Egyptian Journal of Ear, Nose, Throat and Allied Sciences · 3. Discussion The middle ear cleft is located at the petrous part of the tempo-ral bone and extends from the tympanic

Egyptian Journal of Ear, Nose, Throat and Allied Sciences xxx (2016) xxx–xxx

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Egyptian Journal of Ear, Nose, Throat and Allied Sciences

journal homepage: www.ejentas .com

Case report

Primary Hodgkin’s lymphoma of the middle ear: A rare cause of facialnerve palsy

http://dx.doi.org/10.1016/j.ejenta.2016.10.0102090-0740/� 2016 Egyptian Society of Ear, Nose, Throat and Allied Sciences. Production and hosting by Elsevier B.V.This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

⇑ Corresponding author at: Department of Otorhinolaryngology-Head and NeckSurgery, School of Medical Sciences, Universiti Sains Malaysia Health Campus,16150 Kota Bharu, Kelantan, Malaysia.

E-mail address: [email protected] (N. Maithrea).Peer review under responsibility of Egyptian Society of Ear, Nose, Throat and AlliedSciences.

Please cite this article in press as: Maithrea N., et al. Primary Hodgkin’s lymphoma of the middle ear: A rare cause of facial nerve palsy. Egypt J EThroat Allied Sci (2016), http://dx.doi.org/10.1016/j.ejenta.2016.10.010

N. Maithrea a,b,⇑, S. Periyathamby a, Irfan Mohamad b

aDepartment of Otorhinolaryngology-Head and Neck Surgery, Penang General Hospital, Jalan Residensi, 10900 Penang, MalaysiabDepartment of Otorhinolaryngology-Head and Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia Health Campus, 16150 Kota Bharu, Kelantan, Malaysia

a r t i c l e i n f o

Article history:Received 11 October 2016Accepted 30 October 2016Available online xxxx

Keywords:Hodgkins lymphomaMiddle earFacial nerve palsy

a b s t r a c t

Facial nerve palsy can occur whenever any part of the facial nerve is affected. It can be complete or par-tial, unilateral or bilateral and upper motor or lower motor neurone type. Common causes of unilaterallower motor neuron facial nerve palsy include trauma, infections of the middle ear, neoplasms of the par-otid, iatrogenic and idiopathic. Hodgkin lymphoma typically presents with an asymptomatic lym-phadenopathy, but associated symptoms include constitutional symptoms, intermittent fever, chestpain, or shortness of breath. We present an extremely rare case of facial nerve palsy secondary to primaryclassical nodular sclerosis Hodgkin’s lymphoma, arising from the left middle ear extending into the exter-nal auditory canal.� 2016 Egyptian Society of Ear, Nose, Throat and Allied Sciences. Production and hosting by Elsevier B.V.This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-

nd/4.0/).

1. Introduction

Lymphomas are the commonest non-epithelial malignancies ofthe head and neck.1 However, these lymphoreticular neoplasmsseldom present in the temporal bone area unless in cases ofacquired immunodeficiency syndrome.2 Temporal bone lym-phomas are typically metastatic in nature or spread from contigu-ous foci.3 Sporadic cases have been reported in literature, and sitesof origin include the middle ear cleft, mastoid, and external audi-tory canals.4,5

2. Case report

A 53-year-old Indian male presented with one month history ofleft-sided lower motor neuron facial nerve palsy associated withleft-sided hearing loss. He was treated initially with a combinationof antibiotic and steroid therapy by a private practitioner. How-ever, the condition did not improve. On examination he had aleft-sided lower motor neurone facial nerve palsy (Grade IIIHouse-Brackmann classification). A mass was visible filling the

external ear canal. Tuning fork test confirmed the hearing loss,which was conductive in nature. The remainder of the physicalexamination was normal. Blood tests were normal. High resolutioncontrast enhanced computed tomography (CT) scan of the brainand temporal bones showed an irregular hypodense lesion in theleft middle ear cavity and left external auditory canal. Bony erosionof left ossicles, mastoid and tegmen tympani was noted withdehiscence of left facial nerve canal (Fig. 1). There was no intracra-nial extension. He underwent exploration of his left ear and mas-toid. A pale fleshy mass of tissue occupied the epitympanum ofthe middle-ear cleft, external auditory canal and mastoid bowl.The dura was not breached.

