likely causes of the CHL.

A HJB may appear as a white mass, resembling a cholesteatoma,when it is covered with bone. However, if the bony cover of the

Fig. 2. High resolution temporal bone CT scans in the axial (a) and in the saggital plains (b

and in contact with the ossicular chain (arrows).

bulb is thin or dehiscent, a red or purplish mass may be seen,mistakenly referred as a glomus tumor. Additional conditions to beincluded in the differential diagnosis of a middle ear mass behindan intact tympanic membrane are: an aberrant internal carotidartery, persistent stapedial artery, cholesterol granuloma, andneoplasms involving the temporal bone [2].

HRTBCT delineates the bony anatomy of the temporal bone andclearly demonstrates HJB, however MR and MR venography arenecessary to distinguish it from other vascular masses [1]. Reportson surgical correction of the CHL, include attempts to compress orrelocate the HJB but have mostly been unsuccessful. Robindescribed a reposition of a high dehiscent jugular bulb compres-sing the ossicular chain and a consequent reconstruction of thefloor of the mesotympanum by a cartilage graft. Postoperatively,the patient’s hearing improved but deteriorated within time [4].Glasscock described two cases of jugular bulb reposition andreconstruction of the floor with mastoid cortical bone graft with nopostoperative hearing improvement in either case [5].

In the current case, any attempt to surgically manipulate thejugular vein would be especially dangerous since the left internaljugular vein exclusively drains the brain.

The combination of pulsatile tinnitus and unilateral conductivehearing loss with a red or blue to purple mass behind the tympanicmembrane should alert the physician. Preoperative assessmentshould include HRTBCT in order to examine the exact anatomy of

). A high jugular bulb is demonstrated on the left side, intruding the middle ear space

Fig. 3. Cranial MRV demonstrating dominance of the dural sinuses on the left side, with a huge internal jugular vein diverticulum. Contralaterally – the right side

demonstrates complete agenesis of the lateral dural sinuses. Parts (a) and (b) are in the axial and coronal plain, respectively, and part (c) demonstrates the coronal cranial and

cervical view of venous vessels.

A. Katz et al. / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 172–174174

the temporal bone with complementary MR and MRV of the brainblood flow, to delineate the finding from other causes as statedabove, and to demonstrate sufficient contralateral blood flow inthe dural sinuses [6].

References

[1] R.L. Weiss, G. Zahtz, E. Godofsky, H. Parnes, M.J. Shikowitz, High jugular bulb andconductive hearing loss, Laryngoscope 107 (1997) 321–327.

[2] M.S. Haupert, D.N. Madgy, W.M. Belenky, J.W. Becker, Unilateral conductivehearing loss secondary to a high jugular bulb in a pediatric patient, Ear NoseThroat J. 76 (7) (1997) 468–469.

[3] J.R. Page, A case of probable injury to the jugular bulb following myringotomy in aninfant ten months old, Ann. Otol. 23 (1914) 161.

[4] P.K. Robin, A case of upwardly situated jugular bulb in the left middle ear, J.Laryngol. 186 (1980) 1241–1246.

[5] M.E. Glasscock, J.R.E. Dickins, C.G. Jackson, et al., Vascular anomalies of the middleear, Laryngoscope 90 (1980) 77–88.

[6] O. Sedat, K. Huseyin, O. Yilmaz, et al., Surgical treatment of the high jugular bulb bycompressing sinus sigmoideus: two cases, Eur. Arch. Otorhinolaryngol. 265 (2008)987–991.