Histopathological analysis showed a nodular growth patternwith areas of necrosis (Fig. 2). The nodules were composed of amixed population of cells including small lymphocytes, eosino-phils, histiocytes and a moderate number of classical, lacunarand occasionally mummified Reed-Sternberg cells. Immunohisto-chemical studies were positive for CD15 and CD30 (Figs. 3 and4), and negative to ALK-1. Impression was classical nodular sclero-sis Hodgkin’s Lymphoma and staging investigations were done,including bone marrow aspirate, and CT neck/abdomen/thoraxwhich revealed no other focus. He was commenced on a 6 cycleregime of ABVD (Adriamycin, Bleomycin, Vinblastine and Dacar-bazine) chemotherapy with a FDG PET scan planned after comple-tion to assess response. After 3 cycles of chemotherapy his facialnerve palsy was noted to have resolved completely, with repeatpure tone audiometry planned after completion of treatment.

ar Nose

Fig. 1. Irregular hypodense lesion in the left middle ear cavity with ossicularerosion.

Fig. 2. Nodular growth pattern with broad collagen bands surrounding nodules.

Fig. 3. positive immunohistochemical staining for CD 15.

Fig. 4. positive immunohistochemical staining for CD 30.

2 N. Maithrea et al. / Egyptian Journal of Ear, Nose, Throat and Allied Sciences xxx (2016) xxx–xxx

3. Discussion

The middle ear cleft is located at the petrous part of the tempo-ral bone and extends from the tympanic membrane to the cochlea.It is often described as a six-sided box with the tegmental wall as

Please cite this article in press as: Maithrea N., et al. Primary Hodgkin’s lymphThroat Allied Sci (2016), http://dx.doi.org/10.1016/j.ejenta.2016.10.010

the roof, the jugular wall as the floor, the tympanic membrane asthe lateral wall and oval window as the medial wall. Anteriorly itis bound by a thin layer of bone that separates the carotid canaland the tympanic cavity called the carotid wall, and posteriorly itcontains the mastoid antrum, communicating with the mastoidair cells.6 It contains the ossicles, important musculature includingthe stapedius, the facial nerve, vascular supply and lymphoid tis-sue, which drains into the retropharyngeal and deep cervical groupof lymph nodes.7

The facial nerve traverses the tympanic cavity in a bony canalon the labyrinthine wall just the footplate of the stapes. It entersthe temporal bone at the internal auditory meatus. Here it passeslaterally and bends backwards and passes in the Fallopian canalabove the oval window until it reaches the aditus where it turnsdownwards and passes vertically until it reaches the stylomastoidforamen and exits out of the temporal bone.8

Lymphomas most commonly present as asymptomatic painlesslymphadenopathy, with 60–80% occurring in the cervical and supr-aclavicular nodes, with other common areas being in the axilla andthe inguinal nodes. However, in our case, the patient did not haveany palpable lymph nodes, and CT scan done also did not show anylymph node enlargement. A large proportion of patients developsystemic symptoms, especially B symptoms like fever, weight lossand night sweats before the finding of lymphadenopathy, presentin up to 50% of those with advanced disease. 9 These symptomswere also absent in our patient.

Hodgkins lymphoma typically starts from a single foci of lym-phatic tissue, and via lymphatic channels, spread to adjacentlymph nodes and finally disseminating to distant non adjacentsites and organs. While lymphomas are the second most commonmalignancies of the head and neck, lymphomas in the middle earare relatively rare.10 Literature review shows that there has onlybeen 18 reported cases of lymphomas in the ear since 1947.11

Nodular sclerosis Hodgkins lymphoma is the most common sub-type of Hodgkin’s lymphoma, accounting for 60–80% of cases.12

Nevertheless, the subtype of lymphoma does not influence treat-ment modality as much as the stage of the disease. In this patient,there was only involvement of one group of lymph node with con-tiguous extranodal involvement, and prognosis for low grade HL isexcellent, with cure rates of up to 90%. Even in high risk HL, curerates are achieved in up to 80% with a multimodal approach ofchemotherapy and radiotherapy.13

4. Conclusion

Malignancy should always be excluded in a setting of therapy-resistant otitis media or lower motor neuron facial paralysis. The

oma of the middle ear: A rare cause of facial nerve palsy. Egypt J Ear Nose

N. Maithrea et al. / Egyptian Journal of Ear, Nose, Throat and Allied Sciences xxx (2016) xxx–xxx 3

atypical presentation and rare location of this case of Hodgkin’slymphoma also highlights the value of correlation of underlyinganatomy to the lesion site in order to guide further investigationand management.

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oma of the middle ear: A rare cause of facial nerve palsy. Egypt J Ear Nose